Beijing Paradise BBS Backup/PARADISE.ISO/software/BBSDOORW/MED3.ZIP/MEDLIB.DAT

Text File | 1996-02-04 | 1.1 MB | 24,234 lines

*
Research Request: Information on Rosacea


Synonyms
-------
Adult Acne
Acne Erythematosa
Rosacea
Hypertrophic Rosacea
Rhinophyma
Acne Conglobata
Acne Vulgaris
Acne Fulminans
Excoriated Acne of Young Women
Chloracne
Atypical Acneiform eruptions

General Description
------------------

Acne Rosacea is a skin disorder limited to the nose, cheeks and forehead,
typically beginning during adulthood.  The facial skin becomes oily, reddened
and bumpy.  Small red blood vessels are visible.  In extreme cases, the nose may
appear very red and bulbous.

Symptoms
--------

Acne Rosacea affects the skin of the nose, surrounding cheeks and forehead
areas.  The skin becomes oily and progressively reddened (erythematous) with
enlarged blood vessels apparent in the skin (telangiectasias).  Typically, small
elevated growths (papules), some containing dead skin cells and fluid (pustules)
develop over the central area of the face. Scarring usually does not occur.  In
very severe cases, the skin of the nose becomes extremely red and bulbous
(rhinophyma).

Causes
------

The exact cause of Acne Rosacea is not known.   Scientists believe a genetic
predisposition to the disorder may exist.  Symptoms can be intensified by hot
liquids, spicy foods, alcohol consumption (although this is not always a cause;
many cases occur among teetotalers), vigorous exercise, heat, exposure to sun
and wind, certain vitamin deficiencies, endocrine disturbances and/or emotional
stress.


Affected Population
-------------------

Acne Rosacea usually begins between the ages of thirty and fifty years of age,
but can occur at any age.  The disorder tends to affect females more than males,
although cases among males tend to be more severe.


Related Disorders
-----------------

Symptoms of the following disorders can be similar to those of Acne Rosacea.
Comparisons may be useful for a differential diagnosis:

Acne Conglobata may occur alone, or may be part of a symptom complex in
conjunction with Hidradenitis Suppurativa and connective tissue inflammation
(cellulitis) of the scalp.  Lesions usually occur on the neck and upper trunk,
but may extend to the upper arms, lower back, buttocks, and thighs. The typical
acne skin eruptions are present, but symptoms are chronic with abscesses and the
development of connective formations between inflamed skin growths (nodules) and
cysts.  The cysts contain foul-smelling fluid, and severe scarring is common.
Although this form of acne resists treatment with systemic antibiotics, symptoms
may be controlled by the use of isotretinoin in some cases.

Acne Vulgaris is the most common form of acne which affects many adolescents
during puberty.  The skin eruptions primarily appear on the face, upper back
and/or chest due to overactive oil-secreting (sebaceous) glands related to
changes in hormonal activity.

Acne Fulminans is a rare variant of acne seen predominately among adolescent
males.  Mild Acne Vulgaris initially develops but it is unexpectedly followed by
markedly inflamed and painful lesions on the upper trunk and occasionally the
face.  Lesions may bleed and/or crust, and may be accompanied by fever and other
systemic abnormalities.  Treatment with isotretinoin, with or without systemic
corticosteroid drugs and antibiotics, can be effective in most cases.

Excoriated Acne of Young Women (Acne Excorie Des Jeunes Filles) is a variant of
acne produced by excessive manipulation of acne lesions resulting in increased
scarring.  Although it is often found among young women, it can be found in any
age group and both sexes.

Chloracne may develop among workers exposed to chlorinated hydrocarbons.
Treatment of the skin lesions should be accompanied by removal of the irritating
substance from the environment.


Atypical Acneiform eruptions are seen in any age group and are not always
limited to oil-secreting (sebaceous) glands.  These eruptions may appear as a
result of corticosteroid, androgen, or progesterone drug therapy. Occupational
problems including ingestion of iodine or bromine salts, or skin contact with
machine oils such as insoluble cutting oils, may also cause this type of acne.
Additionally, drugs such as diphenylhydantoin and lithium can induce this skin
condition.

Therapies:  Standard
--------------------

Although there is no cure for Acne Rosacea, symptoms can be controlled.
Antibiotics can control inflammation, but may cause adverse reactions in some
patients.  Carbon Dioxide laser and conventional surgery are used to remove
excess skin growth (rhinophyma) as a temporary measure.  Argon lasers have been
effective in reducing redness in the nose area, but only in mild cases which
have not progressed to rhinophyma.  The orphan drug metronidazole (Metrogel) was
approved in 1988 by the FDA for treatment of Acne Rosacea. This drug is
manufactured by Curatek Pharmaceuticals of Elk Village, IL. Other treatment is
symptomatic and supportive.

The orphan drug Sprelin injection was recently approved by the FDA for the
treatment of Precocious Puberty.

Resources
---------

For more information on Acne Rosacea, please contact:

Acne Research Institute
1236 Somerset Lane
Newport Beach, CA  92260
[phone]

NIH/National Arthritis and Musculoskeletal and Skin Diseases (NIAMS) Information
Clearinghouse
9000 Rockville Pike
Bethesda, MD  20892
[phone]
!
*
Research Request:        ADDISON'S DISEASE
Sources of Information:  National Adrenal Diseases
                         Foundation; The Mosby Medical
                         Encyclopedia; NORD


Synonyms
--------
Addisonism
Addison's Syndrome
Adrenal Crisis
Adrenal Hypoplasia
Adrenocortical Hypofunction
Adrenocortical Insufficiency
Chronic Adrenocortical Insufficiency
Hypoadenalism
Primary Adrenal Insufficiency
Primary Failure Adrenocortical Insufficiency

Other diseases which are similar or may be linked to Addison's
Disease are:

Adrenoleukodystrophy
Amyloidosis
ACTH Deficiency
Adrenal Hyperplasia
Cushing's Syndrome
Schmidt Syndrome



DESCRIPTION:

Addison's disease is a life-threatening disease caused by
partial or complete failure of the outer layer of the adrenal
glands, this outer layer is called the adrenal cortex.  This gland
produces hormones that control many body functions. In
Addison's Disease, there is a deficiency of cortisol and
aldosterone (hormones made in the adrenal glands).  When the
body is deficient in these hormones, the result is high levels
of potassium and low levels of salt, which causes an
electrolyte imbalance in the body. This electrolyte imbalance
can cause hypotension (low blood pressure) and loss of water.

SYMPTOMS:
--------
Addison's Disease is marked by weakness, loss of strength, loss
of hunger, loss of water, weight loss and darkening
of the skin, especially that of scars, wrinkled portions of the
skin and mucous membranes.  Dark freckles may appear on the
head and shoulders of Addison's Disease sufferers.  Other
symptoms which may appear later are:  stomach and intestinal
pain due to nausea, vomiting, dizziness and diarrhea. Restlessness,
depression, and extreme sensitivity to cold may also occur
later in the disease.  The onset of Addison's Disease is usually
gradual, over a period of weeks or months.

An extreme insufficiency of adrenocortical hormones (adrenal
crisis) may occur in people with Addison's Disease.  This crisis
may begin as a sudden loss of strength, severe
pain in the abdomen, lower back, and/or kidney failure.  Adrenal
crisis is  LIFE THREATENING.  If an Addison sufferer experiences
any of these  symptoms, SEEK IMMEDIATE ATTENTION FROM YOUR
PHYSICIAN.

A person in adrenal crisis needs immediate intravenous
administration of high-dose hydrocortisone succinate or phosphate
and electrolyte replacement;  drugs called vasopressors
may also be needed to maintain blood pressure. Patients are
urged to carry a card or wear an identification necklace
or bracelet, stating that they have Addison's disease, so that
they will receive proper medical treatment in case of emergency.


CAUSES OF ADDISON'S DISEASE:
------
Although the exact cause of Addison's Disease is not known, it is
believed to be an autoimmune disease. An Autoimmune Disease is when the
body's own immune system (antibodies,lymphocytes, etc.), begins
attacking healthy tissue in error. Approximately 75 percent of cases
of Addison's Disease are thought to be autoimmune related. Other
disorders which can cause the partial destruction of the adrenal
cortex and can trigger the onset of Addison's Disease are: tumor,
tuberculosis, or amyloidosis, which is the abnormal accumulation of a
fatty-like substance within the adrenal glands.

METHODS OF TREATMENT:
--------------------

Addison's Disease is usually treated with replacement therapy that
consists of cortisone and fludrocortisone; these replacement hormones
are taken with meals.  The dosage of these drugs should be increased
during times of infection, trauma, surgery, and other stress periods
in order to prevent an acute adrenal crisis.


Resources
---------

For more information on Addison's Disease, please contact:


The National Adrenal Diseases Foundation, Inc.
500 Northern Blvd., Suite 200
Great Neck, NY  11021
[phone]

NIH/National Digestive Diseases Information Clearinghouse (NDDIC)
9000 Rockville Pike
Bethesda, MD  20892
[phone]

National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT  06812-1783
[phone]
[phone] (TDD for the hearing impaired)
!
*
ADULT IMMUNIZATIONS

QUESTION: I am a 28-year-old woman in good health.  How can I tell if I am
adequately immunized against serious disease?  Should I be receiving any
vaccinations at my age?

    Thanks to the very successful immunization programs we have in this
country, young children rarely get measles, rubella (German measles), mumps,
tetanus (lockjaw) or diphtheria.  Unfortunately, however, a surprising number
of adults still do get them; they may have moved here from another country, a
childhood disease may have been misdiagnosed, or certain vaccines may have
been unavailable when they were children.

    Inadequate immunization against measles and mumps has proven to be a
particular problem among people your age, who may have been too old to receive
the vaccine when it was introduced, and may have missed getting these
diseases.  Also, before the live virus vaccine was perfected in the 1960s,
many children received a vaccine made of killed  viruses, which was
ineffective.

    If you have no memory or record of having these diseases or of being
vaccinated, you can find out through a blood test if you are immune.  If the
test shows that you are not immune, you should be vaccinated, since adults who
get measles and mumps run a higher risk than children of developing serious
complications.

    A surprising number of adults either have not had rubella or were never
vaccinated against that disease.  This disease, ordinarily mild in children,
can cause devastating birth defects in fetuses carried by women who get
infected during pregnancy.

All women of childbearing age should have a blood test to see if they are
immune to rubella; if not, they should be vaccinated at least three months
before they plan on becoming pregnant.

In fact, some states require a rubella test prior to marriage.  Health workers
who come into contact with pregnant women also should make sure they are
immune to rubella.

    Although tetanus and diphtheria are quite rare thanks to longstanding
immunization practices, occasional cases do occur in adults who have never
been vaccinated. After primary immunization from both diseases during
childhood, a booster shot is recommended every 10 years to preserve your
immunity.  Many doctors suggest getting it on your mid-decade birthdays--
25,35,45, and so on--to help you remember.

    Since you are not in a high-risk group by virtue of age or chronic
illness, these are probably the only immunizations you should be concerned
about.  An annual "flu shot" is strongly recommended for people over 65, as
well as people of any age with a chronic disease, especially of the heart or
lungs.  This same high-risk group also should receive a vaccine every five
years against bacterial pneumonia and other related infections.

    In general, healthy young adults do not need to be immunized against
influenza, since the disease is not as debiliating for them, and the vaccine
is available only in limited quantities.  If you work in a hospital or health-
care setting, however, it may be wise to receive the shot so that you do not
transmit the flu viruses to your patients.

A vaccine is now available that offers protection from acute hepatitis B, a
viral infection of the liver that may eventually cause such serious
complications as scarring of the liver.  The disease is contagious only
through blood and contact with blood, and therefore is recommended only for
certain health-care workers, travelers to parts of the world where the disease
is endemic, homosexually active men and drug abusers.  It is not recommended
for anyone whose immune system is already compromised by such a disease as
AIDS.
!
*
                         Aging Research:
                 Practice, Promise, & Priorities
                   National Institute on Aging
                  National Institutes of Health

     NIA is responsible for the "conduct and support of
     biomedical, social, and behavioral research, training,
     health information dissemination, and other programs with
     respect to aging process and the diseases and other special
     problems and needs of the aged."

Research on Aging Act of 1974, as amended in 1990 by P.L. 101-557

                         Aging Research:
                 Practice, Promise, & Priorities


    Are scientists close to finding a cure for Alzheimer's
disease? Do older people need as much sleep as younger people?
Should drivers be retested regularly after age 65 in order to
keep their license? These are a few of the questions scientists
hope to answer as we come to understand aging, basic aging
processes, the difference between normal aging and disease, and
ways to slow deterioration in various body systems. Increasingly
research holds the promise that illnesses and disabilities
associated with old age can be reduced or eliminated.

    Aging Research: Practice, Promise, & Priorities looks at the
vital role research plays in improving the health of older
Americans. Fundamental to the practice of clinical medicine,
research enables doctors to effectively diagnose and treat their
older patients. For example, recent studies on incontinence show
symptoms can be reduced in a majority of people through
behavioral therapy, without resorting to drug treatment or
invasive surgical procedures. Research also yields information on
how to prevent health problems-such as the use of exercise by
frail older people to develop the body strength necessary for
preventing fall accidents and related injuries.

    The National Institute on Aging (NIA) was established in
1974 when Congress authorized the Research on Aging Act, and
through later amendments to the Public Health Service Act
(sections 443-445F). NIA is one of 19 institutes at the National
Institutes of Health (NIH) and leads the federal effort on aging
research. To carry out its mission, NIA engages in collaborative
activities with other NIH institutes and federal agencies.


NIA Priorities

The field of aging includes a broad range of health issues that
affect older people. Which topics are designated priorities
depends on such factors as severity of the condition, the number
of Americans affected, and the burden of cost. While the
institute supports studies in a large number of areas, it focuses
particular attention on the following topics.

Alzheimer's Disease

Alzheimer's disease (AD) is a progressive disorder characterized
by changes in brain function that impairs the cognitive capacity
of patients. Researchers estimate that more than 4 million people
in the United States have some form of dementia, costing the
Nation over $90 billion annually. NIA is especially interested in
identifying interventions that may delay the development of
clinical signs of AD. Even a 5-year delay could substantially cut
the prevalence of the disease, saving immense suffering and
billions of dollars each year.

Understanding Aging

NIA-funded research seeks to identify genetic and environmental
factors associated with aging processes. Scientists are isolating
genes believed responsible for the onset of age-related diseases,
as well as those conferring health and longevity. Other areas of
interest are the modifications proteins undergo with age; control
of cell proliferation; biomarkers of aging; relationships between
healthy aging and disease; effects of aging on the body's immune,
endocrine, and other physiologic systems; the neuropsychology and
cognitive psychology of aging; and lifestyle factors affecting
health and disease, such as diet and exercise.

Frailty, Disability, and Rehabilitation

NIA supports studies on osteoporosis, as well as on falls and
gait disorders which so often lead to frailty and disabilities in
older people.

Hip fractures in frail older people are a major epidemic today,
causing extended hospitalization, institutionalization, and the
loss of independence-all at great personal and financial cost.
The institute sponsors clinical trials to identify interventions
for reducing the physical frailty and balance problems associated
with falls and hip fractures. NIA also sponsors clinical trials
to develop treatments for preventing osteoporotic bone loss in
the hips.

Health and Independence in Later Years

Research supported by NIA emphasizes strategies for maintaining
health and independence, improving quality of life, and
preventing or postponing disabilities during the later years.

Adequate nutrition is important to ensure good health throughout
life. In this area, researchers are studying changes in the
ability of older individuals to taste and smell, changes in their
eating patterns, and metabolic disease (such as diabetes) or
dietary factors that are associated with depressed
immunocompetence and cognitive function.

Research in exercise physiology is designed to assess the effects
of physical activity on the health of older people, as well as
the ability of exercise to prevent physical decline and various
diseases-including diabetes, hypertension, osteoporosis, and
stroke.

Long-Tern Care for Older Adults

Researchers are looking for ways to reduce the need for
long-terms care and institutionalization. They are also studying
ways to enhance the quality of long-term care, ease the burden of
family care, and forecast the requirements for long-term care.

Minority Populations

Improving the health and longevity of minority populations in
possible with continuing progress in research-such as on life
expectancy, family structures and social networks, and
environmental influences, as well as the development of special
interventions for minorities. NIA also actively encourages the
participation of minorities as researchers in the field of aging.

Demographics of Aging

Institute research examines the socioeconomic and demographic
characteristics of older people, with special attention to
populations such as the oldest old, minorities, older adults who
are handicapped, and rural populations.

Women's Health and Aging

Because the majority of people over age 65 are women, the
institute eagerly supports research on older women's health.
Activities include the longitudinal Women's Health and Aging
Study,m which examines the causes and progression of disability
in older women. Other institute studies examine osteoporosis,
menopause, and hormone replacement therapy.


Training and Career Development

NIA uses a variety of funding mechanisms to train individuals for
careers in geriatric research and teaching, offering special
encouragement of minority members developing health science
careers. The Claude D. Pepper Older Americans Independence
Centers is one approach taken by the institute for training
future academic leaders in geriatric medicine.

International Activities

International studies on the health of different racial and
ethnic populations offer scientists unique research
opportunities. NIA scientists working in intramural research
programs host foreign investigators under NIH's Fogarty
International Visiting Program. The institute sponsors
cross-national research by foreign and domestic investigators
through its extramural program. Support for these research
activities, including conferences, is provided by a number of
international agreements. NIA is designated as a World Health
Organization Collaborating Center for Research on Health of
Elderly. Researchers participate in a comprehensive,
multinational program that focuses on dementia, determinants of
healthy aging, immunology, and nutritional aspects of
osteoporosis.

Health Information Dissemination

Educating the public about new research findings and the benefits
of a healthy lifestyle is an important institute objective. NIA's
Public Information Office produces materials (such as articles,
brochures, reports, and fact sheets) covering a broad range of
aging topics for older people and their families, health
professionals, media reporters, government agencies, the general
public, and groups with a special interest in aging. Special
activities and media presentations also help increase public
awareness of health issues, reinforce knowledge of helpful
behaviors, and encourage Americans to become active in
safeguarding their health.


Organization

NIA sponsors research through intramural and extramural programs.
The intramural program conducts basic and clinical research in
Baltimore, MD, and on the NIH campus in Bethesda, MD. The
extramural program funds research and training at universities,
hospitals, medical centers, and other public and private
organizations nationwide.





NIA Organization Chart

<the organizational chart was deleted as it could not be
formatted as text--the major organizations are presented in
outline form>


Office of the Director and Deputy Director

     Office of Administrative Management

     Office of Alzheimer's Disease Research

     Public Information Office

     Office of Extramural Affairs

     Office of Planning, Analysis, and International
     Activities


Extramural Programs

     Behavioral and Social Program

     Biology of Aging Program

     Geriatrics Program

     Neuroscience and Neuropsychology
     of Aging Program


Intramural Programs

     Intramural Research Program
     (Gerontology Research Center)

          Laboratory of Behavior Sciences
          Laboratory of Biological Chemistry
          Laboratory of Cardiovascular Science
          Laboratory of Cellular and Molecular Biology
          Laboratory of Clinical Physiology
          Laboratory of Molecular Genetics
          Laboratory of Neuroscience
          Laboratory of Personality and Cognition
          Longitudinal Studies Branch
          Research Resources Branch

     Epidemiology, Demography, and Biometry Program





Intramural Research Program

The intramural program is located at the Gerontology Research
Center in Baltimore (the Laboratory of Neurosciences and the
Epidemiology, Demography, and Biometry Program are in Bethesda).
Intramural scientists study aging processes, develop
interventions that will restore or maintain normal function in
older individuals, and contribute new knowledge to prevent and
treat age-associated diseases and disabilities. The intramural
program offers postdoctoral training to promising investigators
(m.D.s and Ph.D.s) for research careers in gerontology and
geriatrics. It also conducts the landmark Baltimore Longitudinal
Study of Aging (BLSA). Begun in 1958, the BLSA seeks to
understand how and why we age by following over, 1,100 men and
women who return to Baltimore every 2 years for intensive
evaluation.

The Epidemiology, Demography, and Biometry Program conducts
intramural research on the epidemiology of health and disease, as
well as on demographic, social, metabolic, and genetic factors
affecting the health of older people.

Staff collect and analyze information from established community
and special populations, surveys conducted by the National Center
for Health Statistics, and other sources.

Extramural Research Program

NIA's extramural research consists of four program areas:
Behavioral and Social Research, Biology of Aging, Geriatrics, and
Neuroscience and Neuropsychology of Aging.

The Behavioral and Social Research Program supports social and
behavioral research and training on aging processes and the role
of older people in society. It focuses on changes, as well as
aspects of stability, in psychological and cognitive functioning
and the relationship of older people to their family and
community. Demographic research on the changing age composition
of the population and the impact of the aging population on
society are also emphasized. Special attention is given to
studies on how to maintain and even enhance health and
independence in later years, improve long-term care, expand
opportunities for older workers, and understand the impact and
implications of retirement.

The Biology of Aging Program supports research on the basic
mechanisms involved in aging processes and the onset of
age-related disease. It funds research and training in
biochemistry, molecular and cell biology, genetics, immunology,
endocrinology, nutrition, and pathobiology. The program also
assists facilities that provide investigators with aging animals
and cell cultures for use in research.


The Geriatrics Program sponsors research on the causes,
prevention, and treatment of older people's health problems. It
supports research on physical frailty, osteoporosis,
musculoskeletal and cardiovascular disorders, nutrition,
infections, and endocrinology. The program also maintains
research training through programs such as the Claude D. Pepper
Older Americans Independence Centers.

The Neuroscience and Neuropsychology of Aging Program supports
research on the structure and function of the aging nervous
system, and the behavioral manifestations of the aging
brain. Research areas include age-related changes in the nervous
system, especially as they affect sensory processes, learning,
memory, and sleep. Of special interest are studies on the
epidemiology, cause, diagnosis, treatment, and management of
symptoms of Alzheimer's disease and other disorders associated
with the aging nervous system.

The Office of Alzheimer's Disease Research (OADR) is located
within NIA. The institute is the lead agency in federal efforts
to study the disease. OADR coordinates research activities and
advises the director and other staff on Alzheimer'-related
issues. It also manages the NIH Coordinating Committee on
Alzheimer's Disease, supports collaborative efforts with outside
agencies, and sponsors international scientific workshops.

The Public Information Office plans and carries out a
legislatively mandated program to educate the general public,
media, physicians and health care workers, other government
agencies, and service organizations about aging research.

The Promise of Research

Today's need for reducing health care costs demands the rapid
passage of new scientific information from laboratory to clinical
practice. Since the institute was established 20 years ago,
NIA-funded scientists have reported progress in many areas of
aging. The research advances on the following pages are a
selection of recent findings.

Brain Changes and Intellectual Function

    Scientists have identified the common gene apolipoprotein E,
usually associated with moving cholesterol in the blood, as an
important risk-factor associated with late-onset Alzheimer's
disease, one of the most frequent forms of this disease.

    Cognitive training can improve the intellectual abilities of
older people. Early training sessions have been found to offer
the greatest benefit for those older people who start at a low,
cognitively disadvantaged level.

    Accidents involving older drivers are a problems, with most
happening at intersections, rights-of-way, and during lane
changes. A strong predictor of driving accidents in older people
is the Useful Field of View test that measures the spatial area
in which someone can rapidly take in information from multiple
sources.

Promoting Good Health

    Research increasingly shows people can stay vigorous into
their later years by avoiding smoking and obesity and by getting
moderate exercise.

    Scientists conduct animal studies to better understand the
role of diet in humans. They found that rodents who consumed 40
percent fewer calories not only lived longer, but also had
significantly fewer signs of disease and cancerous tumors than
those on a regular diet.

    Those men and women with higher levels of
education-regardless of race-can expect to stay healthier 2 1/2
to 4 years longer than people with less schooling.

    A higher bone mass protects against osteoporotic fractures
in both older women and men. A recent study added to our
understanding of the effects of bone density by showing greater
bone mass is linked with increased calcium intake during
childhood.

    Falls and impaired mobility in frail older people are often
linked to muscle weakness in the lower body. Scientists looking
for ways to increase lower body strength found that even
nursing-home residents in their nineties participating in
strength-training exercises improved their strength, gait speed,
and ability to climb stairs. Their peers who took nutritional
supplements instead of exercising had no improvement.

    Older people with risk factors for falling can also
dramatically reduce the chance of a fall by learning safer ways
to walk and by substituting or stopping certain medicines.

    Physicians may be able to detect prostate cancer years
earlier than was previously possible by monitoring yearly changes
in the level of an enzyme, prostate specific antigen (PSA).

Women' Health

    The number of people age 85 and older is growing rapidly and
is soon expected to make up almost a quarter of the older
population, with women outnumbering men 5 to 2. Disability rates
in this group are high and poverty is prevalent. Moreover, women
85 and over are among those most likely to be poor and
institutionalized.

    Biofeedback and exercise can be highly effective in reducing
incontinent episodes for many women. Scientists found that both
biofeedback and pelvic muscle exercises could reduce incidents by
as much as 50 to 99 percent in some individuals.

The Heart

    Older people with isolated systolic hypertension (in which
the upper number [systolic] in the blood pressure ratio is
elevated while the lower number [diastolic] remains normal)
benefit from a low dose of a diuretic reduces the systolic
pressure, lowering the risk of stroke and coronary heart disease.

    Scientists are learning more about atherosclerosis by
examining the smooth cells that make up blood vessels. When these
cells are injured by fatty streaks or plaques, they tend to
migrate toward the inside surface of the vessel and quickly
divide. This altered structure and function of smooth cells
contributes to the destruction of normal tissue and disease.

Genetics and Aging

    Gene therapy allows the manufacture of a product, such as a
missing enzyme or a desired hormone, in the patient's won body.
The recent discovery of an effective cellular vehicle to deliver
such a therapeutic product brings scientists closer to being able
to use gene therapy to retard or reverse some of the consequences
of aging.

    Older people are often more susceptible to environmental
stresses, such s hot or cold temperatures, than younger people.
Shedding light on a possible cause for this susceptibility,
rodent studies show the heat shock response (a selective
expression of specific genes to heat or other types of stress)
becomes altered in older animals and this could reduce the
animal's ability to respond effectively to extreme temperatures.


Additional Information:

NIA Programs and Research
Public Information Office
Building 31, Room 5C27
Bethesda, MD 2089
[phone]

Publications by NIA
NIA Information Center
[phone]
[phone] for TTY

Alzheimer's Disease
Alzheimer's Disease Education
and Referral Center (ADEAR)
P.O. Box 8250
Silver Spring, MD 20910
[phone]

U.S. Department of Health and Human Services
Public Health Service
National Institutes of Health
March 1994

!
*
What's Your Aging I.Q.?

True or False

1.Baby boomers are the fastest growing segment of the population.
2.Families don't bother with their older relatives.
3.Everyone becomes confused or forgetful if they live long
  enough.
4.You can be too old to exercise.
5.Heart disease is a much bigger problem for older men than for
  older women.
6.The older you get, the less you sleep.
7.People should watch their weight as they age.
8.Most older people are depressed.  Why shouldn't they be?
9.There's no point in screening older people for cancer because
  they can't be treated.
10.Older people take more medications than younger people.
11.People begin to lose interest in sex around age 55.
12.If your parents had Alzheimer's disease, you will inevitably
   get it.
13.Diet and exercise reduce the risk for osteoporosis.
14.As your body changes with age, so does your personality.
15.Older people might as well accept urinary accidents as a fact
   of life.
16.Suicide is mainly a problem for teenagers.
17.Falls and injuries "just happen" to older people.
18.Everybody gets cataracts.
19.Extremes of heat and cold can be especially dangerous for older
   people.
20."You can't teach an old dog new tricks."


Answers

1.  False.
There are more than 3 million Americans over the age of 85.  That
number is expected to quadruple by the year 2040, when there will
be more than 12 million people in that age group.  The population
age 85 and older is the fastest growing age group in the U.S.

2.  False.
Most older people live close to their children and see them often.
Many live with their spouses.  An estimated 80 percent of men and
60 percent of women live in family settings.  Only 5 percent of the
older population lives in nursing homes.

3.  False.
Confusion and serious forgetfulness in old age can be caused by
Alzheimer's disease or other conditions that result in irreversible
damage to the brain.  But at least 100 other problems can bring on
the same symptoms.  A minor head injury, high fever, poor
nutrition, adverse drug reactions, and depression also can lead to
confusion.  These conditions are treatable, however, and the
confusion they cause can be eliminated.

4.  False.
Exercise at any age can help strengthen the heart and lungs and
lower blood pressure.  It also can improve muscle strength and, if
carefully chosen, lessen bone loss with age.  See a physician
before beginning a new exercise program.

5.  False.
The risk of heart disease increases dramatically for women after
menopause.  By age 65, both men and women have a one in three
chance of showing symptoms.  But risks can be significantly reduced
by following a healthy diet and exercising.

6.  False.
In later life, it's the quality of sleep that declines, not total
sleep time.  Researchers have found that sleep tends to become more
fragmented as people age.  A number of reports suggest that older
people are less likely than younger people to stay awake throughout
the day and that older people tend to take more naps than younger
people.

7.  True.
Most people gain weight as they age.  Because of changes in the
body and decreasing physical activity, older people usually need
fewer calories.  Still, a balanced diet is important.  Older people
require essential nutrients just like younger adults.  You should
be concerned about your weight if there has been an involuntary
gain or loss of 10 pounds in the past 6 months.

8.  False.
Most older people are not depressed.  When it does occur,
depression is treatable throughout the life cycle using a variety
of approaches, such as family support, psychotherapy, or
antidepressant medications.  A physician can determine whether the
depression is caused by medication an older person might be taking,
by physical illness, stress, or other factors.

9.  False.
Many older people can beat cancer, especially if it's found early.
Over half of all cancers occur in people 65 and older, which means
that screening for cancer in this age group is especially
important.

10  True.
Older people often have a combination of conditions that require
drugs.  They consume 25 percent of all medications and can have
many more problems with adverse reactions.  Check with your doctor
to make sure all drugs and dosages are appropriate.

11. False.
Most older people can lead an active, satisfying sex life.

12. False.
The overwhelming number of people with Alzheimer's disease have not
inherited the disorder.  In a few families, scientists have seen an
extremely high incidence of the disease and have identified genes
in these families which they think may be responsible.

13. True.
Women are at particular risk for osteoporosis.  They can help
prevent bone loss by eating foods rich in calcium and exercising
regularly throughout life.  Foods such as milk and other dairy
products, dark green leafy vegetables, salmon, sardines, and tofu
promote new bone growth.  Activities such as walking, biking, and
simple exercises to strengthen the upper body also can be
effective.

14. False.
Research has found that, except for the changes that can result
from Alzheimer's disease and other forms of dementia, personality
is one of the few constants of life.  That is, you are likely to
age much as you've lived.

15. False.
Urinary incontinence is a symptom, not a disease.  Usually, it is
caused by specific changes in body function that can result from
infection, diseases, pregnancy, or the use of certain medications.
A variety of treatment options are available for people who seek
medical attention.

16. False.
Suicide is most prevalent among people age 65 and older.  An older
person's concern with suicide should be taken very seriously and
professional help should be sought quickly.

17. False.
Falls are the most common cause of injuries among people over age
65.  But many of these injuries, which result in broken bones, can
be avoided.  Regular vision and hearing tests and good safety
habits can help prevent accidents.  Knowing whether your
medications affect balance and coordination`1 also is a good idea.

18. False.
Not everyone gets cataracts, although a great many older people do.
Some 18 percent of people between the ages of 65 and 74 have
cataracts, while more than 40 percent of those between 75 and 85
have the problem.  Cataracts can be treated very successfully with
surgery; more than 90 percent of people say they can see better
after the procedure.

19. True.
The body's thermostat tends to function less efficiently with age,
making the older person's body less able to adapt to heat or cold.

20. False.
People at any age can learn new information and skills.  Research
indicates that older people can obtain new skills and improve old
ones, including how to use a computer.



U.S. Department of Health and Human Services
Public Health Service
National Institutes of Health

October 1991
!
*
SOMETHING IN THE AIR ABOUT AIRBORNE ALLERGENS

National Institutes of Health
National Institute of Allergy and Infectious Diseases


Contents

Introduction
Symptoms of Allergies to Airborne Substances
Pollen Allergy
Mold Allergy
Dust Allergy
Animal Allergy
Chemical Sensitivity
Diagnosing Allergic Diseases
Treating Allergic Diseases
Allergy Research


Introduction

When is sneezing not a symptom of a cold? Very often, when it
represents an allergic reaction to something in the air.  It is
estimated that 35 million Americans suffer from upper respiratory
allergic reactions to airborne pollen. Pollen allergy, commonly
called hay fever, is one of the most common chronic diseases in
the United States.  Worldwide, airborne dust causes the most
problems for people with allergies.  The respiratory symptoms of
asthma, which affects approximately 15 million Americans, are
often provoked by airborne allergens (substances that cause an
allergic reaction).
     Allergic diseases are among the major causes of illness and
disability in the United States, affecting as many as 40 to 50
million Americans.  The National Institute of Allergy and
Infectious Diseases, a component of the National Institutes of
Health, conducts and supports research on allergic diseases.  The
goals of this research are to provide a better understanding of
the cause of allergy, to improve the methods for diagnosing and
treating allergic reactions, and eventually to prevent allergies.
This booklet summarizes what is known about the causes and
symptoms of allergic reactions to airborne allergens, how these
reactions are diagnosed and treated, and what medical researchers
are doing to help people who suffer from these allergies.

What is an allergy?

An allergy is a specific immunologic reaction to a normally
harmless substance, one that does not bother most people.
Allergic people often are sensitive to more than one substance.
Types of allergens that cause allergic reactions include food,
dust particles, medicines, insect venom, mold spores, or pollen.


Why are some people allergic to these substances while others are
not?

Scientists think that people inherit a tendency to be allergic,
although not to any specific allergen.  Children are much more
likely to develop allergies if their parents have allergies.
Even if only one parent is allergic, a child has a one in four
chance of developing allergies.  Exposure to allergens at certain
times when the body's defenses are lowered or weakened, such as
after a viral infection, during puberty, or during pregnancy,
seems to contribute to the development of allergies.

WHAT IS AN ALLERGIC REACTION?

Normally, the immune system functions as the body's defense
against invading agents such as bacteria and viruses.  In most
allergic reactions, however, the immune system is responding to a
false alarm.  When allergic persons first come into contact with
an allergen, their immune systems treat the allergen as an
invader and mobilize to attack.  The immune system does this by
generating large amounts of a type of antibody (a
disease-fighting protein) called immunoglobulin E, or IgE.  Only
small amounts of IgE are produced in nonallergic people.  Each
IgE antibody is specific for one particular allergenic
(allergy-producing) substance. in the case of pollen allergy, the
antibody is specific for each type of pollen: one antibody may be
produced to react against oak pollen and another against ragweed
pollen, for example.

    These IgE molecules attach themselves to the body's mast
cells, which are tissue cells, and to basophils, which are blood
cells.  When the allergen next encounters the IgE, it attaches to
the antibody like a key fitting into a lock, signalling the cell
to which the IgE is attached to release (and in some cases to
produce) powerful inflammatory chemicals like histamine,
prostaglandins, and leukotrienes.  These chemicals move into
various parts of the body, such as the respiratory system, and
cause the symptoms of allergy.

    Some people with allergy develop asthma.  The symptoms of
asthma include coughing, wheezing, and shortness of breath due to
a narrowing of the bronchial passages (airways) in the lungs and
to excess mucus production.  Asthma can be disabling and
sometimes can be fatal.  If wheezing and shortness of breath
accompany allergy symptoms, it is a signal that the bronchial
tubes also have become involved, indicating the need for medical
attention.







SYMPTOMS OF ALLERGIES TO AIRBORNE SUBSTANCES


The signs and symptoms are familiar to many:

     Sneezing often accompanied by a runny or clogged nose

     Coughing and postnasal drip

     Itching eyes, nose, and throat

     Allergic shiners (dark circles under the eyes caused by
     increased blood flow near the sinuses)

     The "allergic salute" (in a child, persistent upward rubbing
     of the nose that causes a crease mark on the nose)

     Watering eyes

     Conjunctivitis (an inflammation of the membrane that lines
     the eyelids, causing red-rimmed, swollen eyes and
     crusting of the eyelids).

     In people who are not allergic, the mucus in the nasal
passages simply moves foreign particles to the throat, where they
are swallowed or coughed out.  But something different happens to
a person who is sensitive to airborne allergens.
     As soon as the allergen lands on the mucous membranes lining
the inside of the nose, a chain reaction occurs that leads the
mast cells in these tissues to release histamine.  This powerful
chemical enlarges the many small blood vessels in the nose.
Fluids escape through these expanded vessel walls, which causes
the nasal passages to swell, resulting in nasal congestion.
     Histamine can also cause sneezing, itching, irritation, and
excess mucus production, which can result in allergic rhinitis
(runny nose).  Other chemicals made and released by mast cells,
including prostaglandins and leukotrienes, also contribute to
allergic symptoms.


POLLEN ALLERGY

Each spring, summer, and fall tiny particles are released from
trees, weeds, and grasses.  These particles, known as pollen,
hitch rides on currents of air.  Although their mission is to
fertilize parts of other plants, many never reach their targets.
Instead, they enter human noses and throats, triggering a type of
seasonal allergic rhinitis called pollen allergy, which many
people  know as hay fever or rose fever (depending on the season
in which the symptoms occur).

     Of all the things that can cause an allergy, pollen is one
of the most widespread.  Many of the foods, drugs, or animals
that cause allergies can be avoided to a great extent-
even insects and household dust are escapable.  Short of staying
indoors when the pollen count is high-and even that may not
help-there is no easy way to evade windborne pollen.
     People with pollen allergies often develop sensitivities to
other troublemakers that are present all year, such as dust.  For
these allergy  sufferers, the "sneezin' season" has no limit.
Year-round airborne allergens cause perennial allergic rhinitis,
as distinguished from seasonal allergic rhinitis.

WHAT IS POLLEN?

Plants produce microscopic round or oval pollen grains to
reproduce.  In some species, the plant uses the pollen from its
own flowers to fertilize itself Other-types must be
cross-pollinated; that is, in order for fertilization to take
place and seeds to form, pollen must be transferred from the
flower of one plant to that of another plant of the same
species.  Insects do this job for certain flowering plants, while
other plants rely on wind transport.
     The types of pollen that most commonly cause allergic
reactions are produced by the plain-looking plants (trees,
grasses, and weeds) that do not have showy flowers.  These plants
manufacture small, light, dry pollen granules that are
custom-made for wind transport.  Samples of ragweed pollen have
been collected 400 miles out at sea and 2 miles high in the air.
Because airborne pollen is carried for long distances, it does
little good to rid an area of an offending plant-the pollen can
drift in from many miles away.  In addition, most allergenic
pollen comes from plants that produce it in huge quantities.  A
single ragweed plant can generate a million grains of pollen a
day.


     The chemical makeup of pollen is the basic factor that
determines whether it is likely to cause hay fever.  For example,
pine tree pollen is produced in large amounts by a common tree,
which would make it a good candidate for causing allergy.  The
chemical composition of pine pollen, however, appears to make it
less allergenic than other types.  Because pine pollen is heavy,
it tends to fall straight down and does not scatter.  Therefore,
it rarely reaches human noses.
     Among North American plants, weeds are the most prolific
producers of allergenic pollen.  Ragweed is the major culprit,
but others of importance are sagebrush, redroot pigweed, lamb's
quarters, Russian thistle (tumbleweed), and English plantain.
     Grasses and trees, too, are important sources of allergenic
pollens.  Although more than 1,000 species of grass grow in North
America, only a few produce highly allergenic pollen.  These
include timothy, Johnson, Bermuda, redtop, orchard, sweet vernal,
and Kentucky bluegrass. Trees that produce allergenic pollen
include oak, ash, elm, hickory, pecaii, box elder, and mountain
cedar.
     It is common to hear people say that they are allergic to
colorful or scented flowers like roses. In fact, only florists,
gardeners, and others who have prolonged, close contact with
flowers are likely to become sensitized to pollen from these
plants.  Most people have little contact with the large, heavy,
waxy pollen grains of many flowering plants because this type of
pollen is not carried by wind but by insects such as butterflies
and bees.

WHEN DO PLANTS MAKE POLLEN?

One of the most obvious features of pollen allergy is its
seasonal nature-people experience its symptoms only when the
pollen grains to which they are allergic are in the air.  Each
plant has a pollinating period that is more or less the same from
year to year.  Exactly when a plant starts to pollinate seems to
depend on the relative length of night and day and therefore on
geographical location-rather than on the weather. (On the other
hand weather conditions during pollination can affect the amount
of pollen produced and distributed in a specific year.) Thus, the
farther north you go, the later the pollinating period and the
later the allergy season.

     A pollen count which is familiar to many people from local
weather reports, is a measure of how much pollen is in the air.
This count represents the concentration of all the pollen (or of
one particular type, like ragweed) in the air in a certain area
at a specific time.  It is expressed in grains of pollen per
square meter of air collected over 24 hours.  Pollen counts tend
to be highest early in the morning on warm, dry, breezy days and
lowest during chilly, wet periods.  Although a pollen count is an
approximate and fluctuating measure, it is useful as a general
guide for when it is advisable to stay indoors and avoid contact
with the pollen.



MOLD ALLERGY

Along with pollens from trees, grasses, and weeds, molds are an
important cause of seasonal allergic rhinitis.  People allergic
to molds may have symptoms from spring to late fall.  The mold
season often peaks from July to late summer.  Unlike pollens,
molds may persist after the first killing frost.  Some can grow
at subfreezing temperatures, but most become dormant.  Snow cover
lowers the outdoor mold count drastically but does not kill
molds.  After the spring thaw, molds thrive on the vegetation
that has been killed by the winter cold.
     In the warmest areas of the United States, however, molds
thrive all year and can cause year-round (perennial) allergic
problems.  In addition, molds growing indoors can cause perennial
allergic rhinitis even in the coldest climates.

WHAT IS MOLD?

There are thousands of types of molds and yeast, the two groups
of plants in the fungus family.  Yeasts are single cells that
divide to form clusters.  Molds consist of many cells that grow
as branching threads called hyphae.  Although both groups can
probably cause allergic reactions only a small number of molds
are widely recognized offenders.

     The seeds or reproductive particles of fungi are called
spores.  They differ in size, shape, and color among species.
Each spore that germinates can give rise to new mold growth,
which in turn can produce millions of spores.



WHAT IS MOLD ALLERGY?

When inhaled, microscopic fungal spores or, sometimes, fragments
of fungi may cause allergic rhinitis.  Because they are so
small, mold spores may evade the protective mechanisms of the
nose and upper respiratory tract to reach the lungs and bring on
asthma symptoms.  Build-up of mucus, wheezing, and difficulty  in
breathing are the result.  Less frequently, exposure to spores or
fragments may lead to a lung disease known as hypersensitivity
pneumonitis, which will be discussed later.
     In a small number of people, symptoms of mold allergy may be
brought on or worsened by eating certain foods, such as cheeses,
processed with fungi.  Occasionally, mushrooms, dried fruits, and
foods containing yeast, soy sauce, or vinegar will produce
allergic symptoms. There is no known relationship, however,
between a respiratory allergy to the mold Penicillium and an
allergy to the drug penicillin, made from the mold.

WHERE DO MOLDS GROW?

Molds can be found wherever there is moisture, oxygen, and a
source of the few other chemicals they need. In the fall they
grow on rotting logs and fallen leaves, especially in moist,
shady areas. In gardens, they can be found in compost piles and
on certain grasses and weeds. Some molds attach to grains such as
wheat, oats, barley, and corn, making farms, grain bins, and
silos likely places to find mold.

  Hot spots of mold growth in the home include damp basements
and closets, bathrooms (especially shower stalls), places where
fresh food is stored, refrigerator drip trays, house plants, air
conditioners, humidifiers, garbage pails, mattresses, upholstered
furniture, and old foam rubber pillows.
  Bakeries, breweries, bams, dairies, and greenhouses are
favorite places for molds to grow. Loggers, mill workers,
carpenters, furniture repairers, and upholsterers often work in
moldy environments.

WHICH MOLDS ARE ALLERGENIC?

Like pollens, mold spores are important airborne allergens only
if they are abundant, easily carried by air currents, and
allergenic in their chemical makeup. Found almost everywhere,
mold spores in some areas are so numerous they often outnumber
the pollens in the air.  Fortunately however only a few dozen
different types are significant allergens.
   In general, Alternaria and Cladosporium (Hormodendrum) are the
molds most commonly found both indoors and outdoors throughout
the United States. Aspergillus, Penicillium, Helminthosporium,
Epicoccum, Fusarium, Mucor, Rhizopus, and Aureobasidium
(Pullularia) are also common.

ARE MOLD COUNTS HELPFUL?

Similar to pollen counts, mold counts may suggest the types and
relative quantities of fungi present at a certain time and place.
For several reasons, however, these counts probably cannot be
used as a constant guide for daily activities. One reason is that
the number and types of spores actually present in the mold count
may have changed considerably in 24 hours because weather and
spore dispersal are directly related. Many of the common
allergenic molds are of the dry spore type-they release, their
spores during dry, windy weather. Other fungi need high humidity,
fog, or dew to release their spores.  Although rain washes many
larger spores out of the air, it also causes
some smaller spores to be shot into the air.
   In addition to the effect of day-to-day weather changes on
mold counts, spore populations may also differ between day and
night. Day favors dispersal by dry spore types and night favors
wet spore types.

ARE THERE OTHER MOLD-RELATED DISORDERS?

Fungi or microorganisms related to them may cause other health
problems similar to allergy. Some kinds of Aspergillus especially
may cause several different illnesses, including both infections
and allergy. These fungi may lodge in the airways or a distant
part of the lung and grow until they form a compact sphere known
as a "fungus ball." In people with lung damage or serious
underlying illnesses, Aspergillus may grasp the opportunity to
invade and actually infect the lungs or the whole body.
   In some individuals, exposure to these fungi can also lead to
asthma or to an illness known as "allergic bronchopulmonary
aspergillosis." This latter condition, which occurs occasionally
in people with asthma, is characterized by wheezing, low-grade
fever, and coughing up of brown-flecked masses or mucous plugs.
Skin testing, blood tests, x-rays, and examination of the sputum
for fungi can help establish the diagnosis. Corticosteroid drugs
are usually effective in treating this reaction; immunotherapy
(allergy shots) is not helpful. The occurrence of allergic
aspergillosis suggests that other fungi might cause similar
respiratory conditions.
   Inhalation of spores from fungus-like bacteria, called actino-
mycetes, and from molds can cause a lung disease called
hypersensitivity pneumonitis. This condition is often associated
with specific occupations. For example, farmers's lung disease
results from inhaling spores growing in moldy hay and grains in
silos. Occasionally, hypersensitivity pneumonitis develops in
people who live or work where an air conditioning or a humidity
unit is contaminated with and emits these spores.
   The symptoms of hypersensitivity pneumonitis may resemble
those of a bacterial or viral infection such as the flu. Bouts of
chills, fever, weakness, muscle pains, cough, and shortness of
breath develop 4 to 8 hours after exposure to the offending
organism. The symptoms gradually disappear when the source of
exposure is removed. If this is not possible, such as in
occupational settings, it may be necessary to increase the
ventilation of the workplace, wear a mask with a filter capable
of removing spores, or change jobs. If hypersensitivity
pneumonitis is allowed to progress, it can lead to serious heart
and lung problems.


DUST ALLERGY

An allergy to dust found in houses is perhaps the most common
cause of perennial allergic rhinitis.  House dust allergy usually
produces symptoms similar to pollen allergy.

What is house dust?

Rather than a single substance, house dust is a varied mixture of
potentially allergenic materials. It may contain fibers from
different types of fabrics; cotton lint, feathers, and other
stuffing materials; bacteria; mold and fungus spores (especially
in damp areas); food particles; bits of plants and insects; and
other allergens peculiar to an individual home.

Dust also may contain microscopic mites. These mites, which also
live in bedding, upholstered furniture, and carpets, thrive in
summer and die in winter. However, in a warm, humid house, they
continue to thrive even in the coldest months. The particles seen
floating in a shaft of sunlight are dead dust mites and their
waste products. These waste-products, which are proteins,
actually provoke the allergic reaction.  House dust mite allergy
is the major year-round allergy in the world, though ragweed
allergy is more prevalent in the United States.
  Waste-products of cockroaches are also an important cause of
allergy symptoms from household allergens, particularly in some
urban areas of the United States.


ANIMAL ALLERGY

Household pets are the main culprits in causing allergic
reactions to animals. It was once thought that pet allergy was
provoked by dander or fur from cats and dogs. Now, however, the
allergen is known to be proteins in the saliva that is present on
the dander or fur. Cats win the prize for causing the most
allergic reactions. One reason may be that cats preen themselves
more than other furry pets. This preening coats
the hairs with saliva containing allergens, which become airborne
when the saliva dries. Also, it may be because cats are held more
and often spend more time in the house, close to humans, than do
dogs.
   Some rodents, such as guinea pigs and gerbils, have become
increasingly popular as household pets. They, too, can cause
allergic reactions in some people. Urine is the major source of
allergens from these animals.
   Allergies to animals can take 2 years or more to develop and
may not subside until 6 months or more after ending contact with
the animal. Carpet and furniture are a reservoir for pet
allergens, and the allergens can remain in them for 4 to 6 weeks.
In addition, these allergens can stay in household air for months
after the animal has been removed. Therefore, it is wise for
people with an animal allergy to check with the landlord or
previous owner to find out if furry pets had lived previously on
the premises.


CHEMICAL SENSITIVITY


"Allergic to the twentieth century" is a phrase that has been
used to describe people who seem to react to everything in their
environment-indoors and outdoors. These allergy-like reactions
can result from exposure to man-made substances, such as those
found in paints or carpeting, or to natural substances, such as
odors emitted by plants and flowers. Although the symptoms may
resemble some of the manifestations of true allergies,
sensitivity to chemicals does not represent a true allergic
reaction.


DIAGNOSING ALLERGIC DISEASES

People with allergy symptoms, such as allergic rhinitis, may at
first suspect they have a cold-but the "cold" lingers on. It is
important to see a doctor about any respiratory illness that
lasts longer than a week or two.  When it appears that the
symptoms are caused by an allergy, the patient should see a
physician who understands the diagnosis and treatment of
allergies. If the patient's medical history indicates that the
symptoms recur at the same time each year, the physician will
work under the theory that a seasonal allergen (like pollen) is
involved.  Properly trained specialists recognize the patterns of
the local seasons and the association between these patterns and
symptoms. The medical history suggests which allergens are the
likely culprits.  The doctor will also examine the mucous
membranes, which often appear swollen and pale or bluish in
persons with allergic conditions.


Skin Tests

To confirm which allergen is responsible, skin testing may be
recommended using extracts from allergens such as dust, pollens,
or molds commonly found in the local area. A diluted extract of
each kind of allergen is injected under the patient's skin or is
applied to a scratch or puncture made on the patient's arm or
back.
   With a positive reaction, a small, raised, reddened area with
a surrounding flush (called a wheal and flare) will appear at the
test site. The size of the wheal can provide the physician with
an important diagnostic clue, but a positive reaction does not
prove that a particular pollen is the cause of a patient's
symptoms. Although such a reaction indicates that IgE antibody to
a specific allergen is present in the skin, respiratory symptoms
do not necessarily result.

Blood Tests

Skin testing is not advisable in some people such as those with
widespread skin conditions like eczema. Diagnostic tests can be
done using a blood sample from the patient to detect levels of
IgE antibody to a particular allergen. One such blood test is
called the RAST (radioallergosorbent test), which can be
performed when eczema is present or if a patient has taken
medications that interfere with skin testing.
   It is expensive to perform, takes several weeks to yield
results, and is somewhat less sensitive than skin testing.
Overall, skin testing is the most sensitive and least costly
diagnostic tool.



TREATING ALLERGIC DISEASES

There are three general approaches to the treatment of these
allergies: avoidance of the allergen, medication to relieve
symptoms, and allergy shots. Although no cure for allergies has
yet been found, one of these strategies or a combination of them
can provide varying degrees of relief from allergy symptoms.

Avoidance

Complete avoidance of allergenic pollen or mold means moving to a
place where the offending substance does not grow and where it is
not present in the air. But even this extreme solution may offer
only temporary relief since a person who is sensitive to a
specific pollen or mold may subsequently develop allergies to new
allergens after repeated exposure. For example, people allergic
to ragweed may leave their ragweed-ridden communities and
relocate to areas where ragweed does not grow, only to develop
allergies to other weeds or even to grasses or trees in their new
surroundings. Because relocating is not a reliable solution,
allergy specialists do not encourage this approach.
   There are other ways to evade the offending pollen: remaining
indoors in the morning, for example, when the outdoor pollen
levels are highest. Sunny, windy days can be especially
troublesome. If persons with pollen allergy must work outdoors,
they can wear face masks designed to filter pollen out of the air
and keep it from reaching their nasal passages. As another
approach, some people take their vacations at the height of the
expected pollinating period and choose a location where such
exposure would be minimal. The seashore, for example, may be an
effective retreat for many with pollen allergies.
   Mold allergens can be difficult to avoid, but some steps can
be taken to at least reduce exposure to them. First, the allergy
sufferer should avoid those hot spots mentioned earlier where
molds tend to be concentrated. The lawn should be mowed and
leaves should be raked up, but someone other than the allergic
person should do these chores. if such work cannot be delegated,
wearing a tightly fitting dust mask can greatly reduce exposure
and resulting symptoms.  Travel in the country, especially on
dry, windy days or while crops are being harvested, should be
avoided as should walks through tall vegetation. A summer cabin
closed up all winter is probably full of molds and should be
aired out and cleaned before a Mold-sensitive person stays there.
   Around the home, a dehumidifier will help dry out the
basement, but the water extracted from the air must be removed
frequently to prevent mold growth in the machine.
   Those with dust allergy should pay careful attention to dust-
proofing their bedroom. The worst things to have in the bedroom
are wall-to-wall carpets, venetian blinds, down-filled blankets,
feather pillows, heating vents with forced hot air, dogs, cats,
and closets full of clothing. Shades are preferred over venetian
blinds because they do not trap dust. Curtains can be used if
they are washed periodically in hot water to kill the dust mites.
Bedding should be encased in a zippered, plastic, airtight, and
dust-proof cover.  Although shag carpets are the worst type for
the dust-sensitive person, all carpets trap dust and make dust
control impossible. In addition, vacuuming can contribute to the
amount of dust, unless the vacuum is equipped with a special
high-efficiency particulate air (HEPA) filter. Wall-to-wall
carpets should be replaced with washable throw rugs over
hardwood, tile, or linoleum floors.

   Reducing the amount of dust in a home may require new
cleaning techniques as well as some changes in furnishings to
eliminate dust collectors. Water is often the secret to effective
dust removal. Washable items should be washed often using water
hotter than 130' Fahrenheit. Dusting with a damp cloth or oiled
mop should be done frequently.
   The best way for a person allergic to pets, especially cats,
to avoid allergic reactions is to find another home for the
animal. There are, however, some suggestions that help keep cat
allergens out of the air: bathe the cat weekly and brush it more
frequently, remove carpets and soft furnishings, and use a vacuum
cleaner with a high-efficiency filter and a room air cleaner (see
section below). Wearing a face mask while house and cat cleaning
and keeping the cat out of the bedroom are other methods that
allow many people to live more happily with their pets.
   Irritants such as chemicals can worsen airborne allergy symp-
toms and should be avoided as much as possible. For example,
during periods of high pollen levels, people with pollen allergy
should try to avoid unnecessary exposure to irritants such as
dust, insect sprays, tobacco smoke, air pollution, and fresh tar
or paint.

AIR CONDITIONERS AND FILTERS

Use of air conditioners inside the home or in a car can help
prevent pollen and mold allergens from entering. Various types of
air-filtering devices made with fiberglass or electrically
charged plates may help reduce allergens produced in the home.
These can be added to the heating and cooling systems. In
addition, portable devices that can be used in individual rooms
are especially helpful in reducing animal allergens.
   An allergy specialist can suggest which kind of filter is best
for the home of a particular patient. Before buying a filtering
device, it is wise to rent one and use it in a closed room (the
bedroom, for instance) for a month or two to see whether allergy
symptoms diminish. The air-flow should be sufficient to exchange
the air in the room five or six times per hour; therefore, the
size and efficiency of the filtering device should be determined
in part by the size of the room.
   Persons with allergies should be wary of exaggerated claims
for appliances that cannot really clean the air. Very small air
cleaners cannot remove dust and pollen-and no air purifier can
prevent viral or bacterial diseases such as influenza, pneumonia,
or tuberculosis.  Buyers of electrostatic precipitators should
compare the machine's ozone output with Federal standards. Ozone
can irritate the nose and airways of persons with allergies,
especially those with asthma, and can increase the allergy
symptoms. Other kinds of air filters such as HEPA filters do not
release ozone into the air.

Medication

For people who find they cannot adequately avoid the allergens,
the symptoms often can be controlled with medications. Effective
medications that can be prescribed by a physician include
antihistamines, topical nasal steroids, and cromolyn sodium-any
of which can be used alone or in combination. Many effective
antihistamines and decongestants also are available without a
prescription.
   Antihistamines. As the name indicates, an antihistamine
counters the effects of histamine, which is released by the mast
cells in the body's tissues and contributes to allergy symptoms.
For many years, antihistamines have proven useful in relieving
sneezing and itching in the nose, throat, and eyes and in
reducing nasal swelling and drainage.
   Many people who take antihistamines experience some distress-
ing side effects: drowsiness and loss of alertness and
coordination. In children, such reactions can be misinterpreted
as behavior problems.  During the last few years, however,
antihistamines that cause fewer of these side effects have become
available by prescription. These new nonsedating antihistamines
are as effective as other antihistamines in preventing
histamine-induced symptoms, but do so without causing sleepiness.
       Topical nasal steroids.  This medication should not be
confused with anabolic steroids that have serious side effects.
Topical nasal steroids are anti-inflammatory drugs that stop the
allergic reaction. In addition to other beneficial actions, they
reduce the number of mast cells in the nose and reduce mucus
secretion and nasal swelling. The combination of antihistamines
and nasal steroids is a very effective way to treat allergic
rhinitis.
  Cromolyn sodium. Cromolyn sodium stops allergic reactions
from starting. It is administered as a nasal spray, and it can
prevent the release of chemicals like histamine from the mast
cell.

IMMUNOTHERAPY

Immunotherapy, or a series of allergy shots, is the only
available treatment that has a chance of reducing the allergy
symptoms over the long haul. Patients receive injections of
increasing concentrations of the allergen(s) to which they are
sensitive. These injections reduce the amount of IgE antibodies
in the blood and cause the body to make a protective antibody
called IgG. About 85 percent of patients with allergic rhinitis
will have a significant reduction in their hay fever symptoms and
in their need for medication within 24 months of starting
immunotherapy. Many patients are able to stop the injections
with good, long-term results. As better allergens for
immunotherapy are produced, this technique will become an even
more effective treatment.


ALLERGY RESEARCH

The National Institute of Allergy and infectious Diseases (NIAID)
conducts and supports research on allergies focused on
understanding what happens to the body during the allergic
process-the sequence of events leading to the allergic response
and the factors responsible for allergic diseases. This
understanding will lead to better methods of preventing and
treating allergies.
   NIAID supports a network of Asthma, Allergic and Immunologic
Diseases Cooperative Research Centers throughout the United
States. The centers encourage close coordination among scientists
studying the immune system, genes, biochemistry, and
pharmacology. This interdisciplinary approach helps move research
knowledge as quickly as possible from research scientists to
physicians and their allergy patients.
   Educating patients and health care workers is an important
tool in controlling allergic diseases. All of these research
centers conduct and evaluate educational programs focused on
methods to control allergic diseases.
   NIAID's National Cooperative Inner-City Asthma Study Centers
are examining ways to prevent asthma in minority children in
inner-city environments. Asthma, a major cause of illness and
death among these children, is provoked by a number of possible
factors, including allergies to airborne substances.
   Although several factors provoke allergic responses,
scientists know that heredity is a major influence on who will
develop an allergy. Therefore, researchers are trying to identify
and describe the genes that make a person susceptible to allergic
diseases.
   Other studies are aimed at seeking better ways to diagnose and
treat people with allergic diseases and to better understand the
factors that regulate IgE production in order to reduce the
allergic response in patients. Several research institutions are
focusing on ways to influence the cells that participate in the
allergic response.
   These studies offer the promise of improving treatment and
control of allergic diseases and the hope that one day allergic
diseases will be preventable as well.

INFORMATION RESOURCES

American Academy of Allergy and Immunology
611 East Wells Street
Milwaukee, WI 53202
1-800-822-ASMA

Asthma and Allergy Foundation of America
1125 15th Street, NW, Suite 502
Washington, DC 20005
1-800-7-ASTHMA

Allergy and Asthma Network
3554 Chain Bridge Road, Suite 200
Fairfax, VA 22030
1-800-878-4403

For information on air-cleaning devices:

Environmental Protection Agency
Public Information Service
401 M Street, SW
Washington, DC 20460
1-800-438-4318

U.S. Dept. of Health and Human Services
Public Health Service
National Institutes of Health
Bethesda, Maryland 20892
!
*
Medicine For The Public: Allergic Diseases

U.S. Department of Health and Human Services
Public Health Service
National Institutes of Health
Warren Grant Magnuson Clinical Center


This patient-oriented publication seeks to increase public
awareness and understanding of allergic diseases and provide
valuable information on the causes and treatments of allergies,
with emphasis on new non-sedating methods of treatment. The
production of this publication was made possible by Marion
Merrell made possible by Marion Merrell Dow Inc., Kansas City,
MO. 64114.

A list of Medicine for the Public booklets and videotapes is
available by calling 301-496-2563; or by sending a postcard to
Clinical Center Communications, National Institutes of Health,
9000 Rockville Pike, building 10, room 1C255, Bethesda, Maryland
20892.

Foreword

Americans continue to demand a greater role in deciding issues
that affect their health.  Increased health awareness and the
convincing evidence linking lifestyle, risk factors, and specific
diseases have accelerated our need to know.

The Clinical Center, recognizing the importance of providing
information to facilitate intelligent decisions on health issues,
created a unique lecture series featuring physician scientists
working at the frontiers of biomedical research at the National
Institutes of Health.

The Medicine for the Public series has provided an opportunity
for millions of people to learn more about how their bodies work
and what they can do to maintain or improve their health.

This publication is one of several adapted from the series.  It
is our sincere hope that you will find this material interesting
and enlightening.

Saul Rosen, Ph.D., M.D.
Acting Director
Warren Grant Magnuson Clinical Center
National Institutes of Health


Allergic Diseases

Who Gets Allergies?

Allergies are incredibly common.  More than 50 million Americans-
I out of 5-suffer from allergic diseases.  One out of every 11
office visits to the doctor is for an allergic disease.

Inheritance has a major influence on allergy.  If one parent has
allergies, the odds are that one in three of the children will
have allergies.  If both parents have allergies, then all the
children will probably have allergies.

Aside from inheritance, it is not known why some people get
allergies and others do not.  Some believe that hormonal
influences, viral infections, smoking, and a number of other
influences affect whether one develops allergies.  No one knows
all the reasons why people with equal likelihood to develop
allergies become allergic to different things, or why some have
hay fever and others have asthma.

Second, a person has to be exposed to an allergen, a foreign
protein that causes allergy.  Ragweed pollen is the major cause
of allergy in the United States.  It is an unusual allergen that
is found in high concentrations only in this country.  Most
people who move to the United States are exposed to ragweed for
the first time.  Many have never had allergies previously in
their families for centuries, yet they develop allergies within
two or three years of living in the United States.  A large part
of why they develop allergies is exposure to ragweed, which is an
incredibly potent allergy-producing plant.

Each ragweed plant produces about one billion pollen grains
during an average allergy season.  Those pollen grains are very
small-microscopic.  They float in the air and may be carried out
to sea as many as 300 or 400 miles.  So it does not matter if
people do not have ragweed plants in their backyards; they are
clearly exposed to ragweed every place in the United States
except for the and southwest and southern California, where
ragweed does not grow.

Other major allergens include grass pollens, tree pollens, dust,
molds, and animal dander.  Worldwide, however, the major allergen
is the dust mite.  All temperate climate areas in the world have
dust mites, which live in carpeting, mattresses, and upholstery.
They have to have temperatures above 60 degrees to reproduce.
Most people keep the temperature in their homes above 60 degrees
all year.  Dust mites also need a relative humidity above 50
percent.

People are not actually allergic to the dust mite; they are
allergic to its feces.  The fecal balls are sticky, heavy
materials that bind to carpeting or upholstery.  One of the worst
ways to bring dust mite allergens into the air is to vacuum the
floor; this blows the dust up into the air, where it floats for a
couple of hours and makes up the moats in a beam of sunlight.

How Do Allergies Work?

There are three components to allergies: mast cells,
which contain chemicals like histamine; antibodies, a specific
type of protein made by the immune system, known as IgE; and
allergens, which trigger the reaction.  Mast cells are the
allergy-causing cells and are found in every tissue throughout
the body, though they are most heavily concentrated in those
tissues that are exposed to the outside world-the skin, linings
of the nose and lungs, gastrointestinal tract, and reproductive
system.

The IgE antibody, which actually causes allergy, sits on the
surface of these mast cells.  A mast cell has about 1,000
histamine-containing granules in its cytoplasm, and on its
surface are between 100,000 and 1 million receptors for IgE.
When the IgE encounters the allergen, it triggers the mast cell
to release granules from its cytoplasm.  Those granules contain
histamine and other chemicals.  These mediators that are released
then interact with the tissues, causing the allergic symptoms.

With ragweed, for example, the pollen grains from the plant are
male gametes equivalent to sperm.  They carry the male genetic
code from a male ragweed plant to a female ragweed plant.  Since
the pollen grains are wind borne and do not have a particular way
of finding a female plant, many excess pollen grains are produced
in the hopes that one or another will find a female plant and
fertilize it.

On the surface of a pollen grain are enzymes that help the pollen
grain enter female plants.  Unfortunately, people breathe in
pollen grains that are floating in the air.  When a pollen grain
gets on the skin that lines the inside of the nose, those enzymes
are released from the pollen grain and work their way through the
mucus in the nose.  The enzymes sensitize the person by
initiating the production of IgE antibodies, which then sensitize
mast cells that are in the nose.  It generally takes about two to
five seasons of allergen exposure before a person makes enough
IgE to result in allergy symptoms.

Everyone makes some IgE.  Only people with genetic
predispositions toward allergies make large quantities.  IgE,
like other antibodies that the body produces, is part of the
body's defense mechanism.  Some antibodies, like IgG, get rid of
Streptococcus and help cure those infected with a strep throat.
Other antibodies get rid of cold viruses.  The IgE antibody is
directed against parasites.  Its function is to protect the body
against parasitic infections.  There are few parasites in the
United States, but the IgE antibody system reacts against the
enzymes from the surface of pollen grains as if they were
parasites and elicits an allergic response.  The body is
misdirecting an extremely important immune response at pollens,
dust, dander, and molds.

Most antibodies last in the body about three weeks, but IgE may
sit on its receptor on mast cells and sensitize the mast cells
for years.  For example, someone who had an adverse allergic
reaction to penicillin as a child could still be allergic to the
drug as an adult.  The IgE antibody the person made as a 6-year-
old child would still be present in the 40-year-old adult.  It is
sitting on the mast cells, which are long-lived cells, and it
conveys incredibly long-lived sensitivity to allergens.

In one research study, we looked at mast cell histamine release
under the electron microscope.  We sensitized a mast cell to IgE,
making it allergic to grass, trees, ragweed, and certain breeds
of cats and dogs.  We then took this mast cell and exposed it to
an extract of ragweed.  Over a 15-minute period, many of the
granules in its cytoplasm disappeared.  Fifteen minutes later,
the granules were all gone.

Although the mast cell had no visible secretary granules in its
cytoplasm, it was alive and well; over the next 6 to 24 hours it
would reconstitute all the granules that had disappeared.  After
releasing histamine from its granules, a mast cell can once again
do damage to the person the next day when he or she breathes in
ragweed.

The chemicals that are released from mast cells are the mediators
that cause allergies.  At last count, there were 28 separate
chemicals released by mast cells that orchestrate allergic
responses.  The allergic symptoms a person experiences depend on
the tissue in which the mediators are released.  For example, if
these chemicals are released in the nose, the person will get hay
fever, allergic rhinitis.  If they are released in the chest, the
person will get asthma or coughing.  Chemicals released in the
skin will produce hives or eczema, in the intestine will produce
food allergy or diarrhea, and in the brain may result in a
migraine.

There is a whole spectrum of problems that these mediators can
cause.

Allergic reactions often take place very quickly.  Those who
experience allergy may go outside on a bright, sunny, windy
morning during the ragweed season and within 15 minutes begin to
have allergic reactions.  This reaction is referred to as
immediate hypersensitivity.  When mast cells release their
chemicals, they cause immediate reactions.

Some people also experience late-phase reactions.  When mast
cells release their chemicals, they cause an inflammatory
response.  The site of the allergic reaction gets red, swollen,
hot, and tender, causing a more prolonged response.  A person may
go out at 8 a.m. and experience a late reaction at 4 p.m. Such
reactions may last one day, two days, one week, or one month from
a single allergen exposure.  They are part of the underlying
problem for chronic asthma, rhinitis, eczema, hives, and other
allergic diseases.

How Are Allergies Diagnosed?

Allergy is diagnosed through skin testing, which shows an
immediate reaction to allergens.  The procedure traditionally
involves introducing a minute amount of allergen into the skin.
The tip of the needle is used to puncture the skin, causing an
interaction between the allergen and a mast cell.

When doctors administer skin tests, they are introducing allergen
into the skin and causing the same reaction that the patients
experience in their noses or lungs during the allergy season.  In
the past, doctors used to take small needles and inject them
under the skin surface and put a minute amount of allergen in the
skin.  Within 15 minutes a welt formed, like a mosquito bite, if
the patient had a positive reaction.  That was minimally
uncomfortable, but it was still uncomfortable.

Today, doctors use a needle to put a drop of allergen on the skin
without breaking the skin surface.  They "tent" the skin by
putting the needle through the droplet and lifting up the skin
without breaking the skin surface.  It is essentially painless,
yet it is very sensitive and specific.

Diagnosis, preferentially, is done by skin testing.  Skin tests
are fast; they take about 5 minutes to administer and can be read
within 15 minutes.  They are very sensitive, relatively cheap,
cause minimal discomfort, but require some medical expertise.

A second way to diagnosis allergy is with a blood test that looks
for IgE antibodies.  This test is relatively expensive, slightly
less sensitive, requires a blood drawing, and does not require
expertise.  It is done frequently by nonallergists who want to
see if the patient has allergies.  In proper hands, both tests
are equally informative.


Allergic Rhinitis

Allergic rhinitis (hay fever) is a disease of incredible
proportion.  Thirty-five million Americans-17 percent of the
population-experience allergic rhinitis.  It is the single most
common chronic disease experienced by human beings.  As a single
entity, 1 out of every 40 doctor office visits (2.5 percent) are
due to allergic rhinitis.

Allergic rhinitis is caused by exposure to airborne allergens.
The process of allergic rhinitis takes place in the nose.  Pollen
grains, dust, and dander are trapped by hairs in the nose and are
trapped in the mucus that lines the inside of the nose.  The
allergens release soluble proteins that reach the mucous
membranes, causing allergic rhinitis.

The skin that lines the inside of the nose is a succulent tissue
full of glands and blood vessels.  The submucous glands in this
living tissue produce the mucus in the nose. In fact, that is why
the lining is called a mucous membrane-it specifically makes
mucus.  Although many people think of mucus as a bother, mucus is
quite helpful.

Mucus is important because it humidities and protects the mucous
membrane.  It contains antimicrobial factors that protect people
from both bacterial and viral infections.  When people get colds,
it is despite the fact that they have mucus; if they did not have
that mucus, they would have infections all the time.

What about nasal congestion? The body has cavities in the lining
of the nose where blood can pool.  The nose can become swollen
with blood pooling in these sinusoids.  When blood is diverted
into these sinusoids, this tissue gets markedly enlarged and the
person cannot breathe through his or her nose.  Everyone
experiences nasal congestion; every 45 minutes to 2 hours, one
side of the nose congests and the other side constricts.  People
breathe preferentially through one side, resting the other side,
and then alternating sides.  One never breathes evenly through
both sides of the nose because of this process of congestion.  Of
course, during allergic rhinitis it gets much worse and the
allergy sufferer experiences more severe, chronic nasal
congestion.

What can the mucous membrane do? It can congest by pooling blood
in these sinusoids; it can become itchy or sneezy by stimulating
some of the sensory nerves in the nose; and it can produce
secretions.  These are the processes that people experience when
they have rhinitis-allergic rhinitis, vasomotor rhinitis,
rhinitis from colds, or from eating hot and spicy foods.

The most common features of allergic rhinitis are sneezing
attacks and itching of the nose, eyes, pharynx, and palate.
Clicking the tongue on the top of the mouth is the way one
scratches the soft palate, and the soft palate itches if one has
allergic rhinitis.  One also gets a runny nose or congestion of
the nose.

To confirm a diagnosis of allergic rhinitis, the doctor performs
a nasal examination and looks for changes in the mucous membrane.
If the patient has allergic rhinitis, the mucous membrane becomes
very pale because it is swollen.  In fact, it takes on a whitish-
blue tint.  It is very wet with a watery secretion.  A smear of
the mucus would be loaded with a type of white blood cell known
as an eosinophil, which is very characteristic of allergic
diseases, and the patient would have an increase of eosinophils
in his or her blood.  If the doctor does a skin test or a blood
test, called a RAST test, which measures an increase in the
patient's IgE antibody, both would be positive.

There are two kinds of allergic rhinitis-seasonal and perennial.
Seasonal allergic rhinitis characteristically occurs as spring-
fall allergies.  Springtime begins with tree allergies.  Grass is
another major springtime allergen.

In the eastern United States, there are few allergens in July and
early August, and individuals with allergic rhinitis get better.
Pollen from plants that bloom in the summer is spread by the
insects, not by the wind, so that is a good time for most people
unless they are allergic to molds.  Seasonal allergies begin
again when ragweed pollinates, starting from mid-August and
lasting until the first frost.

Some people also have seasonal allergies to dust during the
winter months when the house is closed up and the dust mite feces
are richest in the air.  But most people with dust allergy have
year-round-perennial- symptoms.

Other things that cause year-round allergies are molds.  Many
people who live in humid areas have damp cellars in which molds
form.  These molds cause major problems for people with mold
allergies.

The other common cause of year-round allergies is allergens from
pets.  Cats are the worst source of pet allergens, much worse
than dogs.  The source of allergen from cats is not their fur or
skin; it is their saliva, or the proteins in their saliva.  And
what do cats do all day? They preen.  They put saliva on their
fur, the saliva dries, aerosolizes, and is the source for the
allergen.  Dogs-sloppy, friendly little animals that they are-
only preen selected parts of their body and are much less likely
to expose humans to salivary allergens.

Dogs are still a major source of allergy, especially if they
slobber, but if one had to choose between the two, one would
choose a dog over a cat.  Ideally, allergy sufferers would not
have any furred animals in the house because they all cause
allergy.  Cockroaches also are a major source of year-round
allergens.

How Is Allergic Rhinitis Treated?

Seasonal allergic rhinitis generally has a better prognosis
because it is not a year-round exposure. It is easier to treat
and tends to be much less severe.  By contrast, perennial disease
tends to be much more difficult to treat and is harder to
control.

When treating patients with allergic rhinitis, doctors try
specifically to take away the causes of the disease.  By using
allergy avoidance, they get rid of the allergen and the patient
is better.  Other common treatments include antihistamines,
allergy immunotherapy (allergy shots), a drug called cromolyn
sodium, and topical corticosteroids.

To avoid pollen allergens, one must know when the pollen counts
are highest.  They are generally highest early in the morning,
about 6 a.m., on a bright, sunny, breezy day.  That is the time
allergy sufferers should try to stay inside.  If they ride in a
car, they should use the car air conditioner.  Allergy sufferers
should use their house air conditioner, too.  Air conditioning
filters the air very well, taking out more than 99 percent of all
the pollen- and allergen-producing material in the air.  If
people who are very sensitive during the ragweed, grass, or tree
season must go outside in the yard, they should wear nuisance
masks, which are like surgical masks and available in drug
stores.  They are comfortable and will reduce the likelihood of
inhaling allergens.

Those with dust allergy should design their bedrooms accordingly.
The worst things to have in the bedroom of an allergic person are
venetian blinds, which are dust traps; down-filled blankets;
feather pillows; heating vents with forced hot air; carpeting;
dogs; cats; and closets full of clothing.

Instead of venetian blinds, people should have shades over the
windows because they cannot trap dust.  If curtains are used,
they should be washable curtains that are cleaned periodically in
hot water to kill the dust mites.  A hardwood or linoleum floor
is best, but a washable throw rug is acceptable if it is cleaned
in hot water regularly.  The bedding should be encased within
allergen-proof encasements that are airtight to keep in dust
mites, along with their feces.  Pillows should be hypoallergenic
and replaced every one to two years because when people sleep,
they sweat and the sweat makes the pillows moldy over time.
Blankets and bedding should be hypoallergenic-definitely not down
filled.  If at all possible, clothing should be kept in another
room.  Ideally, the closets would be empty in order to remain
dust free.  Heat registers should be covered with a filter.  New
products are appearing in the market that can kill dust mites in
carpeting and may prove to be a boon to allergy sufferers.

Allergy shots are extremely effective.  Eighty-five percent of
the people who receive allergy shots to treat hay fever due to
grass, ragweed, trees, and dust get better.  It usually takes one
to two years.  Many people get better for years, and some even
permanently.  Allergy shots, which are the only known way to turn
off allergic disease, reduce the production of IgE and cause the
body to make another class of antibody called IgG, which actually
protects people from allergic diseases.  Shots are the only
method available for long-lasting protection from allergies.

Patients with problems only two to four weeks of the year usually
are treated with medications.  Those with perennial disease are
more likely to be put on allergy shots unless their condition can
be controlled completely with allergy medications.

There are several medications available to treat allergic
rhinitis.  One is cromolyn sodium administered in the nose, which
prevents allergic reactions from taking place.  This drug
actually stops the release of chemical mediators like histamine
from the mast cell.  It is often a very effective therapy.
Unfortunately, it must be used about four times a day as a nasal
spray.

A second major improvement in therapy of allergic rhinitis has
been the use of topical nasal steroids.  These steroids are not
anabolic steroids.  They are anti-inflammatory steroids that stop
the late phase reaction.  They reduce the number of mast cells in
the nose, reduce mucus secretion and swelling, and have other
beneficial actions.  Because they are given topically, they have
no effects elsewhere in the body-only in the nose.

The other major medication used is antihistamines.  Over the last
five or six years, nonsedating antihistamines have become
available.  These antihistamines are just as effective as older
antihistamines, but they do not cause sleepiness.  However, they
are considerably more expensive than some of the over-the-counter
antihistamines.

Antihistamines work beautifully for immediate hypersensitivity.
They reduce sneezing, itching, runny nose, and partially reduce
the congestion of allergic rhinitis.  Unfortunately, they have no
effect on late phase reactions.  Antihistamines and nasal
steroids are effective combinations to treat allergic rhinitis.

Asthma

Fifteen million people-seven percent of those who live in the
United States-have asthma.  It is the number one cause for school
absenteeism among all chronic diseases.  It is the number six
cause for hospitalization of all diseases and the number one
cause for hospitalization of children.

It is estimated that $4.5 billion is spent every year on
medically related charges for the treatment of asthma.  That
includes hospital and doctor visits.  This is an extremely
important disease that kills as many as 4,000 Americans a year.

Asthma is a disease of the airways, the tubes through which
people breathe.  The causes of airflow obstruction in asthma are
swelling of the airways, excessive mucus production, inflammation
of the airways with eosinophils and neutrophils, and airway
smooth muscle contraction.  Asthmatic airways are full of
secretions, mucus containing eosinophils and neutrophils-white
blood cells-that reflect the underlying inflammation.  The
epithelial cells that line the airways have been lifted off and
the airways are denuded.  The muscles contract, closing the
airways, and the glands are very reactive and produce large
quantities of mucus.

Asthma is an inability to breathe out.  Normally when people
breathe in, they lower their diaphragms, raise their ribs, and
breathe in.  It is an active process.  To breathe out, they stop
breathing in.  Breathing out is passive.  They breathe out
because the lungs are made of elastic tissue, like a rubber band.
When a person stops breathing in, the lungs try to assume their
relaxed size and do so by letting the air out-if there is no
obstruction.

Asthmatic airways have excess mucus, are swollen and inflamed,
and have their muscles contracted.  As people with asthmatic
airways breathe in, they open up their chests and their lungs get
bigger.  As a result, the airways get bigger and they can move
air around these obstructions.  They have opened up the airways.
When they stop breathing in to breathe out, these obstructions
close, thereby trapping the air in the lungs.

Take a deep breath to the maximum and do not let it out for the
next minute.  Breathe at the top of your lungs and do not let out
any air.  That is what it feels like to have asthma.  Asthmatics
trap 2 liters of air in their chests, which is the amount of air
in a basketball.  They have to breathe at the top of their lungs.
It is exhausting and feels terrible.

What Causes Asthma?

The most common cause of asthma is allergy.  Of the children
under 16 years old who have asthma, 90 percent are allergic.  Of
the people under 30 who have asthma, 70 percent are allergic.  Of
the people over 30, 50 percent have allergies.

Asthma also may be caused by infections such as bronchiolitis,
which is a wheezing disease that effects children less than 2
years old and is caused by a viral infection of the airways.
This disease leads to asthma; more than half the children who get
bronchiolitis have asthma until they are at least 7 years old.

Adults with asthma also get infections that make their asthma
worse.  They will have colds that commonly develop into
bronchitis.  Because their lungs are inflamed, the lungs get
irritated by a cold very readily and go into an asthmatic attack.

Drugs like aspirin cause asthma in 10 percent of asthmatics.
Aspirin and aspirin like drugs specifically cause asthma in a
population of patients that have recurring sinusitis, or
infections of the sinuses, and have nasal polyps.

Other drugs that may cause asthma or make it worse are beta-
adrenergic blocking agents, which are used for treatment of such
conditions as migraine, too rapid a heart rate, congestive heart
failure, tremor, and glaucoma.

A third type of agent that may cause asthma is sulfiting agents,
which are chemicals that are added to processed foods to keep
them from turning brown.  If a food should ordinarily turn brown
and has not, it has sulfiting agents in it. This includes dried
fruits, fruit juices, vegetables, and wines.  As sulfites are
eaten, they mix with acids in the stomach and become sulfuric
acid, which is a gas.  The gas travels up through the esophagus,
is breathed in, and provokes asthma.

Industrial and occupational exposures also have a bad effect on
asthma.  On smoggy days, the air is loaded with exhaust from
motor vehicles.  This is a major source of pollutants, and people
with asthma experience increased asthmatic symptoms as a result.

How Is Asthma Diagnosed?

An allergist will do spirometry to measure the patient's ability
to blow air out of the chest.  The patient takes a deep breath
and blows into a machine called a spirometer.  The doctor then
measures how much and how quickly air was blown out.  Most people
blow all the air in their chest out within 3 seconds; 75 percent
of the air is exhaled within 1 second.  The point of maximum
expiration is called the "peak flow."

Asthmatics have trouble blowing out the air.  That is because of
airflow obstruction during breathing out.  As a result, they have
a very hard time blowing out air and it takes much longer.

Over the past few years, the availability of inexpensive and
accurate peak flow meters has made life easier for doctors.
Patients can measure their own peak flow in the morning and
evening.  The patient blows into the flow meter and it measures
the peak expiratory flow rate.

For home use it is reliable and inexpensive.  A patient can use
it before and after taking bronchodilators.  It tells the doctor
how much airflow increased with the use of bronchodilators and
can be used to regulate medications.  It also tells the doctor
when the patient is doing well, and warns when trouble is coming.

Airway hyperresponsiveness is increased reactivity of the
airways, a "twitchiness" of the airways.  Asthmatics have
nonspecific airway responsiveness.  They react when breathing in
certain chemicals, including irritants and chemical mediators, or
under certain physical conditions like exercise.  The importance
of airway hyperreactivity is that it clearly separates those who
do not have hyperreactivity from asthmatics.  Airway
hyperreactivity actually predicts who will develop asthma.
Patients who have abnormal hyperreactivity are very likely to be
predisposed to developing asthma under the right conditions.

There is a range of abnormal airway hyperreactivity among people
with asthma, ranging from mild to severe, and doctors can use
that range of near normal to very abnormal to determine which
medications are required.

Airway hyperreactivity gets worse with allergic reactions that
cause late phase reactions.  But airway hyperreactivity can get
better if the patient avoids allergens, goes on allergy shots,
and uses inhaled cromolyn or corticosteroids.  Recently, it has
been recognized that one of the major targets for the treatment
of asthma is airway reactivity.

How Is Asthma Treated?

In order to treat asthma and airway reactivity, doctors recommend
that asthma patients avoid the allergens to which they are
sensitive whenever possible.  They put the patients on allergy
shots if they have allergic asthma in hopes of reducing the
allergic contribution to the asthma.  They use inhaled cromolyn,
which stops allergic reactions, and often use inhaled
corticosteroids, which stop inflammation of the airways and
reduce airway hyperreactivity.

With all patients, doctors also use symptomatic treatment of
asthma involving agents that relax the airways.  These agents
include beta-adrenergic agonists, theophylline, anti-
cholinergics, and occasionally expectorants and mucolytics.
These medications are employed to try to reverse the airflow
obstruction, but they do not have any effect on the underlying
causes of asthma.

Until recently, the therapy of asthma was based on the drug
theophylline.  This drug is an excellent, time-proven, time-
tested, and very reliable bronchodilator that opens up asthmatic
airways.  It was the foundation of asthma therapy from 1970 to
1990.

In the 1980's beta-adrenergic agents became available.  These
medications relax the airways and make many people better.  They
became very important in asthma therapy, and have become the
predominant drug used to relax airways.

In 1990, asthma therapy shifted so that all asthmatics are now
treated with specific therapy aimed at the underlying causes of
the disease-allergy avoidance, inhaled corticosteroids, perhaps
allergy shots, and perhaps inhaled cromolyn.  Nearly all patients
use inhaled corticosteroids or cromolyn for the treatment of
their airway inflammation.  For control of airway obstruction,
patients use beta-adrenergic agonists, as well as theophylline
and ipratropium, which is an anti-cholinergic agent.
Theophylline, however, has gone from being the foundation of
treatment to a second-line therapy It is added later and taken
away sooner.

What about general treatments? It is recommended that all
asthmatics exercise.  Some patients have exercise-induced asthma,
so it sounds contradictory, but with the proper medications every
asthmatic-particularly children-can and should exercise.
Swimming is the preferred form of exercise; biking is the second
preferred; and running is the least preferred.  It is recommended
that asthmatics do not smoke, and that people with asthmatic
children stop smoking.  Patients are advised to monitor their
pulmonary function with a home peak flow meter and learn as much
as possible about asthma.


Questions and Answers


Q.    Nobel Laureate Linus Pauling made mention of the fact that
vitamin C has some effect on allergic reactions.  What is the
latest on that?

A.   Scientifically it has not been proven that vitamin C has any
effect, beneficial or detrimental, on allergies.  It is not
harmful, at least in reasonable doses, but it will not help the
disease.

Q.   How are food allergies effectively identified and then
treated?

A.    Dr. Dean Metcalfe, here at the National Institute of
Allergy and Infectious Diseases, screened adults for food
allergy, and he found that when people came in and said, "I'm
allergic to strawberries," they were likely to be allergic to
strawberries.  If people came in and said, "I'm allergic to
food," they were almost never allergic to food.  When people come
in and can identify the food to which they are sensitive, we can
confirm that by skin testing.  In the case of a specific
allergen, the skin tests are positive almost all the time.  And,
if we do a double-blind food challenge, it is frequently
positive.

     We have many examples where people have said, "I haven't
been able to eat eggs for 25 years," and we skin tested them and
the tests were negative.  We did a provocation challenge, and the
only response was they did not care for the taste of eggs.  So,
the skin test plus a good history is how we screen for food
allergies.

Q.    Have you ever observed any ophthalmological effects from
allergies such as double vision?

A.   The eye is always involved in allergy, but the symptoms are
generally itching, swelling, and redness.  Eye movement and
visual acuity are not affected.

Q.   What advice would you give to parents, both of whom suffer
from allergies, regarding the introduction of foods for their
infant children?

A.    Our instructions are very simple.  We would recommend,
especially if you have two allergic parents, that solids not be
introduced until the children are at least 6 months of age.  We
recommend exclusive breast-feeding through at least the first 6
months.  There is some suggestion that very allergenic foods,
like cow's milk and peanuts, should be avoided by the mother
during breast-feeding because she can transfer some allergens in
breast milk.  But the opportunity for a child to become allergic
to allergens in breast milk is very limited versus drinking cow's
milk.  Thus, breast-feeding is highly recommended.

Q.   I have two questions.  First, what are the possible side
effects of allergy medications and how often do they occur?
Second, I read that in some cases either asthma or hay fever, or
some combination, can be due to emotional problems.  Is there any
information about that?

A.   It has often been claimed that asthma is a psychological
disease.  That is not the case.  Asthma is a real, very important
disease that can kill you.  It may be worsened by psychological
stresses, but it is not caused by stress.  It is not
psychological.  The parents of asthmatics often feel very guilty
that their child has asthma, and they wonder if it is their fault
in some way that influenced their child's asthma.  That is not
the case.

     The side effects for drugs is a very broad question, very
hard to answer, but I can tell you that the agents that we use
today have been selected from many others because of their
efficacy versus their very limited side effects.  And the reason
that theophylline is being used less today is because it has some
side effects we wish to avoid.

Q.   1 would like your comment on sinusitis.  Is it associated
with allergies?

A.   Sinusitis is an infection of the sinus areas of the skull,
which are between the eyes, on either side of the cheeks, in the
forehead, and center of the head.  They are frequently associated
with allergies and the development of sinus congestion; sinus
infections are frequent accompaniments of allergy

Q.    What about the long-term effects of corticosteroids? Did I
understand you to say that was no problem?

A.    Corticosteroids have major complications associated with
them.  In the past, we balanced the use of oral corticosteroids,
which have systemic side effects, with the effects of the
disease.  When the disease was bad enough, we gave oral steroids
and accepted the side effects if we had to.  Now, with topical,
inhaled steroids we have little or no systemic effects, so we get
all the beneficial capabilities of steroids with none of the
unwanted side effects.

Q.    Why do some medications like Benadryl, which you might give
a child for hives, say, "Do not give to a child with asthma"? Is
it dangerous to give a child Benadryl when he or she has hives
and not asthma?

A.   That is an interesting and good question.  The classical
(older) antihistamines, like Benadryl, if you read the label, are
not supposed to be used in asthma.  Testing in the early 1950's
suggested that antihistamines made asthma worse.  So there is a
label insert that says they should not be used in asthma.  We do
not use any of the classical or older antihistamines for the
treatment of asthma because they do not work very well.  Despite
the labeling, however, we do not believe they have any danger for
people with asthma.

     Some of the new, nonsedating antihistamines do have some
efficacy in asthma, and they are being used cautiously for asthma
treatment.  These new antihistamines have no limitations on their
use in asthma and may, indeed, be useful.

Q.   I wondered if there has ever been a program to eradicate
ragweed.

A.   Yes, there has been.  A woman in the Pennsylvania area and
her family had terrible ragweed allergies and she organized the
community to eradicate ragweed.  It did not make any difference
because ragweed moves through the air for such a long distance.

Q.   Many of the over-the-counter antihistamines say, "Do not
take it for more than seven days." If I want to take them longer,
should I get a prescription from a doctor?

A.    Antihistamines have been available since the 1940's and
many people have taken antihistamines for more than 30 years,
They have been among the world's safest drugs.  That does not
mean that you should use an over-the-counter antihistamine
without caution.  By and large, we would recommend that everyone
would benefit from an appropriate diagnosis and proper therapy,
which might include over-the-counter drugs, but might also
include prescription drugs, which are really quite a bit more
potent and maybe more specific.



Biographical Note

Michael A. Kaliner, M.D.
Head, Allergic Diseases Section
Laboratory of Clinical Investigation
National Institute of Allergy and Infectious Diseases

A native of Baltimore, Dr. Michael Kaliner received his
bachelor's degree and medical degree from the University of
Maryland.  He completed his residency in medicine at the
University of California at San Francisco, and his fellowship in
allergy/immunology and rheumatology at Robert B. Brigham Hospital
in Boston.  He joined the National Institute of Allergy and
Infectious Diseases in 1975, became head of the Allergic Disease
Section and director of the Allergic Diseases Center in 1978, and
was named director of the Outpatient Department, NIAID, in 1981.
He currently serves as a consultant at Walter Reed Army Medical
Center and at the U.S. Department of Health and Human Services.

Dr. Kaliner's research focuses on the mechanics and controls of
allergic asthma, rhinitis, anaphylaxis, and urticaria.  By
understanding the origin and physiology of these diseases,
researchers can employ available therapy more effectively and
develop new treatments.

Respected internationally, Dr. Kaliner recently was appointed for
two years to the International Scientific Board of the Pharmacia
Allergy Research Foundation.  He is active on many committees,
including the Executive Committee of the American Academy of
Allergy and Immunology, the Presidential Advisory Committee and
Executive Committee of the International Association of
Allergology and Clinical Immunology.  He is treasurer of the
American Academy of Allergy and Immunology.  He serves on the
editorial board of several professional journals.  Dr. Kaliner
serves on the Executive Committee of the National Allergy and
Asthma Network, the organization that publishes the Mothers of
Asthmatics report.

He received many distinguished awards during his career,
including the USPHS Commendation Medal for Meritorious Service,
USPHS Outstanding Service Award, Roy Patterson Lectureship with
American Academy of Allergy and Immunology, and the Von Pirquet
Lectureship and Award from the Georgetown University Medical
School.

Clinical Center Office of Communications
National Institutes of Health
April 1991
!
*
American College of Allergy, Asthma and Immunology
Advice From Your Allergist: Allergic Rhinitis

You may have allergic rhinitis if . . .
You sneeze a lot, often in rapid succession . . .  your nose is runny, congested or
stuffy . . . you're bothered by itching of the nose, eyes, throat or roof of the mouth
.
. . you're nagged by postnasal drip . . . your ears pop or are plugged . . . you have
a decreased sense of smell . . . and/or you get sinus headaches.

What is allergic rhinitis?
Known to most people as hay fever (see box below), allergic rhinitis is a very
common medical problem affecting more than 15% of the population, both adults
and children.

Allergic rhinitis takes two different forms╨seasonal and perennial. Symptoms of
seasonal allergic rhinitis surface in spring, summer and/or early fall and are
usually caused by allergic sensitivity to pollens from trees, grasses or weeds, or to
airborne mold spores. Other people experience symptoms year-round, a condition
called perennial allergic rhinitis. It's generally caused by sensitivity to house dust,
house dust mites, animal danders and/or mold spores. Underlying or hidden food
allergies are considered a controversial cause of perennial nasal symptoms.

Some people may experience both types of rhinitis, with perennial symptoms
worsening during specific pollen seasons. As will be discussed later, there are also
other causes for rhinitis.

What causes the sneezing, itchy eyes and other symptoms?
When a sensitive person inhales an allergen (allergy-causing substance) like
ragweed pollen, the body's immune system reacts abnormally with the allergen.
The allergen binds to allergic antibodies (immunoglobulin E) that are attached to
cells that produce histamine and other chemicals. The pollen "triggers" these cells
in the nasal membranes, causing them to release histamine and the other
chemicals. Histamine dilates the small blood vessels of the nose and fluids leak
out into the surrounding tissues, causing runny noses, watery eyes, itching,
swelling and other allergy symptoms.

Is allergic rhinitis ever the cause of other problems?
Some known complications include ear infections, sinusitis, recurrent sore throats,
cough, headache, fatigue, irritability, altered sleep patterns and poor school
performance. Occasionally, children may develop altered facial growth and
orthodontic problems. Allergy treatment can eliminate or alleviate most of these
problems.

Are all cases of rhinitis caused by allergies?
No. Not all rhinitis symptoms are the result of allergies. Below are listed the three
most common causes of rhinitis with some of their characteristics.

 Rhinitis    Common    Allergic                       Duration of
 Type        Name      Sensitivity Causes             Symptoms

 Allergic    Hay fever Yes         Dusts, animals     Perennial
                                   pollens, molds,    and/or
                                   foods              seasonal

 Infectious  Colds-flu No          Viruses, bacteria  3-7 days

 Nonallergic Irritant  No          Smoke, air pollu-  Perennial
                                   tion, exhaust      and/or
                                   fumes, aerosol     following
                                   sprays, fragrance, exposure
                                   paint fumes, etc.

No Hay, No Fever ╨ So Why Hay Fever?
"Hay fever" is a turn-of-the-century term which has come to describe the
symptoms of allergic rhinitis, especially when it occurs in the late summer.
However, the symptoms are not caused by hay (ragweed is one of the main
culprits) and are not accompanied by fever. So physicians prefer the term "allergic
rhinitis" because it's more accurate. Similarly, springtime symptoms are
sometimes called "rose fever," but it's just coincidental that roses are in full-bloom
during the grass-pollinating season. Roses and other sweet-smelling, showy
flowers rely on bees, not the wind, for pollination, so not much of their pollen gets
into the air to cause allergies.

Ñ The common cold is a good example of an infectious rhinitis. Most infections
are relatively short-lived, lasting from 3 to 7 days.

Ñ Irritant rhinitis also is called vasomotor or nonallergic rhinitis. People with
irritant rhinitis develop swelling and congestion of the nose when they are
exposed to irritating substances that may not cause symptoms in other people.

The symptoms of allergic and irritant rhinitis can be the same. Many people
experience a combination of both types of rhinitis.

How is allergic rhinitis diagnosed?
Your allergist may begin by taking a detailed history, looking for clues in your
lifestyle that will help pinpoint the cause of your symptoms. You'll be asked about
your work and home environments . . . your eating habits . . . your family's
medical history . . .  the frequency and severity of your symptoms . . . and
miscellaneous matters such as if you have pets. Then, you may require some tests.
Your allergist may employ skin testing, in which small amounts of suspected
allergen are introduced into the skin. Skin-testing is the easiest, most sensitive and
generally least expensive way of making the diagnosis. Another advantage is that
results are available immediately. In rare cases, it also may be necessary to do a
special blood test for allergens, using the RAST or other methods.

Once allergic rhinitis is diagnosed, what can be done to alleviate the symptoms?
Avoidance, medication and immunotherapy ╨ or allergy shots ╨ are the main
treatments for allergic rhinitis.
Ñ Avoidance ╨ a single ragweed plant may release one million pollen grains in just
one day. The pollen from ragweed, grasses and trees is so small and buoyant that
the wind may carry it miles from its source. Mold spores, which grow outdoors in
fields and on dead leaves, also are everywhere and may outnumber pollen grains
in the air even when the pollen season is at its worst.

While it's difficult to escape pollen and molds, here are some ways to lessen
exposure.

╨Keep windows closed and use air-conditioning in the summer, if possible. A
HEPA (High Energy Particulate Air) filter or an electrostatic precipitator may
help clean pollen and mold from the indoor air. Automobile air conditioners help,
too.

╨ Don't hang clothing outdoors to dry. Pollen may cling to towels and sheets.

╨ The outdoor air is most heavily saturated with pollen and mold between 5 and
10 a.m., so early morning is a good time to limit outdoor activities.

╨ Wear a dust mask when mowing the lawn, raking leaves or gardening, and take
appropriate medication beforehand.

Ñ Medication ╨ When avoidance measures don't control symptoms, medication
may be the answer. Antihistamines and decongestants are the most commonly
used medications for allergic rhinitis. Newer medications, such as cromolyn,
inhibit the release of chemicals that cause allergic reactions. Medications help to
alleviate nasal congestion, runny nose, sneezing and itching. They are available in
many forms, including tablets, nasal sprays, eye drops and liquids. Some
medications may cause side effects so its best to consult your allergist if there's a
problem.

Ñ Immunotherapy╨Allergen immunotherapy, known as "allergy shots," may be
recommended for persons who don't respond well to treatment with medications,
experience side-effects from medications or have allergen exposure which is
unavoidable. Immunotherapy does not cure allergies but can be very effective in
controlling allergic symptoms. Allergy injections are usually given at variable
intervals over a period of two to five years.

There are many ways of treating allergies, and each person's treatment must be
individualized based on the frequency, severity and duration of symptoms and on
the degree of allergic sensitivity. If you have more questions, your allergist will be
happy to answer them.

RAGWEED DENSITY IN THE UNITED STATES

Is There Any Escape?
A common question from allergic rhinitis sufferers is: Can I move someplace
where my allergies will go away?

Some allergens are tough to escape. The above map shows how thoroughly
ragweed╨which affects 75% of allergic rhinitis sufferers╨blankets most of the
United States. Less ragweed is found in a band along the West Cost, the southern-
most tip of Florida and northern Maine, but it is still present. Even Alaska and
Hawaii have a little ragweed.

Allergists seldom recommend moving to another locale as a cure for allergies. A
move may be of questionable value because a person may escape one allergy╨to
ragweed, for example╨only to develop sensitivity to grasses or other allergens in
the new location. Since moving can have a disrupting effect on a family
financially and emotionally, relocation should be undertaken only after careful
consultation with an allergist.

For more information, please contact:
American College of Allergy, Asthma and Immunology
85 West Algonquin Road, Suite 550
Arlington Heights, IL  60005
[phone]
!
*
American College of Allergy, Asthma and Immunology
Advice From Your Allergist: Here's some food for thought

Allergies, including hay fever, asthma and skin disorders, affect 58.7 million
Americans, or 25% of the population. Although the true incidence of food allergy
is unknown, a recent study suggests that about 5% of the population may suffer
from adverse reactions to food.

Historical records show that people have long been aware of food allergy.
Hippocrates noted that cow's milk could cause gastric upset and hives. The
Roman poet Lucretius said, "One man's meat is another man's poison." And some
scholars believe Old Testament dietary restrictions were based upon the
awareness that certain foods could cause adverse reactions.

However, the serious study of food allergy began at the start of this century. Since
then, research has led to effective procedures for the diagnosis and treatment of
food allergy, relieving the symptoms of millions with this problem.

What are some common symptoms of food allergy?
Vomiting, nausea, stomach cramps, indigestion, diarrhea, hives, eczema,
headaches, asthma, earaches and rhinitis (itchy, stuffy, runny nose; sneezing, and
phlegm in the throat) are among the most common symptoms. In fact, many parts
of the body can be affected by food allergy. The frequency and severity of
symptoms vary widely from one person to another. Highly allergic persons may
experience severe and life-threatening reactions such as upper airway swelling of
the tongue and lips while, in milder cases, other may only suffer a minor case of
sniffles. Because these symptoms can be caused by a number of diseases, your
allergist may want to examine you to rule them out as the source of your problem.
Not all adverse reactions to foods are due to allergy. Some reactions to milk, for
example, are related to a deficiency of an enzyme (lactase) which normally breaks
down a sugar in milk (i.e. lactose). This deficiency in some individuals causes a
reaction similar to food allergy when milk is ingested.

What causes my symptoms?
Allergy is caused when your body's immune system over-reacts to the
introduction of offending substances. Normally, your body's defense mechanisms
(antibodies) keep you healthy by fighting such invaders as bacteria and viruses,
which can cause infections, and inactivating allergens, which can cause allergic
reactions. The body produces several types of antibodies, or immunoglobulins.
When antibodies battle with allergens, chemicals, including histamine, are
released as a part of the body's reaction to these substances. One antibody in
particular, immunoglobulin E╨or IgE for short╨is produced in greater quantities
than normal by persons with allergies. In large amounts, IgE-released chemicals
can cause blood vessels to widen, smooth muscles to contract and affected skin
areas to become red, itchy and swollen, resulting in an allergic reaction. In fact,
these IgE antibodies found in tissues and secretions are thought to be responsible
for classic allergy symptoms such as hives, swollen nasal membranes, diarrhea
and wheezing.

Why me? Why have I developed food allergy?
You probably inherited it. Heredity seems to be the prime reason some people
have allergies and others don't. If both your parents have allergies, you have a
75% chance of being allergic. If one parent is allergic╨or you have relatives on
one side with allergies╨you have a 50% chance of becoming allergic. Although
food allergy occurs most often in infants and children, it can appear at any age . . .
and can be caused by foods that have been eaten for years without any problems.

Other factors also come into play. For instance, excessive exposure to a particular
food may create sensitivity to that food as testified to by the high incidence of fish
allergy among Scandinavians . . . and of rice allergy among the Japanese.

Some foods are more potent allergens than others. More people suffer reactions
from peas than from carrots, from citrus fruits than from pears. Also, your
reaction to a particular food can be affected by your physical condition at the
time. If  you have a cold, an upset stomach or a non-food allergy╨hay fever, for
example, during high pollen season╨you may be more susceptible to food allergy.
Emotional stress also may aggravate allergic symptoms.

What foods are most likely to cause allergy?
Eggs, milk, nuts, soy, seafood, fish, corn and wheat are the most common allergy-
causing foods, but almost any food can cause allergy. Keep in mind that, if you
are allergic to a particular food, you might be allergic to related foods. For
example, a person allergic to avocado also may react to bay leaves because they're
in the same botanical family. Likewise, a person allergic to peanuts often cannot
tolerate other members of the legume family whose members include peas, beans
and licorice.

How do allergists determine which foods make me sick?
The procedure varies from patient to patient. Some persons know exactly what
food causes their allergies. They eat strawberries and immediately break out with
hives, or they drink milk and immediately begin vomiting or get diarrhea.
Sometimes, however, they need their allergist's help in determining the "culprit,"
especially when symptoms show up many hours after ingesting an offending food.

Your allergist may begin by taking a detailed history. He'll look for clues in your
lifestyle that will help pinpoint the cause of your problem. You'll be asked about
your work and home environments . . . your eating habits . . . your family's
medical history and miscellaneous matters, such as what kind of heating and
cooking fuels you use, and if you have pets!

But some of these questions don't have anything to do with food!
These questions are necessary because your allergist wants to eliminate the
possibility that another problem or multiple allergies may be causing your
symptoms. For example, a patient's allergy to pollen or mold may trigger his
allergy to eggs, so that in the summer, when mold and pollen are everywhere, he
may not be able to eat eggs even though he can eat them safely the rest of the
year.

What's next, after taking the history?
You may require some tests. Your allergist may employ skin testing, in which
small amounts of the suspected allergen are introduced into the skin. A positive
reaction╨a wheal, swelling or flare in the surrounding red area╨indicates the
presence of allergic antibodies. Some patients are given RAST tests, which use
blood samples to determine the extent of antibody production against an allergen.

Will these allergy tests pinpoint my problem?
Although these tests give your allergist further clues for the diagnosis, they are
not 100% reliable. With the information gained from your history, physical exam
and the tests, your allergist may further narrow down the suspected foods by
placing you on a special diet.

If your symptoms occur only occasionally, the culprit is a food that is eaten
infrequently. Your allergist may ask  you to keep a daily food diary listing all
food and medication ingested, along with your symptoms for the day. By
comparing "good days" with "bad days," you and your allergist can determine
which foods are prime suspects.

Then you may be asked to follow a trial diet, which alternates days of
consumption and avoidance of suspected foods. If your symptoms subside during
abstinence╨and flare up when you eat the food again╨the problem food has been
identified.

But I have allergy symptoms every day. How do my allergist and I begin to
identify what foods I'm allergic to?
Your allergist may ask you to go on an elimination diet tailor-made for you. You
may be asked to eliminate foods implicated on the basis of your history, foods
known to be highly allergenic and foods indicated in positive skin tests.  After
several weeks on the elimination diet, your system should be cleared of allergenic
foods. When your symptoms cease, your allergist may reintroduce foods one at a
time to your diet╨and watch for reactions.

This reintroduction is called a "challenge." Many challenges may be necessary to
identify which foods are safe for you and which will make you sorry. In some
cases, not all offending foods or additives can be identified. Then your allergist
may prescribe drugs to ease the symptoms.

Once my allergy is identified, how is it treated?
Avoidance of the allergenic food is the best policy. You must be vigilant in
checking ingredient labels of food products to make sure an offending food or
food additive is not present. For example, a person allergic to milk must avoid ice
cream, cheese, chocolate bars, many cake mixes, and luncheon meats in which
milk is used as a filler. If you are allergic to fish, be aware that glue on envelopes
sometimes is made with fish products and could cause an allergic reaction.

Special food-allergy cookbooks are widely available. Your allergist can
recommend some to you.

Will I ever be able to eat these foods again?
If you have a severe immediate-type reaction to certain foods, NEVER taste them
again. In some very sensitive persons a small taste of an allergenic food can
produce a life-threatening reaction.

However, if your symptoms are milder, you may be able to try a problem food
again. After you have abstained from allergenic foods for a period of at least six
months or longer, your allergist may suggest that you try a small portion of a
problem food. If you have no reaction, you may be able to eat this food
occasionally and in moderation. Allergic reactions to foods have been known to
disappear for months or years, then reappear. In some cases, the allergy
disappears for good.

If you use caution and carefully follow your allergist's advice, you can bring your
food allergy under control. If you have more questions, your allergist will be
happy to answer them.

For more information, please contact:
American College of Allergy, Asthma and Immunology
85 West Algonquin Road, Suite 550
Arlington Heights, IL  60005
[phone]
!
*

American College of Allergy, Asthma and Immunology
Advice From Your Allergist:
Pets May Cause Havoc for Allergic and Asthmatic Patients

Keeping domestic animals as household pets is a universal activity. Pets provide
companionship, security, and a sense of comfort. Children often learn
responsibility and lessons about life and death from pets. However, people with
allergies should be cautious in deciding what type of animal can safely be brought
into their home.

Pets can cause problems to allergic patients in several ways. Their dander (skin
flakes) can cause an allergic reaction, as well as their saliva and urine. The animal
hair itself is not considered to be a very significant allergen. However, the hair or
fur can collect pollen, dust, mold, and other allergens.

The best types of pets for an allergic patient are tropical fish, snakes, lizards,
turtles, salamanders, ants and certain types of insects, frogs, spiders and tortoises.
All of these pets do not shed dander, have hair or fur, nor does their excrement
create allergic problems. However, patients should keep in mind that large
aquariums can add to the amount of water vapor in a room, thus increasing mold
and house dust mite concentrations in their home.

Most Common Pets
The most common household pets are dogs, cats, birds, hamsters, rabbits, mice,
rats and guinea pigs. Larger animals such as horses, goats, cows, chickens, ducks,
and geese, even though kept outdoors, can also cause problems if kept as pets. It
is estimated that 25% of children are exposed to horses either by riding them or by
being in their barns or pens.

The number of pets in the United States is estimated at more than 100,000,000.
This large number also increases the likelihood of accidental exposure to animals
by the allergic patient when visiting homes, farms, etc.

Both feathers and the droppings from birds, another common pet, can increase the
allergen exposure. The allergic patient should not use feather pillows or down
comforters. If a feather pillow is used, it should be placed in a plastic encasing.
An encasing with a zipper is recommended, so none of the feathers can escape.

Bird Droppings
Bird droppings can be a source of bacteria, dust, fungi and mold. This also applies
to the droppings of other caged pets, such as gerbils, hamsters and mice.

The ideal situation for an allergic patient would be to have no pet at all. However,
many pet owners feel strongly about their pets, and would rather remove the
allergic individual from the home than the pet! A pet such as a dog or cat should,
at the very least, be kept out of the allergic patient's bedroom.

The avid pet owner may claim that exposure to his or her pet does not cause them
any problems. This however, should be viewed skeptically, since pet ownership is
an emotionally-charged subject. Also, many allergic pet owners are rarely away
from their pets, so an accurate reporting of pet-related symptoms may not be
possible.

Testing to determine an allergy to animals is not always accurate. Skin tests or
radioallergosorbent tests (a blood test for allergens) can give a "false" negative
reaction. To gain confirmation about a pet's significance as an allergen, the pet
should be removed from the home for several weeks and a thorough cleaning
done to remove the hair and dander. It should be understood that it can take weeks
of meticulous cleaning to remove all the animal hair and dander before a change
in the allergic patient is noted.

A frequently mistaken idea is that short-haired animals cause fewer problems. It is
the dander (flakes of skin) that cause the most significant allergic reactions, not
the length or amount of hair on the pet. As stated previously, allergens are also
found in the pet's saliva and urine. In addition, dogs have been reported to cause
acute symptoms of allergic conjunctivitis (inflammation of the eye) and hay fever
after running through fields and then coming back into contact with their owners.

Severe Reactions
Some allergic patients may have severe reactions (wheezing and shortness of
breath) after exposure to such pets as cats, rats, horses and guinea pigs. Also, a
chronic, slowly progressive feeling of shortness of breath, loss of energy and
feeling of fatigue can result from long-term exposure to birds and their droppings.
This type of disease is known as hypersensitivity pneumonitis and can result in
severe disability. In the event of these severe cases, removal of the offending
animal is mandatory.

In summary, pets can cause direct and indirect allergic problems, both from the
dander and from pollen accumulated on the fur. Allergic children and adults
should not pet, hug or kiss their pets, because of the allergens on the animal's fur
or saliva. Those pets that are known to cause allergic reactions should be removed
from the home of the allergic patient. If the family is unwilling to do this, the pet
should be kept out of the patient's bedroom and, if possible, outdoors.

Limit Pets to Few Rooms
Indoor pets should be restricted to few rooms in the home if possible. Isolating the
pet to one room, however, will not limit the allergens to that room. Air currents
from forced-air heating and air-conditioning will spread the allergens throughout
the house. Homes with forced-air heating and/or air-conditioning may be fitted
with a central air cleaner. This may remove significant amounts of pet allergens
from the home. The air cleaner should be used at least four hours per day.

Text developed by ACAAI members: Gerald L. Klein, M.D., Robert W. Ziering,
M.D., Vista, CA.

For more information, please contact:
American College of Allergy, Asthma and Immunology
85 West Algonquin Road, Suite 550
Arlington Heights, IL  60005
[phone]
!
*

American College of Allergy, Asthma and Immunology
Advice From Your Allergist: What is rhinitis?

Rhinitis is not a disease; it is simply a term describing the symptoms produced by
nasal irritation or inflammation. Symptoms of rhinitis include runny nose
(rhinorrhea), itching (pruritus), sneezing and stuffy nose (blockage or
congestion). These symptoms are the nose's natural response to inflammation and
irritation.

The nose normally produces mucus, which traps substances like dust, pollen,
pollution, and germs such as bacteria and viruses. Mucus flows from the front of
the nose to the back and is swallowed, but when mucus production is excessive, it
can flow from the front (runny nose) or become noticeable from the back (post-
nasal drip). Nasal mucus, normally a thin, clear liquid, can become thick or
colored, perhaps due to dryness, infection, or pollution. When post-nasal drip is
excessive, thick, or contains irritating substances, cough is the natural response
for clearing the throat.

Itching and sneezing are also natural responses to irritation caused by allergic
reactions, chemical exposures (like cigarette smoke), temperature changes,
infections and other factors.

The nasal tissues congest and decongest periodically. In most people, nasal
congestion switches back and forth from side to side of the nose in a cycle several
hours long. Some people, especially those with narrow nasal passages, notice this
nasal cycle more than others. Strenuous exercise or changes in head position can
affect nasal congestion. Nasal congestion is also the natural response to irritation
and inflammation. Severe congestion can result in facial pressure and pain, as well
as dark circles under the eyes.

What is sinusitis?
Sinusitis is inflammation or infection of any of the four groups of air sinus
cavities in the skull, which open into the nasal passages. Sinusitis is not the same
as rhinitis, although the two may be associated and their symptoms may be
similar. The terms "sinus trouble" or "sinus congestion" are sometimes wrongly
used to mean congestion of the nasal passage itself. Most cases of nasal
congestion, though, are not associated with sinusitis.

What causes rhinitis?
Arbitrarily, rhinitis lasting less than six weeks is called acute rhinitis, and
persistent symptoms are called chronic rhinitis. Acute rhinitis is usually caused by
infections or chemical irritation. Chronic rhinitis may be caused by allergy or a
variety of other factors.

The symptoms of allergic rhinitis (called "hay fever" sometimes, but not caused
by hay and not associated with fever) are caused by exposure to substances
(allergens) to which the patient has become allergic. Common allergens are tree,
grass, and weed pollens, molds, animal hair and dander, and house dust mites. In
general, pollen (and sometimes mold) allergy causes symptoms at certain times of
the year, while mold, animal dander, and dust mites cause symptoms year round.
People with allergies have developed antibodies to these allergens.  These
antibodies circulate in the blood stream, but localize in the tissues of the nose and
in the skin. This makes it possible to demonstrate presence of these antibodies by
skin testing, or less commonly, by a special blood test. A positive skin test mirrors
the type of reaction going on in the nose. Not everyone with symptoms of rhinitis
has allergies, and not everyone with allergies has rhinitis.

Rhinitis may result from many causes other than allergic reaction. In fact, the
most common condition causing rhinitis is the common cold. Colds can be caused
by any of more than 200 viruses. Children, particularly young children in school
or day care centers, may have from 8-12 colds each year.  Fortunately, the
frequency of colds lessens after immunity has been produced from exposure to
many viruses.

Colds usually begin with a sensation of decongestion, rapidly followed by runny
nose and sneezing. Over the next few days, congestion becomes more prominent,
the nasal mucus may become colored, and there may be a slight fever and cough.
Cold symptoms resolve within a couple of weeks, although a cough may
sometimes persist. Cold symptoms that last longer may be due to other causes of
chronic rhinitis or to sinusitis.

What are other causes of rhinitis?
Not all symptoms in the nasal passage are caused by allergy or infection. Similar
symptoms can be caused by mechanical blockage, use of certain medications,
irritants, temperature changes, or other physical factors. Rhinitis can also be a
feature of other diseases and medical conditions.

Drug-induced nasal congestion, called "rhinitis medicamentosa," can be caused
by birth control pills and other female hormone preparations, certain blood
pressure medications (beta blockers and vasodilators), and prolonged use of
decongestant nasal sprays.

Decongestant nasal sprays work quickly and effectively, but they alter normal
nasal physiology. After a few weeks of use, nasal tissues swell after the
medication wears off. The only thing that seems to relieve the obstruction is more
of the medicine, and the medication's effect lasts shorter lengths of time.
Permanent damage to the nasal tissues may result. Consultation with a physician
to "get off" the medication is often necessary.

Cocaine also alters normal physiology, causing a condition identical to that
produced by decongestant nasal sprays. If you use cocaine, it is important to tell
your physician so that appropriate therapy can be prescribed.

"Vasomotor rhinitis" is a term used to describe a group of poorly understood
causes of rhinitis, with symptoms not caused by infection or allergy. Many people
have recurrent nasal congestion, excess mucus production, itching, and other nasal
symptoms similar to those of allergic rhinitis, but the disorder is not caused by
allergy.

What triggers vasomotor rhinitis?
Irritants that can trigger vasomotor rhinitis include cigarette smoke, strong odors
and fumes (perfume, hair spray, other cosmetics, laundry detergents, cleaning
solutions, pool chlorine, car exhaust, and other air pollution). Spices used in
cooking can cause nasal irritation, producing a condition called "gustatory
rhinitis."

Other things that can aggravate vasomotor rhinitis are alcoholic beverages
(particularly beer and wine), aspirin, and certain blood pressure medications, such
as reserpine and propranolol. Some people are very sensitive to abrupt changes in
weather or temperature. Skiers often develop a runny nose ╨ "skier's nose" ╨ but
in some people any cold exposure may cause a runny nose. Others start sneezing
when leaving a cold, air conditioned room. These agents are not allergens, do not
induce formation of allergic antibodies, and do not produce positive skin test
reactions. Occasionally, one or two positive skin tests may be observed, but they
do not correlate with the history and are not relevant or significant.

The cause of vasomotor rhinitis is not well understood. In a sufficiently high
concentration, many odors will cause nasal irritation in almost anyone. Some
people, though, are unusually sensitive to irritation, and will have significant nasal
symptoms even when exposed to low concentrations of irritants. Thus, vasomotor
rhinitis seems to be an exaggeration of the normal nasal response to irritation,
occurring at levels of exposure which don't bother most people. It occurs more
often in smokers and older individuals.

As is the case with allergic rhinitis, vasomotor rhinitis often can't be cured.
Fortunately, symptoms can be kept under control by avoiding or reducing
exposure to substances that cause symptoms, and by taking medications when
needed. Patients with vasomotor rhinitis should not smoke or permit smoking in
their homes.

Dryness of the nasal tissues can be a normal effect of aging, or a characteristic of
"rhinitis sicca, " which may be associated with a foul smelling nasal discharge.
Rhinitis can also be a feature of endocrine disease like hypothyroidism or can
occur during pregnancy. Rhinitis can be made worse or even improved during
pregnancy. Alcoholic beverages can cause the blood vessels in the nose to enlarge
temporarily and produce significant nasal congestion.

How do you know what kind of rhinitis you have?
Consult your physician. Sometimes several of the above conditions can coexist in
the same person. In a single individual, allergic rhinitis could be complicated by
vasomotor rhinitis, septal deviation (curvature of the bone separating the two
sides of the nose) or nasal polyps. Use of spray decongestants because of chronic
sinusitis, septal deviation or vasomotor rhinitis may cause rhinitis medicamentosa.
Any of these conditions will be made worse by catching a cold. Nasal symptoms
caused by more than one problem can be difficult to treat, often requiring the
collaboration of an allergist and an otolaryngologist (ear, nose and throat
specialist).

How is rhinitis treated?
Nasal surgery will usually cure or improve symptoms caused by mechanical
blockage or chronic sinusitis not responsive to prolonged antibiotics and nasal
steroid sprays. Stopping the use of offending medications will cure rhinitis
medicamentosa, providing that there is no underlying disorder.

When no specific cure is available, options are ignoring your symptoms, avoiding
or decreasing exposure to irritants or allergens to the extent practical, and taking
medications for symptom relief.

Antihistamines are the most inexpensive and commonly used treatment for
rhinitis. They dry excess secretions and reduce itching and sneezing, but may not
do much for nasal congestion. There are dozens of different antihistamines and
wide variations in how individual patients respond to them. Some are available
over the counter and others require a prescription. Generally, they work well and
produce only minor side effects. Persons with nasal dryness or thick nasal mucus
should avoid taking antihistamines without consulting a physician. Contact your
physician for advice if an antihistamine causes drowsiness or other side effects.

Non-prescription decongestant nasal sprays work within minutes and lasts for
hours, but can't be used for more than a few days at a time without a physician's
order.

Oral decongestants are found in many over the counter and prescription
medications, and may be the treatment of choice for nasal congestion. They don't
cause rhinitis medicamentosa, but need to be avoided be some patients with high
blood pressure. If you have high blood pressure, you should check with your
physician before using them.

Using an over the counter saline nasal spray will help counteract symptoms of dry
nasal passages or thick nasal mucus. Unlike decongestant nose sprays, a saline
nose spray can be used as often as needed. Sometimes, your physician may
recommend washing (douching) of the nasal passage.

Corticosteroids counteract the inflammation caused by release of allergic
mediators, as well as that caused by other nonallergic factors. Thus, they generally
work for many causes of rhinitis symptoms and are sometimes useful for chronic
sinusitis. Corticosteroids are sometimes injected or taken orally, but usually on a
short term basis for extremely severe symptoms. Physicians warn that injected or
oral steroids may produce severe side effects when used for long periods or used
repeatedly and, for this reason, they should be used with extreme caution. In
rhinitis, a corticosteroid is much safer when used topically be spraying it into the
nose.

Cromolyn is a medication that blocks the release of chemical mediators. It does
not work in all patients. It must be used at least four times daily, and improvement
may take several weeks to occur.

Atropine and the related drug ipratropium bromide are sometimes used to relieve
symptoms of rhinitis; in fact, most antihistamines have a slight atropine-like
effect. Atropine can be taken orally, and it is a component of some antihistamine
decongestant preparations. Atropine and ipratropium are not yet commercially
available as nasal sprays.

Antibiotics are for the treatment of bacterial infections. They do not affect the
course of uncomplicated viral upper respiratory infections (common colds), and
are of no benefit for non-infectious rhinitis, including allergic rhinitis. In chronic
sinusitis, antibiotics may help only temporarily, and surgery may be needed.

One or more of the above medications, accompanied by appropriate avoidance
measures, will usually control most symptoms of nasal allergy. If not,
immunotherapy (allergy shots, desensitization) may provide additional relief. The
treatment program consists of injections of a diluted extract, administered
frequently in increasing doses until a maintenance dose is reached. Then, the
injection schedule is changed so that the same dose is given with longer intervals
between injections. Immunotherapy helps the body build resistance tot he effects
of the allergen, reduces the intensity of symptoms provoked by allergen exposure,
and sometimes can actually make skin test reactions disappear. As resistance
develops, symptoms should improve, but the improvement from immunotherapy
will take several months to occur. Immunotherapy does not help the symptoms
produced by nonallergic rhinitis.

For more information, please contact:
American College of Allergy, Asthma and Immunology
85 West Algonquin Road, Suite 550
Arlington Heights, IL  60005
[phone]
!
*
Tipsheet:  What's New in Allergy Research

NIAID TIPSHEET
May 1995

       One out of five Americans -- some 50 million people --
suffers from an allergic disease, which results from the immune
system reacting to a normally innocuous substance such as pollen or
dust.  Most of these people have allergies that affect nasal
passages and sinuses, including allergies to pollen ("hay fever"),
and allergic reactions in the airways of the lungs, which
contribute to asthma.

       Hay fever alone affects some 22 million Americans, who in
total see their doctors 9.4 million times a year.  Asthma, which
afflicts 10 to 15 million Americans, costs the country more than
$6.2 billion annually in direct and indirect costs.

       Although researchers have developed useful therapies to
treat allergic diseases, much remains to be learned about the basic
nature of the allergic response and how to better treat and prevent
allergies and related conditions.

       Recently, investigators supported by the National Institute
of Allergy and Infectious Diseases (NIAID) have reported important
findings that may lead to new ways to reduce the burden of allergic
diseases.  Some of these are summarized below.  To pursue these
or related stories, call Greg Folkers in the NIAID Office of
Communications at [phone].  In this tipsheet:

       The Basics of Allergy - a Brief Primer
       Is It All in the Genes?
       New Hope for Allergic Seafood Lovers
       Why Both Nuts and Beans Bedevil Some Allergic People
       The Dope on the Docking Molecule
       How do Allergy Shots Work?
       Combatting Cat Allergy
       Managing Mast Cells

       NIAID is one of 17 institutes of the National Institutes of
Health (NIH), the federal focal point for biomedical research.  NIH
is an agency of the U.S. Public Health Service, Department of Health
and Human Services.  NIAID conducts allergy research in its
intramural laboratories in Bethesda and Rockville, Md., as well as
through an extensive extramural program administered by the
Division of Allergy, Immunology and Transplantation.


THE BASICS OF ALLERGY - A BRIEF PRIMER

       An allergic response begins with a process called
sensitization.  When a foreign substance such as pollen (an
allergen) first enters the body of an allergic person, cells called
macrophages engulf the invader, chop it into pieces and display the
pieces on their surfaces.  T-helper cells recognize certain allergen
fragments and bind to the macrophages.  This process causes the
T-helper cells to secrete signalling molecules, including
interleukin-4 (IL-4). IL-4, in turn spurs nearby B cells to mature
into plasma cells.  Plasma cells produce Y-shaped antibody proteins.

       One class of antibodies of great importance in allergic
diseases and asthma is immunoglobulin E (IgE).  The two "arms"
of IgE are tailor-made to specifically attach to the allergen.  The
"stem" of the IgE molecule attaches to two classes of immune cells:
mast cells, which concentrate in tissues exposed to the outside
world, especially the skin, and the linings of the nose, lungs and
gastrointestinal tract, and basophils, which circulate in the
blood.

       When the same allergen next enters the person's body, it
binds to the arms of the IgE molecules protruding from the surfaces
of mast cells and basophils.  The interaction of an allergen with
two IgE molecules triggers enzymes associated with the cell membrane
such as tyrosine kinases.  The enzymes start a series of biochemical
reactions in the cell, which causes the cell to release chemicals,
including histamine, from storage pouches in the cell interior called
granules.  These chemicals allow fluid to leak from blood vessels,
producing symptoms such as redness and swelling in nearby tissues,
and also constrict smooth muscles and stimulate mucus production.
The substances also cause such symptoms as a runny nose, sneezing,
itching, hives or abdominal cramps.  In severe cases, anaphylactic
shock may occur following the massive release of such chemicals.

       Other enzymes in the cell membrane cause mast cells and
basophils to produce lipid molecules called prostaglandins and
leukotrienes, which, like histamine, can constrict the lungs'
airways. In addition, mast cells secrete chemical messengers that
recruit other cells from the bloodstream into the tissues, including T
lymphocytes, basophils and eosinophils.

       In the tissues, some of the recently arrived cells release
substances that can increase and prolong early symptoms and may
injure and inflame local tissue.  Such responses often occur
several hours after the initial encounter with an allergen. Collectively,
they are called the late-phase reaction.  The cells present in
late-phase reactions are quite similar to those cells found in the tissues
of patients with chronic allergic rhinitis and asthma.


IS IT ALL IN THE GENES?

       Many studies suggest that some allergic diseases and
asthma can be inherited.  Until recently, however, the specific
genes responsible for these diseases had not been identified.  Now,
investigators have found abnormalities of genes associated with
asthma and allergic diseases, offering the possibility of treating
the primary causes of allergy rather than merely the symptoms.  One
day, it may be possible to identify, even before birth, people at
risk for allergic diseases.  This would make efforts such as allergen
avoidance more efficient by identifying susceptible individuals.


Studies in the Amish and Dutch.
       At The Johns Hopkins University, NIAID grantee David
Marsh, M.D., and colleagues have used genetic analyses of Amish
families to show that a gene regulating the level of IgE in the
bloodstream is located in a specific region of chromosome 5.
Eugene Bleecker, M.D., of the University of Maryland and
colleagues report similar findings in their studies of families in
the Netherlands.  Because high IgE levels in the blood are associated
with allergies, as well as with bronchial hyperactivity and asthma,
it is possible that abnormalities in this gene are associated with
allergic conditions.

       Previous research shows that the gene for IL-4, an important
regulator of IgE levels is on the same region of chromosome 5.
Researchers now speculate that the IL-4 gene and the gene on
chromosome 5 associated with IgE levels might be one and the
same.

Zeroing in on an "allergy gene."
       Grantee Lanny Rosenwasser, M.D., and colleagues at the
National Jewish Center for Immunology and Respiratory Medicine in
Denver, Colo., have found a specific segment of the IL-4 gene that
appears to be different in members of families prone to asthma and
allergy compared to non-allergic individuals.  If this gene segment
can be shown to correlate with high IgE levels, a strong link
between the IL-4 gene and the chromosomal region studied by Dr. Marsh
would be established.  Researchers might then be able to design
strategies to use the gene or its protein products as a target for
therapy in allergic individuals.


NEW HOPE FOR ALLERGIC SEAFOOD LOVERS

       NIAID intramural researcher Dean Metcalfe, M.D., and his
colleagues are working to characterize the specific parts, or
epitopes, of food proteins that bind to IgE and cause allergic
reactions in susceptible people.  Very few of these epitopes are
known, but the identification of these allergy-causing molecules is
an important step in designing approaches to immunotherapy and
diagnostic tools for food allergy.

Muscle protein the culprit in shrimp allergy.
       Recently, Dr. Metcalfe and collaborators in the United
States and India identified two specific regions of a shrimp muscle
protein called tropomyosin that may trigger the adverse reactions suffered
by people allergic to shrimp.  The researchers also found similar
regions in muscle proteins of other shellfish, which may explain
why people allergic to shrimp often are allergic to lobster, crab and
other crustaceans as well.

       The researchers have created small synthetic proteins that
correspond to the allergy-causing regions of the shrimp protein.
These peptides may prove useful in immunotherapy and in
developing a specific test for shrimp allergy.


WHY BOTH NUTS AND BEANS BEDEVIL SOME ALLERGIC PEOPLE

       Many people who are allergic to peanuts are allergic to
other legumes (peas, nuts, beans) as well.  Research by Wesley Burks,
M.D., and his colleagues at the University of Arkansas has helped
explain why.

       Dr. Burks and his colleagues have shown that the gene that
codes for Ara h I, one of the two main peanut allergens, is similar
to genes from legumes that produce proteins called vicilins.  The
researchers found that IgE from peanut-allergic patients also bound
to vicilins from other plants, which explains the cross-reactivity
of peanuts and legumes in allergic people.


THE DOPE ON THE DOCKING MOLECULE

       NIAID researcher Jean-Pierre Kinet, M.D., and his
colleagues have proven that a docking molecule on mast cells and
basophils to which IgE attaches, the high-affinity IgE receptor, is
necessary for allergic reactions.  A therapy that blocks this
receptor could be effective in treating allergies.

       They developed a mouse that lacks a gene to make part of
the high-affinity IgE receptor called the alpha chain.  Without the
alpha chain, the IgE receptor of these mice is unable to bind to
IgE, thwarting an allergic reaction.

       Under experimental conditions, Dr. Kinet's "knockout mice"
(the gene is "knocked out" using laboratory techniques) did not
suffer allergic reactions, whereas ordinary mice with the receptor had the
expected allergic reactions in similar experiments.  These results
confirmed that the high-affinity IgE receptor has a key role in
allergy.

       In other experiments, Dr. Kinet and his team demonstrated
that the other chains of the high-affinity IgE receptor, the beta
and gamma chains, work together to activate the cascade of enzymes
that result in mast cells and basophils releasing inflammatory
chemicals following an encounter with an allergen.  A further
understanding of the interactions of the molecules in the receptor
and the activation of signals in the mast cells and basophils could
provide targets for therapies to prevent allergic reactions.

A mouse with human genes.
       Dr. Kinet and his team are developing a mouse with genes
for the human high-affinity IgE receptor, which differs slightly
from the mouse receptor.  These mice will allow scientists to test
potential allergy treatments that inhibit the interaction of human IgE with
the human high-affinity IgE receptor, or the signalling pathways in the
cell.


HOW DO ALLERGY SHOTS WORK?

       Allergen immunotherapy has been used since 1911, but the
reasons why it works are unclear.  Researchers are now beginning
to understand the mechanisms behind the proven effectiveness of
allergy shots.

       NIAID grantee Dale Umetsu, M.D., Ph.D., and his colleagues
at Stanford University have demonstrated that T cells taken from
patients with allergies produce large amounts of IL-4, but that
IL-4 production is greatly reduced in allergic patients given allergy
shots.  Reduction in IL-4 production correlated with improvement in
allergy symptoms, indicating that decreased IL-4 may be a key to future
therapies for allergies.  Dr. Umetsu and his colleagues are now
studying how IL-4 production is regulated, and looking for more
efficient ways to reduce IL-4 synthesis in T cells.


COMBATTING CAT ALLERGY

       NIAID grantee Philip Norman, M.D., of Johns Hopkins
University and his colleagues are studying immunotherapy using
small portions of allergens called peptides, synthesized in the
laboratory.  Peptides may have fewer side effects than whole
allergens when given as allergy shots, allowing for higher doses
that may improve the effectiveness of immunotherapy.

       Recently, Dr. Norman and his co-workers reported results of
the first clinical trial using peptide immunotherapy for people
allergic to cats, employing peptides based on the Fel d 1, the principal cat
allergen found in the animals' saliva.

       The investigators gave 91 cat-allergic patients four weekly
injections of the peptides or a placebo. Six weeks later, the team
evaluated results of the treatment by exposing the subjects to live
cats in the Hopkins "cat room."  In patients receiving high doses
of the peptides, the treatment reduced allergy symptoms by about 50
percent.  Among eight patients receiving the highest dose and
followed for eight months, four had reductions in symptoms that
were maintained or improved, two maintained some benefit, and two lost
benefit.

A low-tech approach.
       NIAID grantee Tom Platts-Mills, M.D., Ph.D., and colleagues
at the University of Virginia have shown that washing a cat with
tap water can reduce the amount of airborne Fel d 1.  Two cats were
washed in tap water for five minutes, and significant amounts of
Fel d 1 were found in the bath water.  Airborne allergen levels were
measured in an airtight room, and reductions in cat allergen ranged
from 7 to 56 percent after the initial wash, and 36 to 60 percent
after repeated immersions.


MANAGING MAST CELLS

       Mast cells are the big guns in the allergic response,
initiating many of the symptoms of allergy and asthma.  A number of
researchers are studying the factors that control mast cell growth
and activation, with an eye toward containing allergic symptoms by
limiting mast cells.

       NIAID intramural researcher Dean Metcalfe, M.D., and his
co-workers showed that the immune system molecules IL-3 and
stem cell factor (SCF) both stimulate the growth of human mast
cells from their bone marrow precursor cells.  IL-3 appears to be
important for early mast cell proliferation, while SCF maintains
the cells' viability and promotes maturation.

       Using cultures of mouse mast cells, the researchers found
that removing IL-3 causes cells to undergo a kind of cellular
suicide called apoptosis.  However, adding SCF to the cultures prevents
the cellular suicide by linking to surface receptors known as
c-kit.  Another immune system molecule, transforming growth factor
beta, prevents the "rescue" of cells by SCF.

       These and other studies of mast cell growth factors suggest
that it may be possible to alleviate allergy symptoms by deleting
some of an allergic person's mast cells.  Possible treatments
include drugs or cytokines that block IL-3 or SCF.  Alternately, it may be
possible to selectively block the action of SCF by using antibodies
that bind to its receptor, or to employ fragments of the receptor
itself that act as decoys for SCF.

       Dr. Metcalfe and his colleagues also have identified several
of the substances to which mast cells bind in the body.  They are
working to develop ways to limit mast cell migration into tissues
and subsequent adhesion, thereby reducing allergic responses.
!
*
When Should I See an Allergist?

Asthma and allergies are among the most common health problems, with as
many as 50 million Americans afflicted with asthma, hay fever or other
allergy-related conditions.

Some allergies -- such as a mild case of hay fever -- may require no
treatment, or may be controlled with the occasional use of over-the-counter
medications. Others, however, may interfere with day-to-day activities,
lessen the quality of life or, sometimes, be life-threatening. In these cases,
individuals should seek the care of a physician to help them manage their
disease.


The allergist treats asthma and allergies
A physician who specializes in the diagnosis and treatment of asthma and
allergies is an allergist. The allergist has passed a qualifying examination
and is specially trained to identify the factors that trigger asthma or allergies,
and help the patient to prevent or treat the condition.

After earning a medical degree, the allergist completes a three-year residency
training program in either internal medicine or pediatrics, followed by a
two- or three-year program of study in the field of allergy and immunology.
You can be certain that your doctor has met these requirements if he or she
is certified by the American Board of Allergy and Immunology.


When to see an allergist
If you suffer from asthma or allergies -- or suspect that you may have either
-- an allergist will help you learn more about your condition and provide
treatment that controls or eliminates your symptoms. Often, the symptoms
of asthma or allergies develop gradually over a period of time.

Allergy sufferers may become so accustomed to chronic symptoms as
sneezing, nasal congestion or wheezing that they do not consider their
symptoms to be unusual. Yet, with the help of an allergist, these symptoms
can usually be prevented or controlled and the patient╒s quality of life greatly
improved.


What is an allergy?
One of the marvels of the human body is that it can defend itself against
harmful invaders such as viruses or bacteria. But sometimes the defenses
are too aggressive, and harmless substances such as dust, mold or pollen
are mistakenly identified as dangerous. The immune system then rallies its
defenses, launching a host of complex chemical weapons to attack and
destroy the supposed enemy. In the process, some unpleasant and, in
extreme cases, life-threatening symptoms may be experienced by the
allergy-prone individual.


The cause of allergic reactions
There are hundreds of ordinary substances that can trigger allergic reactions.
Among the most common are plant pollens, molds, household dust (dust
mites), animal dander, industrial chemicals, foods, medicines, feathers, and
insect stings. These triggers are called ╥allergens.╙

An allergic reaction may occur anywhere in the body, but usually appears in
the skin, eyes, lining of the stomach, nose, sinuses, throat and lungs --
places where special immune system cells are stationed to fight off invaders
that are inhaled, swallowed or come in contact with the skin.


Who develops asthma or allergies?
Asthma and allergies can affect anyone, regardless of age, gender, race or
socioeconomic factors. While it╒s true that asthma and allergies are more
common in children, they can occur for the first time at any age or, in some
cases, recur after many years of remission.

Although the exact genetic factors are not yet understood, the tendency to
asthma and allergies is linked to heredity. In susceptible people, factors
such as hormones, stress, smoke, perfume or other environmental irritants
may also play a role.


How an allergist can help
Effectively controlling asthma and allergies requires planning, skill and
patience. The allergist, with his or her specialized training and expertise in
managing asthma and allergies, can develop a treatment plan for your
individual condition. The goal will be to enable you to lead a life that is
normal and symptom-free as possible.


Some of the methods that may be used by the allergist include:
Allergy testing. The allergist will usually perform tests to determine what
allergens are involved.

Prevention. The most effective approach to treating asthma or allergies is to
avoid the factors that trigger the condition in the first place. Even when it is
not possible to completely avoid allergens, an allergist can design strategies
for reducing exposure and preventing symptoms.

Prescription Medications. A number of new and effective medications are
available to treat both asthma and allergies.

Immunotherapy (Allergy Shots). In this treatment, patients are injected over
the course of time with gradually increasing doses of the substance to which
they are allergic. In many cases, the body╒s reaction to the substance
becomes less pronounced over time.


Types of Allergies
Allergic Rhinitis (Hay Fever). Allergic rhinitis is a general term used to
describe the allergic reactions that take place when an airborne allergen,
such as pollen or mold, is inhaled through the mouth or nose. Symptoms
may include sneezing, congestion, runny nose, excess mucus, weepy eyes
and a scratchy or burning palate and throat. These may be seasonal
symptoms triggered by plant pollens or molds, in which case the condition
is often called ╥hay fever.╙ Sometimes the problem is year-round, in which
case the culprit may be dust, dander from household pets or other animals,
or, less frequently, a food allergy.

Contact Dermatitis, Skin Allergies. Contact dermatitis, eczema and hives are
skin conditions that can be caused by allergens or other irritants. When the
skin comes in direct contact with an allergen, usually a chemical, contact
dermatitis may result. Often the reaction may take hours or days to develop,
as in the case of poison ivy. The most common allergic causes of rashes
such as eczema are pollen, foods and animals. Hives, or skin welts, may be
triggered by emotional stress or an underlying disease. Allergies to foods,
pets and medication, especially penicillin and aspirin, can also be the cause.

Asthma. Asthma is caused when muscle spasms constrict the flow of air to
the lungs. The linings of airways become inflamed and swollen, and excess
mucus may clog the airways. An asthma attack is characterized by labored
or restricted breathing, a tight feeling in the chest, coughing and wheezing.
The condition can develop quickly and may vary in severity from mild
discomfort to life-threatening attacks in which breathing stops altogether.
Sometimes a chronic cough is the only symptom, and many cases of the
disease go undiagnosed.

Anaphylaxis. Anaphylaxis is a rare, potentially fatal allergic reaction that
affects many parts of the body at once. The trigger may be an insect sting, a
food, such as nuts or shellfish, or medications. Symptoms may include a
dangerous drop in blood pressure, flushing, difficulty breathing, swelling
of the throat, tongue and nose, and loss of consciousness. Usually these
symptoms are immediate and progress rapidly. Emergency medical attention
should be sought at the first sign of an anaphylactic reaction.


You should see an allergist if:
- Your allergies are causing secondary symptoms such as chronic sinus
infections, nasal congestion or difficulty breathing.
- You experience hay fever or other allergy symptoms several months out of
the year.
- Antihistamines and other over-the-counter medications do not control your
allergy symptoms, or create unacceptable side effects, such as drowsiness.
- Your asthma or allergies are interfering with your ability to carry on day-
to-day activities.
- Your asthma or allergies decrease the quality of your life
- You are experiencing warning signs of asthma such as:
-- You sometimes have to struggle to catch your breath.
-- You often wheeze or cough, especially at night or after exercise.
-- You are frequently short of breath of feel a tightness in your chest.
-- You have previously been diagnosed with asthma, but despite treatment,
you have frequent acute asthma attacks.


For more information about asthma and allergies, or a list of board certified
allergists in your area, call 1-800-842-7777.

!
*
Alopecia:

A skin disease characterized by loss of hair, partial or total.

 ---------------------------------------------------
Alopecia

Alopecia, or hair loss, can be caused by a large number of
diverse causes.  Alopecia is divided into two basic groups:
nonscarring and scarring alopecia.

 NONSCARRING ALOPECIA: the hair shafts are gone, but the hair
follicles are preserved (generally reversible).  Examples
include:

 Primary skin disorders

1. Androgenetic alopecia--This is a form of alopecia that
results from an increase in circulating androgens
(testosterone).  This is a common familial form of alopecia
that is seen in males.  In women, this may occur secondary to
dysfunction of the adrenal glands or ovaries.  In some cases,
the possibility of an adrenal gland tumor must be excluded.  A
receding hairline is a common finding in androgenetic alopecia.

2. Alopecia areata--alopecia of unknown cause, but there are
distinctive pathologic findings on microscopic examination of
the skin.  Some feel stress and anxiety play a role in this
form of alopecia.  Baldness is usually patchy and temporary

3. Tinea capitis--a fungal infection that may appear as an area
of scaly skin with minimal hair loss, or patches of hair loss
with broken hairs and pustules.

4. Traumatic alopecia--broken hairs are commonly seen.  Often
due to traction with curlers, rubber bands, braiding, exposure
to heat or chemicals, or hair pulling.

 Drug Side effects--generally temporary and regrowth occurs in
most cases after stopping the offending agent.  Examples
include:

1. Warfarin
2. Heparin
3. propylthiouracil
4. Vitamin A
5. Carbimazole
6. Isotretinoin
7. Lithium
8. Beta-blockers
9. Levodopa
10. Amphetamines
11. Thallium
12. Chemotherapeutic agents

  Systemic Diseases
1. Lupus (also a scarring form of alopecia, see below)
2. Secondary syphilis (scattered hair loss)
3. Hypothyroidism (diffuse thinning)
4. Hyperthyroidism (diffuse thinning)
5. Deficiencies of protein, iron, biotin, or zinc (diffuse thinning)

  SCARRING ALOPECIA: inflammation, fibrosis, and loss of hair
follicles.  A smooth scalp with a decreased number of
follicular openings.  Examples include:

 Primary Skin Disorders
1. Lichen planus
2. Scleroderma

 Systemic Diseases
1. Lupus (discoid lupus)
2. Sarcoidosis
3. Skin metastases (cancer)
!
*
                 ALZHEIMER'S DISEASE: AN OVERVIEW


                  What is Alzheimer's disease?

     Alzheimer's disease (pronounced Altz-hi-merz) is a
progressive, degenerative disease that attacks the brain and
results in impaired memory, thinking and behavior.  It affects an
estimated 2.5 million American adults.
     When it was first described by German physician Alois
Alzheimer in 1907, Alzheimer's disease was considered a rare
disorder.  Today, it is recognized as the most common cause of
dementia.
     Dementia is not a disease itself but a group of symptoms
that characterize certain diseases and conditions.  In the case
of Alzheimer's disease, the dementia includes a decline in
intellectual functioning that is severe enough to interfere with
the ability to perform routine activities.
     The second most common form of dementia is multi-infarct
dementia, which is caused by vascular disease and strokes.  Other
causes of dementia are Huntington's disease, Parkinson's disease,
Pick's disease and Creutzfeldt-Jakob disease.
     There also are a number of conditions that cause dementia-
like symptoms:  depression, drug reactions, thyroid disorders,
nutritional deficiencies, brain tumors, head injuries,
alcoholism, infections (meningitis, syphilis, AIDS) and
hydrocephalus.
     Alzheimer's disease is distinguished from other forms of
dementia by characteristic changes in the brain that are visible
only upon microscopic examination.
     At autopsy, Alzheimer's disease brains show the presence of
tangles of fibers (neurofibrillary tangles) and clusters of
degenerating nerve endings (neuritic plaques) in areas of the
brain that are important for memory and intellectual functions.
     Another characteristic of Alzheimer's disease is the reduced
production of certain brain chemicals, especially acetylcholine
and somatostatin.  These chemicals are necessary for normal
communication between nerve cells.


             Who is affected by Alzheimer's disease?

     Alzheimer's disease is more likely to occur as a person gets
older.  Although less than one percent of people age 65 are
affected by Alzheimer's disease, it is present in an estimated 25
percent of those age 85 or older.  Alzheimer's disease can occur
in middle age as well.  The youngest documented case is that of a
28-year-old individual.
     Alzheimer's disease also affects the family of an Alzheimer
patient.  The emotional, social and financial costs of caring for
an Alzheimer patient are high.  Family members also often risk
their own health in order to care for the Alzheimer patient at
home.


                     What are the symptoms?

     Alzheimer's disease has a gradual onset.  Symptoms include
difficulty with memory and loss of intellectual abilities severe
enough to interfere with routine work or social activities.  The
Alzheimer patient also may experience confusion, language
problems (such as trouble finding words), poor or decreased
judgment, disorientation in place and time and changes in
behavior or personality.
     How quickly these changes occur in an Alzheimer patient will
vary from person to person.  Eventually, the disease leaves its
victims totally unable to care for themselves.  The course of the
disease averages eight years from the time symptoms first appear,
although Alzheimer's disease has been known to last as long as 25
years.


             How is Alzheimer's disease diagnosed?

     At this time, there is no single diagnostic test for
Alzheimer's disease.  A complete physical, psychiatric and
neurologic evaluation by a physician experienced in the diagnosis
of dementing disorders should be obtained when symptoms are
noticed.
     The examination should include a detailed medical history,
mental status test, neuropsychological testing, blood work,
urinalysis, chest x-ray, electroencephalography (EEG),
computerized tomography (CT scan) and electrocardiogram (EKG).
Such an evaluation is essential to determine whether the dementia
is the result of a treatable illness.
     When this kind of a detailed examination is done, the
accuracy of diagnosis is about 90 percent.  However, the only way
to confirm a diagnosis of Alzheimer's disease is to examine brain
tissue under a microscope.


                What causes Alzheimer's disease?

     Scientists are still looking for the cause of Alzheimer's
disease, and their research is pointing in several directions.
     For one form of the disease called Familial or Uncommon
Alzheimer's Disease, there is strong evidence that a defect
exists in a single gene on Chromosome 21.
     However, for most Alzheimer's disease patients the genetic
involvement is less clear.  Although there does seem to be a
genetic predisposition for the disease, other factors influence
whether or not an individual develops Alzheimer's disease.
     Scientists continue to explore the importance of such things
as a slow virus; environmental toxins, such as aluminum; and
   other physical conditions of an individual that may interact with
the genetic defect.


                  Is Alzheimer's disease fatal?

     Although Alzheimer's disease is associated with a shortened
lifespan, the slow, progressive nature of the disease often means
the patient will live for several years after diagnosis.
     Alzheimer's disease eventually leaves the patient physically
less resistant to infections, such as pneumonia, which may be
fatal.  In addition, Alzheimer's disease generally affects an
older population that is subject to other chronic illnesses, and
those illnesses, too, may often be the cause of death.


          Can Alzheimer's disease be treated or cured?

     At this time, there is no treatment or cure available to
stop or reverse the mental deterioration of Alzheimer's disease.
However, new research findings give reason for hope.  Several
drugs used to relieve the symptoms of memory loss are in clinical
trials.
     Other medications are available now to assist in managing
some of the most troubling symptoms of Alzheimer's disease.
Under a doctor's supervision, medication can be used to control
depression, behavioral disturbances and sleeplessness.


              Can Alzheimer's disease be prevented?

     Prevention is not possible at this time because the cause of
the disease is not known.  However, advances in science are
bringing us closer to answers that can lead to treatments and/or
strategies for prevention.  Meanwhile, focus on improved care and
support for the caregiver are helping to ease the burden of
Alzheimer's disease.

    Is there help for people affected by Alzheimer's disease?

     The Alzheimer's Association is the only national voluntary
organization dedicated to providing support and assistance to
Alzheimer patients and their families.  The Alzheimer's
Association is continuing research into the cause, treatment and
cure of Alzheimer's disease, as well as ways to improve the care
and management of current Alzheimer patients.
     The Alzheimer's Association has more than 1,200 support
groups and 190 Chapters nationwide.  For more information on
Alzheimer's disease and the Alzheimer's Association or to
volunteer in the fight against Alzheimer's disease, call the
Association's toll-free number for the location of the Chapter
nearest you.



                            Glossary

  Dementia -- This is not a disease in itself but a group of
symptoms that characterize certain diseases and conditions.  The
major symptoms involve a decline in intellectual functioning that
is severe enough to interfere with routine activities.
Alzheimer's disease is the most common form of dementia.

  Senility -- This label often was used to describe an individual
65 years or older with dementia.  Senility used to be considered
a normal part of aging.  Today, physicians recognize that
dementia is not a normal part of aging but the result of a
disease such as Alzheimer's disease.

  Senile dementia/pre-senile dementia -- Senile dementia is a label
that used to apply to patients whose symptoms of dementia
appeared after age 65.  Pre-senile dementia is a label that used
to apply to dementia victims who were younger than age 65.  These
distinctions have largely been eliminated.  Today, most cases of
dementia in both groups of patients are diagnosed as Alzheimer's
disease.

  Chronic organic brain syndrome -- This label is sometimes given
to patients with a collection of symptoms such as memory loss,
disorientation, confusion, personality changes and inability to
carry out normal daily activities.  The preferred term for these
symptoms is dementia or dementing illness.

  Hardening of the arteries -- The correct medical term is
arteriosclerosis.  The dementia symptoms associated with
arteriosclerosis result only when multiple cerebral infarcts
(strokes) have occurred.  This condition is called multi-infarct
dementia.

     This brochure is one of a series made available by the
Alzheimer's Association with underwriting from American Contract
Bridge League, Louis N. Cassett Foundation, the Coca Cola
Foundation, Ittleson Foundation, Inc., and Million Dollar Round
Table Foundation.
!
*
                      ALZHEIMER'S QUESTION & ANSWER SHEET

             Alzheimer's Disease and Related Disorders Assoc. Inc.
                         70 E. Lake Street, Suite 600
                            Chicago, Illinois 60601


                         What is Alzheimer's Disease?

The most common form of dementing  illness,  Alzheimer's  Disease  (AD)  is  a
progressive,  degenerative  disease  that attacks the brain,  causing impaired
memory,  thinking and behavior.  The person with AD may experience  confusion,
personality  and behavior changes,  impaired judgment,  and difficulty finding
words,  finishing thoughts or following directions.  It eventually leaves  its
victims incapable of caring for themselves.

               What happens to the brain in Alzheimer's Disease?

The nerve cells in the part of the brain that controls memory,  thinking,  are
damaged, interrupting the passage of messages between cells. The cells develop
distinctive changes that are called neuritic plaques (clusters of degenerating
nerve cell ends) and neurofibrillary  tangles  (masses  of  twisted  filaments
which  accumulate  in  previously  health  nerve cells).  The cortex (thinking
center) of the brain shrinks (atrophies),  The spaces in  the  center  of  the
brain become enlarged, also reducing surface area in the brain.

                 What are the symptoms of Alzheimer's Disease?

Alzheimer's Disease is a dementing illness which leads to loss of intellectual
capacity.  Symptoms  usually  occur  in older adults (although people in their
40s and 5Os may also be affected) and include loss of language skills --  such
as trouble finding words,  problems with abstract thinking,  poor or decreased
judgment,  disorientation in place and time,  changes in mood or behavior  and
changes in personality. The overall result is a noticeable decline in personal
activities or work performance.

                    Who is affected by Alzheimer's Disease?

Alzheimer's Disease knows no social or economic boundaries and affects men and
women  almost  equally.  The  disease  strikes  older persons more frequently,
affecting approximately 10% of Americans over age 65 and 47% of those over age
85.

                       What causes Alzheimer's Disease?

The cause of Alzheimer's Disease is not known.  Researchers are  investigating
suspected causes such as neurological damage,  chemical deficiencies, viruses,
genetic abnormalities,  environmental toxins and malfunctions  in  the  body's
disease defense systems.

                      Is Alzheimer's Disease hereditary?

There  is  a slightly increased risk that children,  brothers,  and sisters of
patients with Alzheimer's Disease will get it,  but most cases  are  the  only
ones in a family.  Some patients who develop the disease in middle age (called
early onset) have a "familial" type -- more than one case in the family. It is
important to note that AD can  only  be  definitively  diagnosed  after  death
through  autopsy  of  brain  tissue.  Thirty  percent  of  autopsies turn up a
different diagnosis.  Families are encouraged to  ask  for  an  autopsy  as  a
contribution to learning more about the genetics of AD.

            Are there treatments available for Alzheimer's Disease?

Presently,  there  is  no  definite cure or treatment for Alzheimer's Disease.
Unfortunately,  there are many unscrupulous individuals who  market  so-called
"cures." These treatments are often expensive and they don't cure AD. However,
since  senility  is such a scary problem and because families are desperate to
find help for loved ones,  these bogus treatments continue to  sell.  Most  of
them have no scientific proof of effectiveness.

                     How is Alzheimer's Disease diagnosed?

There  is no single clinical test for Alzheimer's Disease.  It is diagnosed by
ruling out all other curable or incurable causes of memory  loss.  A  positive
diagnosis  of Alzheimer's Disease can only be made by microscopically studying
a small piece of brain tissue after death. The cerebral cortex of an Alzheimer
brain will have characteristic abnormalities -- cells marred  by  plaques  and
tangles.  However,  a  working  diagnosis  can be made through various testing
procedures that include a  thorough  physical  as  well  as  neurological  and
psychological examinations.

               How long do people with Alzheimer's Disease live?

People  diagnosed with Alzheimer's Disease may live from two to 20 years after
the onset of memory loss symptoms.  It shortens one's expected life span,  but
given appropriate care and medical attention,  patients often survive for many
years at home or in a nursing home. Death can't usually be predicted until the
very terminal stages.  It is common for patients in terminal-stage Alzheimer's
to  lose  weight,  and to have difficulty swallowing,  controlling bladder and
bowels, walking and speaking.  They may curl into a fetal position.  Alzheimer
victims  often  succumb  to  a  series  of repeated infections such as bladder
infections or pneumonia.

                   What is the scope of Alzheimer's Disease?

Alzheimer's afflicts approximately 4 million Americans and it's estimated that
one in three of us will face this disease in  an  older  relative.  More  than
100,000  die annually,  making Alzheimer's Disease the fourth leading cause of
death among adults.  Half of all current nursing home patients  are  affected,
making AD a costly public health and long term care problem.  An estimated $80
billion is spent annually on  the  care  of  AD,  including  costs  diagnosis,
treatment,  nursing home care,  at-home care and lost wages.  Alzheimer's also
affects the patient's caregivers, who become the second victims.  Persons with
AD  often  require 24-hour care and supervision,  most of which is provided in
the home by family and friends.  In  addition  to  the  tremendous  stress  of
providing  care,  families  also  bear  most  of  the financial burdens of the
disease as well.

                 Aren't memory problems normal in old people?

Benign,  or normal,  forgetfulness is part of the  normal  aging  process  and
usually  begins  in  early  middle  age.  Most  people  have  some  experience
forgetting names, appointments or where they left their keys.  However, normal
forgetfulness  differs  from  Alzheimer's Disease in some very important ways.
The Alzheimer patient will frequently become lost  in  familiar  surroundings;
forget  names of familiar people;  have problems handing money;  forget how to
dress,  read or write;  and lose the ability to use the tools of daily  living
such as a key or radio.
!
*
                    AMYOTROPHIC LATERAL SCLEROSIS

The literal meaning of Amyotrophic Lateral Sclerosis is: A -
Lack of, MYO - muscle, TROPHIC - nourishment, and SCLEROSIS -
hardening of areas in the LATERAL - side (as well as the
forward) portions of the spinal cord.

Amyotrophic Lateral Sclerosis is commonly reffered to as
A.L.S. or Lou  Gehrig's Disease (after the famous baseball
player who had this illness).  Considering the fact that ALS
has been known to physicians since the late  1860's, and that
during any year approximately 2 of every 125,000 people  will
develop ALS, the general public has little or no knowlege of
the disease.  Therefore, the purpose of this article is to help
familiarize the public with  this disease.

ALS typically strikes people between 40 and 70 years old, but
can strike  people as young as 20 and as old as 80.  Men seem
to be affected about one  and a half times more often than
women.  Any race or nationality can be  affected.  The average
course of ALS is three to four years.  However, recent  reports
have indicated that 50 percent of ALS patients were living at
three  years after diagnosis and 10 percent of these were
living after 10 years, and  1 percent of these actually
improved and appear to no longer have the disease.

ALS is a progressive, degenerative muscle disease. In that
the weakening  of the affected muscles never ceases. ALS is a
disease that affects the motor  neurons (nerve cells that
control muscles) in the brain and spinal cord.  Eventually
these motor neurons are unable to transmit the signals which
the  muscles need to work. The easiest way to understand how
this works is to  think of the motor neurons in ALS as
something like worn-out electrical power  lines.  In a normal
motor neuron, electrical signals flow easily to the muscle  and
the muscle responds by contracting.  In an ALS affected motor
neuron,  the electrical signal has trouble getting through, or
may not get to the  muscle at all, resulting in the muscle not
contracting.  An unused muscle will  eventually atrophy (shrink
and waste-away).

The early symptoms of ALS vary, but typically appear as a
general feeling  of fatigue.  As the muscles lose their energy
source the patient may  experience muscle twitching or muscle
cramping.  Usually at this point the  patient does not even
realize there is anything wrong, and therefore, ALS can  be
present for some time before more extreme symptoms appear.
THIS DOES NOT  MEAN that everyone who experiences muscle
cramping, twitching, and fatigue has  ALS.  The aforementioned
symptoms also could occur in someone who, unused to a
vigourous workout, vigourously worked out.  More advanced
symptoms may appear  in any external muscle of the body except
those supplying the eyes.  Advanced  symptoms often begin in
the hands and feet and then extend closer to the trunk.

Symptoms in the hand may include having difficulty holding unto
objects,using  all fingers to type, play piano, etc.  Symptoms
in the feet, may include,  foot drop(foot can't be held at a 90
degree angle to the leg). As the disease  progresses to the
legs the patient usually experiences difficulty with walking,
falling down, and rising from a sitting position. I would like
to say at this  time, that the human body has some amazing
resources it applys in the early  stages of ALS.   While the
ALS victim may have moderatley severe weakness in  certain
muscles, muscles that have not yet been affected will
compensate for  the affected muscles, i.e. if shoulder muscles
are not yet affected they will  compensate for weak arm
muscles.  Therefore, the ALS patient will be able to  continue
to do ordinary things for a longer period of time.  Eventually,
as  the disease progresses all muscles will be affected except
the bladder muscle,  the sphincter muscle, and the eye
muscles.  Typically, the progression starts  with hands, and
feet, moves to legs, arms and trunk, then to the speech and
swallowing process, and finally to the lungs, which results in
death.

The cause of ALS is unknown at present, therefore, there is no
cure.  Research is being done and there are various theories,
but to date, there is  nothing concrete.

For more information on ALS you can contact the Muscular
Dystrophy  Association, 810 Seventh Avenue, New York, NY
10019, or The ALS Association,  15300 Ventura Blvd., Suite 315,
Sherman Oaks, CA   91403
!
*
ANEURYSM FAQ'S

Frequently asked questions/answers prepared by:

M. David Tilson, MD
Professor of Surgery
Columbia University in the City of New York
I-way address: [e-mail]
Compuserve: 73443,244

1. What is an aneurysm?

     An aneurysm is a dilation of a blood vessel (similar
to a balloon) that poses a risk to health from the
potential for rupture, clotting, or dissecting.  Rupture
of an aneurysm in the brain causes stroke, and rupture of
an aneurysm in the abdomen causes shock.  The abdominal
aortic aneurysm (AAA) is the most common, and the rest of
this discussion will focus on the AAA.

2. What causes aneurysms?

     Several new theories have developed over the last 15
years.  It appears that the disease probably requires a
basic genetic susceptibility that may be traceable to a
single major locus, in other words, a single dominant
gene.  The disease unequivocally runs in families.  In
addition, there are probably other contributing causes,
like smoking and high blood pressure.

3. Who is at greatest risk?

     White men over age 55 are at the greatest risk.  In
fact, aneurysms are among the top ten causes of death
among this group.
By about age 80, over 5% of white males will have
developed an aneurysm.  AAA's occur less frequently in
white women, and they are relatively uncommon in African
Americans of both sexes.

4. Why are aneurysms so dangerous?

     AAA's cause many deaths because they are usually
silent until a medical emergency occurs.  One author has
referred to an AAA as a "U-boat" in the belly, because
they are silent, deep, and deadly.
5. How can I find out if I have one?

     If you are thin and have a moderately large-sized
AAA, you or your doctor may be able to feel it below your
rib cage.  Many are incidentally discovered as a result
of medical imaging for other conditions, by ultrasound
exams, CAT scans, MRI's, or even plain films of the
abdomen.  If you are over 55 and other members of your
family have had one or more AAA's, you should advise your
doctor and have an ultrasound.  It is safe, fast and
painless.

6. I have an aneurysm.  What is the risk of death from
rupture?

     If rupture occurs, few survive.  Among celebrities,
Roy Rogers survived a rupture, but he was the exception
and not the rule.  The best predictor of risk of rupture
is the size of the aneurysm. The diameter of a normal
aorta is about 2 centimeters (cm)(a little less than an
inch).   Once a AAA has reached 5-6 centimeters (cm) in
diameter, about the size of an orange, the risk of
rupture is very substantial, probably about 50/50 over
the next few years.  I think all vascular surgeons would
agree that a 5-6 cm aneurysm should be repaired, unless
the patient is a prohibitive risk.  There is less
unanimity of opinion about smaller AAA's, since the risk
of rupture is much lower.  A few surgeons are now
recommending repair of aneurysms over 3 cm, but most
would advise watchful waiting for AAA's that small.
There is presently a controlled, randomized, multi-center
trial being carried out in the Veterans Administration
Hospitals to try to answer the question as to when the
surgical risk for the patient becomes less than the risk
from rupture, depending on the size of the AAA.

7. What is "watchful waiting?"

     Most vascular surgeons feel comfortable following
patients with small AAA's every six months with an
ultrasound examination.  The average rate of growth of an
aneurysm is less than one-half of a centimeter per year,
and some grow much slower, remaining relatively stable
for fairly long periods of time.  Others may enlarge
rapidly, and a "growth spurt" is a serious warning sign.

8. Is there anything one can do while "waiting"?

     Giving up tobacco, making sure of reasonable blood
pressure control, and improving physical fitness with a
mild exercise program are all prudent.  So far, no
medication has been proven in a prospective scientific
experiment to reduce the growth rate of AAA's in people,
although propranolol (a beta-blocker) has been shown to
prevent ruptured aneurysms in turkeys and delay aneurysm
growth in mice.  Retrospective studies at Yale and at the
University of Vermont have suggested that propranolol
might be beneficial in people, but proof will await a
prospective trial.  One is being planned now by
investigators in Canada and at the University of Vermont.

9. What are the risks of surgical repair?

     The risk of death from surgery is related to
hospital expertise and experience, the skill of the
surgeon, and the basic underlying health of the patient.
Mortality rates are frequently reported to be as low as
0 to 2% in academic medical centers with vascular
specialists and superior intensive care.  Rates may be
higher in small community hospitals without dedicated
vascular specialists.  Patients without any history
or signs of heart disease generally do very well,
because heart attack postoperatively is the leading cause
of surgical mortality.  Patients with known coronary
artery disease should have a thorough cardiological
evaluation prior to surgery.

10. How long does it take to recover, and then will I
have a normal life?

     The average hospital stay is 7-10 days, and most
patients take about 6 weeks off before returning to work.
By that time, they have usually regained their sense of
well being, although some bounce back much faster.  The
vast majority of patients are back on a normal survival
curve for life expectancy, consistent with their cohort
of persons of similar age and with similar underlying
health (e.g., heart condition, renal function, etc.)

11. I've heard of "minimally invasive surgery".  Does
this work for AAA?

     Not yet, but progress is being made.  An
"endovascular" repair (e.g., using tubes, stents, and
wires threaded up into the aneurysm from leg arteries)
was developed by a surgeon in Argentina and is presently
coming into use in the United States.  It is presently
considered experimental and offered only in selected
centers to patients who present a prohibitive risk for
a standard surgical approach.

12. Where can I get more information on this subject?

     I have posted additional files on Compuserve and on
the Internet (URL "http://www.cc.columbia.edu/~mdt1/").
Comprehensive home medical books, like the one published
by the staff at the Mayo Clinic, are in most large
bookstores and may be helpful.

     If you want to take a plunge into the underlying
scientific literature, a gate to Medline like Paperchase
on Compuserve will allow you to search thousands of
scientific and medical communications (with abstracts
available for downloading).

     Last month (Aug 94) I participated in the organizational
meeting of the American Aneurysm Foundation, which has
received a tax-exempt charitable charter to raise public
awareness of the aneurysm problem and to support the
development of new knowledge related to aneurysm prevention
and treatment.  We will be developing a library of
information for both conventional and also electronic
distribution.  If you would like to be on our mailing list
as we get started, please E-mail to [e-mail],
or use my Compuserve address (73443,244).
!
*
                          Facts About Angina?

  What is angina?

  ANGINA PECTORIS ("ANGINA") IS A recurring pain or discomfort
  in the chest that happens when some part of the heart does
  not receive enough blood.  It is common symptom of coronary
  heart disease (CHD), which occurs when vessels that carry
  blood to the heart become narrowed and blocked due to
  atherosclerosis.

  Angina feels like a pressing or squeezing pain, usually in
  the chest under the breast bone, but sometimes in the
  shoulders, arms, neck, jaws, or back.  Angina is usually
  precipitated by exertion.  It is usually relieved within a
  few minutes by resting or by taking prescribed angina
  medicine.

  What brings on angina?

  Episodes of angina occur when the heart's need for oxygen
  increases beyond the oxygen available from the blood
  nourishing the heart.  Physical exertion is the most common
  trigger for angina.  Other triggers can be emotional stress,
  extreme cold or heat, heavy meals, alcohol, and cigarette
  smoking.

  Does angina mean a heart attack is about to happen?

  An episode of angina is not a heart attack.  Angina pain
  means that some of the heart muscle in not getting enough
  blood temporarily-for example, during exercise, when the
  heart has to work harder.  The pain dose NOT mean that the
  heart muscle is suffering irreversible, permanent damage.
  Episodes of angina seldom cause permanent damage to heart
  muscle.

  In contrast, a heart attack occurs when the blood flow to a
  part of the heart is suddenly and permanently cut off.  This
  causes permanent damage to the heart muscle.  Typically, the
  chest pain is more severe lasts longer, and does not go away
  with rest or with medicine that was previously effective.
  It may be accompanied by indigestion, nausea, weakness, and
  sweating.  However, the symptoms of a heart attack are
  varied and may be considerably milder.

  When someone has a repeating but stable pattern of angina, an
  episode of angina does not mean that a heart attack is about
  to happen.  Angina means that there is underlying coronary
  heart disease.  Patients with angina are at an increased
  risk of heart attack compared with those who have no
  symptoms of cardiovascular disease, but the episode of
  angina is not a signal that a heart attack is about to
  happen.  In contrast, when the pattern of angina changes--if
  episodes become more frequent, last longer, or occur without
  exercise--the risk of heart attack in subsequent days or
  weeks is much higher.

  A person who has angina should learn the pattern of his or
  her angina--what cause an angina attack, what it feels like,
  how long episodes usually last, and whether medication
  relieves the attack.  If the pattern changes sharply of if
  the symptoms are those of a heart attack, one should get
  medical help immediately, perhaps best done by seeking an
  evaluation at a nearby hospital emergency room.

  Is all chest pain "angina"?

  No, not at all.  Not all chest pain is from the heart, and
  not all pain from the heart is angina.  For example, if the
  pain lasts for less that 30 seconds or if it goes away
  during a deep breath, after drinking a glass water, or by
  changing position, it almost certainly in NOT angina and
  should not cause concern.  But prolonged pain, unrelieved by
  rest and accompanied by other symptoms may signal a heart
  attack.

  How is angina diagnosed?

  Usually the doctor can diagnose angina by noting the
  symptoms and how they arise.  However one or more diagnostic
  tests may be needed to exclude angina or to establish the
  severity of the underlying coronary disease.  These include
  the electrocardiogram (ECG) at rest, the stress test, and x-
  rays of the coronary arteries (coronary "arteriogram" or
  "angiogram").  The ECG records electrical impulses of the
  heart.  These may indicate that the heart muscle is not
  getting as much oxygen as it needs ("ischemia"); they may
  also indicate abnormalities in heart rhythm or some of the
  other possible abnormal features of the heart.  To record
  the ECG, a technician positions a number of small contacts
  on the patient's arms, legs, and across the chest to connect
  them to an ECG machine.  For many patients with angina, the
  ECG at rest is normal.  This is not surprising because the
  symptoms of angina occur during stress.  Therefore, the
  functioning of the heart may be tested under stress,
  typically exercise.  In the simplest stress test, the ECG is
  taken before, during, and after exercise to look for stress
  related abnormalities.  Blood pressure is also measured
  during the stress test and symptoms are noted.  A more
  complex stress test involves picturing the blood flow
  pattern in the heart muscle during peak exercise and after
  rest.  A tiny amount of a radioisotope, usually thallium, is
  injected into a vein at peak exercise and is taken up by
  normal heart muscle.  A radioactivity detector and computer
  record the pattern of radioactivity distribution to various
  parts of the heart muscle.  Regional differences in
  radioisotope concentration and in the rates at which the
  radioisotopes disappear are measures of unequal blood flow
  due to coronary artery narrowing, or due to failure of
  uptake in scarred heart muscle.  The most accurate way to
  assess the presence and severity of coronary angiogram, an
  x-ray of the coronary artery.  A long thin flexible tube (a
  "catheter") is threaded into an artery in the groin or
  forearm and advanced through the arterial system into one of
  the two major coronary arteries.  A fluid that blocks x-rays
  (a "contrast medium" or "dye") is injected.  X-rays of its
  distribution show the coronary arteries and their narrowing.

  How is angina treated?

  The underlying coronary artery disease that causes angina
  should be attacked by controlling existing "risk factors."
  These include high blood pressure, cigarette smoking, high
  blood cholesterol levels, and excess weight.  If the doctor
  has prescribed a drug to lower blood pressure, it should be
  taken as directed.  Advice is available on how to eat to
  control weight, blood cholesterol levels, and blood
  pressure.  A physician can also help patients to stop
  smoking.  Taking these steps reduces the likelihood that
  coronary artery disease will lead to a heart attack.  Most
  people with angina learn to adjust their lives to minimize
  episodes of angina, by taking sensible precautions and using
  medications if necessary.  Usually the first line of defense
  involves changing one's living habits to avoid bringing on
  attacks of angina.  Controlling physical activity, adopting
  good eating habits, moderating alcohol consumption, and not
  smoking are some of the precautions that can help patients
  live more comfortably and with less angina.  For example, if
  angina comes on with strenuous exercise, exercise a little
  less strenuously, but do exercise.  If angina occurs after
  heavy meals, avoid large meals and rich foods that leave one
  feeling stuffed.  Controlling weight, reducing the amount of
  fat in the diet, and avoiding emotional upsets may also
  help.

  Angina is often controlled by drugs.  The most commonly
  prescribed drug for angina is nitroglycerin, which relieves
  pain by widening blood vessels.  This allows more blood to
  flow to the heart muscle and also decreases the work load of
  the heart.  Nitroglycerin is taken when discomfort occurs or
  is expected.  Doctors frequently prescribe other drugs, to
  be taken regularly, that reduce the heart's workload.  Beta
  blockers slow the heart rate and lessen the force of the
  heart muscle contraction.  Calcium channel blockers are also
  effective in reducing the frequency and severity of angina
  attacks.

  What if medication fail to control angina?

  Doctors may recommend surgery or angioplasty if drugs fail
  to ease angina or if the risk of heart attack is high.
  Coronary artery bypass surgery is an operation in which a
  blood vessel is grafted onto the blocked artery to bypass
  the blocked or diseased section so that blood can get to the
  heart muscle.  An artery from inside the chest (an "internal
  mammary" graft) or long vein from the leg (a "saphenous
  vein" graft) may be used.

  Balloon angioplasty involves inserting a catheter with a
  tiny balloon at the end into a forearm or groin artery.  The
  balloon is inflated briefly to open the vessel in places
  where the artery is narrowed.  Other catheter techniques are
  also being developed for opening narrowed coronary arteries,
  including laser and mechanical devices applied by means of
  catheters.

  Can a person with angina exercise?

  Yes.  It is important to work with the doctor to develop an
  exercise plan.  Exercise may increase the level of pain-free
  activity, relieve stress, improve the heart's blood supply,
  and help control weight.  A person with angina should start
  an exercise program only with the doctor's advice.  Many
  doctors tell angina patients to gradually build up their
  fitness level-for example, start with a 5 minute walk and
  increase over a weeks or months to 30 minutes or 1 hour.
  the idea is to gradually increase stamina by working at a
  steady pace, but avoiding sudden burst of effort.

  What is the difference between "stable" and "unstable"
  angina?

  It is important to distinguish between the typical stable
  pattern of angina and "unstable" angina.  Angina pectoris
  often recurs in a regular or characteristic pattern.
  Commonly a person recognizes that he or she is having angina
  only after several episodes have occurred, and a pattern has
  evolved.  the level of activity of stress that provokes the
  angina is somewhat predictable, and the pattern changes only
  slowly.  This is "stable" angina, the most common variety.

  Instead of appearing gradually, angina may first appear as a
  very severe episode or as frequently recurring bouts of
  angina.  Or, an established stable pattern of angina may
  change sharply; it may by provoked by far less exercise that
  in the past, or it may appear at rest.  Angina in these
  forms is referred to as "unstable angina" and needs prompt
  medical attention.

  The term "unstable angina" is also used when symptoms
  suggest a heart attack but hospital tests so not support
  that diagnosis.  For example, a patient may have typical but
  prolonged chest pain and poor response to rest and
  medication, but there is no evidence of heart muscle damage
  either on the electrocardiogram or in blood enzyme tests.

  Are there other types of angina?

  There are two other forms of angina pectoris.  One, long
  recognized but quite rare, is called Prinzmetal's or variant
  angina.  This type is caused by vasospasm, a spasm that
  narrows the coronary artery and lessens the flow of blood to
  the heart.  the other is a recently discovered type of angina
  called microvascular angina.  Patients with this condition
  experience chest pain but have no apparent coronary artery
  blockages.  Doctors have found that the pain results from
  poor function of tiny blood vessels nourishing the heart as
  well as the arms and legs.  Microvascular angina can be
  treated with some of the same medications used for angina
  pectoris.
!
*
Ankle Sprains

Created 1989

THIS IS A HEALTH-LINE MESSAGE FROM THE UNIVERSITY OF WISCONSIN-MADISON.

     Ankle sprains are common injuries that usually result from a turning-in of
the foot.  A sprain involves a partial or complete tear of a ligament, the
tough tissue that holds together the bones that form joints.  When a ligament
is partially torn, the intact portion holds the joint together.  A complete
tear of the ligament may result in abnormal motion of the joint, causing an
unstable feeling.
     When your weight comes down on your turned-in foot you might hear a pop or
snap, and feel pain on the outer side of the ankle.  Within hours, a moderate
amount of swelling is seen.  Bleeding from the torn ligaments may cause a
purplish discoloration of the skin within 24 hours.
     Most ankle sprains are of minor severity.  They involve moderate pain and
swelling on the outside of the ankle without pain on the inner side.  If you
think you have sprained your ankle, stop the activity you are participating in
and protect the weakened ligament from further injury.  A plastic bag of ice
wrapped in an elastic bandage should then be applied for a period of twenty
minutes, four to five times daily, for seventy-two hours.
     The ice constricts the bleeding vessels in the torn ligaments, preventing
swelling.  In addition, it relieves pain.  Elevation of the affected ankle
above the level of the heart and application of an elastic bandage, such as an
ace wrap, around the ankle also helps prevent swelling.  After 72 hours, a
heating pad set at 101-102 degrees Fahrenheit applied over a moist towel for 20
minute periods, 3 times a day, will aid in improving the blood supply to
healing ligaments.  Crutches with partial weight bearing on the ankle should be
used for 4-5 days to protect the damaged ligament and for comfort.
     Pain on both the inside and outside of the ankle, pain above the ankle, a
feeling of instability, gross deformity, or severe swelling are all indications
of a more severe injury involving rupture of the ligaments or fracture.  These
injuries should initially be protected with use of crutches, and treated with
ice, elevation, and an ace wrap.  Such injuries should also be evaluated by a
physician.  A completely torn ligament needs to be protected in a cast for 4-6
weeks in order to prevent the ligament from healing in an elongated position.
     A neglected sprained ankle can cause persistent ankle and leg pain, and
can encourage successive sprains.  Walking on a sprained ankle does not prove
you are brave.
     If you are uncertain whether you have a sprain or a fracture, be sure to
consult a physician.  Thank you.

!
*
 Anorexia Nervosa And Bulimia Nervosa
THIS IS A HEALTH-LINE MESSAGE FROM THE UNIVERSITY OF WISCONSIN-MADISON.

Anorexia nervosa and bulimia nervosa are complex
psychophysiologic disorders.  Individuals with anorexia have an
intense fear of obesity, a disturbed body image, and pursue
thinness regardless of their emaciated state. A companion
disorder, bulimia nervosa, consists of uncontrollable binge
eating, usually followed by self-induced purging such as
vomiting or use of laxatives. Bulimia is called the binge/purge
syndrome, and occurs across all weight ranges.

     Both anorexia and bulimia occur at a much higher frequency
in females than in males, and a significant increase in their
incidence has occurred during the past decade.

     The cause of eating disorders is still not clear, but we
do know there is no single physical or psychological cause.
Considerable attention is now being focused on the possible
connection between bulimia and depression.  It is essential to
approach the understanding of eating disorders from a combined
biological, psychological, and social perspective.  Individuals
with eating disorders have learned to use their behaviors to
cope, although unsuccessfully, with life's struggles.  Problems
in self-esteem, self-identity, and assertiveness are all
related to eating disorders and are treated with
psychotherapy.  Close to half of those suffering from eating
disorders will have poor psychological and social adjustment to
adult life as well as prolonged abnormal eating patterns.

     Eating disorders can be severe and potentially life
threatening.  Although they are psychiatric disorders, a wide
variety of physical complications may occur as a result of
self-induced starvation, vomiting and other behaviors intended
to achieve weight loss.  The physical complications seen in
individuals who have eating disorders are a result of the
starvation process itself; the binging behavior; and the
attempts by the individual to control weight artificially
through self-induced vomiting, and the use of laxatives,
diuretics, or diet pills.  Almost every organ system in the
body can be affected.  One of the most frequent complications
is the loss of or increasing irregularity of menstrual periods.

     The long term mortality rate for anorexia nervosa is
higher than in any other psychiatric disorder.  Death is
usually the result of the direct effects of starvation,
chemical disturbances affecting the heart, or suicide.
Frequent vomiting may cause damage to the esophagus, stomach,
teeth and gums, as well as lead to other imbalances of vital
body fluids and electrolytes, imbalances which could lead to
death.  Abuse of laxatives or water pills can also cause these
fluid and electrolyte imbalances, and can lead to death.  Many
other complications have been reported making careful medical
management of these conditions essential.

     The overall treatment of eating disorders requires both
medical and psychological support.  Less severe cases can be
followed with some success by individual physicians and
psychotherapists.  However, in recent years, multidisciplinary
teams have developed to better manage those who are more
severely affected.  Most individuals with eating disorders can
be treated as outpatients.  A few individuals who have lost
control over their eating or purging habits and are unstable
physically or psychologically, may require hospitalization.
Many medical centers now offer specialized care in this area.
Treatment should include symptom management, medical
consultation, nutritional education, and individual and/or
family psychotherapy.  Cognitive behavioral group psychotherapy
has also been helpful, particularly for bulimia.

     Support groups such as those facilitated by the
organization ANAD, which stands for Anorexia Nervosa and
Associated Disorders, may be beneficial for both victims and
their families.  Support groups, however, should not substitute
for individualized treatment programming.  Thank you.  ID:
036-1560.
!
*
Appendicitis

      The appendix is the remnant of a primitive extension of the
digestive system, and occurs as a small, worm-like pouch arising
from the first part of the colon, just after it begins to attach
to the small intestine.  It has no important function in human
digestion.

   When the opening of the appendix becomes obstructed with a
particle of undigestible food, a small calcium-containing stone,
or other matter, the chemicals and bacteria within it begin to
interact, and swelling and pressure build up.  The pressure
eventually compresses the draining veins, and the process
accelerates.  The stretched walls are then invaded by the
bacteria, pus is produced from the white cells rushed to the
areaby the body, and ultimately the appendix may burst.  If this
happens, contamination of the usually sterile abdominal cavity
and its surrounding sac, the peritoneum, occur.  This so-called
"acute abdomen" may rapidly be fatal, as blood poisoning nd
shock develop.

   Most victims are between the ages of 5 and 35.  Males and
females are both affected.  No definite causative factors are
known; although grape seeds, popcorn and other similar particles
are sometimes found to in the appendiceal opening, there is no
good evidence linking these statistically with the disease.

    Symptoms

   Classically, but by no means consistently, there is a
relatively abrupt onset of pain in the upper abdomen or navel
area, which later radiates to the right lower abdomen.  Nausea or
vomiting often will follow. Low grade fever is noted in many
patients, rising markedly if perforation occurs.  As the disease
and symptoms progress in frequency, perforation becomes a
concern, and urgency of treatment is obvious.

   The physician may also look for elevated white blood cell
counts, abdominal tenderness, rigidity, and characteristic sounds
(or lack of sounds) in the abdomen.  In the classical case, the
diagnosis is rarely missed.

   Unfortunately, many cases are not typical.  The pain may not
occur in the right lower abdomen, or may occur there as well as
other areas such as the upper abdomen or back. Differential
considerations may include diseases of the colon, gall bladder,
ovary, pancreas, small intestine, kidney, or other organs.  It is
generally recognized that even the most wise, conscientious, and
concerned physician will occasionally miss or misdiagnose
appendicitis.  In fact, most surgeons would agree that the
occasional removal of a normal appendix in sincere efforts to
remove the diseased ones at an appropriate time is an unfortunate
but acceptable price to pay for careful medical practice.  The
price of NOT removing a truly infected appendix is far greater.

    Treatment

  Appendectomy--the removal of the appendix-- is the treatment of
choice unless there is some strong medical reason to the
contrary.  It is interesting that in other countries, treatment
is often more conservative, with antibiotics, intravenous fluids,
and other measures.  This leaves the potential for recurrences,
and is only recommended for rare exceptions in current American
practice.

   If perforation has not yet occurred, some 15% of patients may
develop some postoperative complication, but these are generally
easily managed.  On the other hand, if perforation has occurred,
up to 50 or 60% of patients have complications. Mortality of
appropriately treated appendicitis today is extremely low, and
usually occurs in elderly or debilitated patients.  The main goal
of the patient is to seek care early.

   It is important to realize that many doctors will take an
observational approach early in the disease; this is wise under
some circumstance to avoid unnecessary surgery, since many
apparent cases will turn out to be some other self-limited
disease such as infection, ovarian pain, etc.  Careful judgment
is called for, and intimate communication between doctor and
patient (sometimes entailing hospitalization) is crucial.

   Preventive appendectomy is not generally necessary (General
Eisenhower is said to have done this before going to the front),
unless an unrelated surgery is necessary and the appendix is
removed incidental to this.  No long-term complications of
uncomplicated appendectomy are recognized.
!
*

                   ARRHYTHMIA/RHYTHM DISORDERS

What is arrhythmia?

An arrhythmia is a change in the regular beat of the heart.
The heart may seem to skip a beat or beat irregularly or
very fast or very slowly.

Does having an arrhythmia mean that a person has heart
disease?

Many times, there is no recognizable cause of an arrhythmia.
Heart disease may cause arrhythmias.  Other causes include:
stress, caffeine, tobacco, diet pills, and cough and cold
medicines.

Are arrhythmias serious?

The vast majority of people with arrhythmias have nothing to
fear.  They do not need extensive exams or special
treatments for their condition.

In some people, arrhythmia are associated with heart
disease.  In these cases, heart disease, not the arrhythmia,
poses the greatest risk to the patient.

In a very small number of people with serious symptoms,
arrhythmia themselves are dangerous.  These arrhythmias
require medical treatment to keep the heartbeat regular.
For example, a few people have a very slow heartbeat
(bradycardia), Causing them to feel lightheaded or faint.
If left untreated, the heart may stop beating and these
people could die.

How common are arrhythmias?

Arrhythmias occur commonly in middle-age adults.  As people
get older, they are more likely to experience an arrhythmia.

What are the symptoms of an arrhythmia?

Most people have felt their heart beat very fast,
experienced a fluttering in their chest, or notice that
their heart skipped a beat.  Almost everyone has also felt
dizzy, faint, or out of breath or had chest pains at one
time of another.  One of the most common arrhythmia is sinus
arrhythmia, the change in heart rate that can occur normally
when we take a breath.  These experiences may cause anxiety,
but for the majority of people, they are completely
harmless.

You should not panic if you experience a few flutters or
your heart races occasionally.  But if you have questions
about your heart rhythm or symptoms, check with your doctor.

Arrhythmia Types

Originating in the Atria

Sinus arrhythmia.  Cyclic changes in the heart rate during
breathing.  Common in children and often found in adults.

Sinus tachycardia.    The sinus node sends out electrical
signal faster than usual, speeding up the heart rate.

Sick sinus syndrome.    The sinus node does not fire its
signals properly, so that the heart rate slows down.
Sometimes the rate changes back and forth between a slow
(bradycardia) and fast (tachycardia) rate.

Premature supraventricular contractions or premature atrial
contraction (PAC).  A beat occurs early in the atria,
causing the heart to beat before the next regular heartbeat.

Supraventricular tachycardia (SVT), paroxysmal atrial
tachycardia (PAT).  A series of early beats in the atria
speed up the heart rate ( the number of times a heart beats
per minutes).  In paroxysmal tachycardia, repeated periods
of very fast heartbeats begin and end suddenly.

Atrial flutter.  Rapidly fired signals cause the muscles in
the atria to contract quickly, leading to a very fast,
steady heartbeat.

Atrial fibrillation.  Electrical signals in the atria are
fired in a very fast and uncontrolled manner. Electrical
signals arrive in the ventricles in a completely irregular
fashion, so the heart beat is completely irregular.

Wolff-Parkinson-White syndrome.   Abnormal pathways between
the atria and ventricles cause the electrical signal to
arrive at the ventricles too soon and to be transmitted back
into the atria.  Very fast heart rates may develop as the
electrical signal ricochets between the atria and
ventricles.


Originating in the Ventricles

Premature ventricular complexes (PVC).  An electrical signal
from the ventricles causes an early heart beat that
generally goes unnoticed.  The heart then seems to pause
until the next beat of the ventricle occurs in a regular
fashion.

Ventricular tachycardia.  The heart beats fast due to
electrical signals arising from the ventricles (rather than
from the atria)

Ventricular fibrillation.  Electrical signals in the
ventricles are fired in a very fast and uncontrolled manner,
causing the heart to quiver rather than beat and pump blood.


What happens in the heart during an arrhythmia?

Describing how the heart beats normally helps to explain
what happens during an arrhythmia.

The heart is a muscular pump divided into four chamber--two
atria located on the top and two ventricles located on the
bottom.

Normally each heartbeat starts in the right atrium.  Here, a
specialized group of cells called the sinus node, or natural
pacemaker, sends an electrical signal.  The signal spreads
throughout the atria to the area between the atria called
the atrioventricular (AV) node.

The AV node connects to a group of special pathways that
conduct the signal to the ventricles below.  As the signal
travels through the heart, the heart contracts.  First the
atria contract, pumping blood into the ventricles.  A
fraction of a second later, the ventricles contract, the
ventricles contract, sending blood throughout the body.

Usually the whole heart contracts between 60 and 100 times
per minute.  Each contraction equals one heartbeat.

An arrhythmia may occur for one of several reasons:

■  Instead of beginning in the sinus node, the heartbeat
   begins in another part of the heart.
■  The sinus node develops an abnormal rate or rhythm.
■  A patient has a heart block.

What is a heart block?

Heart block is condition in which the electrical signal
cannot travel normally down the special pathways to the
ventricles.  For examples, the signal from the atria to the
ventricle may be (1) delayed, but each one conducted; (2)
delayed with only some getting through; or (3) completely
interrupted.  If there is no conduction, the beat generally
originates from the ventricles and is very slow.

What are the different types of arrhythmias?

There are different types of arrhythmia's.  Arrhythmias are
identified by where they occur in the heart (atria or
ventricles) and by what happens to the heart's rhythm when
they occur.

Arrhythmia arising in the atria are called atria or
supraventricular (above the ventricles) arrhythmias.
Ventricular arrhythmias begin in the ventricles.  In
general, ventricular arrhythmias caused by heart disease are
the most serious.

How does the doctor know that I have arrhythmia?

Sometimes an arrhythmia can be detected by listening to the
heart with a stethoscope.  However, the electrocardiogram is
the most precise method for diagnosing the arrhythmia.

An arrhythmia may not occur at the time of the exam even
though symptoms are present at other times.  In such cases,
tests will be done if necessary to find out whether an
arrhythmia is causing the symptoms.

What tests can be done?

First the doctor will take a medical history and do a
thorough physical exam.  Then one or more tests may be used
to check for an arrhythmia and to decide whether it is
caused by heart disease.


Tests for Detecting Arrhythmias

■  Electrocardiogram (EGG or EKG).  A record of the
   electrical activity of the heart.  Disks are placed on
   the chest and connected by wires to a recording machine.
   The heart's electrical signals cause a pen to draw lines
   across a strip of graph paper in the ECG machine.  The
   doctor studies the shapes of these lines to check for any
   changes in the normal rhythm.  The types of ECGs are:

---    Resting ECG.  The patient lies down for a few
     minutes while a record is made.  In this type of ECG,
     disks are attached to the patient's arms and legs as
     well as to the chest.

---  Exercise ECG (stress test).  The patient exercises
   either on a treadmill machine or bicycle while connected
   to the ECG machine.  This test tells whether exercise
   causes arrhythmias or makes them worse or whether there
   is evidence of inadequate blood flow to the heart muscle
   ("ischemia").

---  24-hour ECG (Holter) monitoring.  the patient goes
   about his or her usual daily activities while wearing a
   small, portable tape recorder that connects to the disks
   on the patient's chest.  Over time, this test shows
   changes in rhythm (or "ischemia") that may not be
   detected during a resting or exercise ECG.

---  Transtelephonic monitoring.  The patient wears the tape
   recorder and disks over a period of several days to
   several weeks.  When the patient feels an arrhythmia, he
   or telephones a monitoring station where the record is
   made.  If access to a telephone is not possible, the
   patient has the option of activating the monitor's memory
   function.  Later, when a telephone is accessible, the
   patient can transmit the recorded information from the
   memory to the monitoring station.  Transtelephonic
   monitoring can reveal arrhythmia that occur only once
   every few days or weeks.


■  Electrophysiologic study (EPS).  A test for arrhythmias
   that involves catheterization.  Very thin, flexible tubes
   (catheters) are placed in a vein of an arm or leg and
   advanced to the right atrium and ventricle.  This
   procedure allows doctors to find the site and type of
   arrhythmia and how it responds to treatment.

How are arrhythmias treated?

Many arrhythmias require no treatment whatsoever.

Serious arrhythmias are treated in several ways depending on
what is causing the arrhythmia.  Sometimes the heart disease
is treated using one or more of the following treatments.

■  Drugs

   There are several kinds of drugs used to treat
   arrhythmias.  One or more drugs may be used.

   Drugs are carefully chosen because they can cause side
   effects.  In some cases, they cause arrhythmias or make
   arrhythmias worse.  For this reason, the benefits of the
   drug are carefully weighed against any risks associated
   with taking it.  It is important not to change the dose
   or type of your medication unless you check with your
   doctor first.

   If you are taking drugs for an arrhythmia, one of the
   following , one of the following tests will probably be
   used to see whether treatment is working: a 24-hour
   electrocardiogram (ECG) while you are on drug therapy, an
   exercise ECG, or a special technique to see how easily
   the arrhythmia drugs may also be checked.

■  Cardioversion

   To quickly restore a heart to its normal rhythm, the
   doctor may apply an electrical shock to the chest wall.
   Called cardioversion, this treatment is most used in
   emergency situations.  After cardioversion, drugs are
   usually prescribed to prevent the arrhythmia from
   recurring.


■  Automatic implantable defibrillators

   These devices are used to correct serious ventricular
   arrhythmia that can lead to sudden death.  The
   defibrillator is surgically placed inside the patient's
   chest. There, it monitors the heart's rhythm and quickly
   identifies serious arrhythmia.  With an electrical shock,
   it immediately disrupts a deadly arrhythmia.

■  Artificial pacemaker

   An artificial pacemaker can take charge of sending
   electrical signals to make the heart beat if the heart's
   natural pacemaker is not working properly or its
   electrical pathway is blocked.  During a simple
   operation, this electrical device is placed under the
   skin.  A lead extends from the device to the right side
   of the heart, where it is permanently anchored.

■  Surgery

   When an arrhythmia cannot be controlled by other
   treatment, doctors may perform surgery.  After locating
   the heart tissue that is causing the arrhythmia, the
   tissue is altered or removed so that it will not  produce
   the arrhythmia.

How can arrhythmias be prevented?

If heart disease ids not causing the arrhythmia, the doctor
may suggest that you avoid what is causing it.  For example,
if caffeine or alcohol is the cause, the doctor may ask you
not to drink coffee, tea, colas, or alcoholic beverages.

Is research on arrhythmias being done?

The National Heart, Lung , and Blood Institute (NHLBI)
supports basic research on normal and abnormal electrical
activity in the heart to understand hoe arrhythmias develop.
Clinical studies with patients aim to improve the diagnosis
and management of different arrhythmias.  These studies will
someday lead to better diagnostic and treatment strategies.

Where can I find publication about heart disease?

To obtain publications about heart disease, you may want to
contact your:

■  local American Heart Association chapter
■  local or state health department

The National Heart, Lung, and Blood Institute also has
publications about heart disease.  For more information,
contact:

■  NHLBI Communications and Public
Information Branch, Building 31, Room 4A21,
Bethesda, Maryland 20892.







NIH Publication No. 91-2264
April 1991



U.S. DEPARTMENT OF HEALTH AND HUMAN SERVICES
Public Health Service
National Institutes of Health
National Heart, Lung, and Blood Institute
!
*
ARTHRITIS IN CHILDHOOD AND ADOLESCENCE
THOMAS J. A. LEHMAN MD
CHIEF, DIVISION OF PEDIATRIC RHEUMATOLOGY
THE HOSPITAL FOR SPECIAL SURGERY, AND
ASSOCIATE PROFESSOR OF PEDIATRICS
CORNELL UNIVERSITY MEDICAL CENTER
212-606-1151

Arthritis in childhood?  IsnÆt that only an old personÆs disease?  For both
children with arthritis and the doctors who care for them public education is
one of the greatest challenges.  Most lay people and many doctors fail to
realize that the problem exists.  Many children suffer for months or years
before the diagnosis of arthritis is thought of and proper treatment begun.
But the problem doesnÆt end there.  Children with arthritis frequently
experience difficulty because their teachers and schoolmates donÆt believe
children can get arthritis and have no idea what to expect from the child with
arthritis or about the nature of the illness.  As a result, when the child is finally
diagnosed with arthritis the family may be told just to put them in a wheel
chair because ænothing can be done.Æ  This is entirely wrong!!
     Arthritis affects approximately 1 child in every thousand in a given year.
Fortunately most of these cases are mild.  However, approximately one child in
every ten thousand will have more severe arthritis that doesnÆt just go away.
Many children have what is called an acute reactive arthritis following a viral or
bacterial infection.  This arthritis is often quite severe for a brief period, but
usually disappears within a few weeks or months.  Juvenile rheumatoid
arthritis (JRA) is the most common type of arthritis which persists for months
or years at a time.
    There are three main forms of JRA which are separated by how they begin.
Pauciarticular JRA is defined by the involvement of less than four joints at the
beginning.  This is the form which often begins in young girls as a swollen
knee or ankle which appears without injury or explanation.  Usually it is
æpainlessÆ but someone will have noticed that the knee looks swollen or the
child is walking funny.  Since arthritis causes morning stiffness parents are
slow to get concerned about this because, æShe always looks okay once she gets
going.Æ  This arthritis is often very mild and treated just with mild nonsteroidal
antiinflammatory drugs, but it can cause two important problems.  The biggest
problem is that many children with pauciarticular JRA develop inflammation
of the eye (iridocyclitis).  The inflammation is not painful, but if not detected
and treated it may lead to scarring of the lens and permanent visual damage
(even blindness).  At the beginning this inflammation cannot be seen except by
an ophthalmologist using a special instrument called a æslit lamp.Æ  Because the
eye disease is more common in children with a positive test for antinuclear
antibodies (ANA) these children are all told they need every three month
examinations by an eye specialist.  All other children with JRA need eye
examinations every six months.  No one has been able to completely explain
the association of eye disease and arthritis or why it is more frequent in
children with ANA.  But we do know it happens and its important to make sure
every childÆs eyes get checked.
     The second important problem with pauciarticular JRA is that it may cause
the bones in the legs to grow at different rates with the result that one leg is
longer than the other.  When this happens children are forced to walk with a
limp.  This damages the knee and the hip leading to premature arthritis from
æwearing outÆ the joints by the time the child is an adult and should be
prevented.  Fortunately we understand how this happens.  When the knee or
another joint is inflamed by the arthritis its blood supply increases.  Then just
like a plant that receives more water than the plants around it, it grows faster
and larger.  We are always trying to stop the inflammation.  Most often the
therapy is successful and the child does not develop a significant leg length
discrepancy.  If he or she does we can do two things.  First we can put a lift in
the shoe on the short side to correct the effect of the different leg lengths.
This doesnÆt do anything for the knee, but it prevents excessive wear on the
hip and allows the child to walk more normally.  The next step is to monitor
growth.  When the child is getting closer to fully grown an orthopedist can
look at X-rays of the legs and try to guess when the bones are going to stop
growing.  If the leg with arthritis is 3 cm longer than the other leg, they will
look at the X-rays and try to guess when there is 3 cm of leg growth left.  Then
you stop the growth on the leg that is too long and allow the short leg to catch
up.  This can be done with a very simple operation.
     Polyarticular JRA is the form in which four or more joints are involved
from the beginning.  This form is more severe both because of the greater
number of joints involved and the fact that it tends to get worse over time.
These children may have a great deal of difficulty with normal activities and
need to be treated aggressively.  From a doctorÆs point of view the most
important thing is to bring the disease under control as quickly as possible.
This may require use of some fairly strong medications, but itÆs important.
One thing to watch out for is using steroids (e.g. prednisone).  In severe cases
this may be necessary, but it is not a ærealÆ solution.  Steroids make patients
with arthritis feel wonderful, but its like sweeping dirt under the rug.
Everything looks good, but it really isnÆt.  Taking too much steroid for a long
period causes lots of problems like short stature and weak bones.  Whenever
we have to put a child on steroids we want to get them off as quickly as
possible.  Nonsteroidal antiinflammatory drugs are enough for many children
with polyarticular JRA, but more severe cases may require ægold shots,Æ or
æsecond lineÆ medications like sulphasalazine or methotrexate.  Severe cases
requiring steroids or æsecond lineÆ medications should be under the care of
experienced physicians.
    The most worrisome form of JRA is systemic onset disease.  This form of JRA
begins with high fevers and a rash.  It is very important in this setting to make
sure the patient really has systemic onset JRA and not an infection of some
kind.  One of the most important findings is that the fever goes away for at
least part of every day in someone with systemic onset JRA.  Usually the fever
is high once or twice each day.  At those times the child looks very sick and
doesnÆt want to be touched, but when the fever goes down to normal again
they look and feel better.  This form of arthritis is puzzling to physicians.
Sometimes it goes completely away and never comes back again.  Other times
the fevers and rash go away, but the arthritis progresses over time and can be
very severe.  This form of JRA can involve the internal organs and rarely is a
ælife threateningÆ disease.  In addition to their other problems these children
have an greater likelihood of bad reactions to medications and must be
monitored very carefully.
    There are several other forms of arthritis which can affect children and
adolescents which some doctors lump together with JRA, but have different
outcomes and should be considered separately.  Interestingly these most often
affect older children (greater than eight years of age) and teenagers while
typical JRA most often affects young children.   One of these is the teenager
who has rheumatoid factor positive arthritis with involvement of the small
joints in the hands and feet.  Rheumatoid factor is a blood test finding which is
present in most adults with rheumatoid arthritis, but is absent in most
children with JRA.  It is present in this group because they usually are
teenagers who have adult type rheumatoid arthritis starting early.  Because it
is starting early this is a very worrisome group and these children need to be
treated aggressively.  Often they will have lifelong arthritis.
     A second form of arthritis which is common in this æolderÆ group is
spondyloarthropathy.  This is a family of diseases in which the arthritis is the
same, but the associated problems are very different.  The typical findings of a
spondyloarthropathy are early involvement of the hips and other large joints.
In addition, these forms of arthritis tend to be asymmetric (i.e. one side of the
body is more severely affected than the other).  The key finding is that these
children not only have inflamed joints, but they also have inflammation
around their tendons.  Often they have ankle or heel pain due to inflammation
of the tendons inserting in the foot.  In some mild cases the tendon
inflammation occurs without obvious swollen joints.
     It is important to recognize the spondyloarthropathies as different from
JRA because the best treatment is different and the outcome is likely to be
different.  In addition, one must look carefully for evidence of the other
diseases that can be associated with spondyloarthropathies.  These include
inflammatory bowel disease, psoriasis, ReiterÆs syndrome, and BehcetÆs
syndrome.  The most worrisome children with spondyloarthropathies are the
HLA B27 positive boys.  They are at risk for developing ankylosing spondylitis.
However, most children with spondyloarthropathies seem to do reasonably
well.  In general for children who are HLA B27 negative and do not have an
associated condition the arthritis is more likely than JRA to come and go
repeatedly over a period of years, but is less likely to be very severe or
destructive.  Unfortunately we have only recognized children with spondylo-
arthropathies as being ædifferentÆ since the middle 1970Æs so good long term
follow-up data is available yet.
     With proper therapy the children with all of these forms of arthritis will
usually get better over time.  Indeed the vast majority of children with
arthritis grow up to lead normal lives without significant difficulty.  Even for
severe cases with proper medications, proper physical and occupational
therapy, and proper surgery if necessary, virtually no one with arthritis should
need a wheel chair.  Everyone doctor knows stories of children who looked
awful, but did very well or looked like it was ænothing serious,Æ who became
very sick, but these are the rare exceptions.  We can take good care of
children with arthritis.  For over 95% of the children with arthritis today we
donÆt need new drugs or miraculous inventions, we just need proper
application of the resources we already have.
    There are three important things for every child or adolescent with arthritis.
First is proper recognition and diagnosis of the disease.  Second is proper
treatment by an experienced physician with a multidisciplinary support
including physical and occupational therapists and orthopedic surgeons.
Third is proper education of the patient and family.  People with arthritis are
no different from everyone else in the world.  They all need to grow up, have
jobs, get married and have families.  Some will have some difficulty with
mechanical problems.  Many will have small things they canÆt do if you watch
them carefully.  Few will go on to be professional athletes or military officers,
but even fewer will be ætotally disabledÆ by their disease.  IÆve seen far more
children who were disabled because they were told they couldnÆt do things
than I have who were really disabled by their disease.  We should never accept
a child with arthritis being told to use a wheel chair.  In almost every case we
ought to be able to correct the problem and get them walking again.  This is
why educating the public and physicians is so important.  We all need to be
aware that children can get arthritis and must be properly diagnosed and
treated.  But once thatÆs been done we need to make sure they reach their full
potential.  We all must remember to treat children with arthritis just like
everyone else.  They need the same discipline, the same allowance, the same
grades, and the same respect as all the other children.  Arthritis might affect
the body, but it must never be allowed to affect the mind.
Arthritis in childhood and adolescence  - Lehman page 4

!
*
Check Your Asthma "I.Q."

The following true-or- false statements test what you know about
asthma.  Be sure to read the correct answers and explanation on
the back of this sheet.

T F  1. Asthma is a common disease among children and adults in
        the United States.

T F  2. Asthma is an emotion or psychological illness.

T F  3. The way that parents raise their children can cause
        asthma.

T F  4. Asthma episodes may cause breathing problems, but these
        episodes are not really harmful or dangerous.

T F  5. Asthma episodes usually occur suddenly without warning.

T F  6. Many different things can bring on an asthma episode.

T F  7. Asthma cannot be cured, but it can be controlled.

T F  8. There are different types of medicine to control asthma.

T F  9. People with asthma have no way to monitor how well their
        lungs are functioning.

T F  10.Both children and adults can have asthma.

T F  11.Tobacco smoke can make an asthma episode worse.

T F  12.People with asthma should not exercise.

11-12 correct =     Congratulations!   You know a lot about
             asthma.  Share this information with your family
             and friends.

10-11 correct =     Very good

Fewer than 10 correct =  Go over the answers and try to learn
more about asthma.

Answers to the Asthma "I.Q." Quiz

1. TRUE.  Asthma is a common disease among children and adults
   in the United States,  and it is increasing.  About 10
   million people have asthma, of whom 3 million are under 18
   years of age.

2. FALSE.  Asthma is not an emotional or psychological disease,
   although strong emotions can sometimes make asthma worse.
   People with asthma have  sensitive lungs that react to
   certain things, causing the airways to tighten, swell, and
   fill with mucus.  The person then has trouble breathing and
   may cough and wheeze.

3. FALSE. The way parents raise their children does not cause
   asthma.  It is not caused by a poor parent-child relationship
   or by being overprotective.

4. FALSE.  Asthma episodes can  be harmful.  People can get very
   sick and need hospitalization.  Some people have died from
   asthma episodes.  Frequent asthma episodes, even if they are
   mild, may cause people to stop being active and living normal
   lives.

5. FALSE.  Sometimes an asthma episode may come on quite
   quickly.  However, before a person has any wheezing or
   shortness of breath there are usually symptoms such as a
   cough, a scratchy throat, or tightness in chest.  Most
   patients learn  to recognize these early symptoms and can
   take medicine to prevent a serious episode.

6. TRUE.  For most people with asthma, an episode can start from
   many different "triggers."  Some of these things are pollen
   from trees or grasses; molds or house dust; weather changes;
   strong odors; cigarette smoke; and certain foods.  Other
   triggers include being upset; laughing or crying  hard;
   having a cold or the flu; or being  near furry or feathered
   animals.  Each person with asthma has an individual set of
   asthma " triggers".

7. TRUE.  There is no cure yet for asthma..  However asthma
   patients can control it to a large degree by:
   ■   Getting advice from a doctor who treats asthma patients
   ■   Learning to notice early signs of an asthma episode and
       to start treatment.
   ■   Avoiding things that can cause asthma episodes
   ■   Taking medicine just as the doctor says
   ■   Knowing when to get medical help with a severe episode
8. TRUE. Several types of medicines are available to control
   asthma.  Some people with mild asthma need to take medication
   only when they have symptoms.  But most people need to take
   medicine every day to prevent symptoms and also to take
   medicine when symptoms do occur.  A doctor needs to decide
   the best type of medicine for each patient and how often it
   should be taken.  Asthma patients and their doctors need to
   work together to manage the disease.

9. FALSE.  People with asthma can monitor how well their lungs
   are functioning with a peak flow meter. This small device can
   be used at home, work, or school.  The peak flow meter may
   show that the asthma is getting worse before the usual
   symptoms appear.

10.  TRUE.  Both children and adults can have asthma .
   Sometimes, but not always, symptoms will go away as children
   get older.  However, many children continue to have asthma
   symptoms throughout adulthood.  In some cases, symptoms of
   asthma are not recognized until a person is an adult.

11.  TRUE. Smoke from cigarettes, cigars and pipes can bring on
   an asthma attack..  Indoor smoky air from fireplaces and
   outdoor smog can make asthma worse.  Some can also "set off"
   other triggers.  Smokers should be asked not to smoke near
   someone with asthma.  Moving to another room may help, but
   smoke travels room to room.  No smoking is best for everyone!

12.  FALSE.  Exercise is goo for most people -with or without
   asthma.  When asthma is under good control, people with
   asthma are able to play most sports.  For people whose asthma
   is brought on by exercise, medicines can be taken before
   exercising to help avoid an episode.  A number of Olympic
   medalists have asthma.

For more information on asthma, write:
National Asthma Education Program
P.O. Box 30105
Bethesda, MD  20824-0105

National Asthma Education ProgramU.S. Department Of Health And
Human Services
Coordinated by the Office of Prevention,Public Health Service
Education, and Control           National Institute of Health
National Heart, Lung, and Blood InstituteNIH Publication No.
92-1128
                                 Reprinted October 1992
!
*

American College of Allergy, Asthma and Immunology
Advice From Your Allergist: Asthma

Asthma affects more than 10 million Americans and is one of the leading causes of
school and work absences. Over one billion dollars is spent each year on health care
for asthma. Although its exact cause remains a mystery and no cure exists, many
excellent treatment options are available to control and reverse this chronic
obstruction of the airways.

What is asthma?
Asthma is a respiratory condition characterized by episodes of airflow obstruction
in the bronchial tubes. Symptoms caused by this obstruction include coughing,
chest tightness, wheezing and shortness of breath. Although problems are often
separated by symptom-free periods, asthma is a chronic illness.

Who gets asthma?
Asthma tends to occur within families. The role of inheritance plays a less clearer
role in adult-onset asthma. People of any age may suffer from asthma, but more
than half the cases are found in children between the ages of 2 and 17. In young
children, boys are nearly twice as likely to develop asthma as girls, but this sex
difference tends to disappear in older age groups.

What causes an asthma attack?
A wide variety of "triggers" may initiate an episode of asthma. The most common
triggers are allergens, aspirin and tartrazine, irritants, food additives and
preservatives, viral respiratory infections and physical exertion.

Allergens are substances to which susceptible individuals may become allergic.
They are a major source of problems in children and adults. Common allergens
include plant pollen (tree, grass and weed), animal dander, housedust mites, molds
and certain foods. When an allergic individual comes in contact with one of these
allergens, a complicated series of events causes the body to release certain
chemicals (mediators). These mediators then trigger asthma.

Aspirin and aspirin-containing products can trigger asthma attacks in susceptible
individuals. The exact cause of the reaction is unclear, but it does not appear to be
an allergic reaction. Ten to twenty percent of asthmatics experience a significant
decrease in their lung function after taking aspirin. Similar reactions can occur with
a related group of medications called nonsteroidal anti-inflammatory agents and
with tartrazine (yellow food dye #5). As a general rule, asthmatics should avoid
these products.

Cold air, smoke, industrial  chemicals, perfume, paint and gasoline fumes are all
examples of irritants that can provoke asthma. These irritants probably trigger
asthma by stimulating irritant receptors in the respiratory tract. These receptors, in
turn, cause the muscles surrounding the airway to constrict, resulting in an asthma
attack.

Although food additives can trigger asthma, this is rare. The most common trigger
is sulfites, which are used to preserve certain foods and medications. Viral
respiratory infections are the leading cause of acute asthma attacks. Surprisingly,
bacterial infections (except sinusitis) do not usually provoke acute asthma attacks.

What happens during an asthma attack?
An asthmatic's breathing tubes are "twitchy." That is, an asthmatic's bronchial
tubes narrow in response to certain triggers. Because individuals without asthma do
not react to these stimuli, an asthmatic's bronchi are described as hyperactive.
During and attack, muscles surrounding the bronchial tubes contract, narrowing the
air passages. Inflammation also occurs along the lining of the airways which
produces swelling and further reduction of airway size. In addition, mucus glands
along the inside of the air passages produce excess mucus which accumulates in the
already narrowed air passages. The end result is that breathing, especially exhaling,
becomes extremely difficult. Air becomes trapped behind the narrowed bronchial
passages and there is a decrease in the oxygen available to the body.

How long does an asthma attack last?
The duration varies according to the severity of the attack. Mild episodes may only
last a few hours. Severe episodes, however, may go on for days or even weeks.
Mild attacks can resolve spontaneously or may require medication. More severe
attacks can also be treated with medications but may require hospitalization.

What should be done during an attack?
Always follow the instructions of your allergist. In general, it is important to stay
calm and take your prescribed medications. Bronchodilators are the most commonly
prescribed drugs to treat asthma. They relax the muscles surrounding the airways,
resulting in dilation of the bronchial tubes. Bronchodilators may be inhaled, taken
orally or injected.

Why does physical exertion cause an attack?
During exercise, rapid breathing occurs through the mouth. As a result, the air
which reaches the bronchial tubes has not been warmed and humidified by passing
through the nose. This cold, dry air can trigger asthma. Asthma symptoms are
generally at their worst after six to eight minutes of aerobic exercise. Over 70% of
all asthmatics suffer some degree of exercise-induced asthma.

Should asthmatics avoid sports and exercise?
By taking preventive measures, asthmatics should be able to compete in any sport.
Not all sports, however, are equally tolerated. In general, exercise and sports that
involve prolonged periods of running are more likely to provoke asthma attacks
than nonaerobic ones. Swimming is one of the best tolerated sports. In most
instances, exercise induced asthma can be controlled to allow participation in any
sport. Many Olympic athletes, including several gold medal winners, have had
asthma.

What is the difference between allergies and asthma?
Asthma is obstruction of airflow in the bronchial tubes that is reversible. Allergies
are one of the factors that can trigger asthma attacks. Not all asthmatics are allergic
and there are many people who are allergic but do not have asthma.

Is there a cure for asthma?
Though it has long been treatable, a cure for asthma remains elusive. Preventive
treatment, however, may minimize the difficulty an individual experiences with
asthma.

What's the best treatment?
Prevention is always the best form of treatment. It is important for an asthmatic to
learn what conditions prompt an attack and avoid them whenever possible. When
avoidance is impossible, preventive treatment is desirable. Various forms of
preventive therapy are available.

Medications may be started prior to exercise or exposure to environments that
predictably produce an attack.

If the asthma attacks are frequent or unpredictable, your allergist may advise you to
take medications on a routine basis. Drugs used for this purpose include long-acting
theophyllines, inhaled or oral beta agonists, cromolyn and inhaled or oral steroids.
For allergic asthmatics, immunotherapy (allergy shots) may offer relief from
allergens that cannot be avoided. Immunotherapy increases a patient's tolerance to
the allergens that prompt asthma symptoms.

Is asthma a psychological disorder?
No, but emotions can worsen asthma. Panic can prevent an asthmatic from relaxing
and following instructions properly, both of which are essential during an attack.
Also, scientists have found that strong emotions can cause an asthmatic's bronchial
tubes to constrict, which may provoke or worsen an attack.

Asthma can cause emotional strain. Depression often sets in when asthmatics
cannot participate in normal activities. Asthma is a leading cause of school
absences, which can have far reaching effects on the child's emotional well-being
and education. Finally, it must also be remembered that asthma can be a major
emotional and financial strain on the entire family.

Will some children outgrow asthma?
The idea that asthma will be outgrown is more a myth than reality. True, some
individuals may reach a point where they no longer suffer asthmatic symptoms as
they did in earlier years. But sophisticated testing would show these individuals still
run the risk of attacks later in life.

Is asthma life-threatening.
In severe cases, asthma can be life threatening. Deaths occur more frequently in
adults. More than 80% of the 3,880 deaths related to asthma in 1985 occurred in
asthmatics over age 45. Usually, however, the airways can be opened and the
attack controlled with medications. In a severe attack, the airways may become
completely blocked leading to respiratory failure. This condition is a medical
emergency and requires immediate attention. It is important for asthmatics to learn
to recognize severe episodes and how to prevent them. Some physicians blame  the
dramatic increase in asthma related deaths to an over-reliance on drugs designed to
open obstructed airways. It appears more likely, however, that deaths are due to the
delay in administering therapy to relieve the attack.

Tips for parents of asthmatic children
- Above all else, learn everything you can about asthma;
- Learn what triggers your child's attacks and avoid them as best you can;
- Recognize the signs of an oncoming attack and learn to judge its severity;
- Provide preventive care so that your child has the least amount of difficulty with
his or her asthma;
- Teach your child how to care for him or herself.

Summary
Although no cure exists for asthma, excellent treatment is available. We learn more
about asthma every year and newer, more effective and safer drugs are always
being developed. As a result, most asthmatics live normal, productive lives.
Research is continuing and the outlook is bright. For more information about
asthma, contact your allergist.

For more information, please contact:
American College of Allergy, Asthma and Immunology
85 West Algonquin Road, Suite 550
Arlington Heights, IL  60005
[phone]
!
*
ASTHMA AND ALLERGIC DISEASES

Research for the Future
National Institute of Allergy and Infectious Diseases
National Institutes of Health



FOREWORD


More than 40 years ago, the United States Congress recognized the
importance of  studying allergic diseases and changed the name  of
the National Microbiological Institute to the new National
Institute of Allergy and Infectious Diseases (NIAID), reflecting
the growing importance of allergic diseases.  Since then, NIAID has
been a leader in biomedical research on allergic diseases and
asthma.

Allergies are the sixth leading cause of chronic disease in the
United States.  Major scientific discoveries by NIAID scientists
and those supported by NIAID have contributed significantly to
our understanding of allergic diseases, including asthma, and have
led to substantially better methods of diagnosing and treating
them.

The Institute is especially concerned about the increasing asthma
morbidity and mortality rates in the United States, particularly in
minority populations. The seven-year-old boy pictured in this
booklet represents the thousands of American inner-city minority
children who have asthma, and who run a high risk of suffering from
the most serious consequences of this treatable and controllable
disease.

To help these children, NIAID has launched a major study in eight
research institutions around the country. The scientists who are
conducting the National Cooperative Inner-City Asthma Study
are looking at many possible reasons why asthma is increasing and
causing severe illness and death in inner-city minority children.
In this creative and innovative study, they will be evaluating
interventions that might be used to reverse this trend. In this
booklet, we examine the challenges faced by scientists studying
asthma and allergic diseases and present some of the exciting
research programs that are under way to meet these challenges. We
also look down the road at how research for the future may help us
reach the Institute's ultimate goal-to prevent asthma and allergic
diseases.



Anthony S. Fauci, M.D.
Director, National Institute of Allergy and
Infectious Diseases



INTRODUCTION

Allergic diseases were once brushed off as "just allergies," but
we are now realizing the significant role that they play in the
health and quality of life of people in this country and around
the world.  Among the major causes of chronic illness and
disability in the United States ,allergic diseases, including
asthma, may affect as many as 50 million Americans, of one in five
people.  The economic burden of these diseases is staggering. For
example, asthma was estimated to cost more than 6.2 billion health
care dollars in 1990.

The National Institute of Allergy and Infectious Diseases (NIAID),
a component of the National Institutes of Health, stands at the
forefront of scientific research on allergic and immunologic
diseases. NIAID scientists and those supported by NIAID are working
to prevent a broad spectrum of disorders of the immune system,
including asthma and allergies.

Until prevention is possible, an intermediate goal of the
Institute's research programs is to determine how allergic diseases
develop and to improve diagnosis and treatment. Achieving this
goal will help reduce the impact these diseases have on health and
quality of life as well as reduce the economic burden imposed on
individuals, families, and the health care system.

At NIAID laboratories in Bethesda, Maryland, scientists are
conducting basic and clinical investigations on asthma and allergic
diseases. NIAID's Division of Allergy, Immunology, and
Transplantation provides research grant, contract, and cooperative
agreement support to scientists at universities and other research
institutions throughout the United States.



ASTHMA

Asthma is a serious respiratory disorder that affects an estimated
10 to 15 million people and claims more than 4,000 lives in the
United States each year. Despite our increased knowledge about
asthma, the epidemiology of this disease in the United States
presents a dismal picture. During the last decade, the prevalence
of asthma cases, hospitalizations, and deaths has been
increasing. For example, in 1 987 twice as many children and
adults, ages 5 to 34, died from asthma than in 1969.

The statistics are particularly grim in minority populations in the
United States. Non-whites are almost three times as likely as
whites to die from asthma. A higher percentage of African
Americans have asthma than do whites. Minorities living in the
inner cities seem to be at particular risk for developing asthma.
For example, the rates of asthma among Puerto Rican children living
in the New York City area are among the highest in the United
States.

(see aaad.gif file)


SOCIOECONOMICS

Of the $6.2 billion spent by Americans in 1990 on asthma-related
care, almost $1.6 billion was for hospital care. Another $1 billion
was spent on medications to treat and control asthma symptoms.
Americans also lose billions of dollars in wages because of
asthma-related problems.  A recent study found that school
absenteeism costs an estimated $1 billion in lost pay for parents
who stayed home to care for asthmatic children. Adult asthmatics
who stayed home from work because of illness lost wages amounting
to $850 million.

Social factors influence the rising incidence of asthma, although
the magnitude of these influences has not yet been determined.
Certain factors, including poverty, family problems, inadequate
treatment, and lack of access to health care, probably increase an
asthma patient's risk of having a severe asthma attack or, more
tragically, of dying from asthma.

Although good, consistent medical attention can reduce
asthma-related hospitalizations, there are 500,000 asthma-related
hospital admissions each year in the United States, with a higher
percentage of african Americans and Hispanics being hospitalized
than whites. In New York City, for example, asthma-related
hospitalizations and deaths cluster in poor, predominantly minority
neighborhoods where there is often a lack of adequate primary
health care.

Environmental factors can also adversely affect people with asthma.
An example is indoor tobacco smoke, a well-known respiratory
irritant that can cause serious problems for asthma patients.
Asthmatic children with parents who smoke are at particular risk of
suffering from the effects of inhaling second-hand tobacco smoke.
The percentage of Americans who smoke cigarettes has been steadily
decreasing, but smoking continues to remain highest among
individuals who often have the least access to good health care.


ALLERGIC DISEASES

In the United States, an estimated 40 to 50 million people suffer
from allergies. An estimated 33 million people have chronic
sinusitis, the most prevalent allergy-related disease. Pollen
allergy, commonly called hay fever, affects an estimated 19 million
Americans and prompts 8 million office visits to physicians. Food
allergies are believed to occur in 8 percent of children younger
than 3 years old. In addition, each year billions of dollars are
spent on treating allergic diseases in the United States.

When an allergic person comes in contact with an allergen
(allergy-provoking substances) cells of the immune system produce
an unusual class of antibody (disease-fighting protein). This class
of antibody is called immunoglobulin E, or IgE, and it starts the
classic allergic response. Among allergic individuals, how and to
what extent their immune systems respond to a particular allergen
is influenced by genetic as well as environmental factors.

While allergic reactions to airborne allergens such as pollen,
mold, or dust usually cause relatively minor discomfort,
sensitivity to substances such as penicillin, insect venom, or
peanuts can cause anaphylaxis, a serious and potentially fatal
allergic reaction. Anaphylactic reactions to penicillin are
responsible for an estimated 1 to 8 deaths per million population.
An estimated 1 to 2 million people experience severe allergic
reactions to insect stings each year. Moreover, severe,
life-threatening allergic reactions to food may occur as frequently
as those to insect stings.



RESEARCH

Although a variety of therapies have been developed to treat asthma
and allergies, we still do not fully understand certain critical
aspects of these diseases.  To develop more effective therapies and
devise methods for preventing these illnesses, researchers are
attempting to discern how the immune system recognizes an allergen,
why people respond differently to allergens, as well as what
environmental, genetic, and other factors might he responsible for
allergic diseases.

Allergy to latex has become an increasingly important health
problem, especially among medical personnel who wear latex gloves
to reduce exposure to human immunodeficiency virus, which causes
AIDS, and hepatitis B virus. Although we know proteins derived from
latex cause allergic reactions, better understanding of the
structure and immunologic function of these proteins would help
scientists devise ways to eliminate the cause of these potentially
dangerous reactions.

NIAID-funded scientists have been at the forefront of discoveries
and advances in the field of allergy. They were the first to
identity the IgE antibody that is the key to the allergic response.
NIAID intramural investigators and NIAID supported investigators
have now identified the complete structure of the IgE receptor, the
molecule on tho surface of mast cells and basophils to which IgE
antibodies attach. (Mast cells in the tissues and basophils in the
blood are cells that together cause allergy symptoms.)  Blocking
the function of the IgE receptor may eventually lead to a new
therapy for allergies.  Investigators have studied the events that
occur after allergic reactions are initiated by allergen binding to
IgE antibody on mast cells and basophils. Perhaps the most
important breakthrough in studying allergic reactions is the
identification by NIAID investigators of the biologic events
responsible for the late phase reaction (LPR). LPR usually occurs
4 to 6 hours after an allergen has entered the body. The discovery
that these late reactions involve inflammatory cells and that they
resemble a allergic reactions has led to the recent recognition
that inflammation is a central feature of allergic diseases and
asthma. This knowledge has provided a significant focus to asthma
research-how the inflammation is produced, how it is regulated, and
how it can be prevented. In addition, NIAID investigators
discovered that inhaled corticosterolds inhibit LPR. That discovery
contributed to the growing use of these drugs to successfully treat
both allergy and asthma.

The inflammatory process is highly complex, but the insights that
have emerged highlight and expand this exciting area of research.
One major key to this process is a group of powerful chemicals
called cytokines, which are produced by certain cells of the immune
system and which help regulate cell growth and function, including
IgE antibody production and allergic and asthmatic inflammation.
Substantial progress has been made recently in understanding how
these chemicals are involved in most aspects of the development and
function of inflammation.

Research studies have opened the door for developing promising
alternative and innovative therapies such as now anti-inflammatory
drugs and agents that block the actions of cytokines.


RESEARCH AND THE COMMUNITY

The NIAID-supported National Cooperative Inner-City Asthma Study
(NCICAS) is composed of eight units in seven cities that are
studying the unusually high morbidity rates of asthma in inner
city children.

The objective of NCICAS phase I is to identify modifiable factors
determining asthma severity and morbidity among inner-city
children. A number of interesting findings have emerged from
pilot studies. For example, allergen surveys of more than 80
inner-city homes revealed that cockroaches may he a more important
trigger of asthma in this population than are house dust mites.

NCICAS is also evaluating the use of peak flow meters, plastic
devices that can be used by patients at home to monitor their
breathing. (Because of airflow obstruction in the lungs,
asthmatics have trouble breathing air out.) From the patient's
monitoring records, the doctor can tell when a person is doing well
and when to take action, such as increasing medication to forestall
trouble. The peak flow meter pilot studies showed that a two-week
monitoring of the variability of peak air flow can he successfully
and accurately carried out.

In addition, an innovative technique has been developed to measure
the quality of asthma care delivered in inner-city emergency rooms.

To reduce asthma morbidity, Demonstration and Education (D & E)
projects develop innovative ways to apply existing knowledge and
programs. The main objectives of the projects are to teach
better self-management skills to those with asthma and to increase
asthma Knowledge among health care providers.

Two school-based D 8 E projects involve now self-management skills.
One project, carried out by investigators at Scripps Clinic in San
Diego, California, is concerned with developing asthma intervention
protocols in fourth through sixth graders in a predominantly
Hispanic school district.

The other, based at The Johns Hopkins University in Baltimore,
Maryland, is studying new evaluation tools for asthma in 13- to 18
year-olds in a predominantly African-American school district.
In addition, a clinic-based project, a computer-driven asthma
education aid for children, is in the final stages of development
at Children's Hospital in Boston, Massachusetts. This program will
teach asthma management skills to African-American asthma patients
under age 15.

NIAID established the Asthma, Allergy, and Immunologic Diseases
Cooperative Research Centers (AAIDCRCS) to promote Interaction
among basic and clinical researchers and to enhance outreach and
demonstration activities. These centers encourage close
coordination between scientists studying fundamental concepts of
Immunology, genetics, biochemistry, and pharmacology and clinical
investigators who treat allergic individuals. This approach will
lead to both a better understanding of the complex mechanisms
underlying asthma and allergies and the clinical application of
this knowledge.  Various allergic problems are under investigation
at the AAIDCBCS: asthma; skin diseases, such as atopic dermatitis;
urticaria; angioedema; and allergic reactions caused by insect
stings, foods, drugs, and airborne allergens.

Studies also focus on the basic mechanisms involved in immune
system function and reactions, including research on antibodies,
particularly IgE, and on the inflammation-inducing chemicals
released during an allergic attack.


FUTURE

The remarkable advances during the last 10 years in our
understanding of the structure and functions of the immune system
are paving the way to an exciting and hopeful future for
diagnosing, treating, and preventing asthma and allergic diseases.
Recent discoveries using modern molecular technology are providing
a stimulus for allergic disease research. For example, scientists
have developed a genetically altered mouse that produces large
amounts of the cytokine interleukin-4 (IL-4). Such mice express an
inflammatory response in several tissues that resembles human
allergic inflammation, and they also can produce large amounts of
lgE antibody.  This type of approach will lead to a clearer
understanding of which genes are responsible for allergic responses.
We are now beginning to understand how cytokines regulate the
production of adhesion molecules that bind inflammatory cells to
the sites of allergic disease, such as the nose, skin, and lungs.
These and other findings suggest that molecules directed against
critical targets in the allergic process such as cytokines and
adhesion molecules-may lead to a new means of therapy to better
treat asthma and allergic diseases.

One of the most exciting areas of research involves unraveling the
genetic mysteries of asthma and allergic diseases in conjunction
with studying how allergens and other environmental factors induce
these diseases. By identifying the genes responsible for them,
prenatal diagnostic tests could he developed to identify persons at
risk for allergic disease. This early diagnostic knowledge could he
used to manipulate the environment or to start early intervention
therapy, thereby eliminating or altering the severity of the
disease process in these individuals. Thus, the overall quality of
life would be greatly improved for these patients.

Recent studies in understanding how and why the immune systems of
allergic individuals produce IgE ant bodies to allergens may lead
to innovative vaccine therapy. In preliminary clinical trials,
scientists are evaluating an approach that could stop the immune
system from responding to allergens. Called peptide induced
allergy, this process could be the "allergy shot" of the future.

Finally, researchers are working to better understand the actions
of the cells and molecules associated with the inflammatory process
in asthma and allergic diseases.  Knowing more precisely how these
cells travel to the site of inflammation in the lungs, nose,
gastrointestinal tract, or skin should pave the way for new drugs
that block this cascade of events.

With the quickening pace of research on asthma and allergic
diseases and on the immune system, we may be able to cure-or even
prevent-these chronic, often severe, and sometimes life-threatening
diseases by the end of the decade.


INFORMATION RESOURCES



Allergy and Asthma Network/
Mothers of Asthmatics, Inc.
3554 Chain Bridge Road, Suite 200
Fairfax, VA 22030
1-800-878-4403

American Academy of Allergy
and Immunology
GI 1 East Wells Street
Milwaukee, WI 53202
1-800-822-ASMA

American College of Allergy
and Immunology
800 E. Northwest Highway
Suite 1080
Palatine, IL 60067
708-359-2800

American College of Chest Physici
3300 Dundee Road
Northbrook, IL 60062-2340
708-498-1400

American Lung Association
American Thoracic Society
1740 Broadway
New York, NY 10019-4374
212-315-8700


Asthma and Allergy Foundation
of America
II 25 ]5th Street, N.W., Suite 502
Washington, DC 20005
1-800-7-ASTHMA

National Asthma Education Program
Information Center
P.O. Box 30105
Bethesda, MD 20824-0105
301-251-1222
!
*
ASTHMA

The one characteristic all asthmatics share is a hypersensitive
airway system.  When an asthma attack is triggered, these
hypersensitive airways become narrow and fill up with mucus, making
it difficult to breathe.  Although mild asthma is sometimes more of
a nuisance than a life-threatening condition, prompt treatment is
necessary to avoid potential complications.

Three percent of the United States population, including both
children and adults, suffers from asthma.  However, thanks to
extensive research and the introduction of new medications, this
condition is indeed treatable, and there is no reason why an
asthmatic should not lead a full and productive life.

What happens during an attack?


Picture the human respiratory system as an upside-down tree.  Air
is taken in through the windpipe, or trachea (the trunk of the
tree), which divides into two main branches called bronchi.  The
bronchi further divide into smaller branches called bronchioles.
The bronchioles end in clusters of air sacs, or alveoli.  Within
the alveoli oxygen-laden air is exchanged for deoxygenated air (air
that has already been used by the body).

During an asthma attack the smooth muscles that wrap around the
bronchioles tighten, shutting off the flow of air to and from the
alveoli.  In addition, because of irritation the soft membranes
that line the bronchioles become inflamed, and the secretion of
mucus increases.  Because this bronchial mucus is unusually sticky,
coughing fails to expel it.  The mucus plugs up the bronchioles,
making it extremely difficult to exhale, and causes the wheezing
sound characteristic of an asthma attack.

Because greater difficulty is experienced in breathing out than in
breathing in, increasing amounts of stale air are retained in the
lungs with each breath.  In a severe attack, this can lead to a
feeling of near suffocation.

Causes

A variety of stimuli can trigger an asthmatic attack in a
susceptible individual.  When asthma runs in the family
(particularly if it begins in childhood) the stimulus is often
found to be an allergen (a substance that produces an allergic
reaction).  The asthma is part of the allergic response.  Common
allergens include pollen, mold spores, house dust, pet dander and
dairy products, especially eggs and milk.

An asthma attack may also be triggered by an irritating substance
in the air (not necessarily an allergen) such as chemical fumes,
excessive air pollution, insecticides, chalk dust, tobacco smoke
and other environmental pollutants.  Many people suffer from asthma
caused by substances to which they are exposed on the job.
Sometimes asthma becomes worse as a result of a respiratory
infection.  Asthma may also be provoked by strenuous exercise or
exposure to cold air.

Scientific opinion is divided as to whether emotional factors play
an important part in causing asthma in either children or adults.
However, one thing is certain:  the emotional complications
resulting from the frustration and fear of being an asthmatic
should be taken as seriously as the disease itself.

Prevention

If a particular allergen has been pinpointed, every effort should
be made to avoid contact with it.  For instance, if the allergen is
house dust, the asthma victim's room should be kept as free as
possible of dust by frequent cleaning.  Objects that collect dust,
such as heavy drapes, thick rugs, wool blankets and knickknacks
should be removed.  Someone other than the asthmatic should do the
cleaning, and the asthmatic should stay out of the room until at
least an hour after the cleaning has been completed.  If the asthma
has been found to be caused by animal dander from a pet, the animal
may have to be given away, or at least kept out of the asthmatic's
bedroom.

Often, however, especially if the allergen is present year-round
(as is true of house dust and pet dander), it is very difficult to
keep from coming into contact with it.  In this case, the asthmatic
may sometimes be helped by a process called immunotherapy.  Over a
period of several weeks, the asthmatic is given a series of
injections containing increasing amounts of the allergen so that
tolerance is built up to it.  These injections are usually given by
an allergist.

Another form of preventive therapy is the use of a medication that
will suppress an asthma attack before it begins.  This type of
treatment is particularly effective for exercise-induced asthma.

Treatment

The most widely used preparations for treating asthmatic attacks
are bronchodilators.  Bronchodilators relax the muscles that
tighten around the bronchioles during an asthmatic episode,
reducing swelling of the mucous membranes and loosening the plugs
of mucus that have collected in the bronchioles.

Bronchodilators are available in oral, injectable and aerosol form.
The oral preparations take somewhat longer to work than the aerosol
forms, but they also last longer.  An adaptor is available for
people who have trouble timing actuation of the aerosol spray with
inspiration.  Inhalant bronchodilators should be used only as
directed in order to avoid side effects.  Consult your doctor
promptly if the inhaler seems to be becoming less effective.

If bronchodilators do not succeed in stopping asthmatic attacks,
oral or inhalant corticosteroids may be prescribed.  Steroids must
be used under close medical supervision because they potentially
can have more serious side effects than other antiasthmatic
preparations.  Some asthmatics are "steroid dependent" and require
long-term steroid therapy.

There are also some other methods that can prevent, ease or shorten
asthmatic attacks.  One of these is getting into a position where
the mucus can drain from the lungs - for example, by lying on your
back on a bed with your head hanging over the side.  Other
techniques include special breathing exercises and biofeedback
methods.

In general, try to stay healthy and particularly avoid getting a
cold or the flu.  Drink plenty of fluids (six to eight glasses of
water daily) to loosen and break up mucus in the bronchial
passages.  If you smoke, it is extremely important to stop.

Complications

Untreated asthma can lead to serious complications.  The continued
stretching of the alveoli by accumulations of stale air can
eventually damage the delicate tissues to the point where they lose
their elasticity and cease to function.  Self-treatment is
inadvisable and especially dangerous for older people, who may
attribute their chronic shortness of breath to asthma, when in fact
they may be suffering from progressive heart failure.  Anyone who
experiences breathing difficulties should consult a doctor.

Summing up

Asthma, if left untreated, can be a disabling disease.  Every
effort should be made to identify the factors responsible for the
attacks, because prevention is the foundation of effective
treatment.

A variety of therapeutic approaches may be tried to control asthma;
effective therapy usually involves a combination of treatments that
may include drugs, avoidance of allergens, and immunotherapy.
Although many people believe that asthma is an emotional disorder,
this does not seem to be the case; however, asthma - like any
chronic disease - can produce emotional problems that involve not
only the victim but other family members as well.  Emotional
support is an important element is the overall treatment of asthma.

!
*
                                    National Institutes of Health

                                          EMBARGOED FOR RELEASE
                                          6:00 p.m. EDT
                                          Thursday, June 22, 1995

                                          Patricia Newman
                                          NCI Press Office
                                          [phone]



Questions and Answers:  Ataxia Telangiectasia


1.    What is ataxia telangiectasia?

      Ataxia telangiectasia (A-T) is a rare, recessive genetic disorder of
      childhood that occurs in about 1/40,000 and 1/100,000 persons worldwide.
      The ailment is progressive.  By their teens, patients with A-T are
      frequently wheelchair-bound.

      The disease is characterized by neurologic problems, particularly
      abnormalities of balance, recurrent serious sinus and respiratory
      infections, and dilated blood vessels in the eyes and on the surface of
      the skin.  Patients usually have immune system abnormalities and are
      very sensitive to the effects of radiation treatments.

      In the United States, where recurrent infections typical of the disorder
      are usually controlled by antibiotics, patients are at high risk of
      developing and dying of cancer, particularly leukemias and lymphomas.

2.    Is the disorder curable?

      There is no cure for A-T at the present time.  With the cloning and
      eventual sequencing of the gene (named ATM), several avenues of research
      to develop better treatment become possible, including:  (1) gene
      therapy;  (2) rational drug design;  and (3) direct replacement of the
      functional protein.

3.    What does it mean that the disorder is recessive?

      A disease is recessive when a person inherits the predisposing gene from
      both parents, each of whom transmits one copy of the abnormal gene to
      their child.


4.    What is a carrier?

      A carrier is a person with one normal and one altered copy of a gene
      that is linked to a particular disease.  These individuals usually do
      not realize they are carriers, because the disease is not present, or
      its signs and symptoms are very mild.

5.    How many A-T carriers are there in the United States?

      An estimated one percent, or 2 1/2 million, of the U.S. population may
      be carriers for A-T.

6.    How do I know if I am a carrier?

      In the past, carriers have been identified chiefly because they are
      parents of a child with A-T.  In a small number of cases, carriers have
      been identified through extensive laboratory and clinical studies of
      families where A-T has occurred.  With the cloning of the ATM gene,
      however, scientists will be able to devise a test to detect A-T carri-
      ers.  To do this, they take advantage of what they know about alter-
      ations to the gene in a particular family.  Because different carriers
      will have different mutations in the gene, devising a diagnostic gene
      test may be difficult.

7.    What cancers are increased in A-T patients?

      At least 10 percent of A-T patients develop cancer.  Most of these are
      cancers of the lymphoid tissues (leukemias and lymphomas), but one-fifth
      of the cancers occur in the stomach, brain, ovary, skin, liver, larynx,
      parotid gland, and breast.

8.    What cancers are increased in A-T carriers?

      Definitive information does not yet exist to answer this question, and
      must await the development of a test to identify ATM gene carriers.
      Some studies of the families of A-T patients have reported a 3-4 fold
      increased risk of cancer, including breast cancer; this finding is
      controversial.  Current estimates of risk are based on studies of
      selected families of A-T patients.  The cloning of the ATM gene now
      allows scientists worldwide to evaluate suggestions of increased risk
      for cancer, and NCI is
      collaborating on several studies that combine epidemiology with molecu-
      lar analysis to address this question.





9.    Are A-T patients sensitive to radiation?

      A-T patients have an increased sensitivity to ionizing radiation, the
      type found in
      X-rays.  When cultured in the laboratory, the blood and skin cells of
      these patients have markedly reduced ability to replicate and form cell
      colonies after X-ray
      exposure.  Scientists believe that the ATM gene is one of at least three
      genes,
      (ATM, p53 and GADD45) involved in this cell cycle arrest.

10.   Are ATM gene carriers also sensitive to the effects of radiation?

      This is a question needing further study, and the cloning of the ATM
      gene will make new studies of carriers possible.  When cultured in the
      laboratory, the blood and skin cells of known ATM gene carriers are
      intermediate in their sensitivity to X-rays, compared to A-T patients
      and the general public.

11.   Does their increased sensitivity to radiation, compared to the general
      public, make it dangerous for carriers to have diagnostic X-rays?

      There is no evidence to support this.  At the dosages of radiation
      delivered by today's technology, diagnostic tests are not considered
      harmful to carriers.

12.   Are X-rays harmful to A-T patients?

      A-T patients are sensitive to the effects of radiation and should be
      monitored for adverse effects.  However, they do receive diagnostic X-
      rays when necessary.

      The information about radiation sensitivity in A-T comes largely from
      observations of A-T patients who undergo radiation treatments, especial-
      ly for cancer, and also from laboratory studies on skin and blood cells.
      Physicians who treat A-T patients limit
      X-ray exposures by using the most modern equipment and techniques
      available, but they do recommend these diagnostic tests when they are
      needed.

      Now that the ATM gene has been isolated, scientists will be able to
      study the nature of the radiation sensitivity observed in cells from A-T
      patients, as well as any
      intermediate level of radiation sensitivity observed in cells from known
      carriers.

13.   Suppose I learn I am an A-T carrier.  Will mammography increase my
      chances of getting breast cancer?

      There is no definitive evidence that A-T carriers of any age have
      increased sensitivity to X-rays from mammograms or other diagnostic
      tests.  For women 50 and older,  th benefits of mammography have been
      demonstrated clearly.
      For younger women, those 40-49, NCI continues to recommend discussing
      individual risk issues with your physician.  The influence, if any, of
      X-rays on the breast cells of premenopausal women is theoretical and
      will be studied further, now that the ATM gene is cloned.

14.   Are there any alternatives to mammography on the horizon?

      Recent technological modifications to mammography result in delivery of
      lower radiation doses than 20 years ago.  In addition, NCI is sponsoring
      the development and clinical testing of non-ionizing breast imaging
      technologies, such as magnetic resonance imaging, ultrasound and PET.

15.   What about environmental sources of radiation, such as cellular phones.
      Should A-T carriers avoid these exposures?

      To date, there have been no definitive studies linking non-ionizing
      radiation, such as that from cellular phones, to cancer.  NCI currently
      is evaluating non-ionizing radiation from a variety of sources to assess
      thoroughly any potential cancer risks.

16.   What studies does NCI have under way or planned to answer these ques-
      tions more definitively?

      NCI is sponsoring a wide variety of research on DNA repair, radiation
      sensitivity and resistance, and A-T.  Clinical scientists at the NCI
      have over 30 years of experience with A-T, and are continuing research
      to develop better diagnostic tests, immune dysfunction analyses, and
      treatments.

      In addition, NCI epidemiologists are collaborating in several studies of
      A-T, including population-based studies of the risk of cancer in A-T
      patients and their relatives in Denmark that are being expanded to other
      Scandinavian countries; a study of
      early-onset breast cancer among X-ray technologists, whose blood samples
      are being examined for mutations in the BRCA1 and ATM genes; and ongoing
      studies for over 20 years of radiation-induced breast cancer.  These
      latter studies include analysis of scoliosis patients who as young girls
      received intense spinal X-rays to monitor the growth spurt; women with
      tuberculosis who received hundreds of chest fluoroscopic X-rays to
      monitor lung collapse; survivors of the atomic bombings in Hiroshima and
      Nagasaki; women with breast cancer who developed secondary breast cancer
      in the contralateral breast; women with Hodgkin's disease treated with
      radiotherapy, and others.  In a number of these studies, blood and tumor
      samples are being evaluated for the  influence of genes such as p53 and
      BRCA1 on radiation-induced breast cancer risk, and ATM gene studies will
      be possible in the near future.

                                   #   #   #

!
*

Athlete's Foot


THIS IS A HEALTH-LINE MESSAGE FROM THE UNIVERSITY OF WISCONSIN-MADISON.
     Athlete's foot is a very common skin condition caused by a fungus which
must have at least a 95% humidity environment in order to multiply.  Signs that
you may have this infection could include cracking, scaling, peeling, itching,
redness and blisters on the soles of your feet or between your toes.  Your
toenails may have become yellowish and thickened if you have had the condition
for a long time.
     If you suspect that you have athlete's foot try the following method of
control.  First, wash your feet carefully in soap and water, rinse well and dry
all areas thoroughly.  Powder your feet heavily, especially between your toes,
and put on clean socks.  While indoors, go around in your stocking feet or bare
feet when at all possible.  This will reduce moisture from perspiration.  You
may wish to purchase a special powder to use.  You will find your pharmacist
helpful in guiding you in your choice.
     Many people with mild athlete's foot find they can treat themselves very
effectively.  It may take a while for all signs of the infection to disappear
completely but it will quickly improve if you keep the areas dry.
     If your condition should become more severe, more uncomfortable, look very
red and angry, and last more than two weeks after beginning treatment, you may
have another type of skin problem.  In this case, you should consult a
physician.
     Other skin ailments can mimic athlete's foot.  You may be allergic to the
synthetic material of your socks and a change to the 100% cotton variety plus
general good foot hygiene may bring relief.  Frequently, people are allergic to
the strong laundry detergents in which socks are washed.  Giving your socks an
extra rinse after laundering may help your condition.  In general, we can say
that any condition present on the tops or your feet or between the great toe
and first toe is NOT likely athlete's foot.  If an inflammation seems to be
related to an open blister or other wound and does not improve after two or
three days of hot water soaks, see your physician.
     You can help prevent, as well as cure, athlete's foot if you keep all
areas of your feet dry at all times.  If you work outside and wear heavy boots
and socks, at lunchtime you may be able to change to clean socks into which you
have previously placed a goodly amount of foot powder.  After putting on the
clean, dry socks, spread your toes and puff the powder around.  Wearing
overshoes all day or wearing tennis shoes without socks will help your feet
perspire and become a perfect environment in which the athlete's foot fungus
can flourish.
     Athlete's foot cannot be just "wished" away.  Face up to the situation and
remember that control and cure as much easier when the problem has just begun.

!
*
FOOD AND WATER BORNE BACTERIAL DISEASES


CENTERS FOR DISEASE CONTROL AND PREVENTION
Date Last Rev'd: March 9, 1995

FOOD AND WATER BORNE BACTERIAL DISEASES


FOODBORNE BACTERIAL DISEASES - GENERAL INFORMATION
More than 250 different diseases have been described that can be caused by
contaminated food or drink. The most common foodborne diseases are infections
caused by bacteria, such as Salmonella and Campylobacter, or by the Norwalk
family of viruses.  A foodborne disease outbreak is defined as a group of
people developing the same illnesses after ingesting the same food. Most
cases of foodborne disease are single cases not associated with a recognized
outbreak.

The great majority of food items which cause foodborne diseases are raw or
undercooked foods of animal origin such as meat, milk, eggs, cheese, fish, or
shellfish.

In 1983, it was estimated that there were approximately 6 million cases of
infectious foodborne diseases which caused 9,000 deaths.  Some foodborne
diseases such as botulism and trichinosis are becoming less common, while
others such as salmonellosis are becoming more common.  Thus, the spectrum of
foodborne disease is changing. New infections not previously known to be
foodborne diseases are emerging.

Approximately 400-500 foodborne disease outbreaks are reported each year.
Not all outbreaks or diseases are equally likely to be reported, and many
cases of foodborne diseases are sporadic.

To prevent contracting foodborne diseases, the consumer can do the following:

    1)         Make sure that food from animal sources (meat, diary, eggs) is
    thoroughly cooked or pasteurized.  Avoid eating such foods raw or
    undercooked.
    2)         Be careful to keep juices or drippings from raw meat, poultry,
    shellfish or eggs,  from contaminating other foods.
    3)         Do not leave potentially contaminated foods for extended
    periods of time at temperatures that permit bacteria to grow.  Promptly
    refrigerate leftovers and food prepared in advance.

Thorough cooking kills almost all foodborne bacteria, viruses and parasites,
and is the single most important step in preventing foodborne disease.
Preventing spread of contamination from raw foods in the kitchen is also
important. Washing one's hands, cutting board, and knife with soap and water
immediately after handling raw meat, raw poultry, raw seafood or raw eggs
will help keep the foodhandler from contaminating any other foods in the
kitchen.  Persons who are ill with diarrhea or vomiting should not prepare
food for others.  Special care is needed in the preparation of food for
infants, the elderly, and persons whose immune systems are compromised by
underlying illness or medical treatment of illness.

While foodborne diseases, their causes and effects are better understood
today, emerging risks need to be monitored for several reasons.

 First, the food supply of the United States is changing dramatically,  The
 conditions under which food animals are raised have changed greatly.  We
 now import 30 billion tons of food a year, including fruit, vegetables,
 seafoods, and canned goods;  these imported foods are an increasing
 proportion of the diet, and often come from developing countries where food
 hygiene and basic sanitation is less advanced.  Food processing
 technologies are constantly evolving.  The centralization of the food
 industry means that a single contaminated product may appear in many
 different foods and many different forms, and infect a considerable number
 of people before it is identified.

 Second, consumers are changing; there are increasing numbers of elderly or
 immunosuppressed persons who are at higher risk of severe illness;
 consumers spend less time cooking than before, and may have received less
 instruction in food handling in home or school than before.

 Finally, new and emerging foodborne pathogens have been identified, which
 can cause diseases unrecognized 50 years ago. These include bacteria,
 parasites, and viruses, along with toxic causes of foodborne illnesses.
 Constant vigilance is necessary to identify new problems requiring new
 solutions as they emerge.

However, despite these new risks, the food supply of the United States is
probably safer now than ever.

ADDITIONAL SOURCES OF INFORMATION
More detailed information on foodborne disease can be found in encyclopedias,
in medical and public health textbooks, and in textbooks on food
microbiology.  CDC Surveillance summaries on foodborne diseases and their
outbreaks are published as supplements to the Morbidity and Mortality Weekly
Report, the MMWR, and are available at many public libraries, and all medical
libraries. These Surveillance summaries include information on salmonellosis,
campylobacteriosis, trichinosis, and viral hepatitis, as well as on foodborne
disease outbreaks. They can also be obtained by writing this office:  Public
Inquires, Office of Public Affairs, Centers for Disease Control, 1600 Clifton
Road, Atlanta, Georgia, 30333.

Questions about the safe handling of food can be addressed to your County
Health Department or County Extension Home Economist.

Questions about the safety of a specific food can be answered by the FDA
Consumer Hotline:  [phone].

Questions specifically about meat and poultry can be answered by the USDA
Meat and Poultry Hotline:  [phone].
!
*
                  DEAR DOCTOR COLUMNS ARE WRITTEN BY UAB
               UNIVERSITY OF ALABAMA AT BIRMINGHAM) EXPERTS
                AND PUBLISHED WEEKLY IN THE BIRMINGHAM NEWS

BOILS
Source:  Ed Varner, M.D.
Writer:  Susan O. Brown


QUESTION:

I have recurrent boils on my buttocks and the back of my thighs.
Although they eventually go away, they are often painful,
becoming red, tender, and filled with pus.  Sometimes I try to
squeeze them to relieve the pressure, but it usually makes them
worse.  What causes boils and what should I do when I get one?

ANSWER:

   Boils, also called furuncles, are an infection of a hair
follicle (a tiny pit in the skin from which a hair grows) by
certain bacteria, usually Staphylococcus aureus, commonly found
on the skin.  When there are multiple boils anywhere on the body,
the condition is called furunculosis.  In contrast, a carbuncle
is either an unusually large, severe boil or a group of boils
joined together by small tunnels in the skin.
    Boils are very common, and virtually everybody is affected
with one at some time.  Staph bacteria can enter the body through
cuts, sores, puncture wounds, skin ulcers, or irritated skin,
causing infection.  They also can be the result of poor
resistance to infection or poor hygiene.  Although they can occur
anywhere on your skin, they are most likely to appear on your
face, neck, armpits, buttocks, or thighs.
   As a hair follicle becomes inflamed and painful, white blood
cells, which form part of the body's defense system against
bacteria, collect at the site to combat the infection.  Starting
as a red, tender lump, which may throb, a boil becomes larger and
painful over the course of a few days.  As pus collects, it
develops a white or yellow head, or center.  The pus is under
pressure, which increases the pain and tenderness.  Occasionally
fever develops.  Eventually, the boil bursts through the skin,
the pain is relieved, and the boil heals.  This process usually
takes about two weeks.  However, the boil may recur later near
the same site and go through the same cycle.
   When boils occur on the upper lip or near the nose, infections
can spread to the sinus cavity.  Rarely, the bacteria can enter
the bloodstream and cause more serious problems.  Physicians warn
that boils should not be touched or squeezed.  Squeezing can
force the infection into deeper tissues.  Permanent scarring can
occur from a boil, especially if it is improperly treated.
   Boils are sometimes signs of a system-wide problem.  For
example, people who have diabetes or kidney disease are more
susceptible to boils and other infections than people without
these conditions.  Whatever the cause, large boils need to be
evaluated and treated by your physician.
   Moist heat helps boils come to a head so they'll drain.
Application of a warm, tap-water compress to the boil every 30
minutes for a few hours will help it burst and drain much sooner.
Occasionally, a physician may have to lance the boil to allow the
pus to drain away.  Depending on the severity of the boil, an
antibiotic pill or injection, or an antibiotic ointment may be
prescribed to kill the bacteria.
   Skin cleansers that kill germs can be used to keep the area
scrupulously clean during healing.  Washcloths and towels should
be laundered after each use.  Underwear and bedclothes should be
laundered after contact with the lesion.  If dressings are
changed at home, discard them in a plastic bag and seal it to
prevent spread of infection.  If you have boils, it's best to
take showers instead of baths to reduce the chance of spreading
the infection to other parts of your body and to others.
   If boils are a recurring problem, physicians recommend a
complete medical evaluation to determine if a system-wide problem
is the cause.
!
*
NIH Healthline--October/November 1994
National Institutes of Health, Office of Communications
[phone], [phone]
Compuserve 74201,1057


PREMATURE GRAYING MAY BE A SIGN OF BONE DISEASE--by
Judy Folkenberg

     Premature graying may be a marker that an individual is at
risk for osteoporosis, according to research supported by NIH's
National Center for Research Resources.
     In this study, researchers led by Clifford J. Rosen, M.D.,
at the Maine Center for Osteoporosis Research and Education,
picked 63 individuals who met the study's criteria out of a
sample of nearly 1,000 patients from a bone clinic in Bangor. The
clinic serves as a referral center for about 300,000 people in
eastern Maine.
     Osteopenia--the term used to describe thinning bones--was
measured by x-rays which scanned the spine.  The scientists found
those persons with premature graying were over 4 times more
likely to have varying degrees of osteopenia when compared to the
control group--those individuals without premature gray hair.
     "Premature graying" meant that an individual's hair had
turned more than 50 percent gray before the age of 40.
     The earlier one's hair turned gray, say the researchers, the
greater the chance of a history of osteoporosis in the family.
For example, individuals with premature graying in their teens
and twenties had a stronger family history of the disorder than
those who had premature graying in their thirties.
     The scientists aren't sure why premature graying and
increased risk for osteoporosis are linked.
     "But we think that perhaps the gene that controls premature
graying is next to the gene that regulates bone density (genes in
close proximity sometimes act together); or perhaps one gene
influences the other," said Rosen.
     This research was published in the Journal of Clinical
Endocrinology and Metabolism (Vol. 79, No. 3, 1994).

                                --An NIH Healthline report
                                  October/November 1994
Contact:  Dr. Clifford Rosen
          [phone]
           Judy Folkenberg
          [phone]
!
*
BOTULISM

CENTERS FOR DISEASE CONTROL AND PREVENTION
Date Last Rev'd: March 9, 1995

FOOD AND WATER BORNE BACTERIAL DISEASES


BOTULISM

Botulism is a rare but serious foodborne disease.  It is caused by
contamination of certain foods by the botulism bacterium commonly found in
the soil.  There are two different illnesses: adult botulism and infant
botulism.  An adult may become ill by eating spoiled food containing the
botulism toxin.  This toxin is produced when the bacteria grow in improperly
canned foods and occasionally in contaminated fish.  Infant botulism is
caused by eating the spores of the botulinum bacterium. For infants one
source of these spores is honey.

When contaminated food is eaten by adults, toxin is absorbed from the
intestines and attaches to the nerves causing the signs and symptoms of
botulism.  Early symptoms include blurred vision, dry mouth, difficulty in
swallowing or speaking, general weakness, and shortness of breath.  The
illness may progress to complete paralysis, respiratory failure, and death.
When infants eat contaminated food, the spores grow in the intestines and
release toxin.

Diagnosis is made by the presence of appropriate neurologic symptoms and by
laboratory tests that detect toxin or by culture of Clostridium botulinum
bacterium from the patient's stool.

Although there are very few cases of botulism poisoning each year, prevention
is extremely important.  Home canning should follow strict hygienic
recommendations to reduce contamination of foods.  In addition, because the
botulism toxin is destroyed by boiling for 10 minutes, people who eat home-
canned foods should consider boiling the food before eating it to ensure
safety. A county extension home economist can provide specific instructions
on safe home canning techniques. To help prevent infant botulism, infants
less than 12 months old should not be fed honey.

Treatment for adults requires care in an intensive care unit; botulism
antitoxin can be helpful if given soon after symptoms begin.  Treatment for
infants requires hospitalization and possibly care in an intensive care unit.
Antitoxin is not recommended for infants.

In 1989, 23 adult and 75 infant botulism cases were reported to CDC from 23
states.
!
*
NEW YORK STATE DEPARTMENT OF HEALTH

Botulism                     (food-borne botulism and infant botulism)

What is botulism?

Botulism is a food poisoning caused by a toxin produced by the bacteria,
Clostridium botulinum.  Only a few cases are reported in New York State
each year.

Who gets botulism?

Food-borne botulism is due to ingestion of a toxin formed in food. It often
involves improperly processed home canned foods. Botulism in infants under
one year of age has been associated with the ingestion of contaminated honey.

How is botulism spread?

Person to person spread does not occur. A person must ingest contaminated
food that has not been properly cooked or reheated after the toxin has been
produced by the bacteria. With infant botulism, an infant must ingest
bacterial spores and then produce the toxin in his/her gastrointestinal tract.

What are the symptoms of botulism?

Food-borne and infant botulism produce symptoms that affect the nervous
system.  The symptoms of food-borne botulism include blurred or double vision,
general weakness, poor reflexes, difficulty swallowing and sometimes death.
Infant botulism has a wide range of symptoms including difficulty breathing,
visual disturbances, poor feeding and poor reflexes.

How soon do symptoms appear?

Symptoms of food-borne botulism usually appear 12-36 hours after ingestion,
but may take several days. The incubation period for infant botulism is
unknown.

What is the treatment for botulism?

Hospital care is necessary. Antitoxin is given in certain cases of food-borne
botulism, but not in cases of infant botulism.

What happens if botulism is not treated?

Untreated botulism may result in death.

How can botulism be prevented?

Identified sources of infant botulism, such as honey, should not be fed to
infants.  All canned and preserved foods should be properly processed and
prepared.  Bulging containers should not be opened and foods with off-odors
should not be eaten or even tasted. Commercial cans with bulging lids should
be returned unopened to the vendor.
!
*
     DEAR DOCTOR COLUMNS ARE WRITTEN BY UAB (UNIVERSITY OF ALABAMA AT
      BIRMINGHAM) EXPERTS AND APPEAR WEEKLY IN THE "BIRMINGHAM NEWS."


QUESTION:

     Lately, I've been waking myself up during the night grinding
     my teeth. Sometimes I grind so hard my jaw is sore the next
     morning. Why do I do this and what can I do to prevent
     damaging my teeth?

ANSWER:
     What you've been experiencing is not uncommon. In fact, an
estimated 95 percent of the U.S. population, including children,
will clinch and grind their teeth, called bruxism, at some point
in their life. But because it occurs during sleep, only a
fraction of people are aware they have this annoying and
potentially harmful problem.
     Because bruxism often results in excessive wear or damage to
your teeth, particularly the eye teeth, your dentist is often the
first to suspect a problem. The first thing that may alert that
you're busy grinding during the night is a stiff jaw or sore
chewing muscles in the morning. Other symptoms and signs that
point to bruxism include achy or painful facial muscles or jaw
(temporomandibular) joints, cracked teeth, and destruction of
dental work.  In severe cases, people can even wake up with their
jaw locked either open or closed.
     Once thought to be caused by problems in occlusion (the way
the upper and lower teeth fit together when the jaws are closed),
recent studies have pinpointed stress as the culprit. The side
effects of certain medications and being physically exhausted
also can cause or aggravate bruxism.
     Researchers, using sensors to detect tooth grinding during
sleep, found that grinding increases during stressful periods of
people's lives such as during school exams or divorces. However,
when antianxiety medications were given, the grinding decreased.
     But medications are only a short-term solution. The long-
term solution to bruxism is learning how to handle the stress in
your life. Stress management programs used to treat bruxism
include biofeedback, counseling, hypnosis, and progressive
relaxation. In addition, your dentist may recommend bite splints
or nightguards, which fit between the biting surfaces of the
teeth to protect them from injury and to keep the jaw relaxed. If
left untreated, bruxism may contribute to the premature loss of
teeth and inflamed gums.  Since you're aware of your problem, see
your dentist right away. oblem, see your dentist right away.
!
*
First Aid For Burns


THIS IS A HEALTH-LINE MESSAGE FROM THE UNIVERSITY OF WISCONSIN-MADISON.
     The proper treatment for burns is based upon a knowledge of the depth, the
locations, the cause, and the extent of the burn.  Burns vary in size and
severity from local redness to extensive destruction of the skin and underlying
tissues.  The first degree burn is like a mild sunburn which produces redness,
warmth, swelling, and pain.  The pain of a small, first degree burn may be
relieved by quickly immersing the area in cold water or by applying a cold
water or ice poultice.  A simple, non-greasy, water soluble burn ointment,
available at most drug stores, may be placed over the washed skin.  Do not use
vaseline, grease, or other greasy products on burns as these can be very
difficult and painful to remove should the burn become infected or be deeper
than first degree.  After a first degree burn, the skin may peel and itch as it
heals.
     A second degree burn is one that blisters, a sign that the injury has
killed some of the surface skin cells.  The second degree burn usually heals by
itself and leaves little scarring.  Small second degree burns should be gently
cleaned with mild soap and running , moderately warm water and covered with a
simple water soluble burn ointment plus a soft sterile dressing which is left
in place for four to five days.  If possible, the blisters should not be
broken.  If the burn is likely to get dirty, some doctors like to give a
tetanus booster shot.  If you have a burn with blistering and haven't had a
recent tetanus shot, you should call your doctor for his opinion.
     A third degree burn kills the full thickness of the skin and therefore
heals slowly and results in a scar.  Very small third degree burns are treated
with an antibiotic ointment and a sterile dressing.  Healing takes a number of
months and the dressing therefore needs to be changed.  Again, some doctors
give a tetanus booster shot.  All third degree burns are best treated by a
health professional rather than at home.
     Sunburn is a common injury which everyone knows something about.  Sunburns
over large areas of the body require medical attention as do burns caused by
sunlamps.  If you are using a sunlamp, cover your eyes completely and use an
automatic timing device attached to the lamp so it will turn off in case you
fall asleep.  Sunburns of the eyes often happen when you are skiing and can be
avoided by wearing special goggles.
     Burns form caustic agents such as lye or acids should be washed with high
quantities of moderately warm water and examined by a physician.  Electrical
burns are serious since they frequently cause wider injury than is first
believed.
     The location of a burn is an important consideration.  A small burn of the
eyelid is much worse than a similar one on the arm.  The larger the burn, the
more serious the injury.  Indeed, a large burn represents the most serious
injury of man.  Large burns, whether first, second or third degree, should be
treated by a physician.  In addition to infections and the need for special
treatments, serious burns can cause shock.  Any patient with a serious burn
should be brought to a doctor's office, clinic, or emergency room as soon as
possible after the occurrence of the injury so that shock can be avoided or
treated.
     The best treatment for a burn is prevention.  Every open flame is a
danger.  Flame retardant fabrics can prevent the tragic injuries which occur
when clothing catches fire.  Scalding burns are also common and all homemakers
should habitually use back burners and turn pot handles so that a child CANNOT
reach them.  Finally, anyone working around flammable agents must obey simple
rules of safety if serious and fatal accidents are to be avoided.  Remember,
fat or grease fires must be extinguished with foam, not water.  In the kitchen,
the grease fires can be suffocated with damp towels or a pan lid.
     If you are concerned about the treatment of a burn, the safest course of
action is to consult your physician or call a local emergency health service.
Thank you.

!
*
NEW YORK STATE DEPARTMENT OF HEALTH

Campylobacteriosis

What is campylobacteriosis?

Campylobacteriosis is a bacterial infection that affects the intestinal tract
and, rarely, the bloodstream.  It is probably the most common cause of
bacterial diarrhea in New York State.  Most cases are seen in the summer
months
and occur assingle cases or outbreaks.

Is this a new disease?

No.  Campylobacteriosis has probably been in existence for many years but has
only recently been recognized as a common infection due to improved laboratory
methods.

Who gets campylobacteriosis?

Anyone can get campylobacter infection.

How is the germ spread?

Campylobacter are generally spread by eating or drinking contaminated food
or water and, occasionally, by contact with infected people or animals.

What are the symptoms of campylobacteriosis?

Campylobacteriosis may cause mild or severe diarrhea, often with fever and
traces of blood in the stool.

How soon after exposure do symptoms appear?

The symptoms generally appear two to five days after the exposure.

Where are the campylobacter germs found?

Many animals including swine, cattle dogs and birds (particularly poultry)
carry the germ in their intestines.  These sources in turn may contaminate
meat products (particularly poultry), water supplies, milk and other items
in the food chain.

For how long can a person carry the campylobacter germ?

Generally, infected people will continue to pass the germ in their feces for
a few days to a week or more.  Certain antibiotics may shorten the carrier
phase.

Do infected people need to be isolated or excluded from school or work?

Since the organism is passed in the feces, only people with active diarrhea
who are unable to control their bowel habits (infants, young children,
certain handicapped individuals, for example) should be isolated.  Most
infected people may return to work or school when their stools become formed
provided that they carefully wash their hands after toilet visits.  Food
handlers, children in day care and health care workers must obtain the
approval of the local or state health department before returning to their
routine activities.

What is the treatment for campylobacteriosis?

Most people infected with campylobacter will recover on their own or require
fluids to prevent dehydration.  Antibiotics are occasionally used to treat
severe cases or to shorten the carrier phase, which may be important for food
handlers, children in day care and health care workers.  Since relapses
occasionally occur, some physicians might treat mild cases with antibiotics
to prevent a recurrence of symptoms.

How can campylobacteriosis be prevented?

1.  Always treat raw poultry, beef and pork as if they are contaminated and
handle accordingly:
        - Wrap fresh meats in plastic bags at the market to prevent blood
        from dripping on other foods.
        - Refrigerate foods promptly; minimize holding at room temperature.
        - Cutting boards and counters used for preparation should be washed
        immediately after use to prevent cross contamination with other foods.
        - Avoid eating raw or undercooked meats.
        - Ensure that the correct internal cooking temperature is
        reached-particularly when using a microwave.
2.  Avoid eating raw eggs or undercooking foods containing raw eggs.
3.  Avoid using raw milk.
4.  Encourage careful handwashing before and after food preparation.
5.  Make sure children, particularly those who handle pets, wash their hands
carefully.
!
*
CARPAL TUNNEL SYNDROME

CENTERS FOR DISEASE CONTROL AND PREVENTION
Date Last Rev'd: March 9, 1995

CARPAL TUNNEL SYNDROME

In recent years, reports of repetitive motion injuries have risen
dramatically in workplaces across the country. These problems, frequently
termed "Cumulative Trauma Disorders" are being reported at alarming rates in
all types of workplaces - from meatpacking plants to newspaper pressrooms. Of
all these disorders, carpal tunnel syndrome is the condition most frequently
reported.

What is Carpal Tunnel Syndrome (CTS)?
The carpal tunnel receives its name from the 8 bones in the wrist, called
carpals, that form a tunnel-like structure.  The tunnel is filled with flexor
tendons which control finger movement.  It also provides a pathway for the
median nerve to reach sensory cells in the hand.  Repetitive flexing and
extension of the wrist may cause a thickening of the protective sheaths which
surround each of the tendons.  The swollen tendon sheaths, or tenosynovitis,
apply increased pressure on the median nerve and produce Carpal Tunnel
Syndrome (CTS).

What are the Symptoms of CTS?
The symptoms of CTS often first appear as painful tingling in one or both
hands during the night, frequently painful enough to disturb sleep.
Accompanying this is a feeling of uselessness in the fingers, which are
sometimes described as feeling swollen, even though little or no swelling is
apparent.  As symptoms increase, tingling may develop during the day,
commonly in the thumb, index, and ring fingers.  A decreased ability and
power to squeeze things may follow.  In advanced cases, the thenar muscle at
the base of the thumb atrophies, and strength is lost.

Many patients with CTS are unable to differentiate hot from cold by touch,
and experience an apparent loss of strength in their fingers.  They appear
clumsy in that they have trouble performing simple tasks such as tying their
shoes or picking up small objects.

What Causes CTS?
As stated earlier, swelling of the tendons that line the carpal tunnel causes
CTS.  Although there are many reasons for developing this swelling of the
tendon, it can result from repetitive and forceful movements of the wrist
during work and leisure activities.  NIOSH research indicates that job tasks
involving highly repetitive manual acts, or necessitating wrist bending or
other stressful wrist postures, are connected with incidents of CTS or
related problems.  Moreover, it is apparent that this hazard is not confined
to a single industry or job but occurs in many occupations--especially those
in the manufacturing sector.  Indeed, jobs involving cutting, small parts
assembly, finishing, sewing, and cleaning seem predominantly associated with
the syndrome.  The factor common in these jobs is the repetitive use of small
hand tools.

How Large of a Problem is CTS?
In the past ten years, more and more cases of workers afflicted with CTS have
been reported in medical literature.  One reason for this increase may be
that automation and job specialization have fragmented workers' tasks to the
point where a given job may involve only a few manipulations performed
thousands of times per workday.  Increased public awareness of work related
risk factors has led to an increased number of requests for NIOSH health
hazard evaluations (HHEs) to investigate suspected problems.  The increased
awareness of work tasks as a factor in the onset of CTS is reflected in the
growing number of requests for health hazard evaluations (HHEs) received by
NIOSH to investigate such suspected problems.  NIOSH received about three
times as many HHE requests related to hand and wrist pain in 1992 as compared
to 1982.
While no nationwide statistics on incidence and cost are available, the
Wisconsin Workers' Compensation Division shows the number of compensated CTS
cases in Wisconsin grew from 230 in 1983 to 1,937 in 1988. Additionally, the
Bureau of National Affairs reports that a case of CTS can cost as much as
$30,000.

Prevention
NIOSH recommendations for controlling carpal tunnel syndrome have focused on
ways to relieve awkward wrist positions and forceful arm and hand movements.
NIOSH recommends redesigning tools or tool handles to enable the user's wrist
to maintain a more natural position during work.  Other recommendations have
involved modified layouts of work stations.  Still other approaches include
altering the existing method for performing the job task, providing more
frequent rest breaks, and rotating workers across jobs.  Tool and process
redesign are preferable to administrative means, such as job rotation, as a
means of prevention.

The incidence and severity of CTS can be minimized through training programs
that increase worker awareness of symptoms and prevention methods, and
through proper medical management of injured workers.

Treatment
Treatment of CTS may involve surgery to release the compression on the median
nerve and/or use of anti-inflammatory drugs and hand splinting to reduce
tendon swelling in the carpal tunnel.  Such medical interventions have met
with mixed success especially when an affected person must return to the same
working conditions.

Current NIOSH Research
In 1992, NIOSH continued to investigate cumulative trauma disorders (CTD's),
such as CTS, among workers using keyboards in the news and telecommunications
industries, direct mail order processors, and grocery store workers using
laser scanning devices.  NIOSH is also developing projects to study the
ergonomic design of keyboards to reduce CTS, the ergonomics of powered hand
tools in the automotive industry, and possibilities for ergonomic
intervention in the beverage container delivery industry.

In addition, NIOSH analyzed the 1988 National Health Interview Survey Data to
estimate the prevalence of CTS in the adult population of the U.S.  The
findings show 2.8 million people self-reported CTS in 1988.  White females
between ages 45 to 54 were at the highest risk of self-reported CTS.  A
projected prevalence of "medically-diagnosed" carpal tunnel syndrome was
about 0.7 million among "recent workers" (72% of U.S. adults).

To improve the surveillance of CTS, NIOSH is assisting health departments in
California, Massachusetts, and Wisconsin to develop pilot state-based
strategies using voluntary reporting from health care providers.
!
*
Cataracts

U.S. Department of Health and Human Services
Public Health Service
National Institutes of Health


What is a cataract?

A cataract is a cloudy or opaque area in the lens of the eye.
The lens is located behind the pupil and iris.  It helps focus
light onto the retina, the light-sensitive tissue that lines the
inside of the back of the eye.  Usually, the lens is transparent.
But if it becomes clouded, the passage of light is obstructed and
vision may be impaired.

What causes a cataract?

When a cataract forms, there is a change in the chemical
composition of the lens, but scientists do not know exactly what
causes these chemical changes.  The most common form of cataract
is related to aging, although this type can occur at age 50 or
even earlier.  Cataracts also may be associated with diabetes,
other systemic diseases, drugs, and eye injuries.  Sometimes
babies are born with congenital cataracts or develop them during
the early years of life.

What are the symptoms of a cataract?

Cataracts usually develop gradually, without pain, redness, or
tearing in the eye.  Some cataracts never progress to the point
where they seriously impair vision, whereas others eventually
block most or all vision in the affected eye.  The effect of a
cataract on vision depends on several things: 1) its size, 2) its
density, 3) its location within the lens.

Among the signs that a cataract may be forming are:

     o    Hazy, fuzzy, or blurred vision.  Double vision
          sometimes occurs, but this usually goes away as the
          cataract worsens.

     o    The need for frequent changes in eyeglass
          prescriptions.  When the cataract progresses beyond a
          certain point, these changes no longer improve vision.

     o    A feeling of having a film over the eyes, or of looking
          through veils or a waterfall.  The person with a
          cataract may blink a lot in an effort to see better.

     o    Changes in the color of the pupil, which is usually
          black.  When the eye is examined, the pupil may look
          grey, yellow, or white, but color changes are not
          always noticeable.

     o    Problems with light.  For example, night driving
          becomes harder because the cloudy part of the lens
          scatters the light from oncoming headlights, making
          these lights appear double or dazzling.  Also, the
          person with a cataract may have trouble finding the
          right amount of light for reading or close work.

     o    "Second sight"-a temporary improvement in reading
          vision experienced by some people when their cataract
          reaches a certain stage of development.  As the
          cataract progresses, vision again worsens.

None of these symptoms necessarily means that a person has a
cataract, or if a cataract is present that it must be removed.
However, people who have any of these symptoms should see an eye
doctor.

When should a cataract be removed?

A cataract should be removed surgically when it has progressed to
the point where resulting vision problems interfere with one's
daily activities.  A second reason for cataract surgery, more
urgent but less common than the first, is that the cataract has
become completely opaque (mature).  It is possible for a mature
cataract to swell and even disintegrate inside the eye.  Such
changes can permanently endanger vision.

With congenital cataracts, it used to be standard practice to
postpone surgery until the child was at least 6 months old.
Recently, however, cataracts have been removed from the eyes of
newborn infants with good results.  Early removal of severe
congenital cataracts is an important advance because it reduces
the risk of visual loss resulting from the disuse of one or both
eyes during childhood.

How are cataracts treated?

Treating cataracts really involves two steps.  The first is
removal of the clouded lens by an ophthalmologist.  Surgery is
the only method proven effective for removing cataracts.  The
second is finding an appropriate substitute for the natural lens.
The decision about which substitute lens to use is usually made
before surgery.

There are two general methods of removing a cataract:
intracapsular and extracapsular extraction of the lens.
Intracapsular extraction is sometimes used to remove senile
cataracts.  In this method, the entire lens, including its
capsule, is removed.

Extracapsular extraction involves removal of most lens tissue but
the back part of the lens capsule is left in place.  In infants
and young children, whose lenses are relatively soft, the lens
tissue may be withdrawn through a hollow needle, a procedure
called aspiration.  A variety of extracapsular techniques are
also used to remove the lens in adults.

One technique is called phacoemulsification.  High-frequency
sound vibrations (ultrasound) are used to soften and liquefy the
lens so it can be aspirated through the needle.

Phacoemulsification should not be confused with another form of
eye surgery, photocoagulation, in which laser light-not ultra-
sound-is used to treat some eye disorders other than cataract.  A
laser cannot remove a cloudy lens or make it dear again.
However, some doctors may use a laser to open the front part of
the lens capsule before removing the lens or to help patients who
develop 66 after-cataract." (See "What happens after surgery?")

How safe is cataract surgery?

Cataract surgery is one of the most successful operations done
today-more than 90 percent of the people who have this surgery
find that they can see better.  Complications may occur, but most
are treatable.  Serious complications that threaten vision are
rare.

Certain people may not benefit much from cataract surgery.  They
include people whose cataracts are not advanced enough to impair
vision seriously and those whose vision is impaired by another
eye disease as well.

In summary, each cataract patient should discuss the possibility
of surgery with the doctor who examines his or her eyes to
determine whether the potential benefits of cataract surgery
outweigh the risks.  It is also very important to decide in
advance, with the help of the doctor, what form of substitute
lens would be most suitable.  Patients may want to get a second
opinion on the advisability of surgery and on the most
appropriate substitute lens to use after surgery.

What are the choices for a substitute lens?

There are three options for replacing the natural lens removed,in
cataract surgery: eyeglasses, contact lenses, or an intraocular
lens implant.  Each has advantages and drawbacks.

Eyeglasses.  This is a safe and time-proven solution to the
problem of seeing without a natural lens.  But cataract
eyeglasses can have some unpleasant effects.  Patients may be
bothered by the fact that these glasses magnify objects 20-35
percent, affect depth perception until the person relearns how to
judge distances, and limit side vision.

If only one eye requires cataract surgery, eyeglasses may well
cause problems because the person is unable to fuse the
different-sized images formed by the operated and unoperated
eyes.  Such patients are often advised before surgery that it
would be best to use a contact lens, or have a lens implant.

Contact lenses.  These usually provide better vision than
eyeglasses and also are quite safe if handled and maintained
properly.  A contact lens may be especially helpful after cata-
ract extraction in one eye.  With a contact lens in the operated
eye, the difference in the size of the images seen by the two
eyes is much smaller.  Soft contact lenses are commonly used for
cataract patients.

Another option is the extended-wear contact lens.  These lenses
can be left in the eye for a longer period of time without being
removed, even for sleep.  They may be especially useful for
people who have trouble inserting and removing a contact lens,
because an eye care specialist can remove and clean them period-
ically.  However, extended-wear lenses have some disadvantages:
They are very fragile; some serious infections have been
reported; their long-term safety is still being assessed; and
they do require periodic removal, cleaning, and reinserting.

Intraocular lenses.  These devices, sometimes called IOLS, are
clear plastic lenses that are implanted in the eye during the
cataract operation.  Lens implants have certain advantages: They
usually eliminate or minimize the problems with image size, side
vision, and depth perception noted by people who wear cataract
eyeglasses.  Also, because lens implants remain in the eye and do
not have to be removed, cleaned, and reinserted, they are more
convenient than contact lenses.  This is particularly true for
people who have physical problems that would make it difficult
for them to carry out the procedures involved in using contact
lenses.

Because of these advantages, lens implants have been used with
increasing frequency in recent years.  About three-fourths of all
people now undergoing cataract surgery have an IOL inserted at
the same time, and the vast majority are very pleased with the
results.  Of course ophthalmologists will continue to study IOLs
for many years in an effort to assess the long-term effects of
implantation on the eye as well as the short-term complications.


What happens after surgery?

Most people who undergo cataract surgery are treated as
outpatients and can go home the same day.  For others, a stay in
the hospital of 1-3 days may be required.  In either case, during
the early stages of recovery, patients need to take special care
to avoid strenuous physical activity.

Sometimes people whose cataract surgery was performed by the
extracapsular method develop a problem called "after-cataract."
After the operation, the back part of the lens capsule left in
the eye may become cloudy and interfere with passage of light to
the retina.

The cloudy material must be cleared away, if possible, so that
full vision can be restored.  Ophthalmologists often treat after-
cataract with an ophthalmic laser called the neodymium-YAG or
"cold" laser.  When this procedure is successful, the patient's
vision is restored without additional eye surgery.

What research is being done on cataracts?

The National Eye Institute supports and conducts research on the
eye and its disorders, including cataracts.  The major goals of
this research are to learn more about how and why cataracts
develop, to find ways of preventing cataracts or slowing their
progress, to evaluate the safety and effectiveness of techniques
for treating cataracts, and to devise better methods of
correcting vision after cataract surgery.


This brochure was prepared by the National Eye Institute, NIH,
Building 31, Room 6A31, Bethesda, MD 20893. The telephone number
is: [phone].

NIH Publication No. 93-201
!
*

                              What are Cataracts?
                                      by
                                  Bruce Tizes


Cataracts affect the young and old and have been documented
for  longer  than 5,000  years.  From  the ancient writings of
the Egyptians and Chinese through the earliest portion of the
seventeenth century we have known  that  cataracts interfere
with  vision  and  occur most frequently in older persons.  In
some countries of the world they are the leading cause of
blindness.  Anatomically the  actual  description of a cataract
was precisely developed only as late at the eighteenth
century,  and the marvelous microsurgical techniques for visual
rehabilitation have only been developed over the past few
decades.

Cataracts are nothing more or less than a clouding of the
natural lens in each of  our  eyes.  When one approaches the
eye anatomically,  from front to back, the structures
encountered are the eye lids,  the cornea (a clear  dome),  the
anterior  (front) chamber,  the iris (colored part of the
eye),  and the pupil (the circular black hole in the center of
the iris).  Behind  the  pupil,  and not  very  deep into the
eye,  is the lens,  a discus shaped crystalline clear object.
Just behind the lens,  occupying the balance of the hollow
eyeball is the  vitreous  (a  clear  jello-like substance),
then the retina (nerve cells which pick up the light
information much as a movie screen picks up the  light from a
movie projector) and the balance of the structural elements.
The optic nerve  transmits  the  visual  information  from the
eye to the brain,  and is roughly analogous to a telephone cord
in  it's  transmission  of  information.  Thus,  light enters
the eye through the open lids,  passes through the cornea, the
anterior chamber, the pupillary space in the iris, the lens,
the vitreous cavity,  and  finally  arrives  at the retina and
is sent to the brain via the optic nerve.  If anything
interferes with the proper functioning of  any  part of this
system deterioration of visual quality will result.

Functionally the lens serves as the fine focusing system of the
eye, much as a magnifying  glass when held in proper position
will focus the sun's light rays to a fine  point  capable  of
burning  a  paper.  The  lens  of  the  eye  is susceptible
to  many  difficulties,  many of which result in clouding.  It
is easy to understand why it is difficult to see clearly
through ground glass  or a dirty automobile windshield,  and it
is equally easy to understand why it is difficult to see
clearly through a cloudy  lens  of  the  eye.  Medically  the
clouding  of  the lens is called a cataract;  many causes for
cataracts exist, and they range from trauma,  aging,  systemic
diseases such as  diabetes,  and some cataracts can even be
present at birth.

Irrespective of the cause,  the functional result of a clouding
of the lens is the same,  namely deterioration of the quality
of the  visual  image.  Several parameters  to  evaluate  the
seriousness of a cataract are commonly employed; they include:
the vision of the eye, the amount of glare the eye suffers, the
possibility  of  damage to the eye by the cataract,  and the
particular visual requirements that an individual patient.

Treatment of cataracts is clearly linked with the seriousness
of the  problem.  If one person has a small cataract,  is not
suffering much visual loss or life activity difficulty,  and
has no other  reason  for  intervention,  then  that person
should probably refrain from a surgical intervention.  The
person with substantial visual loss,  inconvenience and
disability has  an  equally  clear choice, the rehabilitation
of vision by cataract surgery.

The  correction  of  vision is a complicated topic,  and much
evolution of the techniques and procedures used is progressing
even as you  read  these  words.  Generally,  surgery  is
required  and  the  person must have the benefit of a skilled
ophthalmic  surgeon.  The  surgical  procedure,  called  a
"cataract extraction," usually lasts under one hour, is
performed under a microscope, in a  hospital or out-patient
surgical center,  and is virtually painless.  It is usually
performed under local anesthesia (i.e.  the patient  does  not
go  to sleep)  and  generally the patient returns home the same
day.  Technically the operation consists of an incision at the
juncture of the sclera (white part of the eye) and the cornea.
The lens is removed after careful manipulation,  and any
residual lens material is carefully removed.  An intraocular
lens implant is  placed,  if  appropriate,   and  the  eye  is
closed  with  fine  sutures approximately  one  half of the
thickness of a typical hair.  Post-operatively the patient is
patched for one or more days,  which is followed by the use  of
an  eye shield to protect the eye from trauma,  and various
types of eye drops and medications to properly govern the
course of recovery.

Visual rehabilitations may occur immediately,  and  is
progressive  over  the weeks   following   surgery;   the
overwhelming  majority  of  patients  have substantial visual
improvement.  Intraocular lens  implants  ("implants")  are
plastic  innert  lenses  that the surgeon insert during the
surgery to improve the visual result.  Although implants will
generally avoid the need for  thick glasses  after  surgery
they  are  not  appropriate for every patient.  Final visual
correction is usually achieved by approximately eight weeks
after  the surgery.
!
*
Cerebral Palsy (aka C.P.) is a nonspecific condition in
which brain damage before or at birth leads to partial
paralysis.

The most common cause of the disorder is traumatic delivery
due to either a small maternal pelvis, large fetal head,
forceps compression of the head, umbilical cord
strangulation, or the partial separation of the placenta
from the uterus before delivery.  Other causes include
oxygen deprivation in the newborn due to excessive
anesthesia, delayed spontaneous respiration, or severe
cardiac malformations.

Certain infections during pregnancy may also increase the
risk of cerebral palsy. These infections include
toxoplasmosis, measles, mumps, cytomegalicinclusion
disease, and syphilis, all of which may affect brain
tissue.  Bacterial meningitis or encephalitis may occur as
a result of nonsterile delivery and cause brain damage.
Similarly, a fetus subjected to radiation or toxic
chemicals, including alcohol, is also a candidate for
cerebral palsy.

As a result of the above, the infant may have a small
brain, hydrocephalus, or focal lesions in the brain.  The
child's appearance ranges from the demented and deformed to
the bright, alert, and happy, with one wasted and and
spastic limb.  Physical Therapy (aka P.T.) may provide some
benefit, but the frequency of cerebral palsy will diminish
only with improved prenatal care and delivery techniques.
A.W. DUDLEY, MD.


Taken from Cruickshank, William M., ed., Cerebral Palsy: A
Developmental Disability, rev. 3rd ed 1976.

________________________________________________________________
General Information about
CEREBRAL PALSY

Fact Sheet Number 2 (FS2), 1993
______________________________________________________________________________

NICHCY
National Information Center for Children and Youth with Disabilities
P.O. Box 1492
Washington, DC 20013
[phone] (Local)
[phone] (TT)
1-800-999-5599 (Toll-free)

SpecialNet User Name:  NICHCY
SCAN User Name:  NICHCY
______________________________________________________________________________

General Information about
CEREBRAL PALSY

Fact Sheet Number 2 (FS2), 1993
______________________________________________________________________________

NICHCY
National Information Center for Children and Youth with Disabilities
P.O. Box 1492
Washington, DC 20013
[phone] (Local)
[phone] (TT)
1-800-999-5599 (Toll-free)

SpecialNet User Name:  NICHCY
SCAN User Name:  NICHCY
______________________________________________________________________________

DEFINITION OF CEREBRAL PALSY

Cerebral palsy is a condition caused by damage to the brain, usually
occurring before, during or shortly following birth.  "Cerebral"
refers to the brain and "palsy" to a disorder of movement or posture.
It is neither progressive nor communicable.  It is also not "curable"
in the accepted sense, although education, therapy and applied
technology can help persons with cerebral palsy lead productive
lives.  It is not a disease and should never be  referred to as such.
It can range from mild to severe.

The causes of cerebral palsy include illness during pregnancy,
premature delivery, or lack of oxygen supply to the baby; or it may
occur early in life as a result of an accident, lead poisoning,
viral infection, child abuse, or other factors.  Chief among the
causes is an insufficient amount of oxygen or poor flow of blood
reaching the fetal or newborn brain. This can be caused by premature
separation of the placenta, an awkward birth position, labor that
goes on too long or is too abrupt, or interference with the umbilical
cord.  Other causes may be associated with premature birth, RH or
A-B-O blood type incompatibility between parents, infection of the
mother with German measles or other viral diseases in early pregnancy,
and microorganisms that attack the newborn╒s central nervous system.
Lack of good prenatal care may also be a factor.  A less common type
is acquired cerebral palsy: head injury is the most frequent cause,
usually the result of motor vehicle accidents, falls, or child abuse.


INCIDENCE

Between 500,000 - 700,000 Americans have some degree of cerebral
palsy.  About 3,000 babies are born with the disorder each year, and
another 500 or so acquire it in the early years of life.

CHARACTERISTICS

There are three main types of cerebral palsy: spastic -- stiff and
difficult movement; athetoid -- involuntary and uncontrolled m
ovement; and ataxic -- disturbed sense of balance and depth
perception.  There may be a combination of these types for any one
individual. Other types do occur, although infrequently.

Cerebral palsy is characterized by an inability to fully control
motor function. Depending on which part of the brain has been damaged
and the degree of involvement of the central nervous system, one or
more of the following may occur: spasms; tonal problems; involuntary
movement; disturbance in gait and mobility; seizures; abnormal
sensation and perception; impairment of sight, hearing or speech;
and mental retardation.

DEVELOPMENTAL, EDUCATIONAL, AND EMPLOYMENT IMPLICATIONS

Early identification of cerebral palsy can lessen developmental
problems and lead to appropriate intervention when it helps the most.
Early intervention programs are family-centered in which
professionals and families work together with the child in specific
activities.  Educators, physical and occupational therapists, social
workers, speech- language pathologists, psychologists and physicians
can assist families by providing information and education.

Activities for children with cerebral palsy may include:
-       speech and language therapy;
-       occupational therapy;
-       physical therapy;
-       medical intervention;
-       family support services;
-       early education; and
-       assistive technology.

 As a child gets older and begins formal schooling, the intensity of
 services will vary from individual to individual.  Persons with
 cerebral palsy are usually able to attain a substantial degree of
 independence but, in some cases, may need considerable assistance.
 Services for the school age child may include continuing therapy,
 regular or special education, counseling, technical support,
 community integration opportunities, recreation and possible personal
 attendants.  A key factor seems to be a supportive family.  People
 extensively affected by cerebral palsy can still be highly
 functional and independent.  The HEATH Resource Center, the
 clearinghouse on postsecondary education for individuals with
 disabilities, states that a significant number of students with
 cerebral palsy are enrolled in colleges and universities.

Important advances have taken place in the last 15 years which have
had a great effect on the long-term well-being of children born with
cerebral palsy.  Advanced technology, including computers and
engineering devices, has been applied to the needs of persons with
cerebral palsy.  Technological innovations have been developed in the
areas of speech and communication, self-care, and adapting living
arrangements and work sites. The future may bring even more
significant applications.

Another important development has been the increased ability of
persons with disabilities, including those who have cerebral palsy
and other severe disabilities, to live independently in the community.
Adults with cerebral palsy are now living, with or without assistance,
in their own apartments or townhouses.  Independent Living Centers
have also proven to be important resources for persons with
disabilities.

RESOURCES

Schleichkorn, J. (1983).  Coping with Cerebral Palsy:  Answers to Questions
Parents Often Ask.  Austin, TX: Pro-Ed, Inc.  (Contact Pro-Ed, 8700 Shoal
Creek Boulevard, Austin, TX 78758.  Telephone: [phone].)

Geralis, E.  (1991).  Children with Cerebral Palsy, A Parent's Guide.
Rockville, MD: Woodbine House.  (Contact Woodbine House, 5615 Fishers Lane,
Rockville, MD 20852.  Telephone: Outside of the DC metropolitan area, call
1-800-843-7323; call [phone] in DC metropolitan area.)

Weiss, S.  (1993).  Each of Us Remembers: Parents of Children with Cerebral
Palsy Answer Your Questions.  Washington, DC: United Cerebral Palsy
Associations, Inc.  (See address and telephone number below.)

ORGANIZATIONS

American Academy for Cerebral Palsy and Developmental Medicine
1910 Byrd Avenue, Suite 100
P.O. Box 11086
Richmond, VA  23230-1086
[phone] (For physician referral)

Independent Living Residential Utilization Project (ILRU)
The Institute for Rehabilitation and Research
2323 South Sheppard, Suite 1000
Houston, TX  77019
[phone]; [phone] (TT)

National Easter Seal Society, Inc.
70 East Lake Street
Chicago, IL  60601
[phone]; [phone] (TT)
[phone] (Outside IL)



National Rehabilitation Information Center (NARIC)
8455 Colesville Road
Suite 935
Silver Spring, MD  20910-3319
[phone] (TT/Voice); [phone]

United Cerebral Palsy Associations, Inc.
1522 K Street, N.W., Suite 1112
Washington, D.C.  20005
[phone] (V/TT); [phone] (V/TT)
!
*
CHICKEN POX (VARICELLA)

CENTERS FOR DISEASE CONTROL AND PREVENTION
Date Last Rev'd: March 9, 1995

CHICKEN POX (VARICELLA) DISEASE INFO


Chickenpox  is  a  common,  highly  contagious  disease  of  childhood.  Most
Americans will have been infected by the virus by age 15, and it is estimated
that approximately 4 million cases occur each year. Children between 5 and  9
years of age account for the majority of all cases, with more cases occurring
during the winter and spring than during other times of the year.

Chickenpox  is easily transmitted between household members or classmates  at
schools  or  day-care  centers  through the drainage  from  the  open  sores,
respiratory  droplets,  or  airborne  particles.  The  virus  can   also   be
transmitted  indirectly  through  contact  with  articles  containing   fresh
drainage from the sores. Persons are most contagious from 1 to 2 days  before
the  rash  appears  through the first 5 to 6 days after the rash.  Generally,
patients are no longer contagious after the sores have crusted over.

Persons  exposed to chickenpox are most likely to develop symptoms 14  to  16
days after exposure. However, for normal individuals, symptoms may appear any
time after 10 days and up to 21 days.

Chickenpox  is  characterized by 1 to 2 days of  fever  up  to  102  degrees,
general  body weakness, and a rash, which in many children is the first  sign
of  the  disease. Only rarely will a person have chickenpox without  a  rash.
Often, the rash begins on the scalp, moves to the trunk, and then to the arms
and legs.

The  chickenpox  rash  usually  is  generalized,  itchy,  and  progresses  to
blisters,  which  become open sores before crusting. The blisters  contain  a
clear  fluid  that  can  transmit the virus to others.  Successive  crops  of
blisters appear over several days.

In  normal  children,  chickenpox is generally a mild  disease  although  the
following  complications do occur. Sometimes bacterial skin  infections  will
develop  from  the open sores associated with chickenpox. Other complications
that  are  more rare but serious include pneumonia, infection  of  the  brain
(encephalitis),  infections of the covering of the brain  (meningitis),  Reye
syndrome, and death.

Serious chickenpox disease and complications are more likely to occur in  the
following types of patients:

1. Those  whose immune systems are not functioning normally, such as  persons
   with  certain  leukemias or cancers, persons with AIDS, or persons  taking
   drugs, including steroids, which suppress their immune systems;

2. Newborn  infants whose mother developed the chickenpox rash close  to  the
   time of delivery; and

3. To a lesser degree, normal adults.

After  a  chickenpox infection, persons generally have lifelong  immunity  to
chickenpox and do not have a second episode of chickenpox. However, the virus
can  reactivate  later in life as a different problem - zoster  or  shingles.
This  is  particularly likely to happen if a person has a problem with  their
immune system such as can occur with older age, disease, or drugs.


CHICKEN POX (VARICELLA) - PREGNANCY AND INFANTS

More than 90% of the adult U.S. population is immune to chickenpox because of
an  infection during childhood. Therefore, for the vast majority of  pregnant
women,  being exposed to chickenpox should pose little concern to either  the
pregnant woman or the unborn child.

Pregnant  women  who  are  not  immune  to  chickenpox,  and  who  have  been
significantly exposed may be at increased risk. Both the pregnant  woman  and
the unborn child have risks including:

1. A  pregnant woman has an increased risk of complications associated with a
   chickenpox illness. However, remember that while chickenpox is  usually  a
   mild  infection  with  few complications for normal children,  for  normal
   adults  the infection can be more severe, and complications are  a  little
   more  frequent.  Therefore, normal adults, including pregnant  women,  may
   have  an  infection with symptoms somewhat more severe than children,  and
   these adults are also at increased risk of complications.

   Pregnant  women who are not immune to chickenpox and who have  significant
   exposure to a person with chickenpox can be evaluated by their doctor  for
   possible  treatment with Varicella-Zoster Immune Globulin, also  known  as
   VZIG.  Treatment  with  VZIG, if given within 96 hours  of  exposure,  can
   prevent  or  moderate a chickenpox infection in the pregnant women.  There
   is,  however,  no evidence that VZIG can prevent or moderate a  chickenpox
   infection  in  the  unborn child. A treatment dose of VZIG  costs  between
   $400 and $500 and physicians can obtain VZIG from The American Red Cross.

2. The  unborn child is at a low risk of birth defects if the pregnant  woman
   is  infected  with  chickenpox during the first  16  weeks  of  pregnancy.
   Infants  whose  mother was infected with chickenpox during  the  first  16
   weeks  of  pregnancy rarely develop congenital varicella  syndrome,  which
   can  include eye, skin, or limb defects in the child. The risk  of  having
   congenital  varicella is low (around 2 to 3 percent), and  is  similar  to
   the  risk  of  developing birth defects in general. Unborn children  whose
   mother  is infected with chickenpox after the first 16 weeks do not appear
   to develop congenital varicella syndrome.

3. The  newborn  child  is  at  increased risk of serious  infection  if  the
   pregnant  woman is infected with chickenpox close to the time of delivery.
   If  the  mother develops chickenpox between 5 days before delivery and  48
   hours  after  delivery, the newborn infant may develop serious  infection.
   Infants whose mothers develop chickenpox outside the "5 days before to  48
   hours  after  delivery" window do not appear to be at  increased  risk  of
   serious illness.

4. Premature  infants who have significant exposure after  birth  may  be  at
   increased  risk for serious illness, especially those infants born  before
   28 weeks of gestation, or whose mothers are not immune to chickenpox.

If  there  is concern that a pregnant woman has been exposed to chicken  pox,
there are 3 ways to figure out if she ia already immune to chickenpox:
1. By  asking  her  parents  or relatives if they remember  whether  she  had
   chickenpox.
2. Check the medical records of her family or pediatric physician.
3. Have  a  laboratory  test performed that would indicate  whether  she  was
   already infected and thus is immune.

If  the  pregnant  woman  is  not known to be immune  to  chickenpox,  it  is
important  to figure out whether she was significantly exposed  to  a  person
with  chickenpox. Significant exposure means being in the presence or  having
prolonged  contact  with  someone  who  is  developing  or  currently  has  a
chickenpox  infection. For example, significant exposure  to  chickenpox  can
include:
      (a)continuous household contact.
      (b)playmate contact of greater than one hour.
      (c)hospital contact involving the same bedroom, or adjacent beds  in  a
      large ward, or
      (d)prolonged face-to-face contact.

If  a  woman  is  pregnant and had a significant exposure to chickenpox,  she
should  consult with her physician about the risk to herself and  her  unborn
child.


CHICKEN POX (VARICELLA) - EXPOSED


Since  3  to 4 million cases of chickenpox occur each year, exposure is  very
difficult to prevent, especially in children. More than 90% of the adult U.S.
population are already immune to chickenpox because they were infected during
childhood.  Therefore, for the vast majority of normal adults, being  exposed
to chickenpox should pose little or no concern.

For  a  person  to  become  infected  with  chickenpox,  they  must  be  both
susceptible to infection (that is, not immune) and have significant  exposure
to an infected person.

Individuals who want to know if they have had chickenpox can do  one  of  the
following:

1. Ask relatives if anyone remembers them having chickenpox.

2. Check the medical records of their family or pediatric physician.

3. Have  a  laboratory test performed that would indicate whether  they  have
   been infected previously and therefore are immune.

Significant exposure to chickenpox can include:
      (a)continuous household contact.
      (b)playmate contact of greater than one hour.
      (c)hospital contact involving the same bedroom, or adjacent beds  in  a
      large ward or
   (d)  prolonged face-to-face contact.

Remember that chickenpox is a mild infection with few complications in normal
children.  However,  certain groups of people are at greater  risk  for  more
serious infection and complications. These groups include the following: high-
risk  newborn infants, which are those newborn infants whose mother developed
chickenpox  between  5 days before delivery to 48 hours after  delivery,  and
persons with a defective immune system, such as cancer and leukemia patients,
persons  with  AIDS,  or  persons  taking drugs,  including  steroids,  which
suppress  their  immune  system. Normal adults  are  at  a  greater  risk  of
developing serious illness or complications, than children, but most  do  not
have serious illness.

Complications  of  chickenpox  include  skin  infections,  pneumonia,   brain
inflammations,  infections of other body organs, Reye  syndrome,  and  death.
Because  Reye  syndrome, a serious brain and liver disease, may  occur  after
using  aspirin  during  an episode of chicken pox  or  flu,  aspirin  is  not
recommended  for treatment of symptoms of chickenpox or flu. In  fact,  never
give aspirin to children without first consulting a physician.

There  is currently no licensed vaccine for chickenpox in the United  States.
Persons  at  high risk of serious chickenpox illness who are  not  immune  to
chickenpox  should be evaluated by their doctor for treatment with Varicella-
Zoster Immune Globulin, also known as VZIG, if they have significant exposure
to infection. In some cases, treatment with VZIG, if given within 96 hours of
exposure, can prevent or moderate a chickenpox infection. A treatment dose of
VZIG  costs  between $400 and $500 and physicians can obtain  VZIG  from  The
American Red Cross. There is no evidence that VZIG can prevent or moderate  a
chickenpox infection in an unborn child.


CHICKEN POX (VARICELLA) - VZIG (Varicella-Zoster Immune Globulin)


Varicella-Zoster Immune Globulin, also know as VZIG, can be used to modify or
prevent chickenpox in susceptible individuals who have had recent significant
exposure to the disease. To be effective, VZIG should be administered  within
96  hours of exposure. Treatment with VZIG may lengthen the incubation period
of chickenpox to as long as 28 days.

VZIG  is  normally used to treat individuals who have a high risk of  serious
disease  or  complications, including immunocompromised children,  high  risk
newborn   children,   premature  infants  with  significant   exposure,   and
immunocompromised adults. Normal adults, including pregnant women, should  be
evaluated on a case by case basis. Because there is no evidence that VZIG can
prevent or modify a chickenpox infection in the unborn child, the decision to
administer  VZIG to a pregnant woman is based on the benefits  to  the  woman
alone.

The duration of protection after VZIG administration is unknown, but VZIG  is
probably  effective for at lease three weeks. VZIG is not known to be  useful
in   treating  clinical  chickenpox  or  shingles  after  the  infection  has
developed,  or  in  preventing  disseminated  shingles,  and  VZIG   is   not
recommended for these uses.

VZIG costs between $400 and $500 for an adult dose. It is available from  the
American Red Cross through its regional offices.



CHICKEN POX (VARICELLA) - PREVENTION

Because   chickenpox  is  highly  contagious  even  before  symptoms  appear,
prevention  is  difficult.  Susceptible persons  who  have  been  exposed  to
chickenpox and may be developing the disease should be kept from contact with
persons at high risk for serious chickenpox infection, such as patients  with
defective  immune  systems,  from  10 days  until  21  days  after  exposure.
Isolating infected persons for 1 week after eruption of sores is prudent  and
somewhat effective in limiting the spread of chickenpox.

Varicella-Zoster Immune Globulin, also know as VZIG, can be used  to  prevent
or  modify chickenpox in susceptible individuals with significant exposure to
the  disease. Ordinarily, VZIG is reserved for use with persons  who  are  at
high  risk  for serious chickenpox disease or complications. To be  effective
VZIG  should be administered within 96 hours of exposure. Persons who develop
mild chickenpox after receipt of VZIG may still be infectious.

A  vaccine  to  prevent chickenpox has been developed by  Merck  Sharp  Dohme
Research  Laboratories  and is being tested for use in  normal  children  and
adults; it may become available within a couple of years.


TREATMENT

Once  a  person  develops  chickenpox,  treatment  may  include  bedrest  and
medication  for  fever, headache, and itching. Your local physician  familiar
with your child is best suited to recommend treatment for chickenpox. In  all
cases,  contact  your  physician  before using  any  treatment  because  some
preparations  are  harmful  if not used in the correct  dose,  especially  in
children.  Remember that aspirin should not be given to children  to  control
fever,  pain,  or  headache associated with chickenpox. In fact,  never  give
aspirin   to   children  without  first  consulting  a  physician.   Instead,
acetaminophen products (like Tylenol), may be used to treat mild  complaints.
Also,  do  not  use skin treatments containing diphenhydramine without  first
consulting a physician.

Acyclovir  and  Arabinoside-A are antiviral drugs used to  treat  individuals
with  serious chickenpox disease and complications. These drugs are sometimes
used  to  treat patients with defective immune systems and high-risk  newborn
infants.  These  drugs  are not usually used to treat  chickenpox  in  normal
healthy children with a routine chickenpox illness.


CHICKEN POX (VARICELLA) - STATISTICS


Chickenpox  is a common, highly contagious disease of childhood  that  occurs
usually  during late winter and early spring. Most Americans will  have  been
infected  by the virus by age 15, and it is estimated that approximately  3.5
to  4  million cases occur each year. Not all states require that health care
workers  report chickenpox cases. Some cases of chickenpox are so  mild  that
medical  attention  is never sought. Therefore, only an  average  of  225,000
cases  of chickenpox are reported to the CDC annually. Even though most cases
of  chickenpox are mild with few complications, between 40 and 90 chickenpox-
related  deaths  occur  annually. About half these  deaths  occur  in  normal
individuals,  and  half in persons whose immune systems are  not  functioning
normally.  A  few  other deaths are from Reye Syndrome, which  can  occur  in
children following the use of aspirin during an episode of chickenpox. During
the  past  few years, the deaths due to chickenpox have been decreasing.  The
decrease  in the death rate may be due to both improved treatment  and  to  a
decrease in Reye Syndrome.
!
*
Chlamydia

                              CHLAMYDIA
                       (Chlamydia trachomatis)

One of the most prevalent and problematic sexually transmitted diseases
(STDs) is Chlamydia.  This bacterial disease causes infections affecting
3-4 million people each year.  Chlamydia may occur alone, but can also
occur simultaneously with other sexually transmitted diseases.

For women, it is the most frequent cause of pelvic inflammatory disease
(PID), an infection of the internal reproductive organs which may result
in infertility if left untreated.  For men, Chlamydia is the most frequent
cause of NGU, or Nongonococcal Urethritis, and can also cause sterility.


Symptoms/Transmission
----------------------
Chlamydia is transmitted through sexual contact, most often intercourse.
The infection causes symptoms for some people, while others have no
symptoms at all.

Women are more likely than men to have Chlamydia without symptoms.
In many cases, the infection is first noticed during a routine pelvic
examination.  Examination is recommended for the presence of any of the
following symptoms:  vaginal itching or discharge; burning on urination;
pain in the abdomen or pelvis; pain with intercourse; or swollen glands
in the groin.

Men may have mild burning with urination and for some men, this symptom
may resolve without treatment.  However, many times the infection is still
present without any evident symptoms and can cause permanent damage.
Examination is recommended if symptoms such as a discharge, painful or
burning urination or swollen glands are noticed.


Diagnosis
----------
If the clinician who examines you suspects the presence of Chlamydia or
other infections, testing will be recommended.  The test involves a
painless procedure in which a swab is used to take a sample of cells
from the cervix (for women) or from the urethra (for men).  This test
sample is sent to the McKinley Health Center Laboratory for evaluation
and the results are available within a few days.  During this waiting
period, it is recommended that you refrain from sexual intercourse.
!
*

     DEAR DOCTOR COLUMNS ARE WRITTEN BY UAB (UNIVERSITY OF ALABAMA AT
      BIRMINGHAM) EXPERTS AND APPEAR WEEKLY IN "THE BIRMINGHAM NEWS"



QUESTION:

   My daughter is on her high school's swim team. She is very
   fair skinned with light blonde hair. Her beautiful hair has
   turned green, and others tell me it's the chlorine in the
   pool. Her hair is also dry. Is there anything she can do to
   protect her hair?

ANSWER:
   It's somewhat misleading to say that chlorine used to keep
swimming pools free of germs turns hair green. Actually, it's
chlorine's oxidation of dissolved metals such as copper in the
pool water that causes blonde and grey hair to take on a greenish
tint.
   As a bleach, chlorine does lighten hair. But it also eats away
at the cuticle -- the protective outer protein layer of the hair
shaft -- causing hair to become split, dry, and brittle. Damaged
cuticle also allows the hair shaft to absorb dissolved metals in
pool water. Permed and color-treated hair intensifies the drying
effects of chlorine.
   Wearing a bathing cap will help protect your daughter's hair
against pool chemicals. And many competitive swimmers use a cap
to streamline their movements through water by cutting down on
drag. Running some protein conditioner or oil treatment through
her hair before putting on a bathing cap, will coat the hair
shaft for extra protection. Your daughter should wash her hair
immediately after getting out of the pool and avoid letting her
hair dry without at least rinsing the pool water out of her hair.
!
*
CHOLERA

CENTERS FOR DISEASE CONTROL AND PREVENTION
Date Last Rev'd: March 9, 1995

FOOD AND WATER BORNE BACTERIAL DISEASES


CHOLERA
CDC has detailed information to help people understand foodborne diseases.
This information is referenced on the Disease Information Directory.

Cholera is an acute intestinal infection. Cholera occurs in many of the
developing countries of Africa, and Asia, where sanitary conditions are less
than optimal. Most recently, cholera outbreaks have occurred in parts of
Latin America.

Most infected persons have no symptoms or only mild diarrhea. However,
persons with severe disease can die within a few hours after onset due to
loss of fluid and salts through profuse diarrhea and, to a lesser extent,
through vomiting.

Only a few cases of cholera have occurred in the United States since 1973.
Even with foreign travel, the risk of infection to the U. S. traveler is very
low, especially for those who are follow the usual tourist itineraries and
stay in standard accommodations. Worldwide cholera activity is characterized
by occasional epidemics in developing countries.

The organism that causes the illness is named Vibrio cholerae type O:1.
During epidemics, it is spread by ingestion of food or water contaminated
directly or indirectly by feces or vomitus from infected persons. Diagnosis
is made by culturing the bacteria from the stool of a patient and confirming
that the organism produces toxin.

The best protection is to avoid consuming food or water that may be
contaminated with feces or vomitus from infected persons. The organism can
grow well in some foods, such as rice, but it will not grow or survive in
very acidic foods, including carbonated beverages, and is killed by heat.

A cholera vaccine is available, but normally is not recommended. Only 50% of
those who take the vaccine develop immunity to cholera, and this immunity
lasts only a few months.

Treatment for cholera involves rehydration with oral rehydration solution or,
in the most severe cases, with intravenous solutions until the patient is
able to ingest fluids.  Treatment with antibiotics (usually tetracycline or
doxycycline) will decrease the duration of illness and the excretion of live
cholera bacteria, and will decrease the volume of fluid lost, but is not
necessary for successful treatment.

CHOLERA VACCINE INFORMATION
A cholera vaccine is available, but normally is not recommended. Only 50% of
those who take the vaccine develop immunity to cholera, and this immunity
lasts only a few months. No country currently requires the cholera vaccine
for entry if arriving from cholera-infected countries. Consult the
International Travelers Hotline for specific recommendations.

The complete vaccination schedule includes 2 doses of vaccine spaced 1 to 4
or more weeks apart. Dosages are age specific.

For infants 0-6 months of age; the vaccine is not recommended.

Data indicate that simultaneous administration of cholera and yellow fever
vaccines produces less-than-normal antibody responses to both vaccines. A
three week minimum interval between cholera and yellow fever vaccines is
recommended. In cases where both vaccines are required and time constraints
exist, then administer simultaneously or as far apart as possible.

Reactions to the vaccine are: 1-2 days of pain, erythema, and induration at
the site of injection; fever, malaise, and headache. Serious reactions are
rare, but if experienced, re-vaccination is not advisable.

No specific information on the safety of cholera vaccine in pregnancy is
available, therefore vaccination should be avoided.
!
*
CHOLESTEROL:  THE NUMBERS

Cholesterol is an essential substance in animal cells.  However,
when present in excess in susceptible animals, it can accumulate
in damaged blood vessel walls.  This can lead to stoppage of
blood flow in the blood vessel.  If the vessel involved is a
coronary artery, a heart attack results.  If a brain artery is
involved, the result is a stroke.  While factors other than high
cholesterol are necessary for atherosclerosis to occur, high
blood cholesterol is a major modifiable factor.  Smoking and high
blood pressure are the other two factors.  The presence of any
one of these factors doubles the risk of heart disease.  Having
two of these factors increases the risk more than four times.
Having all three factors increases the risk of heart disease and
stroke more than eight times normal.  A goal of modern preventive
medicine is to identify those individuals with high cholesterol
and to provide them with the means to reduce this to a normal or
low level.  Here are the recommended maximum levels for total
cholesterol.




  AGE         RECOMMENDED         MODERATE RISK       HIGH RISK

20-29          Under 180             200-220             220+
30+            Under 200             201-239             240+

The total cholesterol level, however, can be misleading.
Cholesterol in the body is attached to molecules called
lipoproteins which transport it.  Some lipoproteins (called LDL
and VLDL) lead to an over accumulation of cholesterol and a risk
of atherosclerosis.  Other lipoproteins (HDL) transport
cholesterol out of the body and thus reduce the risk of
atherosclerosis.  Total cholesterol is a quantitative measurement
of all the cholesterol bound to all of the lipoprotein, both the
"good" and the "bad".  Thus, to better evaluate one's risk, we
need to separately measure the protective and the dangerous
lipoprotein.  A simplified way to do this is to independently
measure the total cholesterol level and the HDL cholesterol
level.  We then divide the total cholesterol by the HDL
cholesterol to give the Cholesterol/HDL Cholesterol Ratio.  This
ratio is a much better predictor of atherosclerosis because it
looks at the balance between the "good" and "bad" cholesterol
which is not possible with only the total cholesterol value.

        Complete cholesterol testing is available for a small
        fee through the Student Health Service for students.
        Faculty & staff can obtain cholesterol testing through
        their private physician or the Wellness Program.

Following is a list of recommended reading for diet and exercise.

GOOD FAT, BAD FAT: HOW TO LOWER YOUR CHOLESTEROL & BEAT THE ODDS
OF HEART ATTACK, Glenn Griffin & William Castelli (ed. by Bill
Fisher), Fisher Books, 1988.

NEW AMERICAN DIET, Conner, Simon and Schuster, 1986

PHYSIOLOGY OF FITNESS, 2nd edition.  Dr. Brian J. Sharkey: Human
Kinetics Publishers, Inc., Illinois, 1984.

ROCKPORT WALKING PROGRAM, Dr. James Rippe & Ann Ward, PhD.,
Prentice Hall PRess, New York, 1988.

THE AEROBICS PROGRAM FOR TOTAL WELLBEING.  Dr. Kenneth Cooper,
Bantam Books, New York, 1962.
!
*
American Heart Association
Cholesterol and Your Health Know Your Numbers

A routine visit to a physician by a healthy American adult
might include having a blood sample drawn and analyzed. Usually
the lab work is completed, the results are reported to the
physician, and the numbers are recorded on the patient's chart.

The American Heart Association suggests that patients ask their
physicians to discuss these results when they are available and
understand what the tests are for and what the numbers mean.

One report the physician generally requests is cholesterol
level. Cholesterol is a fat- like substance carried in the
blood which comes from two sources. "Blood cholesterol" is
produced primarily by the liver, while "dietary cholesterol" is
found in foods from animals. These foods include egg yolks,
meat, fish, poultry and whole milk dairy products such as ice
cream and cheese. A certain amount of cholesterol is needed to
generate hormones and cells.

However, when the blood cholesterol climbs above the desirable
level, cholesterol can build up over a period of time with some
other substances on the inner lining of blood vessels like rust
in water pipes. During that time, the buildup can narrow those
vessels, keeping sufficient oxygen-carrying blood from getting
to the heart or brain. It is easy for a clot to form that will
clog the vessels, cutting off the flow of blood. The result can
be heart attack or stroke.

To understand what the numbers resulting from a blood
cholesterol test mean, first know that cholesterol is measured
in milligrams per deciliter of blood, expressed as "mg/dl."
Also be aware that the level varies depending on age, sex, and
the presence of certain disease. If other risk factors are
present-smoking, high blood pressure, diabetes, glucose
imbalances, excess weight or a history of heart disease in the
patient's family, the probability of having a heart attack
increases regardless of cholesterol level.

The American Heart Association considers a healthy American
adult, 20 years or older, at "low risk" for cardiovascular
disease when cholesterol measures 200 mg/dl or lower, if no
other phase of cardiovasculardisease or any other
cardiovascular risk factors are present. The same adult is at
"moderate risk" if cholesterol level is between 200 and 239
mg/dl, and at "high risk" with a reading of 240 mg/dl or higher.

If it is determined that a patient has a high cholesterol
level, the AHA recommends that the physician determine the
appropriate treatment. This will usually include a low-fat,
low-cholesterol diet. The physician should also address other
major risk factors, including smoking and high blood pressure.
More than 50 percent of middle-aged Americans have cholesterol
levels 200 mg/dl and above. It is prudent for all adults to
follow a heart-healthy, low-fat, low- cholesterol diet as a
preventive measure. Have your cholesterol checked regularly by
a physician and know your numbers.
!
*
            So You Have High Blood Cholesterol...

        U.S. DEPARTMENT OF HEALTH AND HUMAN SERVICES

                  Public Health Service
              National Institutes of Health

               NIH Publication No. 92-2922
                   Reprinted April 1992

                     [Graphic Omitted]

            National Cholesterol Education Program
           National Heart, Lung, and Blood Institute
                National Institutes of Health


Know Your Cholesterol Level


     Lowering your high blood cholesterol is an important step
to reducing your risk of coronary heart disease, Remember to
ask your doctor to tell you your total cholesterol,
LDL-cholesterol, and HDL-cholesterol levels. Use the charts
below to record your progress.


Date _________________________   Date _________________________

Total Cholesterol ____________   Total Cholesterol ____________

HDL __________________________   HDL __________________________

LDL __________________________   LDL __________________________


Date _________________________   Date _________________________

Total Cholesterol ____________   Total Cholesterol ____________

HDL __________________________   HDL __________________________

LDL __________________________   LDL __________________________


Date _________________________   Date _________________________

Total Cholesterol ____________   Total Cholesterol ____________

HDL __________________________   HDL __________________________

LDL __________________________   LDL __________________________


Table of Contents


So You Have High Blood Cholesterol

High Blood Cholesterol: What It Means

How high does your blood cholesterol level have to be to affect
     your health?
How does high blood cholesterol lead to coronary heart disease?
What will lowering your high blood cholesterol level do?
What factors influence your blood cholesterol level?

Blood Cholesterol and Lipoproteins:
  What They Are

Is there "good" and "bad" cholesterol?
How are LDL and HDL levels determined?
What do your LDL and HDL levels mean?

How To Lower Your High Blood Cholesterol

What foods contain fat and cholesterol?
What are some practical guidelines for changing your diet?

What You Can Expect

How soon and how much can you expect your blood cholesterol
     levels to change?
How long do you need to follow this diet?
What should your blood cholesterol goal be?
How do you check your progress?
How often should you have your blood cholesterol levels
     checked?
Can you eat before your test?

Other Steps You Can Take

Do you need to take special medications along with your diet?
What are these medications?
Do you need to make other changes to lower your blood
     cholesterol level?
What else can you do to lower your risk for coronary head
     disease?

Where You Can Go For Help

What other health professionals, in addition to your doctor,
     can give you help?
How can you get more materials about high blood cholesterol?

Glossary


So You Have High Blood Cholesterol...

[Graphic Omitted]

     Anyone can develop high blood cholesterol regardless of
age, sex, race, or ethnic background. But, because there are no
warning symptoms or signs, you are likely to be surprised at
such a diagnosis. Don't be alarmed, but do take it seriously.
Like high blood pressure, most people are unaware that their
blood cholesterol levels are high until they learn it from
their doctor. And, like high blood pressure, it is a potential
threat to your health that you can do something about.

     If you have just learned that you have high blood
cholesterol, there are some important facts you need to know to
protect your health. First, you need to find out what high
blood cholesterol is, how high your level is, and what you can
do to lower it. Then prepare to make some changes. Although
these changes will depend on many factors considered by your
doctor, modifying your diet is the preferred way to lower blood
cholesterol.

     This brochure will answer many of the questions you may
have about your blood cholesterol level. The glossary will help
you become familiar with the terms used in this brochure and in
other information about cholesterol.


High Blood Cholesterol: What It Means


     High blood cholesterol is one of the three major risk
factors for coronary heart disease (cigarette smoking and high
blood pressure are the other two). In other words, high blood
cholesterol can significantly increase your risk of developing
heart disease. Fortunately all three risk factors are
"modifiable"; that is, you can do something about them. You can
take steps to lower your cholesterol level and thus lower your
risk for coronary heart disease.

     High blood cholesterol occurs when there is too much
cholesterol in your blood. Your cholesterol level is determined
partly by your genetic makeup and the saturated fat and
cholesterol in the foods you eat. Even if you didn't eat any
cholesterol, your body would manufacture enough for its needs.


How High Does Your Blood Cholesterol Level Have To Be To Affect
Your Health?


     The risk of developing coronary heart disease increases as
your blood cholesterol level rises. This is why it is so
important that you have your blood cholesterol level measured.
Currently, more than half of all adult Americans have blood
cholesterol levels of 200 mg/dl or greater, which places them
at an increased risk for coronary heart disease. Approximately
25 percent of the adult population 20 years of age or older has
blood cholesterol levels that are considered "high," that is,
240 mg/dl or greater.

[Graphic Omitted]

     Your doctor will measure your level with a blood sample
taken from your finger or your arm and will confirm this result
with a second test if it is greater than 200 mg/dl. The
following table can help you see how the results of your total
blood cholesterol tests relate to your risk of developing
coronary heart disease.


Does Your Total Blood Cholesterol Increase Your Risk For
Developing Coronary Heart Disease?


    Desirable          Borderline-High             High
Blood Cholesterol     Blood Cholesterol      Blood Cholesterol

Less than 200 mg/dl    200 to 239 mg/dl     240 mg/dl and above


     Note: These categories apply to anyone 20 years of age or
     older.


     A blood cholesterol level of 240 mg/dl or greater is
considered "high" blood cholesterol. But any level above 200
mg/dl, even in the "borderline-high" category, increases your
risk for heart disease. If your blood cholesterol is 240 mg/dl
or greater, you have more than twice the risk of someone whose
cholesterol is 200 mg/dl, and you need medical attention and
further testing.

[Graphic Omitted]

     When your high blood cholesterol level is combined with
another major risk factor (either high blood pressure or
cigarette smoking), your risk for coronary head disease
increases even further. For example, if your cholesterol level
is in the "high" category and you have high blood pressure,
your risk for coronary head disease increases six times. If you
also smoke, your risk increases more than 20 fold. Other
factors that increase your risk for coronary head disease
include a family history of coronary heart disease before the
age of 55, diabetes, vascular (blood vessel) disease, obesity,
and being male. Whether your total blood cholesterol is in the
"borderline-high" category or "high" category, you should make
some changes in your diet to lower your level. More
specifically, if your level is in the "borderline-high"
category and you have coronary head disease or two other risk
factors for coronary heart disease or is in the "high"
category, your physician will prescribe more aggressive
treatment and follow your cholesterol levels more closely. If
your cholesterol level is desirable, you should have your level
checked again in 5 years and take steps to prevent it from
rising.


Risk Factors for Coronary Hearth disease


High blood cholesterol
High blood pressure
Cigarette smoking
Family history of coronary hearth disease before the age of 55
Diabetes
Vascular disease
Obesity
Being male


How Does High Blood Cholesterol Lead to Coronary Heart Disease?


     Most coronary heart disease is caused by atherosclerosis,
which occurs when cholesterol, fat, and other substances build
up in the walls of the arteries that supply blood to the heart.
These deposits narrow the arteries and can slow or block the
flow of blood. Among many things, blood carries a constant
supply of oxygen to the heart. Without oxygen, heart muscle
weakens, resulting in chest pain (angina), a heart attack
(myocardial infarction), or even death. Atherosclerosis is a
slow progressive disease that may start very early in life yet
might not produce symptoms for many years.

[Graphic Omitted]

What Will Lowering Your High Blood Cholesterol Level Do?


     Lowering your high blood cholesterol level will slow fatty
buildup in the walls of the arteries and reduce your risk of a
heart attack and death caused by a heart attack. In fact, some
studies have shown that, in adults with "high" blood
cholesterol levels, for each 1 percent reduction in total
cholesterol levels, there is a 2 percent reduction in the
number of heart attacks. In other words, if you reduce your
cholesterol level 15 percent, your risk of coronary heart
disease could drop by 30 percent.


What Factors Influence Your Blood Cholesterol Level?


   * Diet. Among the factors you can do something about, diet
     has the largest effect on your blood cholesterol level.
     Saturated fat raises your blood cholesterol level more
     than anything else you eat. Dietary cholesterol also
     increases your blood cholesterol level. If you have high
     blood cholesterol, changing your diet will be a very
     important step to lower it.

   * Weight. Being overweight may also increase your blood
     cholesterol level. Most overweight patients with high
     levels of cholesterol can help lower their levels by
     weight reduction.

   * Physical activity/exercise. Although it is not clear
     whether physical activity can prevent atherosclerosis,
     regular exercise may help you control weight, lower your
     blood pressure, and increase your level of HDL cholesterol,
     the "good" type of blood cholesterol. (HDL cholesterol is
     explained in a later section.)

   * Genetic factors. Genetic factors play a major role in
     determining your blood cholesterol level and can determine
     your ability to lower your level by diet. A small number
     of people have an inherited tendency to have a high blood
     cholesterol level. If you have a genetic disorder
     contributing to a high blood cholesterol level, then your
     parents, children, brothers, and sisters should also have
     their blood cholesterol levels measured.

   * Sex/age. Coronary heart disease is the leading cause of
     death and disability for both men and women in the United
     States. Estimates are that 1 out of 5 men and 1 out of 17
     women will have symptoms of heart disease before the age
     of 60. This means that men have two to three times the
     risk of developing heart disease as women. However, in
     women as in men, cholesterol levels are predictive of
     coronary heart disease.

[Graphic Omitted]

     In the United States, blood cholesterol levels in men and
women start to rise at about age 20. Women's blood cholesterol
levels prior to menopause (45-60 years) are lower than those of
men of the same age. After menopause, however, the cholesterol
level of women usually increases to a level higher than that of
men. In men, blood cholesterol levels off around age 50 and the
average blood cholesterol level declines slightly after age 50.
Since the risk of coronary head disease is especially high in
the later decades of life, reducing blood cholesterol levels
may be important in the elderly.

     In addition, oral contraceptives and pregnancy can
increase blood cholesterol levels in some women. For pregnant
women, blood cholesterol levels should return to normal 20
weeks after delivery.

[Graphic Omitted]

   * Alcohol. You may have heard that modest amounts of alcohol
     can improve your cholesterol profile by increasing your
     HDL-cholesterol level. However, it is not known whether
     the higher level produced by alcohol protects against
     coronary head disease. With this in mind and because
     drinking can have serious adverse effects, alcohol is not
     recommended in the prevention of coronary head disease.

   * Stress. Although stress has been reported to raise blood
     cholesterol levels, there may be other explanations for
     this effect. For example, during periods of stress, people
     may eat more foods that are high in saturated fat and
     cholesterol, which may increase their blood cholesterol
     levels -- rather than the stress itself.

     While all of these factors can influence your blood
cholesterol level, clearly you can do something about a number
of them. In fact, most people are able to lower their blood
cholesterol levels with diet alone.


Blood Cholesterol and Lipoproteins: What They Are


     Cholesterol is an odorless, soft, waxy substance. Your
body needs cholesterol to function normally (for example, as a
component of cell membranes and for the production of many
hormones, vitamin D, and bile acids -- which are important for
the absorption of fat). Cholesterol is present in all parts of
the body, including the brain and nervous system, muscle, skin,
liver, intestines, heart, skeleton, etc.


The cholesterol level of your blood is affected by:

   * The cholesterol your body produces and

   * The saturated fat and cholesterol in your diet.

[Graphic Omitted]

  Your blood cholesterol level is affected not only by the
saturated fat and cholesterol in your diet, but also by the
cholesterol your body produces. As a matter of fact, your
body produces all the cholesterol it needs, and the saturated
fat and cholesterol in your diet only serve to increase your
blood cholesterol level.


Is There "Good" and "Bad" Blood Cholesterol?


     Cholesterol travels in the blood in packages called
lipoproteins. All lipoproteins are formed in the liver and
carry cholesterol through the body.

     Blood cholesterol packaged in low density lipoproteins
(LDLs) is transported from the liver to other parts of the body
where it can be used. LDLs carry most of the cholesterol in the
blood, and if not removed from the blood, cholesterol and fat
can build up in the arteries contributing to atherosclerosis.
This is why LDL-cholesterol is often called "bad cholesterol."

     Cholesterol is also packaged in high density lipoproteins
(HDLs). HDLs carry cholesterol back to the liver for processing
or removal from the body. HDLs therefore help remove
cholesterol from the blood, preventing the accumulation of
cholesterol in the walls of the arteries. Thus they are often
referred to as "good cholesterol."


How are LDL and HDL Levels Determined?


     If your total cholesterol level is either in the "high"
category or in the "borderline-high" category and you have
coronary head disease or two other risk factors for coronary
heart disease, your doctor will want a more complete
"cholesterol profile" that includes LDL-cholesterol, HDL
cholesterol levels, and triglyceride levels. A blood test
provides this information: you will have to fast for 12 hours
prior to the test. (You may notice that your LDL -- and HDL
cholesterol values do not add up to your total blood
cholesterol level. LDLs usually carry about 60-70 percent and
HDLs about 25 percent of the total cholesterol in your blood.
Other lipoproteins carry the rest.)

     Some laboratories may calculate your cholesterol ratio.
This measurement is actually just your total cholesterol or
LDL-cholesterol divided by your HDL-cholesterol. For example,
if your LDL-cholesterol level is 140 mg/dl and your
HDL-cholesterol level is 35 mg/dl, your cholesterol ratio is 1
40/35, or 4. However, HDL-, LDL-, and total cholesterol levels
are independent predictors of your risk for coronary heart
disease. Because combining these values into a ratio can
conceal information useful to you and your physician, it is
more important to know each value separately.


What Do Your LDL and HDL Levels Mean?


     Along with your total blood cholesterol level, your LDL
and HDL levels provide more information on your risk of
developing coronary heart disease. A high LDL-cholesterol level
or a low HDL-cholesterol level puts you at increased risk.
LDL- and HDL-cholesterol levels more accurately predict your risk
for coronary heart disease than a total cholesterol level
alone.

     If your doctor measured your LDL-cholesterol level, use
the chart below to see how your LDL-cholesterol level measures
up.


Does Your Total Blood Cholesterol Increase Your Risk For
Developing Coronary Heart Disease?


    Desirable        Borderline-High Risk       High Risk

Less than 130 mg/gl    130 to 159 mg/dl     160 mg/dl and above


    If your LDL-cholesterol level is in the "desirable"
category, you are at an acceptable level of risk. If your LDL
level is in the "borderline-high risk" category, you could
benefit from lowering your blood cholesterol level by making
some dietary changes. If your LDL level is in the
"borderline-high risk" category and you have coronary heart
disease or two risk factors for coronary head disease, you
should begin diet treatment under your physician's supervision,
as should a person in the "high risk" category. In general,
this means you will be paying closer attention to your
cholesterol level and making more dietary changes than a person
at lower risk.

[Graphic Omitted]

     HDL-cholesterol will also be measured if your total blood
cholesterol puts you in a high risk category. The lower your
HDL-cholesterol level, the greater your risk for coronary head
disease. Any HDL-cholesterol level lower than 35 mg/dl is
considered too low. Quitting smoking, losing weight, and
becoming physically active may help raise your HDL-cholesterol
level. Although it is not known for certain that raising HDL
levels in this way will reduce the risk of coronary heart
disease, these measures are likely to be good for your heart in
any case.


Sample Lab Report


Patient      Sex  Age   Account     ID    Recieved    Reported
Kevin Stone   M    38   01300542   2944   25-Oct-87   26-Oct-87


Test Name             Result           Ranges
CHOL                   250       <200 mg/dl (desirable)
                                 200-239 mg/dl (borderline-high)
                                 >240 mg/dl (high)

HDL CHOL                43       <35 mg/dl (increased risk)
                                 >35 mg/dl (acceptable)

LDL CHOL               171       <130 mg/dl (desirable)
                                 130-159 mg/dl (borderline-risk)
                                 >160 mg/dl (high risk)


How To Lower Your High Blood Cholesterol


     The primary treatment for high blood cholesterol is a diet
that is low in saturated fat and low in cholesterol. This new
way of eating is also nutritious, with all the protein,
carbohydrate, fat, vitamins, and minerals your body needs. To
lower your blood cholesterol, you will:

   * Eat less high-fat food (especially those high in saturated
     fat);

   * Replace part of the saturated fat in your diet with
     unsaturated fat;

   * Eat less high-cholesterol food;

   * Choose foods high in complex carbohydrates (starch and
     fiber); and

   * Reduce your weight, if you are overweight.


     Saturated fats raise your cholesterol level more than
anything else in your diet. Dietary cholesterol also raises
blood cholesterol levels. Instead of eating foods rich in
saturated fat and cholesterol, try more breads, cereals, and
other foods high in complex carbohydrates as well as more
fruits and vegetables. Using unsaturated fats in place of
saturated fats can also help lower your blood cholesterol.

     Fortunately, these dietary changes work together. For
example, eating less saturated fat may also help you decrease
the amount of cholesterol you eat and may help you lose weight.
This is because foods high in saturated fat are often high in
cholesterol as well and are high in calories. In fact, all fats
have more than twice as many calories as either carbohydrate or
protein. And, by losing weight if you are overweight, you can
help lower your LDL-cholesterol level and increase your
HDL-cholesterol level.


What Foods Contain Fat and Cholesterol?


Saturated fats are found primarily in animal products,
particularly fatty meats and many dairy products.

[Graphic Omitted]

Coconut oil, palm kernel oil, and palm oil are also very
saturated.

[Graphic Omitted]

Some of the unsaturated fats in vegetable oils are also made
more saturated by a process called hydrogenation.

[Graphic Omitted]

Commercially prepared and processed foods made with these
vegetable oils or with saturated fats like butter and lard can
also be high in saturated fat.

[Graphic Omitted]

     There are two kinds of unsaturated fat: polyunsaturated
and monounsaturated. You should substitute both of these for
saturated fat in your diet. polyunsaturated fats are found
primarily in plant products -- including safflower, sunflower,
corn, soybean, and cottonseed oils; nuts; and seeds -- and in
fatty fish. Major vegetable oil sources of monounsaturated fats
are primarily olive oil and canola oil.

     Cholesterol is found only in foods of animal origin, both
high-fat foods (like hotdogs and cheddar cheese) and lowfat
foods (like liver and other organ meats). And the amount of
cholesterol in these foods varies. A daily intake of less than
300 mg is recommended. A 3-ounce piece of meat, fish, or
poultry has 60-90 mg of cholesterol; one egg yolk contains
about 270 mg; and a 3-ounce serving of liver has about 390 mg
of cholesterol.


What Are Some Practical Guidelines for Changing Your Diet?


     Again, to reduce your blood cholesterol level, your diet
should be low in fat, particularly saturated fat, and low in
cholesterol. Use the following guidelines as you plan your new
diet.


  1. To cut back on saturated fats:

[Graphic omitted]

   * Choose poultry, fish and lean cuts of meat more often;
     remove the skin from chicken and trim the fat from the
     meat.

   * Drink skim milk or 1% milk instead of 2% milk or whole
     milk. And eat cheeses with no more than 2-6 gram of fat
     per ounce (like low-fat cottage or low-fat farmer cheese)
     instead of processed, natural, and hard cheeses (like
     American, brie, and cheddar).

   * Use tub margarines or liquid vegetable oils that are high
     in unsaturated fat (like sunflower, corn, and olive oil)
     instead of butter, lard, and hydrogenated vegetable
     shortening that are high in saturated fat. Choose products
     that list more unsaturated fat than saturated fat on the
     label.

   * Cut down on commercially prepared and processed foods made
     with saturated fats or oils. Read labels to choose those
     low in saturated fats.


  2. To cut back on dietary cholesterol:


   * Eat less organ meat such as liver, brain, and kidney.

   * Eat fewer egg yolks; try substituting two egg whites for
     each whole egg in recipes.


  3. To increase complex carbohydrates (starch and fiber):


   * Eat more whole grain breads and cereals, pasta, rice, and
     dried peas and beans.

   * Eat vegetables and fruits more often.

[Graphic omitted]

  4. To lose weight:


   * Eat fewer daily calories (cutting back on fat in your diet
     will really help):

   * Burn extra calories by exercising regularly.


What You Can Expect


How Soon and How Much Can You Expect Your Blood Cholesterol
Levels to Change?


     Remember, by closely following your diet and monitoring
your progress with regular checkups, you can lower your blood
cholesterol level and greatly reduce your risk of developing
coronary heart disease.

     Generally, both your total and LDL-cholesterol levels will
begin to drop 2-3 weeks after you begin your cholesterol
lowering diet. Over time, you may reduce your cholesterol
levels by 30-55 mg/dl or even more. The more you reduce your
level, the more you will reduce your risk of developing
coronary heart disease.

[Graphic Omitted]

     How much you reduce your blood cholesterol levels depends
on how much fat, specifically saturated fat, and how much
cholesterol you were eating before starting your
cholesterol-lowering diet; how well you follow your new diet;
and how responsive your body is to the diet. Also, the higher
your blood cholesterol level is to begin with, the greater or
more dramatic reduction you can expect with your new diet.


How Long Do You Need to Follow This Diet?


     Your new cholesterol-lowering diet should be continued for
life. While eating some foods high in saturated fat and
cholesterol for 1 day or at one meal will not raise blood
cholesterol levels, resuming old eating patterns will.
Surprisingly, after a while your new way of eating won't seem
like a "diet" at all, but simply like your regular
routine--full of appealing and appetizing foods.


What Should Your Blood Cholesterol Goal Be?


     Before starting your cholesterol-lowering diet, your
physician will determine your blood cholesterol goal,
specifically the LDL-cholesterol level, that is right for you.
This goal will vary depending on whether you have coronary
heart disease or any of the other risk factors for coronary
head disease. Even though achieving your LDL-cholesterol goal
is more important that the total cholesterol goal, your
physician can check your progress by measuring your total
cholesterol level because it is a good deal simpler and because
you do not have to fast before its measurement. Remember, a
total cholesterol level below 200 mg/dl and an LDL-cholesterol
below 130 mg/dl are desirable.


How Do You Check your progress?


     To get your blood cholesterol measured, see your doctor or
local clinic. They will take a blood sample and send it to a
laboratory. There may be differences in your blood cholesterol
level from day to day. Cholesterol measurements may also differ
somewhat from one laboratory to the next. Your doctor will
consider these factors when you return to check your progress.
It is important that you have your cholesterol level measured
at the same place each time.


How Often Should You Have Your Blood Cholesterol Levels
Checked?


     Your physician will probably want to measure your
cholesterol level after you have been on the diet for 4-6 weeks
and again after 3 months. If the total cholesterol goal is met
after 3 months, measuring your LDL-cholesterol level will
confirm that the LDL-cholesterol goal has been met. If your
response to the diet has been satisfactory (both total
cholesterol and LDL-cholesterol goals have been met), you will
enter a phase of long-term monitoring. Long-term monitoring may
involve remeasuring total cholesterol twice a year and
LDL-cholesterol once a year.

     If you have not met your blood cholesterol goal in 3
months, your doctor may further restrict the saturated fat and
cholesterol in your diet and enlist the help of a dietitian.
Then, after 4-6 weeks more and again after 3 months, your
doctor may measure your cholesterol level. If you have attained
your goal, long-term monitoring can begin. If not, your doctor
may decide you need medication along with your dietary changes.

[Graphic Omitted]

Can You Eat Before Your Test?


     Since total cholesterol levels do not change much after a
meal, total cholesterol can be measured at any time of day,
whether or not you have just eaten. Therefore, you do not need
to fast. However, you should not eat or drink anything except
water or black coffee for at least 12 hours before having your
lipoprotein levels determined (LDL, HDL, triglycerides).
Therefore, it may be convenient to eat your last meal at about
9 p.m. and have your test the next day before breakfast.


Other Steps You Can Take


     While dietary change is the first and most important
action you will be taking to lower your blood cholesterol
level, your doctor may suggest other steps you can take. These
will depend on how well the diet lowers your blood cholesterol
level and whether or not you have any other risk factors for
coronary head disease.


Do You Need to Take Special Medications Along With Your Diet?.


     In most cases, a blood cholesterol-lowering diet is the
only step necessary to lower blood cholesterol levels. However,
if your LDL-cholesterol level is still too high after you've
been on your diet for 6 months, your doctor may decide to
include medication as part of your treatment. In addition, if
your cholesterol level is unusually high or if you have other
major risk factors for coronary heart disease, your doctor may
prescribe medications to lower blood cholesterol even sooner.

     If your doctor does prescribe medications, you must
continue your cholesterol-lowering diet since the combination
may allow you to take less medication to lower your levels.
And, because diet is still the safest treatment, you should
always try to lower your levels with diet alone before adding
medication.


What Are These Medications?


     There are several medications your physician can prescribe
to help you lower your blood cholesterol levels. The report
issued by the National Cholesterol Education Program cited the
bile acid sequestrants--cholestyramine and colestipol--and
nicotinic acid as the drugs of first choice, The report
underscored the effectiveness and the long-term safety of these
drugs as demonstrated in research studies. The report also
cited a new class of drug--HMG CoA reductase--which has
demonstrated considerable effectiveness in lowering cholesterol
levels. One drug in this class--lovastatin--has been approved
for use by the Food and Drug Administration. Because of its
newness, long-term safety data has not yet been established.
Other drugs cited in the report which were not considered as
efficacious in lowering LDL-cholesterol as those mentioned
above include gemfibrozil and probucol. All
cholesterol-lowering medications should be taken only with the
advice of and under the supervision of your physician.


Do You Need to Make Other Changes to Lower Your Blood
Cholesterol Level?


     The key changes you need to make are the ones discussed
above. To review:

   * Follow a diet that is low in saturated fat and
     cholesterol. This is the most important step you will take
     to reduce your high blood cholesterol level.

   * Maintain a desirable weight. If you need to lose weight,
     your new diet will help since it is likely to be lower in
     calories than your current diet.

   * Finally, exercise to help you lose weight and improve
     overall physical fitness. Exercise may also help you raise
     your HDL level.

     Unless your doctor prescribes a cholesterol-lowering
medication, these are the only steps you will need to take to
lower your blood cholesterol level.


What Else Can You Do to Lower Your Risk for Coronary Heart
Disease?


     Fortunately, you can do something about all three of the
major risk factors for coronary head disease-high blood
cholesterol, cigarette smoking, and high blood pressure. Thus,
in addition to lowering your blood cholesterol level, it's a
good idea to quit smoking and control your blood pressure.
Maintaining your desirable weight will also help you lower your
risk for coronary head disease. And all of these steps will
help you to feel better.


Where You Can Go for Help


     Need more help? Want to know more? There are many places
you can go to get information about your new diet, your
diagnosis, and the latest findings about treatment and
medications for high blood cholesterol.


What Other Health Professionals, in Addition to Your Doctor,
Can Give You Help?

[Graphic Omitted]

     If you want some help following your recommended diet,
talk to a registered dietitian or qualified nutritionist. They
can explain the diet to you in greater detail and show you ways
to follow it. They can give you advice on shopping and
preparing foods, eating away from home, and changing your
eating habits to help you stay on your new diet. They will also
help you set goals for dietary change so that you can
successfully lower your high blood cholesterol levels without
drastically changing your eating pattern and overall lifestyle
all at one time. The Division of Practice of the American
Dietetic Association [[phone]] can help you find a
registered dietitian in your area. State and local branches of
the American Dietetic Association,your local hospital, or your
doctor can recommend a dietitian for you.

     There are also other resources. The nurse in your doctor's
office can answer questions you may have about your high blood
cholesterol or your new diet. If your blood cholesterol level
is not lowered by diet and medication, your doctor may refer
you to a physician who is a lipid specialist. Lipid specialists
are experts in the management of high blood cholesterol and
other lipid disorders. If you have questions about drug therapy
or the medication your doctor is prescribing, ask your doctor.
Finally, pharmacists are also aware of the best ways to take
medication, of ways to minimize side effects, and of the latest
research about specific drugs.


How Can You Get More Materials About High Blood Cholesterol?


     If you would like more information to help you start your
new approach to healthy eating, contact the National
Cholesterol Education Program (NCEP) of the National Heart,
Lung, and Blood Institute, Another NCEP pamphlet that can help
you, Eating to Lower Your High Blood Cholesterol, provides more
specific information on how to lower your blood cholesterol
levels through diet. NCEP also has developed a resource list of
agencies and organizations that can answer your questions.
These and other materials can be requested by writing:

National Cholesterol Education Program
National Head, Lung, and Blood Institute
C-200
Bethesda, MD 20892


Glossary


  1. Atherosclerosis -- A type of "hardening of the arteries" in
     which cholesterol, fat, and other blood components build
     up in the walls of arteries. As atherosclerosis
     progresses, the arteries to the heart may narrow so that
     oxygen-rich blood and nutrients have difficulty reaching
     the heart.

  2. Bile acid sequestrants -- One type of cholesterol lowering
     medication, including cholestyramine and colestipol. The
     sequestrants bind with cholesterol containing bile acids
     in the intestine and remove them in bowel movements.

  3. Carbohydrate -- One of the three nutrients that supply
     calories (energy) to the body. Carbohydrate provides 4
     calories per gram -- the same number of calories as pure
     protein and less than half the calories of fat.
     Carbohydrate is essential for normal body function. There
     are two basic kinds of carbohydrate -- simple carbohydrate
     (or sugars) and complex carbohydrate (starches and fiber).
     In nature, both the simple sugars and the complex starches
     come packaged in foods like oranges, apples, corn, wheat,
     and milk. Refined or processed carbohydrates are found in
     cookies, cakes, and pies.

     * Complex carbohydrate -- Starch and fiber. Complex
       carbohydrate comes from plants. When complex carbohydrate
       is substituted for saturated fat, the saturated fat
       reduction lowers blood cholesterol. Foods high in starch
       include breads, cereals, pasta, rice, dried beans and
       peas, corn, and lima beans.

     * Fiber -- A nondigestible type of complex carbohydrate.
       High-fiber foods are usually low in calories. Foods high
       in fiber include whole grain breads and cereals, whole
       fruits, and dried beans. The type of fiber found in foods
       such as oat and barley bran, some fruits like apples and
       oranges, and some dried beans may help reduce blood
       cholesterol.

  4. Cholesterol -- A soft, waxy substance. It is made in
     sufficient quantity by the body for normal body function,
     including the manufacture of hormones, bile acid, and
     vitamin D. It is present in all pads of the body,
     including the nervous system, muscle, skin, liver,
     intestines, head, etc.

     * Blood cholesterol -- Cholesterol that is manufactured in
       the liver and absorbed from the food you eat and is
       carried in the blood for use by all parts of the body. A
       high level of blood cholesterol leads to atherosclerosis
       and coronary heart disease.

     * Dietary cholesterol -- Cholesterol that is in the food
       you eat. It is present only in foods of animal origin,
       not in foods of plant origin. Dietary cholesterol, like
       dietary saturated fat, tends to raise blood cholesterol,
       which increases the risk for heart disease.

  5. Coronary head disease -- Heart ailment caused by narrowing
     of the coronary arteries (arteries that supply oxygen and
     nutrients directly to the head muscle). Coronary heart
     disease is caused by atherosclerosis, which decreases the
     blood supply to the head muscle. The inadequate supply of
     oxygen-rich blood and nutrients damages the head muscle
     and can lead to chest pain, heart attack, and death.

  6. Fat -- One of the three nutrients that supply calories to
     the body. Fat provides 9 calories per gram, more than
     twice the number provided by carbohydrate or protein. In
     addition to providing calories, fat helps in the
     absorption of certain vitamins. Small amounts of fat are
     necessary for normal body function.

     * Total fat -- The sum of the saturated, monounsaturated,
       and polyunsaturated fats present in food. A mixture of
       all three in varying amounts is found in most foods.

     * Saturated fat -- A type of fat found in greatest amounts
       in foods from animals such as meat, poultry, and
       whole-milk dairy products like cream, milk, ice cream,
       and cheese. Other examples of saturated fat include
       butter, the marbling and fat along the edges of meat, and
       lard and the saturated fat content is high in some
       vegetable oils-like coconut, palm kernel, and palm oils.
       Saturated fat raises blood cholesterol more than anything
       else in the diet.

     * Unsaturated fat -- A type of fat that is usually liquid
       at refrigerator temperature. Monounsaturated fat and
       polyunsaturated fat are two kinds of unsaturated fat.

     * Monounsatumted fat -- A slightly unsaturated fat that is
       found in greatest amounts in foods from plants, including
       olive and canola (rapeseed) oil. When substituted for
       saturated fat, monounsaturated fat helps reduce blood
       cholesterol.

     * Polyunsaturated fat -- An highly unsaturated fat that is
       found in greatest amounts in foods from plants, including
       safflower, sunflower, corn, and soybean oils. When
       substituted for saturated fat, polyunsaturated fat helps
       reduce blood cholesterol.

  7. Gram (g) -- A unit of weight. There are about 28 g in 1
     ounce. Dietary fat, protein, and carbohydrate are measured
     in grams.

  8. Hydrogenation -- A chemical process that changes liquid
     vegetable oils (unsaturated fat)into a more solid
     saturated fat. This process improves the shelf life of the
     product -- but also increases the saturated fat content.
     Many commercial food products contain hydrogenated
     vegetable oil. Selection should be made based on
     information found on the label.

  9. Lipids -- Fatty substances, including cholesterol and
     triglycerides, that are present in blood and body tissues.

 10. Lipoproteins -- Protein-coated packages that carry fat and
     cholesterol through the blood. Lipoproteins are classified
     according to their density.

     * High density lipoproteins (HDL) -- Lipoproteins that
       contain a small amount of cholesterol and carry
       cholesterol away from body cells and tissues to the liver
       for excretion from the body. Low levels of HDL are
       associated with an increased risk of coronary head
       disease. Therefore the higher the HDL level, the better.

     * Low density lipoproteins (LDL) -- Lipoproteins that
       contain the largest amount of cholesterol in the blood.
       LDL is responsible for depositing cholesterol in the
       artery walls. High levels of LDL are associated with an
       increased risk of coronary head disease and are therefore
       referred to as "bad cholesterol."

 11. Milligram (mg) -- A unit of weight equal to one thousandth
     of a gram. There are about 28,350 mg in 1 ounce. Dietary
     cholesterol is measured in milligrams.

 12. Milligrams/deciliter (mg/dl) -- A way of expressing
     concentration: in blood cholesterol measurements, the
     weight of cholesterol (in milligrams)in a deciliter of
     blood. A deciliter is about one-tenth of a quart.

 13. Niacin -- A B vitamin essential for energy production in
     cells. The recommended daily allowance is about 14 mg for
     adult females and about 18 mg for adult males. When used
     in massive quantities under a physician's guidance, niacin
     is considered a cholesterol-lowering medication.

 14. Protein -- One of the three nutrients that supply calories
     to the body. Protein provides 4 calories per gram, which
     is less than half the calories of fat. Protein is an
     essential nutrient that becomes a component of many parts
     of the body, including muscle, bone, skin, and blood.

 15. Risk factor -- A habit, trait, or condition in a person
     that is associated with an increased chance (or risk) of
     developing a disease.

 16. Triglycerides -- Lipids (fat-like substances) carried
     through the bloodstream to the tissues. The bulk of the
     body's fat tissue is in the form of triglycerides, stored
     for later use as energy. We get triglycerides primarily
     from the fat in our diet.

 17. Vascular disease -- An ailment of the blood vessels often
     caused by atherosclerosis. Vascular disease may occur in
     the arteries to the brain and the major leg arteries.


     Limited quantities of this brochure are available free of
charge.

National Cholesterol Education Program
National Heart, Lung, and Blood Institute
C-200
Bethesda, Maryland 20892
!
*
             Study confirms lowering cholesterol can save lives

By Craig M. Walker, M.D.
Medical Director,
Cardiocascular Institute of the South

A Scandinavian study has confirmed that bringing the cholesterol levels of
heart patients under control could prolong their lives.

     We've known for a long time that there was a statistical link between
     high blood levels of cholesterol and the accumulation of cholesterol
     plaque in the coronary arteries -- the cause of most heart pain and
     heart attacks. About one-fourth of heart patients in this country take
     one of four similar drugs to control the problem. However, some
     physicians have been reluctant to prescribe them because there was
     little independent research to prove that these fairly expensive
     medications really had much effect.

     The study by doctors at 94 hospitals in Denmark, Finland, Iceland,
     Norway and Sweden appears to provide that proof. The study involved
     4,444 men and women who had heart-related chest pain (angina) or who
     had previously suffered heart attacks. They were randomly assigned to
     receive either the drug simvastatin or a placebo -- an
     identical-looking pill containing no medication.

     After five years, it was found that the overall death rate among those
     receiving the cholesterol-fighting drug was 30 percent lower and the
     death rate from heart disease was 42 percent lower than it was among
     those receiving the placebo.

     Those receiving simvastatin also needed 37 percent fewer coronary
     bypass surgeries or angioplasties than those in the placebo group.

     It's estimated that nine out of every 100 heart attack and angina
     patients will die over a six-year period. The physicians who conducted
     the Scandinavian study estimate that the use of
     cholesterol-controlling drugs could prevent four of those deaths. One
     of the study's authors went so far as to say that controlling
     cholesterol could be as important as stopping smoking in preventing
     further heart attacks.

     While these medications aren't cheap, they are far less expensive than
     bypass surgery or balloon angioplasty required to clear the blockages
     they can prevent. So their appropriate use should help reduce medical
     costs.

     The study does not address whether these medications have a similar
     benefit for patients who have high cholesterol, but who have not had
     heart attacks or angina. So the effectiveness -- or, at least, the
     cost-effectiveness -- of their use in such patients still might be
     debated.

     Nevertheless, the study underscores for all of us the importance of
     controlling cholesterol -- by diet and exercise or medication --
     before a heart attack or other manifestation of coronary artery
     disease compels us to do so.

!
*
        You can control high cholesterol, and the sooner, the better

By Craig M. Walker, M.D.
Medical Director,
Cardiocascular Institute of the South

Here's the problem with cholesterol: you're feeding cheeseburgers to a body
that thinks you're a caveman living on roots, nuts and berries, with just
the occasional mastodon steak thrown in. It still behaves as if obtaining
cholesterol -- a vital nutrient -- is a much bigger problem than getting
rid of it.

     In our prosperous society with its fat and cholesterol-rich diet, the
     reverse is actually true, and it is a concern for everyone, not just
     for the middle-aged and elderly. Vietnam War era studies found that a
     significant number of physically fit American men in their late teens
     and early 20s had already developed advanced levels of atherosclerosis
     -- the buildup of waxy cholesterol plaque in arteries that ultimately
     leads to heart attacks.

     We're more aware today of the risks posed by excessive cholesterol, a
     form of fat which comes entirely from animal products, and saturated
     fats, which have both animal and vegetable sources. Most people have
     even heard that there is a "good cholesterol" (HDL) and a "bad
     cholesterol" (LDL).

     Unfortunately, that effort to provide a simple explanation of
     cholesterol measurement actually misrepresents it. HDL and LDL aren't
     cholesterol, and both are vital to our survival. They are
     lipoproteins, a low-density form (LDL), which transports cholesterol
     to the body's cells, and a high-density form (HDL), which carries away
     excess cholesterol the cells don't use. Chemically, they are
     detergents -- molecules which have one fat-soluble (lipo) end and one
     water-soluble (protein) end that allow them to hold fats in suspension
     in a water-based medium -- blood -- which, by itself, couldn't
     dissolve or transport them.

     What we call cholesterol measurement actually measures the levels of
     these two transporting detergents, not the cholesterol itself.
     Theoretically, the higher the level of HDL, which carries away excess
     cholesterol, compared to LDL, which carries it to the cells, the less
     likely there will be excess cholesterol left around to start building
     up plaque accumulations in our arteries.

     You can, with some effort, actually improve your own HDL-to-LDL ratio.
     Aerobic exercise -- jogging, walking or any other regimen which
     features lots of motion against little resistance -- can be one of the
     biggest factors in shifting your body's LDL /HDL ratio in your favor.
     Losing weight can also have a positive effect.

     And, yes, there is evidence that alcohol, in moderation, can have some
     small beneficial effect in raising HDL levels. But the important word
     in this regard is moderation. If your alcohol consumption exceeds one
     or two drinks a day, you can raise your blood pressure and increase
     the levels of fatty triglycerides in your blood stream, thus undoing
     the benefit of more restrained consumption.

     It has been learned recently that a third lipoprotein, called LP(a),
     figures in the type of high cholesterol that runs in some families
     with a high incidence of atherosclerotic cardiovascular disease and in
     some ethnic groups, including African-Americans. If you fall into one
     of these high-risk groups, it would be worthwhile for you to schedule
     a special test, called a vertical analytical profile, which can detect
     elevated LP(a) levels.. LP(a)-related high cholesterol can be
     controlled by physician-monitored administration of niacin, one of the
     B-complex vitamins.

     If none of these remedies suffice to bring your cholesterol level into
     the "safe" zone, there are a number of medications your doctor can
     prescribe that are effective in controlling the condition.

     I almost hesitate to mention that a sufficiently high level of HDL can
     even slightly reduce plaque accumulation in arteries, because so many
     patients make too much of the fact. HDL isn't some sort of biological
     Drano that can clear out badly clogged arteries. And you can't
     administer it like a drug. You have to make your own -- the hard way.
!
*

Chronic Pain: Hope Through Research

U.S. Department of Health and Human Services
Public Health Service
National Institutes of Health
National Institute of Neurological Disorders and Stroke


     What was the worst pain you can remember?  Was it the time
you scratched the cornea of your eye?  Was it a kidney stone?
Childbirth?  Rare is the person who has not experienced some
beyond-belief episode of pain and misery.  Mercifully, relief
finally came.  Your eye healed, the stone was passed, the baby
born.  In each of those cases pain flared up in response to a
known cause.  With treatment, or with the body's healing powers
alone, you got better and the pain went away.  Doctors call that
kind of pain acute pain.  It is a normal sensation triggered in
the nervous system to alert you to possible injury and the need
to take care of yourself.

     Chronic pain is different.  Chronic pain persists.
Fiendishly, uselessly, pain signals keep firing in the nervous
system for weeks, months, even years.  There may have been an
initial mishap-a sprained back, a serious infection-from which
you've long since recovered.  There may be an ongoing cause of
pain-arthritis, cancer, ear infection.  But some people suffer
chronic pain in the absence of any past injury or evidence of
body damage.  Whatever the matter may be, chronic pain is real,
unremitting, and demoralizing-the kind of pain New England poet
Emily Dickinson had in mind when she wrote:

     Pain-has an Element of Blank-
     It cannot recollect
     When it begun-or if there were
     A time when it was not

The terrible triad

     Pain of such proportions overwhelms all other symptoms and
becomes the problem.  People so afflicted often cannot work.
Their appetite falls off.  Physical activity of any kind is
exhausting and may aggravate the pain.  Soon the person becomes
the victim of a vicious circle in which total preoccupation with
pain leads to irritability and depression.  The sufferer can't
sleep at night and the next day's weariness compounds the
problem-leading to more irritability, depression, and pain.
Specialists call that unhappy state the "terrible triad" of
suffering, sleeplessness, and sadness, a calamity that is as hard
on the family as it is on the victim.  The urge to do
something-anything-to stop the pain makes some patients drug
dependent, drives others to undergo repeated operations or worse,
resort to questionable practitioners who promise quick and
permanent "cures."

     "Chronic pain is the most costly health problem in America,"
says one of the world's authorities on pain.  He and others
estimate annual costs, including direct medical expenses, lost
income, lost productivity, compensation payments and legal
charges, at close to $50 billion.  Here's how that adds up:

     * Headache.  At least 40 million Americans suffer chronic
recurrent headaches and spend $4 billion a year on medications.
Migraine sufferers alone account for 65 million workdays lost
annually.

     * Low back pain.  Fifteen percent of the adult U.S.
population have had persistent low back pain at some time in
their lives.  Five million Americans are partially disabled by
back problems, and another 2 million are so severely  disabled
they cannot work.  Low back pain accounts for 93 million workdays
lost every year and costs over $5 billion in health care.

     * Cancer pain.  The majority of patients in intermediate or
advanced stages of cancer suffer moderate to severe pain.  More
than 800,000 new cases of cancer are diagnosed each year in the
U.S., and some 430,000 people die.

     * Arthritis pain.  The great crippler affects 20 million
Americans and costs over $4 billion in lost income, productivity
and health care.

     Other pain disorders like the neuralgias and neuropathies
that affect nerves throughout the body, pain due to damage to the
central nervous system (the brain and spinal cord), as well as
pain where no physical cause can be found-psychogenic pain-swell
the total to that $50 billion figure.

     Many chronic pain conditions affect older adults.
Arthritis, cancer, angina-the chest-binding, breath-catching
spasms of pain associated with coronary artery disease-commonly
take their greatest toll among the middle-aged and elderly.  Tic
douloureux   (trigeminal neuralgia) is a recurrent, stabbing
facial pain that is  rare among young adults.  But ask any
resident of housing for retired persons if there are any tic
sufferers around and you are sure to hear of cases.  So the fact
that Americans are living longer contributes to a widespread and
growing concern about pain.

     Neuroscientists share that concern.  At a time when people
are living longer and painful conditions abound, the scientists
who study the brain have made landmark discoveries that are
leading to a better understanding of pain and more effective
treatments.

     In the forefront of pain research are scientists supported
by the National Institute of Neurological Disorders and Stroke
(NINDS), the leading Federal agency supporting research on pain.
Other Federal agencies important in pain research include the
National Institute of Mental Health (NIMH), the National
Institute of Dental Research (NIDR) and the National Cancer
Institute (NCI).  Within the last decade both the International
Association for the Study of Pain and the American Pain Society
have been established and grown into flourishing professional
organizations attracting young as well as established research
investigators and practicing
physicians.


Sounding the pain alarm

     Part of the inspiration for the new groups has come from a
deeper understanding of pain made possible by advances in
research techniques.  Not long ago neuroscientists debated
whether pain was a separate sense at all, supplied with its own
nerve cells and brain centers like the senses of hearing or taste
or touch.  Maybe you hurt, the scientists reasoned, because nerve
endings sensitive to touch are pressed very hard.  To some
extent, that is true: Some nerve fibers in your skin will be
stimulated by a painful pinch as well as a gentle touch.  But
neuroscientists now know that there are many small nerve cells
with extremely fine nerve fibers that are excited exclusively by
intense, potentially harmful stimulation.  Scientists call the
nerve cells nociceptors, from the word noxious, meaning
physically harmful or destructive.

     Some nociceptors sound off to several kinds of painful
stimulations hammer blow that hits your thumb instead of a nail;
a drop of acid; a flaming match.  Other nociceptors are more
selective.  They are excited by a pinprick but ignore painful
heat or chemical stimulation.  It's as though nature had
sprinkled your skin and your insides with a variety of
pain-sensitive cells, not only to report what kind of damage
you're experiencing, but to make sure the message gets through on
at least one channel.

Broadcasting the news

     That same dispersion of forces continues once pain messages
reach the central nervous system.  Suppose you touch a hot stove.
Some incoming pain signals are immediately routed to nerve cells
that signal muscles to contract, so you pull your hand back.
That streamlined pathway is a reflex, one of many protective
circuits wired into your nervous system at birth.

     Meanwhile the message informing you that you've touched the
stove travels along other pathways to higher centers in the
brain.  One path is an express route that reports the facts:
where it hurts; how bad it is; whether the pain is sharp or
burning.  Other pain pathways plod along more slowly, the nerve
fibers branching to make connections with many nerve cells
(neurons) en route.  Scientists think that these more meandering
pathways act as warning systems alerting you of impending damage
and in other ways filling out the pain picture.  All the pathways
combined contribute to the emotional impact of pain-whether you
feel frightened, anxious, angry, annoyed.  Experts called those
feelings the "suffering" component of pain.

     Still other branches of the pain news network are alerting
another major division of the nervous system, the autonomic
nervous system.  That division handles the body's vital functions
like breathing, blood flow, pulse rate, digestion, elimination.
Pain can sound a general alarm in that system, causing you to
sweat or stop digesting your food, increasing your pulse rate and
blood pressure, dilating the pupils of your eye, and signaling
the release of hormones like epinephrine (adrenaline).
Epinephrine aids and abets all those responses as well as
triggering the release of sugar stored in the liver to provide an
extra boost of energy in an emergency.

Censoring the news

     Obviously not every source of pain creates a full-blown
emergency with adrenaline-surging, sweat-pouring, pulse-racing
responses.  Moreover, observers are well aware of times and
places when excruciating pain is ignored.  Think of the
quarterback's ability to finish a game oblivious of a torn
ligament, or a fakir sitting on a bed of spikes.  One of the
foremost pioneers in pain research adds his personal tale, too,
of the time he landed a salmon after a long and hearty struggle,
only then to discover the deep blood-dripping gash on his leg.

     Acknowledging such events, neuroscientists have long
suspected that there are built-in nervous system mechanisms that
can block pain messages.

     Now it seems that just as there is more than one way to
spread the news of pain, there is more than one way to censor the
news.  These control systems involve pathways that come down from
the brain to prevent pain signals from getting through.


The gate theory of pain

     Interestingly, a pair of Canadian and English investigators
speculated that such pain-suppressing pathways must exist when
they devised a new "gate theory of pain" in the midsixties.
Their idea was that when pain signals first reach the nervous
system they excite activity in a group of small neurons that form
a kind of pain "pool."  When the total activity of these neurons
reaches a certain minimal level, a hypothetical "gate" opens up
that allows the pain signals to be sent to higher brain centers.
But nearby neurons in contact with the pain cells can suppress
activity in the pain pool so that the gate stays closed.  The
gate-closing cells include large neurons that are stimulated by
nonpainful touching or pressing of your skin.  The gate could
also be closed from above, by brain cells activating a descending
pathway to block pain.

     The theory explained such everyday behavior as scratching a
scab, or rubbing a sprained ankle: the scratching and rubbing
excite just those nerve cells sensitive to touch and pressure
that can suppress the pain pool cells.  The scientists
conjectured that brain-based pain control systems were activated
when people behaved heroically-ignoring pain to finish a football
game, or to help a more severely wounded soldier on the
battlefield.

     The gate theory aroused both interest and controversy when
it was first announced.  Most importantly, it stimulated research
to find the conjectured pathways and mechanisms.  Pain studies
got an added boost when investigators made the surprising
discovery that the brain itself produces chemicals that can
control pain.

     The landmark discovery of the pain-suppressing chemicals
came about because scientists in Aberdeen, Scotland, and at the
Johns Hopkins University Hospital in Baltimore were curious about
how morphine and other opium-derived painkillers, or analgesics,
work.

     For some time neuroscientists had known that chemicals were
important in conducting nerve signals (small bursts of electric
current) from cell to cell.  In order for the signal from one
cell to reach the next in line, the first cell secretes a
chemical "neurotransmitter" from the tip of a long fiber that
extends from the cell body.  The transmitter molecules cross the
gap separating the two cells and attach to special receptor sites
on the neighboring cell surface.  Some neurotransmitters excite
the second cell-allowing it to generate an electrical signal.
Others inhibit the second cell--preventing it from
generating a signal.

     When investigators in Scotland and at Johns Hopkins injected
morphine into experimental animals, they found that the morphine
molecules fitted snugly into receptors on certain brain and
spinal cord neurons.  Why, the scientists wondered, should the
human brain-the product of millions of years of evolution-come
equipped with receptors for a man-made drug?  Perhaps there were
naturally occurring brain chemicals that behaved exactly like
morphine.

The brain's own opiates

     Both groups of scientists found not just one
pain-suppressing chemical in the brain, but a whole family of
such  proteins.  The Aberdeen investigators called the smaller
members of the family enkephalins (meaning "in the head").  In
time, the larger proteins were isolated and called endorphins,
meaning the "morphine within."  The term endorphins is now often
used to describe the group as a whole.

     The discovery of the endorphins lent weight to the general
concept of the gate theory.  Endorphins released from brain nerve
cells might inhibit spinal cord pain cells through pathways
descending from the brain to the spinal cord.  Endorphins might
also be activated when you rub or scratch your itching skin or
aching joints.  Laboratory experiments subsequently confirmed
that painful stimulation led to the release of endorphins from
nerve cells.  Some of these chemicals then turned up in
cerebrospinal fluid, the liquid that circulates in the spinal
cord and brain.  Laced with endorphins, the fluid could bring a
soothing balm to quiet nerve cells.

A new look at pain treatments

     Further evidence that endorphins figure importantly in pain
control comes from a new look at some of the oldest and newest
pain treatments.  The new look frequently involves the use of a
drug that prevents endorphins and morphine from working.
Injections of this drug, naloxone, can result in a return of pain
which had been relieved by morphine and certain other treatments.
But, interestingly, some pain treatments are not affected by
naloxone: Their success in controlling pain apparently does not
depend on endorphins.  Thus nature has provided us with more than
one means of achieving pain relief.

     * Acupuncture.  Probably no therapy for pain has stirred
more controversy in recent years than acupuncture, the
2,000-year-old Chinese technique of inserting fine needles under
the skin at selected points in the body.  The needles are
agitated by the practitioner to produce pain relief which some
individuals report lasts for hours, or even days.  Does
acupuncture really work?  Opinion is divided.  Many specialists
agree that patients report benefit when the needles are placed
near where it hurts, not at the body points indicated on
traditional Chinese acupuncture charts.  The case for acupuncture
has been made by investigators who argue that local needling of
the skin excites endorphin systems of pain control.  Wiring the
needles to stimulate nerve endings electrically
(electroacupuncture) also activates endorphin systems, they
believe.  Further, some experiments have shown that there are
higher levels of endorphins in cerebrospinal fluid following
acupuncture.

     Those same investigators note that naloxone injections can
block pain relief produced by acupuncture.  Others have not been
able to repeat those findings.  Skeptics also cite long-term
studies of chronic pain patients that showed no lasting benefit
from acupuncture treatments.  Current opinion is that more
controlled trials are needed to define which pain conditions
might be helped by acupuncture and which patients are most likely
to benefit.

     * Local electrical stimulation.  Applying brief pulses of
electricity to nerve endings under the skin, a procedure called
transcutaneous electrical nerve stimulation (TENS), yields
excellent pain relief in some chronic pain patients.  The
stimulation works best when applied to the skin near where the
pain is felt and where other sensibilities like touch or pressure
have not been damaged. Both the frequency and voltage of the
electrical stimulation are important in obtaining pain relief.

     * Brain stimulation.  Another electrical method for
controlling pain, especially the widespread and severe pain of
advanced cancer, is through surgically implanted electrodes in
the brain.  The patient determines when and how much stimulation
is needed by operating an external transmitter that beams
electronic signals to a receiver under the skin that is connected
to the electrodes.  The brain sites where the electrodes are
placed are areas known to be rich in opiate receptors and in
endorphin-containing cells or fibers.  Stimulation-produced
analgesia (SPA) is a costly procedure that involves the risk of
brain surgery.  However, patients who have used this technique
report that their pain "seems to melt away."  The pain relief is
also remarkably specific: The other senses remain intact, and
there is no mental confusion or cloudiness as with opiate drugs.
NINDS is currently supporting research on how SPA works and is
also investigating problems of tolerance: Pain may return after
repeated stimulation.

     * Placebo effects.  For years doctors have known that a
harmless sugar pill or an injection of salt water can make many a
patient feel better-even after major surgery.  The placebo
effect, as it has been called, has been thought to be due to
suggestion, distraction, the patient's optimism that something is
being done, or the desire to please the doctor (placebo means "I
will please" in Latin).

     Now experiments suggest that the placebo effect may be
neurochemical, and that people who respond to a placebo for pain
relief-a remarkably consistent 35 percent in any experiment using
placebos-are able to tap into their brains' endorphin systems.
To evaluate it, two NINDS- and NIDR-supported investigators at
the University of California at San Francisco designed an
ingenious experiment.  They asked adults scheduled for wisdom
teeth removal to volunteer in a pain experiment.  Following
surgery, some patients were given morphine, some naloxone, and
some a placebo.  As expected, about a third of those given the
placebo reported pain relief.  The investigators then gave these
people naloxone.  All reported a return of pain.

     How people who benefit from placebos gain access to pain
control systems in the brain is not known.  Scientists cannot
even predict whether someone who responds to a placebo in one
situation will respond in another.  The  San Francisco
investigators suspect that stress may be a factor.  Patients who
are very anxious or under stress are more likely to react to a
placebo for pain than those who are more calm, cool, and
collected.  But dental surgery itself may be sufficiently
stressful to trigger the release of endorphins-with or without
the effects of placebo.  For that reason, many specialists
believe further studies are indicated to analyze the placebo
effect.

     As research continues to reveal the role of endorphins in
the brain, neuroscientists have been able to draw more detailed
brain maps of the areas and pathways important in pain perception
and control.  They have even found new members of the endorphin
family: Dynorphin, the newest endorphin, is reported to be 10
times more potent a painkiller than morphine.

     At the same time, clinical investigators have tested chronic
pain patients and found that they often have lower-than-normal
levels of endorphins in their spinal fluid.  If you could just
boost their stores with man-made endorphins, perhaps the problems
of chronic pain patients could be solved.

     Not so easy.  Some endorphins are quickly broken down after
release  from nerve cells.  Other endorphins are longer lasting,
but there are problems in manufacturing the compounds in quantity
and getting them into the right places in the brain or spinal
cord.  In a few promising studies, clinical investigators have
injected an endorphin called beta-endorphin under the membranes
surrounding the spinal cord.  Patients reported excellent pain
relief lasting for many hours.  Morphine compounds injected in
the same area are similarly effective in producing long-lasting
pain relief.

     But spinal cord injections or other techniques designed to
raise the level of endorphins circulating in the brain require
surgery and hospitalization.  And even if less,drastic means of
getting endorphins into the nervous system could be found, they
are probably not the ideal answer to chronic pain.  Endorphins
are also involved in other nervous system activities such as
controlling blood flow.  Increasing the amount of endorphins
might have undesirable effects on these other body activities.
Endorphins also appear to share with morphine a potential for
addiction or tolerance.

     Meanwhile, chemists are synthesizing new analgesics and
discovering painkilling virtues in drugs not normally prescribed
for pain.  Much of the drug research is aimed at developing
nonnarcotic painkillers.  The motivation for the research is not
only to avoid introducing potentially addictive drugs on the
market, but is based on the observation that narcotic drugs are
simply not effective in treating a variety of chronic pain
conditions.  Developments in nondrug treatments are also
progressing, ranging from new surgical techniques to physical and
psychological therapies like exercise, hypnosis, and biofeedback.

New and old drugs for pain

     When you complain of headache or low back pain and the
doctor says take two aspirins every 4 hours and stay in bed, you
may think your pain is being dismissed lightly.  Not at all.
Aspirin, one of the most universally used medications is an
excellent painkiller.  Scientists still cannot explain all the
ways aspirin works, but they do know that it interferes with pain
signals where they usually originate, at the nociceptive nerve
endings outside the brain and spinal cord: peripheral nerves.
Aspirin also inhibits the production of chemicals manufactured in
the blood to promote blood clotting and wound healing:
Prostaglandins.  Unfortunately, prostaglandins, released from
cells at the site of injury, are pain-causing substances.  They
actually sensitize nerve endings, making them-and you-feel more
pain Along with increasing the blood supply to the area, the
chemicals contribute to inflammation-the pain, heat, redness and
swelling of tissue damage.

     Some investigators now think that the continued release of
pain-causing substances in chronic pain conditions may lead to
long-term  nervous system changes in some patients that make them
hypersensitive to pain.  People suffering such hyperalgesia can
cry out in pain at the gentlest touch, or even when a soft breeze
blows over the affected area.  In addition to the prostaglandins,
blister fluid and certain insect and snake venoms also contain
pain-causing substances.  Presumably these chemicals alert you to
the need for care-a fine reaction in an emergency, but not in
chronic pain.

     There are several prescription drugs that usually can
provide stronger pain relief than aspirin.  These include the
opiate-related compounds codeine, propoxyphene (Darvon),
morphine, and meperidine (Demerol).  All these drugs have some
potential for abuse, and may have unpleasant and even harmful
side effects.  In combination with other medications or alcohol,
some can be dangerous.  Used  wisely, however, they are important
recruits in the chemical fight against pain.

     In the search for effective analgesics physicians have
discovered pain-relieving benefits from drugs not normally
prescribed for pain.  Certain antidepressants as well as
antiepileptic drugs are used to treat several particularly severe
pain conditions, notably the pain of shingles and of facial
neuralgias like tic douloureux.

     Interestingly, pain patients who benefit from
anti-depressants report pain relief before any uplift in mood.
Pain specialists think that the antidepressant works because it
increases the supply of a naturally produced neurotransmitter,
serotonin.  (Doctors have long associated  decreased amounts of
serotonin with severe depression.) But now scientists have
evidence that cells using serotonin are also an integral part of
a pain-controlling pathway that starts with endorphin-rich nerve
cells high up in the brain and ends with inhibition of
pain-conducting nerve cells lower in the brain or spinal cord.
Antidepressant drugs have been used successfully in treating the
excruciating pain that can follow an attack of shingles.

     Antiepileptic drugs have been used successfully in treating
tic douloureux, the riveting attacks of facial pain that affect
older adults.  The rationale for the use of the antiepileptic
drugs (principally carbamazepine-Tegreto) does not involve the
endorphin system.  It is based on the theory that a healthy
nervous system depends on a proper balance of incoming and
outgoing nerve signals.  Tic and other facial pains or neuralgias
are thought to result from damage to facial nerves.  That means
that the normal flow of messages to and from the brain is
disturbed.  The nervous system may react by becoming
hypersensitive: It may create its own powerful discharge of nerve
signals, as though screaming to the outside world "Why aren't you
contacting me?"  Antiepileptic drugs-used to quiet the excessive
brain discharges associated with epileptic seizures-quiet the
distress signals associated with tic and may relieve pain that
way.


Psychological methods

     Psychological treatment for pain can range from
psychoanalysis and other forms of psychotherapy to relaxation
training, meditation, hypnosis, biofeedback, or behavior
modification.  The philosophy common to all these varied
approaches is the belief that patients can do something on their
own to control their pain.  That something may mean changing
attitudes, feelings, or behaviors associated with pain, or
understanding how unconscious forces and past events have
contributed to the present painful predicament.

     * Psychotherapy.  Freud  was celebrated for demonstrating
that for some individuals physical pain symbolizes real or
imagined emotional hurts.  He also noted that some individuals
develop pain or paralysis as a form of self-punishment for what
they consider to be past sins or bad behavior.  Sometimes, too,
pain may be a way of punishing others.  This doesn't mean that
the pain is any less real; it does mean that some pain patients
may benefit from psychoanalysis or individual or group
psychotherapy to gain insights into the meaning of their pain.

     * Relaxation and meditation therapies.  These forms of
training enable people to relax tense muscles, reduce anxiety,
and alter mental state.  Both physical and mental tension can
make any pain worse, and in conditions such as headache or back
pain, tension may be at the root of the problem.  Meditation,
which aims at producing a state of relaxed but alert awareness,
is sometimes combined with therapies that encourage people to
think of pain as something remote and apart from them.  The
methods promote a sense of detachment so that the patient thinks
of the pain as confined to a particular body part over which he
or she has marvelous control.  The approach may be particularly
helpful when pain is associated with fear and dread, as in
cancer.

     * Hypnosis.  No longer considered magic, hypnosis is a
technique in which an individual's susceptibility to suggestion
is heightened.  Normal volunteers who prove to be excellent
subjects for hypnosis often report a marked reduction or
obliteration of experimentally induced pain, such as that
produced by a mild electric shock.  The hypnotic state does not
lower the volunteer's heart rate, respiration, or other autonomic
responses.  These physical reactions show the expected increases
normally associated with painful stimulation.

     The role of hypnosis in treating chronic pain patients is
uncertain.  Some studies have shown that 15 to 20 percent of
hypnotizable patients with moderate to severe pain can achieve
total relief with hypnosis.  Other studies report that hypnosis
reduces anxiety and depression.  By lowering the burden of
emotional suffering, pain may become more bearable.

     * Biofeedback.  Some individuals can learn voluntary control
over certain body activities if they are provided with
information about how the system is working-how fast their heart
is beating, how tense are their head or neck muscles, how cold
are their hands.  The information is usually supplied through
visual or auditory cues that code the body activity in some
obvious way-a louder sound meaning an increase in muscle tension,
for example.  How people use this "biofeed-back" to learn control
is not understood, but some masters of the art report that
imagery helps: They may think of a warm tropical beach, for
example, when they want to raise the temperature of their hands.
Biofeed-back may be a logical approach in pain conditions that
involve tense muscles, like tension headache or low back pain.
But results are mixed.

     * Behavior modification.  This psychological technique
(sometimes called operant conditioning) is aimed at changing
habits, behaviors, and attitudes that can develop in chronic pain
patients.  Some patients become dependent, anxious, and
homebound-if not bedridden.  For some, too, chronic pain may be a
welcome friend, relieving them of the boredom of a dull job or
the burden of family responsibilities.  These psychological
rewards-sometimes combined with financial gains from compensation
payments or insurance-work against improvements in the patient's
condition, and can encourage increased drug dependency, repeated
surgery, and multiple doctor and clinic visits.

     There is no question that the patient feels pain.  The hope
of behavior modification is that pain relief can be obtained from
a program aimed at changing the individual's lifestyle.  The
program begins with a complete assessment of the painful
condition and a  thorough explanation of how the program works.
It is essential to enlist the full cooperation of both the
patient and family members.  The treatment is aimed at reducing
pain medication and increasing mobility and independence through
a graduated program of exercise, diet, and other activities.  The
patient is rewarded for positive efforts with praise and
attention.  Rewards are withheld when the patient retreats into
negative attitudes or demanding and dependent behavior.

     How effective are any of these psychological treatments? Are
some superior to others?  Who is most likely to benefit? Do the
benefits last? The answers are not yet in hand.  Patient
selection and patient cooperation are all-important.  Analysis of
individuals who have improved dramatically  with one or another
of these approaches is helping to pinpoint what factors are
likely to lead to successful treatment.


Surgery to relieve pain

     Surgery is often considered the court of last resort for
pain: When all else fails, cut the nerve endings.  Surgery can
bring about instant, almost magical release from pain.  But
surgery may also destroy other sensations as well, or,
inadvertently, become the source of new pain.  Further, relief is
not necessarily permanent.  After 6 months or a year, pain may
return.

     For all those reasons, the decision for surgery must always
involve a careful weighing of the patient's condition and the
outlook for the future.  If surgery can mean the difference
between a pain-wracked existence ending in death, versus a
pain-free time in which to compose one's life and see friends and
family, then surgery is clearly a humane and compassionate
choice.

     There are a variety of operations to relieve pain.  The most
common is cordotomy:  severing the nerve fibers on one or both
sides of the spinal cord that travel the express routes to the
brain.  Cordotomy affects the sense of temperature as well as
pain, since the fibers travel together in the express route.

     Besides cordotomy, surgery within the brain or spinal cord
to  relieve pain includes severing connections at major junctions
in pain pathways, such as at the places where pain fibers cross
from one side of the cord to the other, or destroying parts of
important relay stations in the brain like the thalamus, an
egg-shaped cluster of nerve cells near the center of the brain.

     In addition, surgeons sometimes can relieve pain by
destroying nerve fibers or their parent cell bodies outside the
brain or spinal cord.  A case in point is the destruction of
sympathetic nerves (a part of the autonomic nervous system) to
relieve the severe pain that sometimes follows a penetrating
wound from a sharp instrument or bullet.

     When pain affects the upper extremities, or is widespread,
the surgeon has fewer options and surgery may not be as
effective.  Still, skilled neurosurgeons have achieved excellent
results with upper spinal cord or brain surgery to treat severe
intractable pain.  These procedures may employ chemicals or use
heat or freezing treatments to destroy tissue, as well as the
more traditional use of the scalpel.

     Recently, Harvard Medical School surgeons reported success
with a new brain operation called cingulotomy to relieve
intractable pain in patients with severe psychiatric problems.
The nerve fibers destroyed are part of a pathway important in
emotions and motivation.  The surgery appears to eliminate the
discomfort and suffering the patient feels, but does not
interfere with other mental faculties such as thinking and
memory.

     Prior to operating, physicians can often test the
effectiveness of surgery by using anesthetic drugs to block like
the pain from a penetrating wound-these temporary blocks can in
themselves be beneficial, promoting repair of nerve damage.

     How do these current treatments apply to the more common
chronic pain conditions?  What follows is a brief survey of major
pain disorders and the treatments most in use today.

The major pains

     * Headache.  Tension headache, involving continued
contractions of head and neck muscles, is one of the most common
forms of headache.  The other common variety is the vascular
headache involving changes in the pressure of blood vessels
serving the head.  Migraine headaches are of the vascular type,
associated with throbbing pain on one side of the head.  Genetic
factors play a role in determining who will be a victim of
migraine, but many other factors are important as well.  A major
difficulty in treating migraine headache is that changes occur
throughout the course of the headache.  Blood vessels may first
constrict and then dilate. Changing levels of neurotransmitters
have also been noted.  While a number of drugs can relieve
migraine pain, their usefulness often depends on when they are
taken.  Some are only effective if taken at the onset.

     Drugs are also the most common treatment for tension
headache, although attempts to use biofeedback to control muscle
tension have had some success.  Physical methods such as heat or
cold applications often provide additional if only temporary
relief.

     * Low back pain.  The combination of aspirin, bed rest, and
modest amounts of a muscle relaxant are usually prescribed for
the first-time low back pain patient.  At the initial
examination, the physician will also note if the patient is
overweight or works at an occupation such as truck-driving or a
desk job that offers little opportunity for exercise.  Some
authorities believe that low back pain is particularly prevalent
in Western society because of the combination of overweight, bad
posture (made worse if there is added weight up front), and
infrequent exercise.  Not surprisingly, then, when the patient
begins to feel better, the suggestion is made to take off pounds
and take on physical exercise.  In some cases, a full
neurological examination may be necessary, including an x-ray of
the spinal cord called a myelogram, to see if there may be a
ruptured disc or other source of pressure on the cord or nerve
roots.

     Sometimes x-rays will show a disc problem which can be
helped by surgery.  But neither the myelogram nor disc surgery is
foolproof.  Milder analgesics (aspirin or stronger nonnarcotic
medications) and electrical stimulation-using TENS or implanted
brain electrodes-can be very effective.  What is not effective is
long-term use of the muscle-relaxant tranquilizers.  Many
specialists are convinced that chronic use of these drugs is
detrimental to the  back patient, adding to depression and
increasing pain.  Massage or manipulative therapy are used by
some clinicians but other than individual patient reports their
usefulness is still undocumented.

     * Cancerpain.  The pain of cancer can result from the
pressure of a growing tumor or the infiltration of tumor cells
into other organs.  Or the pain can come about as the result of
radiation or chemotherapy.  These treatments can cause fluid
accumulation and swelling (edema), irritate or destroy healthy
tissue causing pain and inflammation, and possibly sensitize
nerve endings.  Ideally, the treatment for cancer pain is to
remove the cancerous tissue.  When that is not possible, pain can
be treated by any or all of the currently available therapies:
electrical stimulation, psychological methods, surgery, and
strong painkillers.

     * Arthritis pain.  Arthritis is a general descriptive term
meaning an affliction of the joints.  The two most common forms
are osteoarthritis that typically affects the fingers and may
spread to important weight-bearing joints in the spine or hips,
and rheumatoid arthritis, an inflammatory joint disease
associated with swelling, congestion, and thickening of the soft
tissue around joints.  Recently, a distinguished panel of pain
experts commenting on arthritis reported that "in all probability
aspirin remains the most widely used ... and important drug ...
although it may cause serious side effects."  In the 1950's  the
steroid drugs were introduced and hailed as lifesavers-important
anti-inflammatory agents modeled after the body's own chemicals
produced in the adrenal glands.  But the long-term use of
steroids has serious consequences, among them the lowering of
resistance to infection, hemorrhaging, and facial
puffiness-producing the so-called "moonface."

     Besides aspirin, current treatments for arthritis include
several nonsteroid anti-inflammatory drugs like indomethacin and
ibuprofen.  But these drugs, too, may have serious side effects.
TENS and acupuncture have been tried with mixed results.  In
cases where tissue has been destroyed, surgery to replace a
diseased joint with an artificial part has been very successful.
The "total hip replacement" operation is an example.

     Arthritis is best treated early, say the experts. A modest
program of drugs combined with exercise can do much to restore
full function and forestall long-term degenerative changes.
Exercise in warm water is especially good since the water is both
relaxing and provides buoyancy that makes exercises easier to
perform.  Physical treatments with warm or cold compresses are
helpful sources of temporary pain relief.

     * Neurogenic pain.  The most difficult pains to treat are
those that result from damage to the peripheral nerves or to the
central nervous system itself.  We have mentioned tic douloureux
and shingles as examples of extraordinarily searing pain, along
with several drugs that can help.  In addition, tic sufferers can
benefit from surgery to destroy the nerve cells that supply
pain-sensation fibers to the face.  "Thermocoagulation"-which
uses heat supplied by an electrical current to destroy nerve
cells-has the advantage that pain fibers are more sensitive to
the  treatment resulting in less destruction of other sensations
(touch and temperature).

     Sometimes specialists treating tic find that certain blood
vessels in the brain lie near the group of nerve cells supplying
sensory fibers to the face, exerting pressure that causes pain.
The surgical insertion of a small sponge between the blood
vessels and the nerve cells can relieve the pressure and
eliminate pain.

     Among other notoriously painful neurogenic disorders is pain
from an amputated or paralyzed limb-so called "phantom" pain-that
affects up to 10 percent of amputees and paraplegia patients.
Various combinations of antidepressants and weak narcotics like
Darvon are sometimes  effective.  Surgery, too, is occasionally
successful.  Many experts now think that the electrical
stimulating techniques hold the greatest promise for relieving
these pains.

     * Psychogenic pain.  Some cases of pain are not due to past
disease or injury, nor is there any detectable sign of damage
inside or outside the nervous system.  Such pain may benefit from
any of the psychological pain therapies listed earlier.  It is
also possible that some new methods used to diagnose pain may be
useful.  One method gaining in popularity is thermography, which
measures the temperature of surface tissue as a reflection of
blood flow.  A color-coded "thermogram" of a person with a
headache or other painful condition often shows an altered blood
supply to the painful area, appearing as a darker or lighter
shade than the surrounding areas or the corresponding part on the
other side of the body.  Thus an abnormal thermogram in a patient
who complains of pain in the absence of any other evidence may
provide a valuable clue that can lead to a diagnosis and
treatment.

Where to go for help

     People with chronic pain have usually seen a family doctor
and several other specialists as well.  Eventually they are
referred to neurologists, orthopedists, or neurosurgeons.  The
patient/doctor relationship is extremely important in dealing
with chronic pain.  Both patients and family members should seek
out knowledgeable specialists who neither dismiss nor indulge the
patient; physicians who understand full well how pain has come to
dominate the patient's life and the lives of everyone else in the
family.

     Many specialists today refer chronic pain patients to pain
clinics for treatment.  Over 800 such clinics have opened their
doors in the United States since a world leader in pain therapy
established a pain clinic at the University of Washington in
Seattle in 1960.

     Pain clinics differ in their approaches.  Generally
speaking, clinics employ a group of specialists who review each
patient's medical history and conduct further tests when
necessary. If the applicant is admitted, the clinic staff designs
a personal treatment program that may include individual and
group psychotherapy, exercise, diet, ice massage for pain
(especially  before bedtime), electrical stimulation techniques,
and the use of a variety of analgesic but nonnarcotic drugs. The
aim is to reduce pain medication and so improve the patient's
pain problem that when he or she leaves the hospital it is with
the prospect of resuming more normal activities with a minimal
requirement for analgesics and a positive self-image.

     Contrary to what many people think, pain clinic patients are
not malingerers or hypochondriacs.  They are men and women of all
ages, education, and social background, suffering a wide variety
of painful conditions.  Patients with low back pain are frequent,
and so are people with the complications of diabetes, stroke,
brain trauma, headache, arthritis, or any of the rarer pain
conditions.  The majority of patients participate for 2 or 3
weeks and usually report substantial improvement at discharge.
One young man who had suffered painful chest injury as a result
of a factory accident said he literally "felt taller" after his
pain clinic experience.

Followup at 3- and 6-month intervals, and at lengthier intervals
thereafter, is an essential part of the program, both to evaluate
the long-term effectiveness of treatment and to initiate a
further course of treatment or counseling if necessary.

     Pain clinics have the virtue that they bring together people
with pain problems that have left them feeling isolated,
helpless, and hopeless.  But not everyone with a pain problem may
need the support of a group or residence in a hospital.  The
important factors are that the pain patient-and the
family-understand all the ramifications of pain, and the many and
varied steps that can now be taken to undo what chronic pain has
done.  As a result of the strides neuroscience has made in
tracking  down pain in the brain-and in the mind-we can expect
more and better treatments in the years to come.  The days when
patients were told "I'm sorry, but you'll have to learn to live
with the pain" will be gone forever.

Voluntary health organizations

     Several lay organizations are directly concerned with pain
problems.  They are excellent sources of additional information,
research updates, and specific help and referrals:

American Chronic Pain Association, Inc.
257 Old Haymaker Road
Monroeville, PA 15146
[phone]

National Chronic Pain Outreach Association, Inc.
4922 Hampden Lane, Dept. P
Bethesda, MD 20814
[phone]

National Headache Foundation
5252 N. Western Avenue
Chicago, IL 60625
[phone]
[phone] (Toll free)
[phone] (Toll free in Illinois)

     In addition, many organizations concerned with specific
diseases, such as arthritis or heart disease, provide information
and advice about attendant pain problems.

Pain clinics

     While there is no official certifying agency accrediting
pain clinics throughout the country, there are many excellent
clinics, often affiliated with university-associated medical
centers.  Your family doctor or university medical center may be
able to refer you to reputable clinics nearby.  If not,
physicians can write to the American Society of
Anesthesiologists, 515 Busse Highway, Park Ridge, IL 60068, which
publishes a worldwide pain clinic directory.

NINDS information
     For additional information concerning NINDS research on pain
write:

Office of Scientific and Health Reports
National Institute  of Neurological Disorders and Stroke
Building 31, Room 8AO6
National Institutes of Health
9000 Rockville Pike
Bethesda, MD 20892
[phone]

Prepared by the Office of
Scientific and Health Reports
National Institute of
Neurological Disorders
and Stroke

NATIONAL INSTITUTES OF HEALTH
Bethesda, Maryland 20892

This file scanned from NIH Publication No. 90-2406
November 1989
!
*
COCHLEAR IMPLANTS IN ADULTS AND CHILDREN

National Institutes of Health Consensus Development Conference Statement
                           May 15-17, 1995

This statement will be published as:
     Cochlear Implants in Adults and Children. NIH Consens Statement 1995
     May 15-17; 13(2):1-__.
For making bibliographic reference to consensus statement no. 100 in the
electronic form displayed here, it is recommended that the following format be
used:
     Cochlear Implants in Adults and Children. NIH Consens Statement
     Online 1995 May 15-17 [cited year month day]; 13(2):1- __.

Introduction

Cochlear implants are now firmly established as effective options in the
habilitation and rehabilitation of individuals with profound hearing
impairment. Worldwide, more than 12,000 people have attained some degree of
sound perception with cochlear implants, and the multichannel cochlear implant
has become a widely accepted auditory prosthesis for both adults and children.
The vast majority of adults who are deaf and have cochlear implants derive
substantial benefit from them when they are used in conjunction with
speechreading. Many of these individuals are able to understand some speech
without speechreading, and some of these individuals are able to communicate by
telephone. Benefits have also been observed in children, including those who
lost their hearing prelingually; moreover, there is evidence that the benefits
derived improve with continued use. New speech-sound processing techniques have
improved the effectiveness of cochlear implants, increasing user performance
levels to ones previously unseen.

The NIH sponsored a Consensus Development Conference on Cochlear Implants in
1988. Since then, implant technology has been continually improved. Questions
unanswered at that time have now been resolved. New issues have emerged that
must be addressed.

For example, the performance of some severely to profoundly hearing-impaired
adults using hearing aids is poorer than that of even more severely
hearing-impaired individuals using cochlear implants with advanced speech-
processing strategies. It is possible that cochlear implants could be
beneficial for some of these individuals. Therefore, the criteria for
implantation should be re-examined. The ability to predict preoperatively the
level of performance at which an individual implant recipient will function is
highly desirable. Currently, the limited prediction of implant efficacy in a
specific individual remains a pressing problem. Agreement does not exist on the
definition of a successful implant user. What are the appropriate expectations
for individuals using cochlear implants? How is benefit defined and measured?
What are the audiological, educational, and psychosocial impacts of this
intervention and is it cost-effective? Advancing technology will allow for the
modification of existing devices or the development of new devices. It is
therefore important to know what risks and benefits are associated with device
explantation/reimplantation. Surgical and other risks and possible long-term
effects of cochlear implants require evaluation.

Implantation of individuals with multiple disabilities, the elderly, and
children, particularly children who are prelingually deaf, engenders special
questions. Longitudinal studies are providing information on the development of
auditory speech perception and production and language skills in children who
are deaf and have a cochlear implant. What educational setting is best for the
development of speech and language in these children? Are cochlear implants
efficacious in children who are prelingually deaf?

To address these new issues since the 1988 Consensus Development Conference
(CDC) on Cochlear Implants, the National Institute on Deafness and Other
Communication Disorders, together with the NIH Office of Medical Applications
of Research, convened a Consensus Development Conference on Cochlear Implants
in Adults and Children, May 15-17, 1995. The conference was cosponsored by the
National Institute on Aging, the National Institute of Child Health and Human
Development, the National Institute of Neurological Disorders and Stroke, and
the Department of Veterans Affairs.

The conference was convened to summarize current knowledge about the range of
benefits and limitations of cochlear implantation that have accrued to date.
Such knowledge is an important basis for informed choices for individuals and
their families whose philosophy of communication is dedicated to spoken
discourse. Issues related to the acquisition of sign language were not directly
addressed by the panel, because the focus of the conference was to synthesize
thoughtfully the new information on cochlear implant technology and its use.
The panel acknowledges the value and contributions of bilingual-bicultural
approaches to deafness.

This conference brought together specialists in auditory anatomy and
physiology, otolaryngology, audiology, aural rehabilitation, education,
speech-language pathology, bioengineering, and other related disciplines as
well as representatives from the public. After 1-1/2 days of presentations and
audience discussion, an independent, non- Federal consensus panel weighed the
scientific evidence and developed a draft statement that addressed the
following five questions:

1. What Factors Affect the Auditory Performance of Cochlear Implant Recipients?

Subject Factors

Auditory performance, defined as the ability to detect, discriminate,
recognize, or identify acoustic signals, including speech, is highly variable
among individuals using cochlear implants. Since the 1988 CDC on Cochlear
Implants, however, some factors associated with outcome variability are now
better understood.

Etiology

Because of a larger subject sample, the effects of etiology can now be
distinguished from other factors such as the duration of deafness and the age
of onset. Meningitic deafness does not necessarily limit the benefit of
cochlear implantation in the absence of central nervous system complications,
cochlear ossification, or cochlear occlusion. Children with congenital deafness
and children with prelingually acquired meningitic deafness, for example,
achieve similar auditory performance if the cochlear implant is received before
age 6 years. In general, etiology does not appear to impact auditory
performance in either children or adults.

Age of Onset of Deafness

The age of onset continues to have important implications for cochlear
implantation, depending on whether the hearing impairment occurred before
(prelingual), during (perilingual), or after (postlingual) learning speech and
language. At the time of the last CDC, data on cochlear implantation suggested
that children or adults with postlingual onset of deafness had better auditory
performance than children or adults with prelingual or perilingual onsets. On
average, current data following auditory performance in children over a longer
period of time support this finding. However, the difference between children
with postlingual and prelingual-perilingual onsets appears to lessen with time.
Large individual differences remain within each group.

Age at Implantation

Previous data suggested that prelingually or perilingually deafened persons who
were implanted in adolescence or adulthood did not achieve as good auditory
performance as those implanted during childhood, although individual
differences were recognized. Current data continue to support the importance of
early detection of hearing loss and implantation for maximal auditory
performance. However, it is still unclear whether implantation at age 2, for
example, ultimately results in better auditory performance than implantation at
age 3.

Duration of Deafness

As deafness endures, even in postlingually deafened individuals, some acquired
skills and knowledge may decline and some behaviors that work against
successful adaptation to a sensory device may develop. Individuals with shorter
durations of auditory deprivation tend to achieve better auditory performance
from any type of sensory aid, including cochlear implants, than individuals
with longer durations of auditory deprivation.

Residual Hearing

Cochlear implants tend to give people with profound deafness a level of
auditory performance that is similar to, or better than, the performance of
people with severe hearing impairment who use hearing aids. These data raise
the issue of whether cochlear implants might give persons with severe hearing
impairment and some residual hearing even better auditory performance than they
can attain with a hearing aid. No residual hearing is typically defined as
profound hearing loss and no open-set speech recognition. However, the degree
of preimplantation residual hearing does not predict postimplantation auditory
performance. Research is now addressing the critical distinction between the
importance of residual pure tone sensitivity compared with that of overall
residual auditory capacities and functional communication status.

Electrophysiological Factors

Some surviving spiral ganglion cells are necessary for auditory performance
with a cochlear implant. Degenerative changes occur in both ganglion cells and
central auditory neurons following sensorineural deafening. Although a
relationship between the number of surviving ganglion cells and psychophysical
performance has been demonstrated in animals, a direct relationship between
ganglion cell survival and level of auditory performance in humans has not been
shown. Animal studies also suggest that electrical stimulation increases
ganglion cell survival and also modifies the functional organization of the
central auditory system. The implications of these new findings remain to be
determined.

Device Factors

The task of representing speech stimuli as electrical stimuli is central to the
design of cochlear implants. Designs vary according to (1) the placement,
number, and relationship among the electrodes; (2) the way in which stimulus
information is conveyed from an external processor to the electrodes; and (3)
how the electrical stimuli are derived from the speech input (and other
signals). Changes in cochlear implant design/processing strategies and their
effects on auditory performance are discussed in Section 3.

2. What Are the Benefits and Limitations of Cochlear Implantation?

Impact on Speech Perception in Adults

Cochlear implantation has a profound impact on hearing and speech reception in
postlingually deafened adults. Most individuals demonstrate significantly
enhanced speech-reading capabilities, attaining scores of 90-100 percent
correct on everyday sentence materials. Speech recognition afforded by the
cochlear implant effectively supplements the information least favorably cued
through speech-reading. A majority of those individuals with the latest speech
processors for their implants will score above 80-percent correct on
high-context sentences without visual cues. Performance on single-word testing
in these individuals is notably poorer, although even these scores have been
significantly improved with newer speech-processing strategies. Recognition of
environmental sounds and even appreciation of music have been repeatedly
observed in adult implant recipients. Noisy environments remain a problem for
cochlear-implanted adults, significantly detracting from speech-perception
abilities. Prelingually deafened adults have generally shown little improvement
in speech perception scores after cochlear implantation, but many of these
individuals derive satisfaction from hearing environmental sounds and continue
to use their implants.

Speech Perception, Speech Production, and Language Acquisition in Children

Improvements in the speech perception and speech production of children
following cochlear implantation are often reported as primary benefits.
Variability across children is substantial. Factors such as age of onset, age
of implantation, the nature and intensity of (re)habilitation, and mode of
communication contribute to this variability. Using tests commonly applied to
children and adults with hearing impairments (e.g., pattern perception,
closed-set word identification, and open-set perception), perceptual
performance increases on average with each succeeding year post implantation.
Shortly after implantation, performance may be broadly comparable to that of
some children with hearing aids and over time may improve to match that of
children who are highly successful hearing aid users. Children implanted at
younger ages are on average more accurate in their production of consonants,
vowels, intonation, and rhythm. Speech produced by children with implants is
more accurate than speech produced by children with comparable hearing losses
using vibro-tactile or hearing aids. One year after implantation, speech
intelligibility is twice that typically reported for children with profound
hearing impairments and continues to improve. Oral-aural communication training
appears to result in substantially greater speech intelligibility than manually
based total communication.

The language outcomes in children with cochlear implants have received less
attention. Reports involving small numbers of children suggest that
implantation in conjunction with education plus habilitation leads to advances
in oral language acquisition. The nature and pace of language acquisition may
be influenced by the age of onset, age at implantation, nature and intensity of
habilitation, and mode of communication.

One current limitation is that children are typically implanted at no earlier
than age 2 years, which is beyond what may be critical periods of auditory
input for the acquisition of oral language. Benefits are not realized
immediately, but rather are manifested over time, with some children continuing
to show improvement over several years.

Few studies have used language as an outcome measure. The assessment of speech
perception, language production, and language comprehension in young children
is particularly challenging. Furthermore, all results in children have been
reported for single-channel or feature-based devices only, despite the
relatively rapid evolution of alternatives in speech-coding strategies. Oral
language development in deaf children, including those with cochlear implants,
remains a slow, training-intensive process, and results will typically be
delayed in comparison with normally hearing peers.

Psychologic and Social Issues in Adults and Children

Although psychological evaluation has previously been a part of the preimplant
evaluation process, comparatively little research has been conducted on the
long-term psychological and social effects of electing for implantation. Still,
the psychological and social impact for adults is generally quite positive, and
there appears to be agreement between preimplantation expectations and later
benefit. This benefit is expressed as a decline in loneliness, depression and
social isolation and an increase in self-esteem, independence, social
integration, and vocational prospects.

Many adults report being able to function socially or vocationally in ways
comparable to those with moderate hearing loss. Furthermore, they describe a
new or renewed curiosity about the experience of hearing and the phenomena of
sound. In some cases the experience of implantation becomes an integral part of
the individual's identity, leading implant users to participate and share
experiences in self-interest and advocacy groups.

Negative psychological and social impact is less frequently observed and is
often related to concerns about the maintenance and/or malfunction of the
implant and external hardware. Other social insecurities may result from the
difficulty of hearing amidst background noise, and from unreasonable
expectations of aural-only benefit on the part of the implant user or his/her
family and friends.

The assessment of psychological impact in children with implants lags behind
that for the adult population, in part because psychological outcome is a
factor of audiological benefit, which is realized more slowly in children.
Additionally, such assessment must consider the child's family setting. Because
language acquisition is closely associated with identity, social development,
and social integration, the impact of implantation on a child's development in
these areas deserves more study in order to produce useful indicators that can
bear upon parental decision-making processes.

Rehabilitation and Educational Issues

Although a cochlear implant can provide dramatic augmentation of the auditory
information perceived by deaf children and adults, it is clear that training
and educational intervention play a fundamental role in optimizing postimplant
benefit. Access to postimplant rehabilitation involving professionals familiar
with cochlear implants must be provided to ensure successful outcomes for
implant recipients.

Rehabilitation efforts must be tailored to meet individual needs, and protocols
should be developed to reflect therapies effective for various types of
individuals receiving implants. Therapeutic intervention with prelingually deaf
adults may differ significantly in both time and content from that of
postlingually deaf recipients.

Pediatric cochlear implantation requires a multidisciplinary team composed of
physicians, audiologists, speech-language pathologists, rehabilitation
specialists, and educators familiar with cochlear implants. These professionals
must work together in a long-term relationship to support the child's auditory
and oral development. Although the effects of communication mode in
implantation habilitation have not been sufficiently documented, it is clear
that the educational programs for children with cochlear implants must include
auditory and speech instruction using the auditory information offered by the
implant.

Cost-Utility

The cost-benefit or cost-utility of cochlear implantation must be calculated
for children and adults separately. For adults, the cost of cochlear
implantation includes the initial costs of assessment, the device,
implantation, rehabilitation, system overhead, and maintenance. The benefit or
utility is estimated as a function of quality of life over time. On this basis,
cochlear implantation whether at age 45 years or 70 years compares quite
favorably to many medical procedures now commonly in use (e.g., implantable
defibrillator insertion).

Although it appears that the cost-utility estimates for children are also quite
favorable, we are still in the early stages of cochlear implant application and
cannot yet estimate the cost or potential cost savings that will accrue in the
area of (re)habilitation and education.

3. What Are the Technical and Safety Considerations of Cochlear Implantation?

Cochlear Implant Design Issues

A cochlear implant works by providing direct electrical stimulation to the
auditory nerve, bypassing the usual transducer cells that are absent or
nonfunctional in a deaf cochlea. Over the past 10 years, significant
improvements have been made in the technology used to accomplish auditory
stimulation.

The best performance in speech recognition occurs with intracochlear electrodes
that are close to the nerves to be stimulated, thus minimizing undesirable side
effects.

Early implants used only a single electrode; it has been found that these
single-channel implants rarely provide open-set speech perception. Most recent
implants have used multielectrode arrays that provide a number of independent
channels of stimulation. Such devices provide more information about the
acoustic signal and give better performance on speech recognition. No agreement
exists on the optimum number of channels, although at least 4-6 channels seem
to be necessary.

Much of the recent progress in implant performance has involved improvements in
the speech processors, which convert sound into the electrical stimulus. The
best performance comes with speech processors that attempt to preserve the
normal frequency code or spectral representation of the cochlea. These are
distinguished from feature-based processors, which attempt to analyze certain
features known to be important to speech perception and present only those
features through the electrodes. A major problem in multichannel implants is
channel interaction, in which two electrodes stimulate overlapping populations
of nerves. Channel interaction has now been minimized with speech processors
that activate the electrodes in a nonsimultaneous or interleaved fashion, which
has been shown to improve speech recognition significantly.

A final design issue is the means by which the stimulus information is passed
through the skin from the speech processor to the electrodes. In a
transcutaneous system, the skin is intact and the coupling is done
electromagnetically to an implanted antenna. In a percutaneous system, the
leads are passed directly through the skin. The two systems have slightly
different surgical complications, which are discussed below. The percutaneous
system (1) provides a more flexible connection to the electrodes in case a
change in speech processor is desired, (2) is easier to troubleshoot in case of
electrode problems, and (3) is magnetic resonance imaging (MRI) compatible.
Currently, percutaneous systems are not commercially available.

Issues Related to Magnetic Resonance Imaging

Magnetic Resonance Imaging (MRI) is increasingly the diagnostic tool of choice
for a variety of medical conditions. Implants that use transcutaneous
connectors contain an implanted magnet and some ferrous materials that are
incompatible with the high magnetic fields of an MRI scanner. Implant
manufacturers are redesigning their devices to circumvent this problem.
Potential MRI risks should be part of the informed consent procedure for
persons considering an implant. The external speech processor cannot be made
MRI compatible and should not be taken into the scanner.

Surgical Issues

Cochlear implantation entails risks common to most surgical procedures, e.g.,
general anesthetic exposure, as well as unique risks that are influenced by
device design, individual anatomy and pathology, and surgical technique.
Comparative data of major complications incurred in adult implantation show a
halving of the complication rate to approximately 5 percent in 1993. The
complication rate in pediatric implantation is less than that currently seen in
adults. Overall, the complication rate compares favorably to the 10 percent
rate seen with pacemaker/defibrillator implantation.

Major complications, i.e., those requiring revision surgery, include flap
problems, device migration or extrusion, and device failure. Facial palsy is
also considered a major complication but is distinctly uncommon and rarely
permanent. Notably, no mortalities have been attributed to cochlear
implantation.

Alterations in surgical technique, especially flap design, have led to a
considerable reduction in the flap complication rate, which is particularly
relevant to transcutaneous devices. Alterations in surgical technique,
particularly in methods used to anchor the device, have contributed to a
decrease in device migration/extrusion.

All implants are potentially prone to failure--either because of manufacturing
defects or use- related trauma. Pedestal fracture is a problem unique to the
percutaneous device, but occurs rarely. Manufacturer redesign has produced
electrode arrays that are smaller but sturdier. For the most commonly implanted
device, 95 percent of implants are still functioning after 9 years. Most
current implants with transcutaneous connectors do not provide self-test
capability for the implanted portion, making it cumbersome to test for simple
electrode failure, such as open and short circuits. Failure detection is
particularly problematic in young children. Device manufacturers should include
self-test circuity in future implant designs.

Minor complications are those that resolve without surgical intervention. The
most common is unwanted facial nerve stimulation with electrode activation,
which is readily rectified by device reprogramming. In percutaneous devices,
pedestal infections are uncommon and can be treated successfully with
antibiotics, but on rare occasions may require explantation.

Reimplantation is necessary in approximately 5 percent of cases because of
improper electrode insertion or migration, device failure, serious flap
complication, or loss of manufacturer support. In general, reimplantation in
the same ear is usually possible, and thus far individual auditory performance
after reimplantation equals or exceeds that seen with the original implant.

Long-term complications of implantation relate to flap breakdown, electrode
migration and receiver/stimulator migration. Particularly in the child, the
potential consequences of otitis media have been of concern, but as the
implanted electrode becomes ensheathed in a fibrous envelope, it appears
protected from the consequences of local infection.

4. Who Is a Candidate for Cochlear Implantation?

Adults

Cochlear implants are often highly successful in postlingually deafened adults
with severe/profound hearing loss with no speech perception benefit from
hearing aids. Previously, individuals receiving marginal benefit from hearing
aids were not considered implant candidates. Ironically, such individuals often
have less speech perception than more severely deafened persons who receive
implants. Recent data show that most marginally successful hearing aid users
implanted with a cochlear implant will have improved speech perception
performance. It is therefore reasonable to extend cochlear implants to
postlingually deafened adult individuals currently obtaining marginal benefit
from other amplification systems. Prelingually deafened adults may also be
suitable for implantation, although these candidates must be counseled
regarding realistic expectations. Existing data indicate that these individuals
achieve minimal improvement in speech recognition skills. However, there may be
other basic benefits such as improved sound awareness that correlate with
psychological satisfaction and safety needs.

Because of the wide variability in speech perception and recognition in persons
with similar hearing impairments, all candidates require indepth counseling of
the surgery, its risks and benefits, rehabilitation, and alternatives to
cochlear implantation. To give adequate informed consent, adult candidates
should understand that large variability in individual audiologic performance
precludes preoperative prediction of success. Determining implant candidacy
requires consideration of both objective audiological variables as well as the
subjective needs and wishes of individual candidates. Specific characteristics
of potential adult cochlear implant recipients are provided below.

Audiologic Criteria

Indications in favor of an implant are a severe-to-profound sensorineural
hearing loss bilaterally and open-set sentence recognition scores less than or
equal to 30 percent under best aided conditions. Duration of deafness and age
of onset have been shown to influence auditory performance with cochlear
implants and should be discussed with potential candidates.

In general, when there is no residual hearing in either ear, the ear with
better closed-set performance, more sensitive electrical thresholds, shorter
period of auditory deprivation, or better radiologic characteristics is
implanted. However, when there is residual hearing, the poorer ear should be
chosen, provided that there is radiologic evidence of cochlear patency.

Medical and Surgical Criteria

Traditionally, implantation candidacy was limited to healthy persons. Although
there may be specific medical contraindications to surgery and implantation
such as poor anesthetic risk, severe mental retardation, severe psychiatric
disorders, and organic brain syndromes, cochlear implantation should be offered
to a wider population of individuals. In some circumstances, such as in
individuals with low vision, implantation may be a tool to promote independence
and other quality-of-life goals.

The medical history, physical examination, and laboratory tests are important
tools in candidacy evaluation. Individuals with active ear pathology require
treatment and re-evaluation prior to implantation. The standard evaluation
includes high-resolution computed tomography (CT) scans that serve to detect
mixed fibrous and bony occlusions and anatomical abnormalities. MRI provides
better resolution of soft tissue structures and should supplement the CT scan
when indicated. These imaging techniques should be used to identify
abnormalities that may compromise or impede implant surgery or device use.

The results of electrophysiologic tests do not predict implant success.
However, in selected individuals, such as those with cochlear obliteration or
in decisions regarding ear of implantation, the results of promontory
stimulation may be useful.

Children

Cochlear implants have also been shown to result in successful speech
perception in children. Currently, the earliest age of implantation is 24
months, but there are reasons to reassess this age threshold. A younger age of
implantation may limit the negative consequences of auditory deprivation and
may allow more efficient acquisition of speech and language. Determining
whether cochlear implant benefits are greater in children implanted at age 2-3
years as compared to those implanted at age 4-5 years might resolve this issue,
but sufficient data are unavailable. It is also not clear that the benefits of
implantation before age 2 years would offset potential liabilities associated
with the increased difficulty in obtaining reliable and valid characterization
of hearing and functional communication status at the younger age. A number of
children under age 2 years have received implants, both internationally and in
the United States, when it was thought that bone growth associated with
meningitis would preclude implantation at a later date. Speech/language data
obtained on such children will be helpful in determining the potential benefits
of early implantation and therefore may help to guide future policy.

Audiologic Criteria

Children age 2 years or older with profound (greater than 90 dBHL)
sensorineural hearing loss bilaterally and minimal speech perception under best
aided conditions may be considered for cochlear implantation. In the young
child, auditory brainstem response, stapedial reflex testing, and/or
otoacoustic emission testing may be useful when combined with auditory
behavioral responses to determine hearing status. Prior to implantation, a
trial period with appropriate amplification combined with intensive auditory
training should have been attempted to ensure that maximal benefit has been
achieved. When the validity of behavioral test results is compromised by
maturational factors, the above criteria should be applied in the most
stringent manner (i.e., worse hearing sensitivity, longer trial periods, and so
on). Current research may broaden audiometric criteria for candidacy to better
reflect functional auditory capacity.

Medical and Surgical Criteria

Children should undergo a complete medical evaluation to rule out the presence
of active disease, which would be a contraindication to surgery. The child must
be otologically stable and free of active middle ear disease prior to cochlear
implantation. The radiologic imaging criteria used in adult candidates can be
applied to children.

Psychosocial Criteria

Preoperative assessment should entail evaluation of the child in the context of
the home and social and educational milieu to assure that implantation is the
proper intervention. In some instances psychosocial factors may be used as
exclusionary criteria; however, in most cases it should serve only as baseline
data for tracking cochlear implant outcomes.

Informed Consent

The parents of a deaf child are responsible for deciding whether to elect
cochlear implantation. The informed consent process should be used to empower
parents in their decision-making. The parents must understand that cochlear
implants do not restore normal hearing and that auditory and speech outcomes
are highly variable and unpredictable. They must be informed of the advantages,
disadvantages, and risks associated with implantation to establish realistic
expectations. Furthermore, the importance of long-term rehabilitation to
success with cochlear implants must be stressed. As part of the process of
informed consent, parents must be told that alternative approaches to
habilitation are available. All children should be included in the informed
consent process to the extent that they are able, as their active participation
is crucial to (re)habilitative success.

5. What Are the Directions for Future Research on Cochlear Implantation?

   * Research must attempt to explain the wide variation in performance across
     individual cochlear implant users. New tools, such as functional imaging
     of the brain, might be applied to unexplored variables such as the ability
     of the implant to activate the central auditory system. Investigations of
     the role of higher level cognitive processes in cochlear implant
     performance are needed.
   * The strides that have been made in improving speech perception of cochlear
     implant users should continue through improvements in electrode design and
     signal processing strategies. Noise-reduction technologies and enhancement
     of performance using binaural implants are promising areas.
   * Studies of the effects of cochlear stimulation on auditory neurons have
     provided clear evidence of plasticity in both the survival of neural
     elements and in receptive field organization. Comparisons of neural
     plasticity in animal experiments and of adaptation to cochlear implant
     electrical stimulation by humans provide a unique opportunity to study the
     relationships between neural activity and auditory perception.
   * Comparative research on language development in children with normal
     hearing, children with hearing impairment who use hearing aids, deaf
     children with cochlear implants, and deaf children using American Sign
     Language should be conducted. These studies should be longitudinal and
     reflect current theoretical and empirical advances in neurolinguistics and
     psycholinguistics.
   * Studies of the relationship between the development of speech perception
     and speech production in cochlear implant users must continue. Implanted
     deaf children provide a unique opportunity to examine these developmental
     processes and their relationship to the acquisition of aural/oral
     language. Such information is crucial to understanding and enhancing the
     performance of implanted prelingual children and may help define optimal
     age for implantation.
   * Adequate tools for the assessment of nonspeech benefits of implantation
     should be applied to gain a better understanding of the full effects of
     implantation on the quality of life of implant recipients. This may be
     particularly useful for implant recipients who do not realize significant
     speech-perception benefit. Such data will help in evaluating the
     cost-utility of cochlear implantation.
   * Identifying the components of successful (re)habilitation approaches will
     facilitate extension of these services to all children and adults
     receiving cochlear implants, as will comparison of model and routine
     service programs.

Conclusions

   * Cochlear implantation improves communication ability in most adults with
     deafness and frequently leads to positive psychological and social
     benefits as well. The greatest benefits seen to date have occurred in
     postlingually deafened adults. Cochlear implantation in prelingually
     deafened adults provides more limited improvement in speech perception,
     but offers important environmental sound awareness. Cochlear implantation
     outcomes are more variable in children. Nonetheless, gradual, steady
     improvement in speech perception, speech production, and language does
     occur. There is substantial unexplained variability in the performance of
     implant users of all ages, and implants are not appropriate for all
     individuals.
   * Currently children at least 2 years old and adults with profound deafness
     are candidates for implantation. Cochlear implant candidacy should be
     extended to adults with severe hearing impairment and poor open-set
     sentence discrimination, i.e., less than or equal to 30 percent in the
     best aided condition. Although there are theoretical reasons to lower the
     age of implantation in children, data are too scarce to justify a change
     in criteria. Additional data may justify a change in age and audiologic
     criteria.
   * Auditory performance with a cochlear implant varies among individuals. The
     data indicate that performance is better in individuals who (1) have
     shorter durations of deafness, (2) were implanted before age 6 years, and
     (3) acquired language before their hearing loss occurred. Auditory
     performance is not affected by etiology of hearing loss.
   * Access to optimal educational and (re)habilitation services is important
     to adults and is critical to children to maximize the benefits available
     from cochlear implantation.
   *  The current generation of intracochlear, multichannel implants with
     spectrally based speech processors provides a substantial improvement over
     the previous generation of devices, especially when nonsimultaneous
     electrode activation is used.
   * The low complication rate and high reliability for cochlear implants
     compares favorably with other implanted electronic devices, and continues
     to improve.
   * Current devices are not MRI compatible, and users and physicians should be
     acutely aware of this problem. Implant manufacturers should include MRI
     compatibility and internal self-test systems in future devices.
   * Percutaneous connectors offer many research and clinical opportunities,
     including MRI compatibility, ease of electrode testing, and processor
     upgrades, and they should not be abandoned.

Consensus Development Panel

George A. Gates, M.D.
     Conference and Panel Chairperson
     Professor of Otolaryngology-Head and Neck Surgery
     Director
     Virginia Merrill Bloedel Hearing Research Center
     University of Washington
     Seattle, Washington
Kathleen Daly, Ph.D.
     Assistant Professor
     Department of Otolaryngology
     University of Minnesota
     Minneapolis, Minnesota
William J. Dichtel, M.D.
     Otolaryngologist
     Roanoke, Virginia
Robert J. Dooling, Ph.D.
     Professor
     Department of Psychology
     University of Maryland
     College Park, Maryland
Aina Julianna Gulya, M.D.
     Professor
     Department of Otolaryngology
     Head and Neck Surgery
     Georgetown University
     Washington, District of Columbia
Joseph W. Hall III, Ph.D.
     Professor
     Department of Surgery
     Division of Otolaryngology
     University of North Carolina at Chapel Hill
     Chapel Hill, North Carolina
Susan W. Jerger, Ph.D.
     Professor and Director
     Children's Special Hearing Section
     Department of Otorhinolaryngology
     Baylor College of Medicine
     Houston, Texas
Jacqueline E. Jones, M.D.
     Assistant Professor
     Department of Otolaryngology
     Cornell University Medical College
     New York Hospital
     New York, New York
Margaret H. Mayer, Ed.D.
     Coordinator for Teacher Education and Research
     Northwest School for Hearing-Impaired Children
     Seattle, Washington
Michael Pierschalla
     Cambridge, Massachusetts
Lainie Friedman Ross, M.D., M.Phil.
     Assistant Professor
     Department of Pediatrics and MacLean Center for Clinical Medical Ethics
     University of Chicago
     Chicago, Illinois
Richard G. Schwartz, Ph.D.
     Professor
     Ph.D. Program in Speech and Hearing Sciences
     City University of New York
     New York, New York
Barbara E. Weinstein, Ph.D.
     Associate Professor of Audiology
     Director, Audiology Program
     Lehman College
     City University of New York
     New York, New York
Eric D. Young, Ph.D.
     Professor
     Department of Biomedical Engineering
     Johns Hopkins University
     Baltimore, Maryland

Speakers

Paul J. Abbas, Ph.D.
     "Factors Affecting Auditory Performance: Electrophysiologic Measures"
     Professor
     Department of Speech Pathology and Audiology
     Department of Otolaryngology
     Head and Neck Surgery
     University of Iowa
     Iowa City, Iowa
Peter Blamey, Ph.D.
     "Factors Affecting Auditory Performance of Postlinguistically Deaf Adults
     Using Cochlear Implants: Etiology, Age, and Duration of Deafness"
     Principal Research Fellow
     Department of Otolaryngology
     University of Melbourne
     East Melbourne, Victoria, Australia
Derald E. Brackmann, M.D., F.A.C.S.
     "Percutaneous Connectors in Cochlear Implantation"
     Clinical Professor of Otolaryngology-Head and Neck Surgery/Neurosurgery
     University of Southern California School of Medicine
     President
     House Ear Clinic and Institute
     Los Angeles, California
Judith A. Brimacombe, M.A.
     "Multichannel Cochlear Implants in Adults With Residual Hearing"
     Vice President
     Clinical and Regulatory Affairs
     Cochlear Corporation
     Englewood, Colorado
Patricia M. Chute, Ed.D.
     "Residual Hearing in Children"
     Director, Cochlear Implant Center
     Manhattan Eye, Ear, and Throat Hospital
     New York, New York
Noel L. Cohen, M.D.
     "Surgical Complications and Considerations"
     Professor and Chairman
     Department of Otolaryngology
     New York University School of Medicine
     New York, New York
Michael F. Dorman, Ph.D.
     "Speech Perception by Adults"
     Professor
     Department of Speech and Hearing Science
     Arizona State University
     Tempe, Arizona
     Adjunct Professor
     Division of Otolaryngology
     University of Utah
     Health Sciences Center
     Salt Lake City, Utah
Donald K. Eddington, Ph.D.
     "Introduction and Overview"
     Cochlear Implant Research Laboratory
     Massachusetts Eye and Ear Infirmary
     Boston, Massachusetts
Bruce J. Gantz, M.D., F.A.C.S.
     "Device Failure"
     Professor and Interim Head
     Department of Otolaryngology
     Head and Neck Surgery
     University of Iowa College of Medicine
     Iowa City, Iowa
James W. Heller, P.E.
     "MRI Considerations"
     Manager of Research
     Cochlear Corporation
     Englewood, Colorado
Darlene R. Ketten, Ph.D.
     "Radiologic Assessment"
     Assistant Professor
     Department of Otology and Laryngology
     Harvard Medical School
     Research Director
     Three-Dimensional Imaging Service
     Massachusetts Eye and Ear Infirmary
     Boston, Massachusetts
John F. Knutson, Ph.D.
     "Psychological and Social Issues in Cochlear Implant Use"
     Professor
     Department of Psychology
     Spence Laboratories of Psychology
     University of Iowa
     Iowa City, Iowa
Patricia A. Leake, Ph.D.
     "Long-Term Effects of Electrical Stimulation"
     Professor in Residence
     Department of Otolaryngology
     Research Director
     Epstein Hearing Research Laboratory
     University of California San Francisco
     San Francisco, California
Hugh J. McDermott, Ph.D.
     "Speech Processing Using Selected Spectral Features"
     Research Fellow
     Department of Otolaryngology
     University of Melbourne
     East Melbourne, Victoria, Australia
Richard T. Miyamoto, M.D., F.A.C.S.
     "Timing of Implantation in Children"
     Arilla Spence DeVault Professor and Chairman
     Department of Otolaryngology-Head and Neck Surgery
     Indiana University School of Medicine
     Indianapolis, Indiana
Jean S. Moog, M.S.
     "Rehabilitation and Educational Issues in Children"
     Director of Deaf Education
     Central Institute for the Deaf
     St. Louis, Missouri
Mary Joe Osberger, Ph.D.
     "Effect of Age at Onset of Deafness on Cochlear Implant Performance"
     "Speech Perception in Children"
     Director, Pediatric Clinical Research
     Advanced Bionics Corporation
     Sylmar, California
Robert V. Shannon, Ph.D.
     "Information Transmission in Cochlear Implants: Analysis Channels, Number
     of Electrodes, and Received Channels"
     Auditory Implant Research
     House Ear Institute
     Los Angeles, California
Margaret W. Skinner, Ph.D.
     "Audiologic Criteria for Cochlear Implantation in Adults and Children"
     Associate Professor
     Director of Audiology
     Director of Cochlear Implant Program
     Department of Otolaryngology-Head and Neck Surgery
     Washington University School of Medicine
     St. Louis, Missouri
Quentin Summerfield, Ph.D.
     "Cost-Effectiveness Considerations in Cochlear Implantation"
     Professor
     Medical Research Council
     Institute of Hearing Research
     Nottingham, United Kingdom
Emily A. Tobey, Ph.D.
     "Speech Production in Children"
     Professor and Nelle C. Johnston Chair in Early Childhood Communication
     Disorders
     Department of Communication Disorders
     Callier Center for Communication Disorders
     University of Texas at Dallas
     Dallas, Texas
Susan B. Waltzman, Ph.D.
     "Comparison of Implant Systems"
     Professor
     Department of Otolaryngology
     New York University
     School of Medicine
     New York, New York
Blake S. Wilson, B.S.E.E.
     "Continuous Interleaved Sampling and Related Strategies"
     Director
     Center for Auditory Prosthesis Research
     Research Triangle Institute
     Research Triangle Park, North Carolina
Teresa A. Zwolan, Ph.D.
     "Factors Affecting Auditory Performance With a Cochlear Implant by
     Prelingually Deafened Adults"
     Assistant Research Scientist
     Director, Cochlear Implant Program
     Department of Otolaryngology-Head and Neck Surgery
     Audiology and Electrophysiology Division
     University of Michigan Medical Center
     Ann Arbor, Michigan

Planning Committee

Amy M. Donahue, Ph.D.
     Chairperson
     Acting Chief, Hearing and Balance/Vestibular Sciences Branch
     Division of Human Communication
     National Institute on Deafness and Other Communication Disorders
     National Institutes of Health
     Bethesda, Maryland
Marin P. Allen, Ph.D.
     Chief
     Program Planning and Health Reports Branch
     National Institute on Deafness and Other Communication Disorders
     National Institutes of Health
     Bethesda, Maryland
Lucille B. Beck, Ph.D.
     Associate Chief
     Audiology and Speech Pathology Service
     Department of Veterans Affairs
     Washington, District of Columbia
Elsa A. Bray
     Program Analyst
     Office of Medical Applications of Research
     National Institutes of Health
     Bethesda, Maryland
Judith A. Cooper, Ph.D.
     Deputy Director
     Division of Human Communication
     National Institute on Deafness and Other Communication Disorders
     National Institutes of Health
     Rockville, Maryland
John H. Ferguson, M.D.
     Director
     Office of Medical Applications of Research
     National Institutes of Health
     Bethesda, Maryland
Marilyn Neder Flack, M.A.
     Senior Scientific Reviewer/Audiologist
     Ear, Nose, and Throat Devices
     Center for Devices and Radiological Health
     Office of Device Evaluation
     Food and Drug Administration
     Rockville, Maryland
George A. Gates, M.D.
     Conference and Panel Chairperson
     Professor of Otolaryngology-Head and Neck Surgery
     Director of Virginia Merrill Bloedel
     Hearing Research Center
     University of Washington
     Seattle, Washington
William H. Hall
     Director of Communications
     Office of Medical Applications of Research
     National Institutes of Health
     Bethesda, Maryland
F. Terry Hambrecht, M.D.
     Head, Neural Prosthesis Program
     National Institute of Neurological Disorders and Stroke
     National Institutes of Health
     Bethesda, Maryland
Norman Krasnegor, Ph.D.
     Chief
     Human Learning and Behavior Branch
     Center for Research for Mothers and Children
     National Institute of Child Health and Human Development
     National Institutes of Health
     Bethesda, Maryland
Andrew A. Monjan, Ph.D., M.P.H.
     Chief
     Neurobiology of Aging Branch
     Neuroscience and Neuropsychology of Aging Program
     National Institute on Aging
     National Institutes of Health
     Bethesda, Maryland
Ralph F. Naunton, M.D.
     Director
     Division of Human Communication
     National Institute on Deafness and Other Communication Disorders
     National Institutes of Health
     Rockville, Maryland

Conference Sponsors

     Office of Medical Applications of Research, NIH
     John H. Ferguson, M.D.
     Director
     National Institute on Deafness and Other Communication Disorders
     James B. Snow, Jr., M.D.
     Director

Cosponsors

     National Institute on Aging
     Richard J. Hodes, M.D.
     Director
     National Institute of Child Health and Human Development
     Duane F. Alexander, M.D.
     Director
     National Institute of Neurological Disorders and Stroke
     Zach W. Hall, Ph.D.
     Director
     U.S. Department of Veterans Affairs
     Jesse Brown
     Secretary

ABOUT THE NIH CONSENSUS DEVELOPMENT PROGRAM

NIH Consensus Development Conferences are convened to evaluate available
scientific information and resolve safety and efficacy issues related to a
biomedical technology. The resultant NIH Consensus Statements are intended to
advance understanding of the technology or issue in question and to be useful
to health professionals and the public.

NIH Consensus Statements are prepared by a nonadvocate, non-Federal panel of
experts, based on (1) presentations by investigators working in areas relevant
to the consensus questions during a 2-day public session, (2) questions and
statements from conference attendees during open discussion periods that are
part of the public session, and (3) closed deliberations by the panel during
the remainder of the second day and morning of the third. This statement is an
independent report of the panel and is not a policy statement of the NIH or the
Federal Government.

Statement Availability

Preparation and distribution of this statement is the responsibility of the
Office of Medical Applications of Research of the National Institutes of
Health. Free copies of this statement and bibliographies prepared by the
National Library of Medicine are available from the Office of Medical
Applications of Research, National Institutes of Health, or the NIH Consensus
Program Information Service by 24-hour voice mail. In addition, free copies of
all other available NIH Consensus Statements and NIH Technology Assessment
Statements may be obtained from the following resources:

     NIH Consensus Program Information Service
     P.O. Box 2577
     Kensington, MD 20891
     Telephone 1-800-NIH-OMAR (644-6627)
     Fax [phone]

     NIH Office of Medical Applications of Research
     Federal Building, Room 618
     7550 Wisconsin Avenue MSC 9120
     Bethesda, MD 20892-9120

!
*
THE "COMMON" COLD: (So named because we all seem to get it)

Usually colds occur in the Fall and Winter.  The "cold" is caused by a number
of different viruses which infect the upper respiratory system: nose, throat,
and trachea (windpipe) and sometimes lower into the lungs.  The influenza (flu)
virus is one of many types of viruses which can cause a "common cold".

The typical "cold" lasts 7-10 days, but may last up to 2-4 weeks.  Symptoms
include: fever (99-101), runny or stuffy nose, sore throat, cough, headache,
muscle aches, weakness and hoarseness.

The common cold is not curable with antibotics.  Your body's defenses will
eventually cure the infection.  Your recovery will be helped by adequate rest,
good nutrition, avoiding excessive physical activity, not smoking, and using
alcohol only in moderation.  Your doctor will often recommend medicine to
alleviate specific symptoms.

Headache, fever and muscle ache can be helped by acetaminophen (Tylenol) or
ibuprofen.  Tylenol is preferred to aspirin because aspirin has been associated
with an illness called Reyes Syndrome.  Runny nose or stuffiness is helped by
decongestants or antihistamines or a combination.  Normally it is best to avoid
the antihistamines which may cause sedation and dry out secretions.  Coughing
can be suppressed by cough medicine containing Dextromethorphan or a codeine
preparation.  Studies have shown that 500 mg. of Vitamin C taken every 4-6
hours will shorten the course of a cold by 36-48 hours.  Vitamin C has a mild
antihistamine effect that will not cause excessive drying.  A stuffy, plugged
nose can be helped by saline (salt water) nose drops.  Other nose drops such as
Afrin can be used to a lesser degree (no more than 3 days).

In general, the common cold can be treated in the dorm room or home.  High
fever, severe headache, prolonged cough or sore throat more than 2-3 days,
cough or runny nose productive of yellow or green colored mucous or shortness
of breath do warrant a visit to the Health Service.

A flu shot, given before the flu season, may help decrease the incidence or
severity of an influenza infection.  This vaccine is recommended primarily for
the elderly or those with chronic diseases such as diabetes.  Amantadine, an
antiviral drug, can be useful in the treatment of influenza but needs to be
started in the first 24-48 hours to be effective.
!
*
While this article was written specifically for the teachers of
students with Crohn's Disease and Ulcerative Colitis, the
information will be helpful to families and friends of persons
with one of these disorders.  Many ideas are applicable to
other health problems as well.

For more information, please contact:

National Foundation for Ileitis & Colitis, Inc. 444 Park Avenue South New
York, NY  10016 [phone]


A TEACHER'S GUIDE TO CROHN'S DISEASE & ULCERATIVE COLITIS

Some Facts You Should Know:

1.  Crohn's disease and ulcerative colitis are lifelong illnesses.
Medications often alleviate inflammation and discomfort, but do not cure
the diseases.

2.  Crohn's disease and ulcerative colitis are NOT caused by emotional
stress.  Young people with these illnesses need the support of teachers as
well as family and friends in order to cope.

3.  Crohn's disease and ulcerative colitis are NOT contagious.
Researchers feel the diseases may be caused by an unknown bacterium or
virus, or by a defect in the body's immune system.

4.  Most children do NOT need special diets.  They can generally eat what
their classmates eat, although they may avoid certain foods which cause
them discomfort.  However, when the disease is active, they may be more
selective in their food intake.

5.  Young people with Crohn's disease and ulcerative colitis need ready
access to a bathroom.  Diarrhea is often a severe problem for these
youngsters, and getting to a bathroom in time can be a great source of
anxiety.

Young people with Crohn's disease and ulcerative colitis need our
understanding and support in order to develop their full academic and
social potential.


It would be wonderful if all young people could enjoy good health and
smooth adjustment during their elementary and high school years.
Unfortunately, some children are plagued with chronic illnesses such as
Crohn's disease and ulcerative colitis.  Just getting through the school
day can be an extremely difficult task without the support and
encouragement of teachers who understand their illness.

ABOUT THE DISEASES

Crohn's disease is a condition in which the wall of the small or large
intestine becomes sore, inflamed, and swollen.  This causes abdominal
pain, diarrhea, fever, and loss of weight.  Some young people experience
pain in their knees, ankles, and other joints.  Crohn's disease is called
ileitis when it affects the lower small intestine, Crohn's colitis when it
affects the large intestine (colon), and ileocolitis when both small and
large intestines are involved.

Ulcerative colitis causes inflammation of the inner lining of the large
intestine.  For this reason, diarrhea is often mixed with blood and may
occur with great urgency to the point of incontinence.  There may also be
abdominal pain and pain in the joints.

Crohn's disease and ulcerative colitis may occur at any age, even in
children under 10 years of age.  Boys and girls appear to be affected
equally.  These diseases are NOT to be confused with "spastic colon," or
irritable bowel syndrome, a far less serious, unrelated condition.

COPING WITH THE DISEASE AND ITS TREATMENT

"The hardest thing for me to cope with was the fact that I was different."

"My disease sort of isolates me from other kids.  So it is hard for me to
make friends.  Deep down I really want to be like everyone else."

Young people with Crohn's disease or ulcerative colitis must cope with
attacks of pain and diarrhea, episodes of fever and nausea, and lack of
appetite.  Because many of these children eat less than their bodies
require, they often grow at a slower rate and may appear younger and
smaller than their classmates.  These combined problems often cause them
to withdraw and become depressed or angry, especially during the
pre-adolescent or adolescent years.

Treatment can cause problems, too.  Cortisone-like drugs are effective in
controlling, but not curing, the inflammation caused by Crohn's disease
and ulcerative colitis.  But these drugs often cause youngsters to gain
weight, to develop a rounded puffy appearance, to have worsening of acne,
and to become moody and restless.  This change in appearance further
isolates them from their classmates, who may not be aware of their illness
and may ridicule or make fun of them.

ABSENCE FROM SCHOOL

"My biggest problem is that most of my teachers don't think I look sick!"

Not all young people with Crohn's disease or ulcerative colitis are small
or have side effects of medication.  They may APPEAR quite well but
actually FEEL too ill to attend school.  They might also feel well one day
and sick the next.  The disease and medications cause either fatigue or
sleeplessness, making it difficult for the child to keep alert during the
school day, and keep up with assignments.  An understanding of these
patterns helps the child to cope with missed assignments and lost school
days.

Many young people with Crohn's disease or ulcerative colitis require
hospitalization from time to time, sometimes for several weeks or even
longer.  Surgery may be necessary to remove diseased intestine or
alleviate a complication.  While in the hospital, they appreciate hearing
from classmates and teachers and frequently can keep up with schoolwork
and assignments.

LEAVING THE CLASSROOM

"Sometimes when I have to leave the classroom, some teachers give me a
hard time, and this forces me into explaining my situation.  Meanwhile, I
am in pain and do not feel I have to explain my condition."

Young people with these illnesses tell us that their most difficult
problem in school is their need to use the bathroom.  Unfortunately for
them, attacks of pain and diarrhea may occur suddenly and without warning.
Children with Crohn's disease or ulcerative colitis MUST be able to leave
the classroom quickly and without attracting attention.  Questions about
their need in front of classmates will only cause intense embarrassment
and shame, and even a short delay may cause an accident.  When treated
like adults, young persons will usually respond maturely and will not
abuse the privilege of leaving the classroom to use the bathroom.

In some schools, bathrooms are locked for long periods during the day to
encourage students to remain in the classroom; in many schools, the stalls
in the bathrooms do not have doors.  These situations present further
obstacles to students needing a bathroom urgently.  One solution might be
to provide a key or a special pass to be used at any time during the
school day; another might be to have the student use the bathroom in the
nurse's office.  these solutions acknowledge the reality of the problem
and show respect for the student's feelings.

CLASS TRIPS

"I was never sure what to do about field trips because I never knew in
advance how I was going to feel that day.  The problem was that the money
had to be collected in advance with no refunds."

Participation in class trips and outings can present problems for the
student with Crohn's disease or ulcerative colitis.  Attacks of pain, gas,
or diarrhea during long bus trips, for example, can cause intense anxiety
even in the most secure student.  Since few young people are willing to
impose their requirements for frequent bathroom stops on the whole group,
a child whose disease is causing problems may decide to skip the trip
altogether.

TAKING MEDICATION DURING SCHOOL HOURS

Students with these conditions often need to take medications during the
school day to help control their diarrhea or pain.  Schools generally
require that these medications be dispensed by the school nurse.  This
often means that the student must take time from a lunch period or may be
late to class as a result.  Ongoing communication between the school nurse
and the teachers will make this routine easier for the student.

PARTICIPATION IN SPORTS

Young people with these illnesses should be as active as they would like
to be.  They can participate in sports if they feel well enough.  However,
some strenuous sports may cause fatigue or aggravate abdominal or joint
pains. If this happens, a modified gym program may be the answer.
Teachers or guidance personnel can usually work closely with parents and
the child's physician in developing an individualized program.  If the
student has had recent surgery, his or her surgeon will make the
determination when it is safe to resume activity.

(For more information on these two disorders, choose "Crohn" and
"Ulcerative Colitis" as your search terms in the Rare Disease Database.)
!
*
                      CONGENITAL HEART DEFECTS

 COMMON CHARACTERISTICS
     A congenital abnormality is a defect that is present at birth.
     It is estimated that eight out of every one thousand babies are
     born with some type of congenital heart defect.  The
     abnormality may be minor, requiring no treatment or restriction
     of activity.  On the other hand, it may be so severe that no
     effective treatment is available and the infant will die.
     Between these extremes, there is a range of congenital heart
     defects of various degrees of severity.  Some have become
     increasingly amenable to surgical treatment, offering new hope
     to families.

 CAUSES
     The fetal heart begins to develop early in pregnancy.  By about
     the fourth week, it starts to twitch and then to beat; by the
     sixth week, the four heart chambers are recognizable; by the end
     of the third month, its development is complete.  Any
     abnormality of development during these weeks may cause a
     congenital heart defect.

     When a baby is born with a heart defect, parents are often
     distressed about their own possible role in causing the
     disorder.  However, it is seldom that anything parents do--or
     fail to do--has any causal relationship to the baby's problem.
     In fact, in 90 percent of cases, the causes of congenital heart
     defects remain unknown.

     Some congenital heart defects are known to be associated with
     disturbances in oxygen supply to the mother, or with infections
     such as German measles (rubella), or with specific injuries or
     nutritional deficiencies.  Sometimes a defective chromosome is
     associated with the problem, as in Down's syndrome.  Several
     drugs, when taken early in pregnancy, have been shown to lead to
     heart abnormalities in babies.  Therefore, no woman who is
     pregnant or thinks she may be pregnant should take any
     medication unless it has been approved by a physician.

     Finally, there is a tendency for congenital heart defects to run
     in families.  If either parent has or has had a congenital heart
     defect, the risk that a child will be affected is about one in
     twenty.  Thus, if you or any of your immediate family members
     has such a problem, you might wish to consult a genetic
     counselor before starting a pregnancy.

 SYMPTOMS
     Frequently, a baby with a congenital heart defect has no
     symptoms, and the abnormality is discovered during a routine
     examination.  In other cases, the symptoms are marked at
     birth--or may become severe in early infancy.  The presence of
     symptoms is not necessarily related to the need for treatment;
     many children with no symptoms at all require surgery to prevent
     later problems, while others who have symptoms may outgrow the
     problem without treatment.

     A classic indication of a congenital heart defect is cyanosis, a
     blueness of the skin and mucous membranes caused by an excess of
     underoxygenated blood in the circulatory system.  The birth of
     such a "blue baby" may be a medical emergency.  More often, the
     symptoms are less obvious.  For example, an infant with mild
     heart failure--a less than normal output of blood from the
     heart--may have problems feeding, because sucking is difficult,
     and may therefore be underweight.  Infants with severe heart
     failure may have rapid and distressed breathing.  Many children
     with congenital heart defects become breathless on exertion, or
     even at rest.  Their physical development is often below
     average.

 DIAGNOSIS
     The physician may detect the presence of a congenital
     abnormality through the simple use of a stethoscope, which picks
     up normal heart sounds and abnormal ones such as murmurs.
     (Murmurs do not necessarily signal heart abnormalities:
     "innocent murmurs" are heard in about 60 percent of normal
     infants.)  Different congenital abnormalities generally produce
     recognizably different and characteristic murmurs, and in most
     cases the doctor can detect which abnormality is present.

     A clearer picture may be derived from diagnostic tests.  These
     include chest X-rays, which show the shape and size of the
     heart; electrocardiograms, which show electrical impulses
     produced by the heart and can reveal thickening of its chambers;
     and ultrasound examinations, which show the thickness of the
     heart chamber walls, the size of the chambers and the condition
     of the valves.  Sometimes cardiac catheterization, an
     examination in which a catheter is passed along a blood vessel
     until it reaches the heart, is required.

 TYPES OF DEFECTS
     Eight types of defects are most common in infants.  CONGENITAL
     AORTIC STENOSIS is a narrowing of the aortic valve and sometimes
     of the aorta itself.  This restricts the flow of blood to the
     body generally.  CONGENITAL PULMONARY STENOSIS is a narrowing of
     the pulmonary valve--or, less frequently, of the pulmonary
     artery or the upper right ventricle--that restricts the flow of
     blood to the lungs.  In VENTRICULAR SEPTAL DEFECTS and ATRIAL
     SEPTAL DEFECTS, there are holes in the wall within the heart that
     separates the left and right chambers.  These holes disturb
     normal flow within the chambers, leading to an excess of blood
     circulating in the lungs, which, in turn, leads to fibrosis of
     the pulmonary vessels.  While about a quarter of all ventricular
     holes close spontaneously, such closure is less common for
     atrial defects.

     COARCTATION OF THE AORTA is a constriction that limits the blood
     supply to the lower parts of the body.  PATENT DUCTUS ARTERIOSUS
     involves a failure of the ductus to close after birth.  In the
     womb, the ductus prevents circulation of blood through the
     fetus's lungs, which are not needed since oxygen is supplied
     through the placenta.  After birth, the ductus normally closes.
     If it does not, blood from the aorta flows through the open
     ductus into the pulmonary artery and results in excess blood
     passing through the lungs.  In FALLOT'S TETRALOGY, four
     abnormalities occur together: a hole between the ventricles,
     aortic displacement, a narrowed pulmonary valve, and a
     thickening of the wall of the right ventricle.  In the defect
     known as TRANSPOSITION OF THE GREAT ARTERIES, the locations of
     the aorta and pulmonary arteries are switched.  Here,
     oxygenated blood coming from the lungs returns to the pulmonary
     arteries instead of passing through the aorta to the peripheral
     tissues.  There is usually a hole in the septum, allowing some
     oxygenated blood to reach the aorta.

 TREATMENT
     Except in the case of very small holes in the heart, or when
     patent ductus arteriosus is detected early and occasionally
     closed with drug treatment, most congenital heart defects
     require surgery to correct the abnormality.  If the disorder is
     not so severe as to demand immediate surgery, the operation may
     be delayed until the child is five years old or older, when he
     or she is larger and better able to withstand the stress of open
     heart surgery.

 SUMMING UP
     In most cases of congenital heart defects, the cause of the
     abnormality is not known.  Drugs, infections such as rubella,
     and hereditary factors are implicated in about 10 percent of the
     cases.

     With modern surgical techniques, the success rate for heart
     operations in children is very high.  Most children who have
     effective surgical treatment for a congenital heart defect can
     expect to live normal lives.
!
*
Conjunctivitis

                      CONJUNCTIVITIS

Introduction
-------------
Conjunctivitis, commonly known as "pink eye", is an inflammation of
the membrane (conjunctiva) that covers the eye and lines the inner
surface of the eyelid.  There are three main causes of conjunctivitis.

One cause involves the introduction of either bacterial or viral
microorganisms into the eye.  These may be transmitted to the eye by
contaminated hands, washcloths or towels, cosmetics (particularly eye
makeup), false eyelashes or extended wear contacts.  It is not uncommon
for a minor conjunctivitis to accompany a viral cold or flu.  Although
bacterial and some of the viral infections (particularly herpes) are
not very common, they are potentially serious.  Both types of infection
are contagious.  Irritants are another cause of conjunctivitis. Offenders
of this type include air pollutants, smoke, soap, hairspray, makeup,
chlorine, cleaning fluids, etc.  Lastly, some individuals acquire
conjunctivitis due to a seasonal allergic response to grass and other
pollens.


Symptoms
---------
Various combinations of the following symptoms may be present: itching,
redness, photophobia (sensitivity to light), swelling of the lids and/or
discharge from the eyes. The consistency of possible discharge may range
from watery to purulent (pus-like), depending on the specific cause of
the conjunctivitis.


Duration
--------
It usually takes from a few days to 2 weeks for most types of
conjunctivitis to clear. Conjunctivitis due to an allergy may continue
as long as the offending pollen is present.  With such conditions,
symptoms are likely to recur each year.


Diagnosis
----------
Diagnosis consists of physical examination of the eye by the clinician.
If a discharge is present, a culture for bacteria may be warranted.


Treatment
---------
Treatment varies depending on the cause.  Medications in the form of
ointments, drops or pills may be recommended to either help kill the
germ infecting the eye, relieve allergic symptoms and/or decrease
discomfort.  In the case of conjunctivitis due to a viral cold or flu,
the practitioner may recommend that you be patient and let it run its
course.

Other measures that should be followed:

  * Apply cool compresses to the eye(s) 3-4 times per day for 10-15
    minutes using a clean washcloth each time.  This should help reduce
    itching and swelling and provide some comfort.
  * Wash your hands frequently and keep them away from your eyes in order
    to reduce or prevent recontamination.
  * Avoid rubbing your eyes to decrease irritation of the area.
  * Wear sunglasses if your eyes are sensitive to the light.
  * Avoid exposure to the irritants which may be causing the conjunctivitis.
  * Dispose of old eye makeup if the culture for bacteria is positive.
  * Use a clean pillow case each night.  (Pillow case can be changed
    every other day and turned over nightly).
  * Avoid wearing contact lenses while you are using medications or if
    your eyes are uncomfortable.


Prevention
-----------
Although many kinds of conjunctivitis are hard to prevent, there are
measures which can be taken to decrease your risk of reacquiring or
spreading it to someone else. These are listed below:

  * Do not share eye makeup or cosmetics of any kind with someone else.
  * Avoid sharing washcloths or towels.
  * Wash hands frequently and keep away from the eyes.
  * Wear protective goggles (i.e., for swimming or working) if you must
    be exposed to chemicals which are irritating.
  * Do not use medication (eye drops, ointment, etc.) which has been
    prescribed for someone else.
  * Avoid swimming in nonchlorinated pools or stagnant lakes or ponds.


Concerns
---------
If any of the following problems should occur, notify your clinician:

  * Visual changes
  * Severe eye pain
  * Pain when moving eyes
  * Fever
  * No improvement with medication within 48-72 hours
  * Drainage continues after you have completed full course of medication
  * Roommates or other family members develop symptoms
  * Eyes become very sensitive to light


Directions for using eye drops or ointment
-------------------------------------------
  1. Wash your hands before touching your eyes or your medications.
  2. Gently, pull your lower lid down with your finger.
  3. Look up toward the ceiling.
  4. To instill drops:  Drop medicine inside center of your lower lid.
     Do not drop it on your eyeball.  Close your eyes gently without
     squeezing the lids shut.  Blink to distribute medication over the
     eye.  To instill ointment:  Starting in the corner of your eye
     closest to the nose, squeeze a thin ribbon of ointment along the
     inside of the lower lid.  Close eyes gently without squeezing the
     lids shut.  Roll eyes to distribute the medication over them.
  5. Remove excess solution or ointment outside your eye with a clean
     tissue, using a separate tissue for each eye.
  6. Wash hands after you have finished your medication, to avoid
     transmitting the infection to others.


If you are concerned about any difference in your treatment plan and the
information in this handout, you are advised to contact your health care
provider.


References:
-----------
Boyd-Monk, H.  (1983).  Conjunctivitis.  In H. K. Hamilton (Ed.), Diseases
(pp. 1184-1186).  Springhouse, PA: Intermed Communications.

Trepeta, J.  (1984).  Conjunctivitis.  In S. L. Shamansky, M. C. Cecere,
and E.  Shellenberger (Eds.), Primary Health Care Handbook (p. 132).
Boston, MA:  Little, Brown.

Vickery, D. M. and Fries, J. F.  (1986).  Eye burning, itching, and
discharge. Take Care of Yourself: A Consumer's Guide to Medical Care
(pp. 310-311).  Reading, MA: Addison-Wesley.
!
*
COPROPORPHYRIA

RESEARCH REQUEST:   COPROPORPHYRIA

October 8, 1995


General Information
------------------

Hereditary Coproporphyria is an autosomal dominant form of hepatic porphyria
that is very similar to Acute Intermittent Porphyria, although it is usually a
less severe disease.  It is caused by an enzyme deficiency. Some patients
develop skin photosensitivity, and must avoid sunlight.  The diagnosis is
established by finding excess coproporphyrin in urine and stool (other types of
porphyrins show little or no increase).  Urinary ALA and PBG are increased
during acute attacks, but may become normal on recovery.

The Porphyrias are a group of at least seven disorders.  The common feature in
all porphyrias is the excess accumulation in the body of "porphyrins" or
"porphyrin precursors."  These are natural chemicals that normally do not
accumulate in the body.  Precisely which one of these porphyrin chemicals builds
up depends upon the type of porphyria that a patient has.

Porphyrias can also be classified into two groups:  the "hepatic" and
"erythropoietic" types.  Porphyrins and related substances originate in excess
amounts from the liver in the hepatic types, and mostly from the bone marrow in
the erythropoietic types.

The porphyrias with skin manifestations are sometimes called "cutaneous
porphyrias."  The "acute porphyrias" are characterized by sudden attacks of pain
and other neurological manifestations.  These "acute symptoms can be both
rapidly-appearing and severe.  An individual may be considered in a "latent"
condition if he or she has the characteristic enzyme deficiency, but has never
developed symptoms.  There can be a wide spectrum of severity between the
"latent" and "active" cases of any particular type of this disorder.

The symptoms and treatments of the different types of porphyrias are not the
same.


Symptoms
--------

The large amount of coproporphyrin present in Hereditary Coproporphyria (HCP)
makes the patient sensitive to sunlight, but skin disease is rarely severe in
this type or porphyria.  Clinically, it resembles variegate porphyria and acute
intermittent porphyria.  Symptoms may include abdominal pain, arm and/or leg
pain, generalized weakness, vomiting, confusion, constipation, increased heart
rate, fluctuating blood pressure, urinary retention, psychosis, hallucinations,
and seizures.  The muscle weakness may progress to respiratory paralysis,
necessitating artificial respiration.

The symptoms of porphyria generally arise from effects on the nervous system
and/or the skin.  Sometimes, the cause of the nervous system symptoms is not
clear, and proper diagnosis is delayed.  Skin manifestations can include
burning, blistering and scarring of sun-exposed areas.

Porphyria Cutanea Tarda is the only type of porphyria that can be either
acquired or inherited.  All other types of Porphyria are caused by genetic
factors.  Environmental factors such as drugs, chemicals, diet and sun exposure
can, depending on the type of the disorder, greatly influence the severity of
symptoms.

The terms "porphyrin" and "porphyria" are derived from the Greek word
"porphyrus," meaning purple.  Urine from some porphyria patients may be reddish
in color due to the presence of excess porphyrins and related substances, and
the urine may darken after being exposed to the light.

Because this disease can mimic a host of other more common conditions, its
presence is often not suspected.  On the other hand, the diagnosis of this and
other types of porphyria is sometimes made incorrectly in patients who do not
have porphyria, particularly if improper laboratory tests are carried out.  The
finding of increased levels of delta-aminolevulinic acid (ALA) in urine
establishes that one of the "acute" porphyrias is present.

When a patient is diagnosed as having HCP, relatives should be examined as well.
Latent cases so identified can then avoid agents known to cause attacks.


Causes
------

Hereditary Coproporphyria is a genetic, non-x-linked disorder inherited as an
autosomal dominant trait.  (In autosomal dominant disorders, a single abnormal
gene, contributed by either parent, "overrides" the normal gene contributed by
the other parent causing disease.  Individuals with one affected parent have a
50% chance of inheriting the disorder.  Males and females will be affected in
equal numbers.)

Drugs such as barbiturates, tranquilizers, anticonvulsants, and estrogens may
precipitate attacks.

Environmental factors may include drugs, chemicals, diet and sun exposure.
Depending on the type of porphyria, these factors can greatly influence the
severity of symptoms.

Because all porphyrias are uncommon, it is very unlikely that more than one type
will occur in the same family, or that someone with one type of porphyria will
go on to develop another.


Affected Population
-------------------

Hereditary Coproporphyria may have its onset at any age, and may affect males
and females in equal numbers.  It is the least common of the "hepatic"
porphyrias.


Related Disorders
-----------------

The Porphyrias are a group of related disorders.  For more information on each
of the following types of the disease, choose "porphyria" as your search term in
the Rare Disease Database.

ALA-D Porphyria is a recently-described form of acute porphyria inherited as an
autosomal recessive trait.  It is apparently extremely rare.  There is a
deficiency of the enzyme delta-aminolevulinic acid dehydratase (ALA-D) and
increased excretion of ALA in the urine of patients with this type or porphyria.

Acute Intermittent Porphyria is a hereditary, possibly metabolic,
usuallyasymptomatic disorder (latent).  It may possibly be provoked into active
disease by the administration of certain drugs, notably barbiturates,
sulfonamides, and estrogenic compounds.

Congenital Erythropoietic Porphyria (CEP) is a hereditary disorder due to an
inborn error of metabolism, and manifested in infancy.  Faulty conversion of the
enzyme PBG to uroporphyrinogen in erythroid cells of bone marrow, and red blood
cells leads to this type of Porphyria.  Increased porphyrins also may be found
in plasma, urine, feces, teeth and bones.

Porphyria Cutanea Tarda (PCT) can be either an acquired or inherited type of
Porphyria.  It may become acute due to exposure to chronic alcoholism,
barbiturates or other chemicals, cirrhosis of the liver, or a hepatic tumor. It
may also stem from a nutritional disorder.

Variegate Porphyria (VP) is a hereditary type of Porphyria due to an inborn
error of metabolism.  Precipitating or aggravating factors may include exposure
to barbiturates, sulfonamides, general anesthetics, excessive amounts of
ethanol, and estrogens.

Erythropoietic Protoporphyria (EPP) is a hereditary type of Porphyria marked by
an accumulation of protoporphyrin in the bone marrow, red blood cells and
sometimes the liver.  Excess protoporphyrin is excreted by the liver into the
bile, which in turn enters the intestine and is excreted in the feces.  There
are no urinary abnormalities.  The diagnosis is established by finding increased
protoporphyrin in red blood cells, plasma and feces.

Therapies:  Standard
--------------------

The orphan drug Hematin (an intravenous drug) is very potent in suppressing
acute attacks of the disease.  It is usually given only after a trial of glucose
therapy.  Attention should be given to salt and water balance during treatment.

Many types of drugs such as aspirin and certain antibiotics are believed to be
safe in patients with some types of porphyria.  Recommendations about drugs for
certain types of the disorder are based on experience with the porphyria
patients in whom attacks have been caused by drugs and by tests in animals.
Since many commonly used drugs have not been tested, they should be avoided if
at all possible.  If a question of drug safety arises, a physician or medical
center specializing in porphyria should be contacted.  A list of these
institutions may be procured from the American Porphyria Foundation (see
Resources).

Pregnancy is tolerated much better than was formerly believed.  Many patients
have a few reservations about family planning.  For those who do, genetic
counseling may be useful.

Wearing a Medic Alert bracelet is advisable in patients who have had attacks,
but is probably not warranted in most latent cases.

Therapies:  Investigational
---------------------------

New treatments for several types of porphyria are under investigation.  For the
most updated information on research, please contact the organizations listed in
the Resources section.

Dr. Karl E. Anderson of the University of Texas Medical Branch, Galveston, TX,
77550, has received orphan drug designation for Histrelin, a drug to treat
various types of Porphyria.

Research is underway on the Finnish product Normasang (heme arginate). Dr. Karl
Anderson of The University of Texas Medical Branch will be directing clinical
studies in the United States.  Patients are needed to participate in this
research.  People interested in this study should have their physician contact:

Dr. Karl Anderson
Ewing Hall (J-09)
University of Texas Medical Branch
700 Strand St.
Galveston, TX  77555
[phone]

The Orphan product Hemin and Zinc Mesoporphyrin (HEMEX) is being used for
treatment of Acute Prophyric Syndromes.  For more information, contact:

Herbert L. Bonkovsky, MD
University of Massachusetts
Medical Ctrl
Worchester, MA  01655

Resources
---------

For more information on Hereditary Coproporphyria, please contact:

American Porphyria Foundation
P.O. Box 22712
Houston, TX  77227
[phone]

Porphyria Support Group
4 Eve Road
Leytonstone, London, England
E11 3JE
Tel:  [phone]

NIH/National Digestive Diseases Information Clearinghouse (NDDIC)
9000 Rockville Pike
Bethesda, MD  20892
[phone]

For information on genetics and genetic counseling referrals, please contact:

March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY  10605
[phone]

Alliance of Genetic Support Groups
35 Wisconsin Circle, Suite 440
Chevy Chase, MD  20815
(800) 336-GENE
[phone]


References
----------

American Porphyria Foundation brochure, "Common Questions About Porphyria."
!
*
CROHN'S DISEASE

Fact Sheet

DD Clearinghouse


Inflammatory bowel disease (IBD) is a group of chronic disorders that cause
inflammation or ulceration in the small and large intestines.  Most often IBD
is classified as ulcerative colitis or Crohn's disease but may be referred to
as colitis, enteritis, ileitis, and proctitis.

Ulcerative colitis causes ulceration and inflammation of the inner lining of
the colon and rectum, while Crohn's disease is an inflammation that extends
into the deeper layers of the intestinal wall.

Ulcerative colitis and Crohn's disease cause similar symptoms that often
resemble other conditions such as irritable bowel syndrome (spastic colitis).
The correct diagnosis may take some time.

Crohn's disease usually involves the small intestine, most often the lower
part (the ileum).  In some cases, both the small and large intestine (colon)
are affected.  In other cases, only the colon is involved.  Sometimes,
inflammation also may affect the mouth, esophagus, stomach, duodenum,
appendix, or anus.  Crohn's disease is a chronic condition and may recur at
various times over a lifetime.  Some people have long periods of remission,
sometimes for years, when they are free of symptoms.  There is no way to
predict when a remission may occur or when symptoms will return.

What Are the Symptoms?

The most common symptoms of Crohn's disease are abdominal pain, often in the
lower right area, and diarrhea.  There also may be rectal bleeding, weight
loss, and fever.  Bleeding may be serious and persistent, leading to anemia
(low red blood cell count).  Children may suffer delayed development and
stunted growth.

What Causes Crohn's Disease and Who Gets It?

There are many theories about what causes Crohn's disease, but none has been
proven.  One theory is that some agent, perhaps a virus or a bacterium,
affects the body's immune system to trigger an inflammatory reaction in the
intestinal wall.  Although there is a lot of evidence that patients with this
disease have abnormalities of the immune system, doctors do not know whether
the immune problems are a cause or a result of the disease.  Doctors believe,
however, that there is little proof that Crohn's disease is caused by
emotional distress or by an unhappy childhood.

Crohn's disease affects males and females equally and appears to run in some
families.  About 20 percent of people with Crohn's disease have a blood
relative with some form of inflammatory bowel disease, most often a brother or
sister and sometimes a parent or child.

How Does Crohn's Disease Affect Children?

Women with Crohn's disease who are considering having children can be
comforted to know that the vast majority of such pregnancies will result in
normal children.  Research has shown that the course of pregnancy and delivery
is usually not impaired in women with Crohn's disease.  Even so, it is a good
idea for women with Crohn's disease to discuss the matter with their doctors
before pregnancy.  Children who do get the disease are sometimes more severely
affected than adults, with slowed growth and delayed sexual development in
some cases.

How Is Crohn's Disease Diagnosed?

If you have experienced chronic abdominal pain, diarrhea, fever, weight loss,
and anemia, the doctor will examine you for signs of Crohn's disease.  The
doctor will take a history and give you a thorough physical exam.  This exam
will include blood tests to find out if you are anemic as a result of blood
loss, or if there is an increased number of white blood cells, suggesting an
inflammatory process in your body.  Examination of a stool sample can tell the
doctor if there is blood loss, or if an infection by a parasite or bacteria is
causing the symptoms.

The doctor may look inside your rectum and colon through a flexible tube
(endoscope) that is inserted through the anus.  During the exam, the doctor
may take a sample of tissue (biopsy) from the lining of the colon to look at
under the microscope.

Later, you also may receive x-ray examinations of the digestive tract to
determine the nature and extent of disease.  These exams may include an upper
gastrointestinal (GI) series, a small intestinal study, and a barium enema
intestinal x-ray.  These procedures are done by putting the barium, a chalky
solution, into the upper or lower intestines.  The barium shows up white on
x-ray film, revealing inflammation or ulceration and other abnormalities in
the intestine.

If you have Crohn's disease, you may need medical care for a long time.  Your
doctor also will want to test you regularly to check on your condition.

What Is the Treatment?

Several drugs are helpful in controlling Crohn's disease, but at this time
there is no cure.  The usual goals of therapy are to correct nutritional
deficiencies; to control inflammation; and to relieve abdominal pain,
diarrhea, and rectal bleeding.

Abdominal cramps and diarrhea may be helped by drugs.  The drug sulfasalazine
often lessens the inflammation, especially in the colon.  This drug can be
used for as long as needed, and it can be used along with other drugs.  Side
effects such as nausea, vomiting, weight loss, heartburn, diarrhea, and
headache occur in a small percentage of cases.  Patients who do not do well on
sulfasalazine often do very well on related drugs known as mesalamine or 5-ASA
agents.  More serious cases may require steroid drugs, antibiotics, or drugs
that affect the body's immune system such as azathioprine or 6-mercaptopurine
(6-MP).

Can Diet Control Crohn's Disease?

No special diet has been proven effective for preventing or treating this
disease.  Some people find their symptoms are made worse by milk, alcohol, hot
spices, or fiber.  But there are no hard and fast rules for most people.
Follow a good nutritious diet and try to avoid any foods that seem to make
your symptoms worse.  Large doses of vitamins are useless and may even cause
harmful side effects.

Your doctor may recommend nutritional supplements, especially for children
with growth retardation.  Special high-calorie liquid formulas are sometimes
used for this purpose.  A small number of patients may need periods of feeding
by vein.  This can help patients who temporarily need extra nutrition, those
whose bowels need to rest, or those whose bowels cannot absorb enough
nourishment from food taken by mouth.


What Are the Complications of Crohn's Disease?

The most common complication is blockage (obstruction) of the intestine.
Blockage occurs because the disease tends to thicken the bowel wall with
swelling and fibrous scar tissue, narrowing the passage.  Crohn's disease also
may cause deep ulcer tracts that burrow all the way through the bowel wall
into surrounding tissues, into adjacent segments of intestine, into other
nearby organs such as the urinary bladder or vagina, or into the skin.  These
tunnels are called fistulas.  They are a common complication and often are
associated with pockets of infection or abscesses (infected areas of pus).
The areas around the anus and rectum often are involved.  Sometimes fistulas
can be treated with medicine, but in many cases they must be treated
surgically.

Crohn's disease also can lead to complications that affect other parts of the
body.  These systemic complications include various forms of arthritis, skin
problems, inflammation in the eyes or mouth, kidney stones, gallstones, or
other diseases of the liver and biliary system.  Some of these problems
respond to the same treatment as the bowel symptoms, but others must be
treated separately.

Is Surgery Often Necessary?

Crohn's disease can be helped by surgery, but it cannot be cured by surgery.
The inflammation tends to return in areas of the intestine next to the area
that has been removed.  Many Crohn's disease patients require surgery, either
to relieve chronic symptoms of active disease that does not respond to medical
therapy or to correct complications such as intestinal blockage, perforation,
abscess. or bleeding.  Drainage of abscesses or resection (removal of a
section of bowel) due to blockage are common surgical procedures.

Sometimes the diseased section of bowel is removed.  In this operation, the
bowel is cut above and below the diseased area and reconnected.  Infrequently
some people must have their colons removed (colectomy) and an ileostomy
created.  In an ileostomy, a small opening is made in the front of the
abdominal wall, and the tip of the lower small intestine (ileum) is brought to
the skin's surface.  This opening, called a stoma, is about the size of a
quarter or a 50-cent piece.  It usually is located in the right lower corner
of the abdomen in the area of the beltline.  A bag is worn over the opening to
collect waste, and the patient empties the bag periodically.  The majority of
patients go on to live normal, active lives with an ostomy.

The fact that Crohn's disease often recurs after surgery makes it very
important for the patient and doctor to consider carefully the benefits and
risks of surgery compared with other treatments.  Remember, most people with
this disease continue to lead useful and productive lives.  Between periods of
disease activity, patients may feel quite well and be free of symptoms.  Even
though there may be long-term needs for medicine and even periods of
hospitalization, most patients are able to hold productive jobs, marry, raise
families, and function successfully at home and in society.


Addition Readings

Brandt, LJ, Steiner-Grossman, P, eds.  Treating IBD: A Patient's Guide to the
Medical and  Surgical Management of Inflammatory Bowel Disease.  New York:
Raven Press, 1989.  General guide for patients with sections on treatment and
descriptions and drawings of surgical procedures.  Available from the Crohn's
& Colitis Foundation of America.

Clayman, CB, ed. The American Medical Association Encyclopedia of Medicine.
New York: Random House, 1989.  Reference guide for patients with sections on
Crohn's disease and other intestinal disorders.  Available in libraries and
bookstores.

Lipshutz, WH.  Exploring the causes of IBD and Crohn's disease.  Contemporary
Gastroenterology 1991; 4(4): 10-14.  Review article for physicians.  Available
in medical and university libraries.

Steiner-Grossman, P, Banks PA, Present, DH, eds.  The New People Not Patients:
A Source Book for Living with IBD.  Dubuque, Iowa: Kendall/Hunt Publishing
Company, 1992.  Authoritative book for patients with sections on diagnostic
tests, medications, nutrition, coping with employment and health insurance
problems, and IBD in children and teenagers, older adults, and during
pregnancy.  Available from the Crohn's & Colitis Foundation of America.

Tapley, DF, et al., eds. ne Columbia University College of Physicians and
Surgeons Complete Home Medical Guide.  New York: Crown Publishers, Inc., 1989.
General medical guide with sections on Crohn's disease and other intestinal
disorders.  Available in libraries and bookstores.

Addition Resources

Crohn's & Colitis Foundation of America, Inc., 444 Park Avenue South, 11th
floor, New York, NY 10016-7374; [phone] or [phone].

Ileitis and Colitis Educational Foundation, Central
DuPage Hospital, 24 North Winfield Road,
Winfield, IL 60190; [phone], Ext. 649

Wound Ostomy and Continence Nurses Society, 2755 Bristol Street, Suite 110,
Costa Mesa, CA 92626; [phone].

United Ostomy, Association, 36 Executive Park, Suite 120, Irvine, CA 92714;
[phone].

National Digestive Diseases Information Clearinghouse

Box NDDIC, 9000 Rockville Pike, Bethesda, MD 20892
[phone]

The National Digestive.  Diseases Information Clearinghouse is a service of
the National Institute of Diabetes and Digestive and Kidney Diseases, part of
the National Institutes of Health, under the U.S. Public Health Service.  The
clearinghouse was authorized by Congress to focus a national effort on
providing information to the public, patients and their families, and doctors
and other health care professionals.  The clearinghouse works with
organizations to educate people about digestive health and disease.  The
clearinghouse answers inquiries; develops, reviews, and distributes
publications; and coordinates informational resources about digestive
diseases.

Publications produced by the clearinghouse are reviewed carefully for
scientific accuracy, appropriateness of content, and readability.
Publications produced by sources other than the clearinghouse also are
reviewed for scientific accuracy and are used, along with clearinghouse
publications, to answer requests.

This publication is not copyrighted.  The clearinghouse urges users of this
fact sheet to duplicate and distribute as many copies as desired.


National Institutes of Health

NATIONAL INSTITUTES OF DIABETES & DIGESTIVE & KIDNEY DISEASES


U.S. DEPARTMENT OF HEALTH AND HUMAN SERVICE
Public Health Service
National Institutes of Health


This file scanned from NIH Publication No. 92-3410
October 1992
!
*

CROHN'S DISEASE

Like ulcerative colitis, Crohn's Disease affects young people
primarily, and is a disease which causes inflammation of the
colon.  In this disease, however, the small intestine is also
commonly affected, and the farthest reaches of the colon,
rectum, and anal regions are less common targets.

Crohn's disease is of unknown cause, and seems to be less
common than ulcerative colitis. Current theories revolve around
a combination of an autoimmune reaction of the body directed
toward the intestinal tissue, perhaps triggered by one or more
infectious or environmental factors.  Around 30 out of 100,000
Americans have the disease, which is more frequent in Jews for
unknown reasons. Some inconsistent familial tendencies have
been identified, but the genetic component is poorly understood.

Under the microscope, the inflammation of Crohn's Disease is
distinct, and resembles the body's reaction to certain types of
infection such as tuberculosis, although no such germs are
associated.  If the colon is the main area affected, which
happens occasionally if not often, the microscopic picture can
be the only way to separate it from ulcerative colitis in some
cases.

SYMPTOMS

Typically, a teenager or young adult experiences a period of
weeks or months of "the blahs," with fatigue, weight loss, and
depression.  Then ther occur a progressive combination of
abdominal cramping and diarrhea, usually in the lower right
abdomen.  A low grade fever may be present, and eventually,
medical attention is sought.

The disease may also cause fistulae; these are small tunnels or
tracts within the intestine, and may result in communicating
infections or abscesses between areas of intestine, or even
between intestine and bladder, skin, or other organs.
Extra-intestinal symptoms of the disease include joint pains,
rashes, eye inflammation, and kidney and gall bladder stones.

The disease may come on at any age, not rarely in the 40's and
50's.  Delay in diagnosis is not rare, since so many diseases
can cause similar symptoms.

DIAGNOSIS

When the history suggests the diagnosis, this is usually
confirmed with upper and lower digestive x-rays, and
occasionally with a biopsy of tissue when necessary; this can
sometimes be done through a special viewing tube swallowed by
the patient as an outpatient procedure (endoscopy) or through
the proctoscope if the colon is involved.  Evaluation of the
extent and complications usually includes special blood tests.

If enough of the last part of the small intestine is inflamed,
it becomes unable to absorb certain nutrients, especially
vitamin B 12, fats, and dairy sugar.  This can lead to anemia,
malnutrition, and calcium deficiency.

Special care must be taken to rule out alternative diagnoses including
intestinal lymph node cancers like Hodgkin's Disease, appendicitis in the
acute cases, tuberculosis of the intestine, and other rare diseases.

TREATMENT

The approach to treatment of this disease includes a careful
combination of medical and surgical modalities.  General
measures include careful nutritional measures such as a high
protein and ample calorie diet.  Activity and rest should be
carefully combined, and undue fatigue will contribute to the
symptoms of the disease.  Foods such as dairy products and
concentrated fats are often poorly tolerated, and should be
taken in moderation.

Sulfasalazine (Azulfidine and others) is a useful agent for the
treatment of mild flares and prevention of future attacks. It
is metabolized in the intestine to chemicals which have both
anti-inflammatory and anti-microbial actions, but its exact
mechanism of action in not known.

In more severe worsenings, corticosteroids (cortisone-like
drugs) are often used, despite their well-recognized side
effects.  It is questionable whether these drugs alter the
long-term outcome of the disease but the do seem to control
symptoms of acute attacks, especially when organs outside the
intestine are involved, such as eye, skin, and joints.  When
possible, their use should be kept as brief as possible.  When
even corticosteroids are not useful in the seriously ill
patient, immunosuppressive drugs, such as those used in the
treatment of some cancers, may be cautiously added.  These are
serious agents with potentially fatal side effects, and only
should be used in expert hands with careful monitoring.

The role of surgery in this disease is important.
Unfortunately, recurrences in regions adjacent to the surgery,
or even distant areas, is very common. If too much surgery is
done, the patient may be left with insufficient intestine to
absorb nutrients (see malabsorption).  Nonethless, selective
surgery can reduce symptoms, and in the more serious
complications of obstruction or internal fistulae, it may be
the only alternative.

PROGNOSIS

The course is highly variable, and generalizations are of
little use.  There is some increased mortality in patients
compared to those without the disease, but this is often in the
few patients with fulminant, unremitting disease. For most
patients, the disease presents a recurrent source of illness,
the need for long-term medication, and a significant burden.
Optimal care, patient cooperation, and attitude adjustment can
keep these intrusions to a minimum during the frequent
remissions.
!
*


                            CROHN'S DISEASE

 COMMON CHARACTERISTICS
     Crohn's disease is a chronic, or long-term, inflammation of part
     of the digestive tract.  The part most commonly affected is the
     final section of the small intestine, although patchy
     inflammation can occur anywhere in the intestines.  The cause of
     the disease is not known, but it does not seem to be either
     hereditary or infectious.  It begins with the development of
     patches of inflammation in the intestinal wall, which sometimes
     grow or spread from one part of the digestive system to another,
     and sometimes develop in only one place.  Some of the spots heal,
     but they may leave scar tissue that thickens intestinal walls
     and narrows the passageways.

 SYMPTOMS
     Crohn's disease usually appears as periodic attacks of cramps,
     abdominal pain (especially after eating), diarrhea, and a
     general sense of feeling ill.  You may also have a slight fever.
     Attacks tend to begin when you are in your twenties and to
     recur, sometimes every few months, sometimes every few years,
     for the rest of your life.  In about one-quarter of cases, the
     symptoms only appear once or twice, and the disease does not
     recur.


 RISKS
     This has been a rare disorder in the Western world, but it is
     gradually becoming more common.  There are about twice as many
     cases of it today as there were 20 years ago.  If Crohn's
     disease continues for years, it causes a gradual deterioration
     of bowel functioning.  Sometimes the inflamed intestinal wall
     may leak, and cause peritonitis (see Peritonitis). There is a
     risk of poor absorption of nutrients, with the associated loss
     of appetite and weight, or of intestinal obstruction.  Severe
     bleeding that causes iron-deficiency anemia can also occur.
     There is also a slight chance that Crohn's disease, if it is
     not treated, can increase your susceptibility to cancer of the
     intestine.

 TREATMENT
     If your symptoms suggest the possibility of Crohn's disease,
     consult your physician, who, after examining you, will probably
     want X-rays of your digestive tract, for which you will need a
     barium swallow and perhaps a barium enema.  You may also have to
     undergo endoscopy of the intestines in order to locate the
     inflamed areas.   Blood and bowel movement samples may be
     required to see if you show signs of internal bleeding such as
     bloody bowel movements or anemia.

     In most cases treatment by drugs eases attacks of Crohn's
     disease.  Among the drugs used are analgesics, antidiarrhea
     tablets, vitamin supplements to ensure adequate nutrition, and a
     drug that reduces inflammation.  Your physician may also
     recommend certain foods and warn against others, and may suggest
     that you modify your daily routine to relieve tension that may
     affect your nervous and digestive systems.

     To guard against further attacks, long-term treatment with
     steroids may be prescribed.  Another drug that helps, especially
     if your colon is affected, is an antibacterial medication.
     Initial high doses of such drugs may be decreased gradually.
     Such treatment should reduce the severity of the attacks.

     If repeated attacks have scarred and narrowed your intestines so
     badly that the inflammation does not respond to drug treatment,
     you will probably need surgery to remove the section of
     intestine that is most affected.  Surgery cannot cure Crohn's
     disease, but it can provide dramatic improvement and postpone
     further progress of the disease for many years.
!
*
CUSHING'S SYNDROME

Cushing's syndrome is an endocrine, or hormonal, disorder. Although symptoms
vary from person to person, most patients have upper-body obesity, severe
fatigue and muscle weakness, high blood pressure, backache, elevated blood
sugar, easy bruising, and bluish-red stretch marks on the skin. In women,
there may be increased growth of facial and body hair, and menstrual periods
may become irregular or stop completely.

Cushing's syndrome is caused by prolonged exposure of the body's tissues to
high levels of the hormone cortisol. For this reason, the disorder is
sometimes called "hypercortisolism."

Cortisol is normally produced by the adrenal glands, located just above the
kidneys. It belongs to a class of hormones called glucocorticoids, which
affect almost every organ and tissue in the body. Scientists think that
cortisol has possibly hundreds of effects in the body. Among its vital tasks,
cortisol:

* helps maintain blood pressure and cardiovascular function;
* helps slow the immune system's inflammatory response;
* helps balance the effects of insulin in breaking down sugar for
  energy; and
* helps regulate the metabolism of proteins, carbohydrates, and
  fats.

One of cortisone's most important jobs is to help the body respond to
stress. In fact, the adrenal glands naturally produce more cortisol
during stress. High levels of the hormone normally occur in women
during the last 3 months of pregnancy and in highly trained athletes.
Increased cortisol levels also are found in people suffering from
depression, alcoholism, malnutrition, and panic disorders.

To understand the different ways that cortisol production can go
wrong, it helps to look at how hormones normally do their work.  Endocrine
glands differ from other organs in the body because they release hormones into
the bloodstream. Hormones travel through the blood, instructing cells in other
parts of the body to release another hormone or to perform a specific
function. Among the major endocrine glands are the thyroid, parathyroid,
thymus, pituitary, and adrenals.

Together, they play a major role in growth, metabolism, reproduction, and
overall health.The leaders in this carefully balanced performance are the
brain's hypothalamus and the pituitary gland, a bean-sized organ at the base
of the brain. First, the hypothalamus sends "releasing hormones" to the
pituitary gland. The pituitary responds by secreting other hormones that
regulate growth, thyroid function, and sex hormones such as estrogen and
testosterone. One of the pituitary's main duties is to secrete ACTH
(adrenocorticotropin), a hormone that stimulates the adrenal glands. When the
adrenals receive the pituitary's signal in the form of ACTH, they respond by
producing cortisol. Completing the cycle, cortisol then signals the pituitary
to lower secretion of ACTH.

Normally, the amount of cortisol released by the adrenals is precisely
balanced to meet the body's daily needs. If something goes wrong during this
process -- for example, with the adrenals, or their regulating switches in the
brain (the pituitary gland or the hypothalamus) -- cortisol production can go
awry.

Cause

Exposure to too much cortisol can occur for different reasons. A common cause
of elevated cortisol is the long-term use of glucocorticoid hormones such as
prednisone for the treatment of inflammatory illnesses like rheumatoid
arthritis. People who have taken these hormones for a long time may develop
the symptoms of Cushing's syndrome, for example, the rounded or "moon face"
and muscle weakness.

Elevated levels of cortisol also can be traced to abnormalities of the
pituitary gland or the adrenal glands. High cortisol production also can be
caused by tumor cells that release ACTH, in turn signaling the adrenals to
overproduce cortisol. Each of these causes is discussed in more detail below.

PITUITARY ADENOMAS

Most cases of Cushing's syndrome are caused by benign, or noncancerous tumors
of the pituitary gland called adenomas, which secrete increased amounts of
ACTH. Most patients have a single adenoma. This form of the syndrome, known as
"Cushing's disease," afflicts more women than men.  Adenomas are unlikely to
spread; rarely, however, some pituitary tumors have the features of cancer,
including the ability to spread.

ECTOPIC ACTH SYNDROME

About 17 percent of Cushing's syndrome cases are due to the production of ACTH
by various types of potentially malignant tumors that arise in different parts
of the body. By far, the most common form of ACTH-producing tumor is oat cell,
or small cell, lung cancer, which accounts for about 25 percent of all lung
cancer cases. Other, less common types of cancer that can produce ACTH are
thymomas, carcinoid tumors, pancreatic islet cell tumors, and medullary
carcinomas of the thyroid.

ADRENAL TUMORS

In about 15 percent of patients with Cushing's syndrome, the cause can be
traced to an abnormality of the adrenal glands, most often an adrenal tumor.
In about one-half of these cases, the tumors are noncancerous growths of
adrenal tissue, called adrenal adenomas, which release excess cortisol into
the blood.

Adrenocortical carcinomas, or adrenal cancers, are the least common cause of
Cushing's syndrome, accounting for about 7 percent of cases.  They tend to
occur in children. Cancer cells secrete excess levels of several adrenal
cortical hormones, causing cortisol and adrenal androgen levels to remain
elevated. Adrenal carcinomas also can be marked by very high hormone levels
and rapid development of symptoms.

Incidence

Cushing's syndrome is relatively rare. It affects about 10 people per  million
population every year, or approximately 1 in 5,000 hospital admissions, with
most cases occurring between the ages of 20 and 50 years.

About 70 percent of reported cases are diagnosed with pituitary adenomas that
overproduce ACTH (Cushing's disease). This form of the syndrome affects more
women than men at a ratio of 5:1. The ectopic ACTH syndrome, caused by
ACTH-producing tumors, is responsible for about 17 percent of the cases of
Cushing's syndrome. Of these, over 50 percent are due to lung tumors, with a
3:1 ratio of males to female.  Adrenal tumors account for the remainder of
cases, occurring in about two per million population annually. The average age
of onset is about 40 years.

Symptoms

There is no single symptom shared by all patients with Cushing's syndrome,
although some features of the disorder occur more often than others.

OBESITY

The most common symptom is weight gain, with rounding of the face and
increased fat in the neck and above the collar bone, while the arms and legs
tend to stay thin. Obesity associated with poor growth is most common in
children.

SKIN

Skin changes also are common in Cushing's syndrome. The cheeks redden as the
skin becomes thin, making the blood vessels more visible. The thin, fragile
skin breaks easily, and ulcers may arise from minor injury. The ulcers may
persist for a long time because of poor wound healing. Easy bruising and
bluish-red stretch marks, which often appear on the abdomen, thighs, buttocks,
arms, armpits, and breasts, result from weakened connective tissue. Connective
tissue not only provides the supportive framework of the body and its internal
organs, but it is a major structural component of arteries and veins as well
as the skin.

Cushing's Syndrome

EXCESS HAIR GROWTH

In women, excess hair growth, or hirsutism, often appears on the face, neck,
chest, abdomen, and thighs. It occurs in about 80 percent of women with
Cushing's syndrome.

MENSTRUAL DISORDERS

Menstrual disorders are common. Periods become irregular and often stop. In
men, there is decreased fertility with diminished or absent libido.

HIGH BLOOD PRESSURE

Blood pressure above the normal range, or hypertension, occurs in 85 percent
of Cushing's patients. More than 50 percent of patients have elevated
diastolic pressure, and practically all patients have elevated systolic
pressure. High blood pressure is associated with increased atherosclerosis, a
buildup of fat in the arteries.

MUSCLE AND BONE WEAKNESS

Severe fatigue and weak, fragile muscles are characteristic of Cushing's
syndrome. Backache is common due to osteoporosis, a weakening of the bones
resulting from decreased bone mass. In Cushing's syndrome, rib fractures are
frequent, and vertebral (spinal column) compression fractures may occur during
routine activities such as bending, lifting, or rising from a chair.

HIGH BLOOD SUGAR

High blood sugar, or hyperglycemia, is seen in 80 percent of patients with
Cushing's syndrome following the oral glucose tolerance test.  However,
diabetes mellitus occurs in less than 20 percent of people with Cushing's
syndrome, and usually only in those with a family history of the disorder.

Diagnosis

The diagnosis of Cushing's syndrome is based on a review of the patient's
medical history, physical examination, laboratory tests, and often x-ray exams
of the adrenal or pituitary glands. The aim of these tests is first to
determine whether excess levels of cortisol are present and then to establish
the cause.

24-HOUR URINARY FREE CORTISOL LEVEL

This is the most specific test for diagnosing Cushing's syndrome. The
patient's urine is collected over a 24-hour period and- then tested for the
amount of cortisol. Levels higher than 100 micrograms a day for an adult
suggest Cushing's syndrome. (Persons suffering from depression or alcoholism,
who tend to produce higher than normal  levels of cortisol, may need further
testing to confirm a diagnosis of  Cushing's syndrome.)

Once Cushing's syndrome has been diagnosed, it is important to  determine its
cause. Various other tests are used to find the  abnormality that leads to
excess cortisol production. The choice of  test depends in part on the
preference of the endocrinologist or the center where the test is performed.
Two very specialized tests that may be used are described below.

DEXAMETHASONE SUPPRESSION TEST

In this test, dexamethasone, a synthetic cortisol, is given by mouth  every 6
hours for a period of 4 days. For the first 2 days, low doses of dexamethasone
are given, and for the last 2 days, higher doses are  given. The normal
response after taking dexamethasone is a drop in blood and urine cortisol
levels. Depending on the cause of Cushing's syndrome, different responses of
cortisol to dexamethasone are obtained. The dexamethasone suppression test
helps to distinguish patients with pituitary adenomas from those with ACTH- or
cortisol-producing tumors.
The dexamethasone test can produce false-positive results in response  to
depression, alcohol abuse, high estrogen levels, acute illness, and stress.
Conversely, drugs such as phenytoin and phenobarbital may cause false results
in response to dexamethasone suppression. For this reason, patients should
stop taking these drugs for at least 1 week  before the test is performed.

CRH STIMULATION TEST

The corticotropinreleasing hormone (CRH) stimulation test is a relatively new
diagnostic tool that also may be used to identify the cause of Cushing's
syndrome. Patients with pituitary adenomas, after  receiving an injection of
CRH, usually have a rise in blood levels of ACTH and cortisol. This response
is rarely seen in patients with  ectopic ACTH syndrome and practically never
in patients with cortisol-secreting adrenal tumors.

DIRECT VISUALIZATION OF ENDOCRINE GLANDS

Doctors use different imaging tools to reveal the size and shape of the
pituitary and adrenal glands. The most common are the CT (computerized
tomography) scan and MRI (magnetic resonance imaging). A CT scan produces a
series of x-ray pictures giving a cross-sectional image of a body part. MRI
also produces images of the internal organs of the body but without exposing
the patient to ionizing radiation.  A CT scan or MRI of the pituitary gland
may help determine if a tumor is present, causing an overproduction of ACTH.
Occasionally, doctors use  a special radioisotope procedure, known as the
iodocholesterol scan, to view the adrenal glands. If these tests do not
establish the source  of ACTH, some centers are experienced in performing
catheterization procedures that sample the blood leaving the pituitary gland
to  determine if the pituitary is the source of high ACTH.

Treatment

Treatment of Cushing's syndrome depends on the specific reason for cortisol
overproduction. If the cause is long-term use of  glucocorticoid hormones to
treat another disorder, the doctor will gradually reduce the dosage until the
symptoms are under control. Once  control is established, the daily dose will
be doubled and given on alternate days.

PITUITARY ADENOMAS

Several therapies are available to treat the ACTH-secreting pituitary adenomas
of Cushing's disease.  The most widely used treatment is removal of the tumor
by surgery, known as transsphenoidal adenomectomy. Using a special microscope
and very fine instruments, the surgeon approaches the pituitary gland through
a nostril or an opening made in the bridge of the nose. Because this is an
extremely delicate procedure, patients are often referred to centers
specializing in this type of surgery. The success, or cure, rate of this
procedure is over 80 percent. When surgery fails, it is usually  because the
CT scan or MRI is unable to identify the small adenoma.  After pituitary
surgery, there is a natural but temporary drop in the production of ACTH, so
patients must be given a synthetic glucocorticoid hormone called
hydrocortisone. Most patients can stop this replacement therapy in less than 1
year.

For patients in whom transsphenoidal surgery has failed or who are not
suitable candidates for surgery, radiotherapy is another possible treatment.
Radiation to the pituitary gland is given over a period of 6 weeks, with
improvement occurring in 40 to 50 percent of adults and up to 80 percent of
children. It may take several months before  patients feel better from
radiation treatment alone. However, the combination of radiation and the drug
mitotane (Lysodren) can help  speed recovery. Mitotane suppresses cortisol
production and lowers plasma and urine hormone levels. Treatment with mitotane
alone can be successful in 30 to 40 percent of patients. Other drugs used
alone or  in combination to control the production of excess cortisol are
aminoglutethimide, metyrapone, and ketoconazole. Like all drugs, each has its
own set of side effects that doctors consider when prescribing therapy for
individual patients.

ECTOPIC ACTH SYNDROME

To cure the overproduction of cortisol caused by ectopic ACTH  syndrome, it is
necessary to eliminate all of the cancerous tissue that is secreting ACTH. The
choice of cancer treatment -- surgery,  radiotherapy, chemotherapy,
immunotherapy, or a combination of these treatments -- depends on the type of
cancer and how far it has spread.  Since ACTH-secreting tumors (for example,
small cell lung cancer) may  be very small or widespread at the time of
diagnosis, rtisol-inhibiting drugs like mitotane form an important part of
treatment. In some cases, if pituitary surgery is not successful    surgical
removal of the adrenal glands (bilateral adrenalectomy) may   take the place
of drug therapy.

Recently, researchers have found that a glucocorticoid antagonist, RU 486, is
effective in fighting the excessive effects of cortisol that cause Cushing's
syndrome.  RU 486 is still an investigational drug however, and its use is
limited to clinical trials.

ADRENAL TUMORS

Surgery is the mainstay of treatment for benign as well as cancerous
tumors of the adrenal glands.

Research in Cushing's Syndrome

The National Institutes of Health (NIH) is the biomedical research arm  of the
Federal Government. It is one of seven health agencies of the  Public Health
Service, which is part of the U.S. Department of Health  and Human Services.
Several components of NIH-the National Institute  of Diabetes and Digestive
and Kidney Diseases (NIDDK), the National Institute of Child Health and Human
Development (NICHD), the National  Institute of Neurological Disorders and
Stroke, and the National Cancer Institute-conduct and support research on
Cushing's syndrome  and other disorders of the endocrine system.

NIH-supported scientists are conducting intensive research into the  normal
and abnormal function of the major endocrine glands and the many hormones of
the endocrine system. One important NIDDK-supported  study by Dr. Wylie W.
Vale and colleagues at the Salk Institute for Biological Studies in La Jolla,
California, led to the identification  of corticotropinreleasing hormone
(CRH), which instructs the pituitary  gland to release ACTH. This finding
enabled Dr. George P. Chrousos and  coworkers at the NICHD to develop the CRH
stimulation test, which is  increasingly being used to identify the cause of
Cushing's syndrome.  Using the dexamethasone suppression test, doctors are
able to diagnose  the cause of Cushing's syndrome in 80 percent of patients.
With the CRH stimulation test alone, they can accurately diagnose the cause in
85 percent of cases. By using the dexamethasone suppression test and CRH
stimulation test together, they are able to diagnose Cushing's syndrome with
98 percent accuracy, according to a recent NIH study.

NIH scientists are continuing their efforts to improve the diagnosis  and
treatment of Cushing's syndrome. Current research is targeted not  only at
identifying new hormones, but also understanding their precise  functions.
Researchers also are focusing on the role of receptors, which are large
complex molecules either on the surface of or within  target cells to which
hormones must attach to be effective.

One goal of future research is to find ways to cure Cushing's syndrome
without surgery. For example, it may be possible to destroy pituitary  tumors
selectively by binding a hormone like CRH, which precisely targets pituitary
tissue, with a tumor-killing toxin or monoclonal antibody.

Current NIH Studies

Scientists are treating patients with Cushing's syndrome at the NIH  Warren
Grant Magnuson Clinical Center in Bethesda, Maryland.   Physicians who are
interested in referring a patient with Cushing's syndrome may contact Dr.
George P. Chrousos, Developmental   Endocrinology Branch, NICHD, Building 10,
Room 10N262, Bethesda, Maryland 20892, telephone [phone].

Suggested Reading

The following materials can be found in medical libraries, many  college and
university libraries, and through interlibrary loan in most public libraries.

Forsham, Peter H. and Tyrrell, J. Blake, "Cushing's Syndrome," in  Current
Diagnosis, edited by Rex B. Conn. Philadelphia, W.B. Saunders  Company, 1985,
pp 863-867.

Chrousos, George P., "Cushing's Syndrome," in Conn's Current Therapy,  edited
by Robert E. Rakel. Philadelphia, W.B. Saunders Company, Philadelphia, 1987,
pp 495-498.

Kohler, Peter O., ed. Clinical Endocrinology, New York, John Wiley &  Sons,
1986.  Braunwald, Eugene et al., eds. Harrison's Principles of Internal
Medicine, 11th edition, New York, McGraw-Hill Book Company, 1987, pp
1760-1764.

NCI Research Report: Cancer of the Lung. Prepared by the Office of Cancer
Communications, National Cancer Institute, NIH Publication No. 86-526.


Other Resources

National Cushing's Association  4620l/2 Van Nuys Boulevard  Sherman Oaks,
California 91403  [phone] or [phone]


This epub was written by Eileen K. Corrigan of NIDDK's Office of Health
Research Reports. The draft was reviewed by Dr. George F. Chrousos, National
Institute of Child Health and Human Development, and by Dr.  Richard Horton,
University of Southern California Medical Center.

This epub is not copyrighted. Readers are encouraged to duplicate and
distribute as many copies as needed. Printed single copies may be
obtained from the Office of Health Research Reports, NIDDK, Building 31, Room
9A04, Bethesda, Maryland 20892.

NIH Publication No. 89-3007 April 1989
!
*


                      Cystic Fibrosis:
             Tests, Treatments Improve Survival
                    by Ricki Lewis, Ph.D.
     Alex Deford had been ill almost from the moment of her
birth on Oct. 30, 1971. Her frequent colds and ear infections
coupled with her small size, despite a healthy appetite,
prompted doctors to vaguely diagnose "failure to thrive." When
Alex developed double pneumonia at 4 months, it was clear that
something was very wrong.
     That something turned out to be cystic fibrosis, the most
common inherited illness among white people of Northern and
Western European ancestry, although it is seen in all ethnic
groups. Symptoms include thick, sticky mucus clogging the
lungs, impairing breathing and attracting infection; a blocked
pancreas that cannot release digestive enzymes, causing pain
after eating; stubbed fingers from poor circulation;
infertility; salty sweat; and other problems. Patients may
have any or all of these symptoms--Alex had quite a list.
     When she was diagnosed at Boston Children's Hospital
early in 1972, Alex was so ill that she was expected to live
only days. She survived eight years, but not easily.
     Alex began each day by inhaling a decongestant. Then her
parents took turns providing "postural drainage," a 30- to 60-
minute pounding and pressing on each of 11 segments of the
lungs, to loosen the mucus, which she coughed up. Alex would
then take drugs--antibiotics to prevent lung infection and
powdered digestive enzymes mixed into applesauce.
     Despite this daily regimen, Alex died in January 1980.
Her father, sportswriter and commentator Frank Deford, tells
her story in his book, Alex, the Life of a Child.
     Cystic fibrosis (CF) is inherited and affects 30,000
Americans. In 1989, scientists discovered the gene that causes
cystic fibrosis (see accompanying article.) This discovery is
enabling researchers to develop new diagnostic tests that will
help identify those who can benefit from traditional as well
as several new treatment approaches being evaluated by FDA.
How CF Is Inherited
     CF is typically passed from parents who each carry the
gene, to children of either sex. Carriers have one faulty copy
of the gene, which is responsible for the illness, plus one
normal copy, which prevents symptoms. Each child of carrier
parents has a 1 in 4 chance of inheriting CF; a 1 in 4 chance
of being completely free of the mutant gene; and a chance of
1 in 2 of being a carrier, like the parents.
     Couples usually learn that they carry CF when they have
an affected child. By 1985, individuals who had a sibling with
CF could find out if they carried the gene by taking a
"genetic marker" (linkage analysis) test that spots a
particular family's CF-carrying chromosome, but not the gene
itself. Finding the CF gene makes it possible to detect most
carriers, even if there are no affected relatives.
     The Office of Technology Assessment estimates that 100
million to 200 million people in the United States might want
to take a CF carrier test. About 8 million people in the
United  States, or 1 in 25 whites, may be carriers.

Diagnosing CF

     The same gene discovery that has led to development of
carrier tests is expected to help to more quickly diagnose CF,
whose symptoms resemble those of other illnesses.
     The most widely used and best-known CF test is the
electrolyte sweat test. It detects the excess sodium,
potassium and chloride (charged chemicals called electrolytes)
found on the skin of many people with CF. A physician would
perform a sweat test in a child with unexplained failure to
gain weight, or with very frequent respiratory infections.
     The sweat test evolved from the observations made by a
physician, Dr. Paul di Sant'Agnese, during a 1953 heat wave in
New York City. He was curious why so many children with CF
were being brought to Babies and Children's Hospital, where he
worked, with heat prostration. The youngsters were unable to
cope with the heat because too much salt exited their bodies
in sweat. The fact that the sweat of a person with CF contains
two to six times as much salt as normal sweat gave him the
idea for the sweat test.
     The sweat test became widely used by the mid-1950s, and
is the only CF test cleared by FDA for marketing. (A
forerunner of the sweat test was the observation that a
child's brow was salty when kissed. At the turn of the
century, this is how midwives identified babies with cystic
fibrosis.)
     Although the sweat test is a critical part of a CF
diagnostic work-up, salty sweat can indicate any of several
disorders. Other tests help focus the diagnosis. Some of these
tests are based on methodologies developed by reference
laboratories, which perform medical tests and send the results
to physicians. According to Freda Yoder of FDA's Center for
Devices and Radiological Health, methodologies developed in-
house have not traditionally been regulated by the agency.
     Explains Tom Tsakeris, director of the division of
clinical laboratory devices at FDA, "FDA regulates products,
not laboratories. As long as they are not marketing the test
itself, we do not regulate the lab." However, he adds, the
Clinical Laboratory Improvement Act, signed into law in 1988
but not yet fully implemented, will regulate reference
laboratories.
     One test developed by reference labs measures the amount
of the protein trypsinogen in a newborn's blood. Trypsinogen
is manufactured by the pancreas and sent to the intestine,
where it is snipped to a shorter form, trypsin, which helps
digest proteins. If the pancreas is clogged by the sticky
mucus of CF, trypsinogen levels are elevated, because the
longer protein cannot be cut down to size.
     In one study conducted by researchers at the University
of Colorado School of Medicine and Children's Hospital in
Denver, the trypsinogen test identified 95.2 percent of
infants with CF who did not have the earliest sign, a greenish
discharge called meconium ileus indicating intestinal
blockage. But in the study there were many false positives--of
96 infants who tested high for trypsinogen on two tests, only
31 had CF. So, although the trypsinogen test alone is not
perfect, combined with a sweat  test and observing symptoms,
it can begin to paint a portrait of CF.
     Another test detects the level of certain fetal
intestinal enzymes in the amniotic fluid (the liquid
surrounding the fetus). Amniotic fluid is collected for
testing by a procedure called amniocentesis (see "Genetic
Screening: Fetal Signposts on a Journey of Discovery" in the
December 1990 FDA Consumer). In a fetus with CF, these enzymes
are decreased. Again, however, other disorders besides CF can
produce this finding, and therefore it is not a specific
disease marker. Researchers have turned to the genetic
material to develop a definitive CF test.

Enter Genetic Testing
     Developing a test to detect the gene that causes CF would
provide a definitive diagnosis, because this mutant gene is
the only cause of the disorder. The first step was to find out
where the gene behind CF lies among the 23 pairs of
chromosomes.
     By 1985, several research teams had narrowed the search
to a part of chromosome 7 (the seventh largest chromosome).
Until the CF gene itself was isolated and characterized in
1989, relatives of patients could take an indirect test that
uses linkage analysis. Because of the complexity of test
interpretation, these tests are primarily performed at
academic centers.
     A genetic linkage test tracks a known DNA sequence (a
genetic marker) that, within a family, always occurs in people
with CF, and never in those who do not have the illness. A
genetic marker and the gene responsible for the disorder
behave like two inseparable friends. If you see one at a
party, you know the other is nearby. Genetic linkage testing
is based on the observation that genes carried close together
on the same chromosome tend to be inherited together.
     Ray White at the Howard Hughes Medical Institute at the
University of Utah in Salt Lake City and Robert Williamson of
St. Mary's Hospital Medical School in London each found a
marker, one on either side of the CF gene. Using these two
markers, a couple who already had a child with CF could have
fetal chromosomes tested in a subsequent pregnancy. If the two
markers on the two chromosome 7's in the fetus matched those
of the affected child, then it, too, has likely inherited the
disease.
     A major limitation of linkage tests is that they only
work on families known to have CF. Because people can carry CF
without having symptoms, a disease-causing gene can be in a
family without anyone in recent memory being ill. Finding the
CF gene itself, however, may make possible a test useful on
anyone, so that carriers could be detected in families where
no one has CF.
     Like other genetic tests, CF tests can be performed on
any type of tissue, because all human cells (except red blood
cells) contain two copies of all of the genes, and sperm and
egg have one copy of each. The first CF tests used white blood
cells. Then Williamson's group in London came up with a
pleasanter alternative--a mouthwash! After swishing a
saltwater solution in the mouth, the person spits into a
bottle. The CF gene can be  spotted in cells dislodged from
the inside of the cheek.
     Taking a cue from London, Genzyme Corp. (Cambridge,
Mass.) developed a cheekbrush test for CF, which is
investigational. A patient swabs cheek cells onto a brush, and
the physician sends the sample to Genzyme. The presence of
both normal and mutant CF genes indicates carrier status. If
only mutant genes are there, CF is indicated.

To Test or Not To Test?
     A carrier test provides information to couples who are
not ill but whose children are at high risk of inheriting the
condition.
     Many experts predict that the day of universal CF
screening is approaching, with several companies developing CF
tests that simultaneously screen for several CF mutations.
     Two factors contribute to the sensitivity of a CF carrier
test. The first is the number of mutations that can be
detected. The more mutations tested for, the more carriers
will be spotted.
     Ethnic background is the other important factor, says
Marisa Ladoulis, a genetic counselor at Collaborative
Diagnostic Services in Waltham, Mass. For example, a 12-
mutation test that spots 84 percent of whites with a Northern
or Western European background will detect 92 to 95 percent of
Ashkenazi Jews, and the 16-mutation test finds 96 to 98
percent of them.

All CF Mutations Are Not Equal
     Checking for an errant CF gene may be easy, but
interpreting the results may not be. Researchers are finding
that different CF mutations cause different degrees of
sickness. Alex Deford probably had two copies of delta F508,
the most common and one of the more serious mutations that can
cause CF. But a researcher in the laboratory of Francis
Collins, the co-discoverer of the CF gene, has a milder case
of CF because he inherited the delta F508 mutation as well as
a different one.
     This young man must perform postural drainage on himself
and take antibiotics and digestive enzymes, but he also plays
the trumpet, bikes, and sings. Still, a respiratory infection
can send him to the hospital for a week or longer. Clinicians
are finding that some people who have frequent bouts of
pneumonia and other respiratory infections actually have CF.
     Some people with CF may not even have lung or digestive
symptoms. Aubrey Milunsky, D.Sc., Director of the Center for
Human Genetics at the Boston University School of Medicine,
found that some men who were referred to him because they were
having difficulty fathering a child actually had CF. In
examining x-rays that had been taken as part of a standard
fertility work-up, Milunsky noticed the men lacked the vas
deferens, the paired tubes that deliver sperm from the body.
Knowing this is a symptom in 90 percent of men with CF,
Milunsky tested their genes and found they had inherited CF.
     "Cystic fibrosis is not a simple single mutation to look
for," says Margaret Wallace, Ph.D., assistant professor in the
division of genetics in the department of pediatrics at the
University of Florida in Gainesville. "There will be a lot of
problems in doing the diagnosis and giving an idea of what it
means," she adds.

Treating CF

     CF symptoms are controlled with a number of drugs.
Antibiotic drugs combat infections to which CF patients are
prone, including Pseudomonas aeruginosa bacteria, a type of
microbe that is attracted to the sticky mucus in the lungs.
The combination of animal enzymes, called Viokase, that Alex
Deford took regularly is still used today by CF patients. It
is approved as a prescription digestive aid for CF patients
and others with pancreatic insufficiencies. Combined with a
high-calorie diet, this enzyme preparation aids digestion,
helping the patient to maintain weight.
     Many patients also take anti-inflammatory prescription
drugs, such as ibuprofen (Motrin and others), prednisone
(Deltasone, Winpred, Orason, and others), and naproxen
(Anaprox, Naprosyn and others).
     The drug amiloride (Midamor, Moduretic), introduced in
1967 and approved as an adjunct to treatment with some
diuretic drugs, is now being tested as a treatment for CF.
Scientists believe amiloride thins lung secretions by blocking
sodium uptake by lung cells. Clinical studies are under way to
assess amiloride as a CF treatment alone, and in combination
with the biological products adenosine triphosphate (ATP) and
uridine triphosphate (UTP). (ATP and UTP are components of the
nucleic acids DNA and RNA.)
     Other investigational products are aimed at tempering the
body's immune response to lung infection, which can be
excessive. One such product is deoxyribonuclease. The March
19, 1992, New England Journal of Medicine reported that in a
pilot study, this protein biologic given in an aerosol helped
clear the lungs of 16 adult CF patients. It is being tested in
900 CF patients at 50 medical centers in the United States.

Gene Therapy
     FDA has designated recombinant cystic fibrosis
transmembrane conductance regulator (the gene's protein
product, abbreviated CFTR) as well as gene therapy as orphan
products. This gives their sponsors special incentives because
they are developing products for a condition affecting
relatively few people.
     The first human gene therapy study of CF got under way
last April 17 at the National Heart, Lung, and Blood Institute
after FDA gave the go-ahead the previous day. An engineered
cold virus (adenovirus) was introduced into the cells lining
the nose and airways of a 23-year-old man with CF. The virus
was altered to carry the normal CFTR gene and lacks the genes
to cause a cold and to replicate.
     The research was the first use of gene therapy for a
common genetic disorder and the first use of a cold virus to
transport genes. The study includes 10 patients age 21 or
older who have mild to moderate CF symptoms.
     Previous experiments in rats indicated that replacing the
CF genes in just 10 percent of the lung lining cells improves
lung function. However, because the genes go to the patients'
lungs but not their sex cells, CF can still be passed to the
patients' children.
     New knowledge of CF is coming so fast that the goals of
carrier screening may change even before the tests are cleared
for marketing.
     Soon, detecting the gene for CF may be a way of finding
who needs treatment, as early as possible, just as is
presently done for high blood pressure and elevated blood
cholesterol. Says Wallace, "CF research is moving so quickly,
with a lot of hope for treatment in the near future. It will
be treatable, and possibly easily."

Ricki Lewis is a genetic counselor and is the author of
textbooks on biology and human genetics.

Box:
For more information, contact The Cystic Fibrosis Foundation,
6000 Executive Blvd., Suite 510, Rockville, MD 20852;
telephone (1-800) FIGHTCS.
Advances and Stumbling Blocks
     The symptoms of CF were first described in medical
journals in 1938. The malady was attributed to a defect in the
channels leading from certain glands--a remarkably accurate
description, it would turn out. But the disorder was
recognized before it was given a name, as illustrated by the
17th century English saying, "A child that is salty to taste
will die shortly after birth."
     In 1960, a CF patient rarely lived past the age of 12. By
1970, only half lived to see their 18th birthdays. In the
1970s, when postural drainage began to be implemented and FDA
approved enzyme replacement and antibiotic therapy, the
average lifespan began to creep upwards. Today, it is 29
years, according to the Cystic Fibrosis Foundation. New, more
targeted therapies may raise survival age higher.
     Cystic fibrosis researchers marked a medical milestone on
Oct. 8, 1989, when Science magazine published a report by
Francis Collins and his co-workers at the University of
Michigan at Ann Arbor and Lap-chee Tsui at the Hospital for
Sick Children in Toronto on precisely how a specific gene
disrupts a certain protein to cause CF.
     The researchers named the protein the "cystic fibrosis
transmembrane conductance regulator," or CFTR for short. CFTR
is normally manufactured inside cells lining glands in the
respiratory passages, small intestine, pancreas, and sweat
glands. The protein travels to the cell's surface, where it
controls the flow of salt in and out of the cell like a
gateway in the cell membrane.
     In the disorder, CFTR protein is abnormal in a way that
prevents it from reaching the cell's surface. Without the
gateway in the membrane, salt is trapped inside cells.
Following a natural chemical tendency to try to dilute the
salty interiors of cells, moisture is drawn inside them
through other gateways. This dries out the surrounding
secretions, causing symptoms. In most people with CF, the
protein is missing just one amino acid building block out of
1,480--a tiny, but devastating, glitch.
     Almost as soon as Collins and Tsui described the mutation
that causes CF, dubbed delta F508, a difficulty arose. Delta
F508 was not the only way that the gene could be altered. (A
gene consists of sequences of four types of building blocks.
Just as a sentence can have an error in any of its letters, a
gene can be altered in many ways. A person with CF inherits
two abnormal forms.)
     But within days of the publication of the Science report,
several biotechnology firms were already devising carrier
tests for delta F508. A test for the disease-causing gene
variant became available on an investigational basis by
November 1989. But on Feb. 1, 1990, Collins, Tsui, and several
others reported in The New England Journal of Medicine that
only 75.9 percent of white CF patients of Northern and Western
European backgrounds had the delta F508 variant. How useful
would a test for delta F508 be, researchers worried, if this
wasn't the only variant responsible for CF? At current count,
more than 200 variants of the gene are known.
     The multiple guises of the CF gene meant that a test to
spot delta F508 would miss about 24 percent of Northern or
Western  European descended whites in the United States who do
carry a CF gene. This, in turn, meant that the test would find
only about half the couples in the United States who risk
passing CF to a child (this figure is derived by multiplying
the chances of each parent having delta F508). But it would be
too costly to develop a test for more than 200 different
mutations, when only a few of them are common.
     Adding to the complexity is that different populations
have different proportions of the CF gene variants. For
example, delta F508 occurs in only 35 percent of African-
Americans and Jews of Central and Eastern European ancestry
(called Ashkenazi) who carry CF, making the test for this
mutation even less valuable than it is for non-Jewish whites.
For Hispanics and Italians, the frequency of delta F508 is 50
percent.
     The potential powder keg of a carrier test for a common
genetic disease that would, at best, only work three-quarters
of the time set off a flurry of statements by professional
medical organizations. On Nov. 13, 1989, the American Society
of Human Genetics urged caution in carrier testing until a
greater percentage of the CF-carrying population could be
identified, calling for pilot programs to test the tests.
Meanwhile, they suggested the test only for those with a close
affected relative.
     In early March 1990, a panel of physicians, geneticists,
genetic counselors, and attorneys met at the National
Institutes of Health in Bethesda, Md., to develop guidelines
for CF carrier testing. This group echoed the earlier call for
pilot programs, adding that widespread testing should wait
until tests could detect 90 to 95 percent of carriers.
     In December 1992, the American Society of Human Genetics
reevaluated their 1989 statement, in light of the ability to
detect many CF mutations. Their advice remains unchanged--for
now, CF testing should be offered only to those with a
relative who has the disorder. The organization also calls for
informed consent and genetic counseling, confidentiality of
results, and quality control of the laboratory performing the
test.
--R.L.
!
*
Diabetic Retinopathy

U.S. Department of Health and Human Services
Public Health Service
National Institutes of Health

Why is it important to know how diabetes affects the eyes?

If you are among the 10 million people in the United States who
have diabetes-or if someone close to you has this disease-you
should know that diabetes can affect the eyes and cause visual
impairment.

Fortunately, there are ways to prevent or lessen the eye damage
caused by diabetes.  That is why it is so important for people
with this disease to have a professional eye examination as soon
as their diabetes is diagnosed, and at least once a year
thereafter.

Regular eye examinations are especially important for people who
have had diabetes 5 years or longer, for those who have difficulty
controlling the level of sugar in their blood, and for diabetic
women who are pregnant.  All of these people are at increased risk
for diabetes-associated eye problems.

What is diabetic retinopathy?

Diabetic retinopathy is a potentially serious eye disease caused
by diabetes.  It affects the retina-the light-sensitive tissue at
the back of the eye that transmits visual messages to the brain.
Damage to this delicate tissue may result in visual impairment or
blindness.

Diabetic retinopathy begins with a slight deterioration in the
small blood vessels of the retina.  Portions of the vessel walls
balloon outward and fluid starts to leak from the vessels into the
surrounding retinal tissue.  Generally, these initial changes in
the retina cause no visual symptoms.  However, they can be
detected by an eye specialist who is trained to recognize subtle
signs of retinal disease.

In many people with diabetic retinopathy, the disease remains mild
and never causes visual problems.  But in some individuals,
continued leakage from the retinal blood vessels leads to macular
edema.  This is a build-up of fluid in the macula-the part of the
retina responsible for the sharp, clear vision used in reading and
driving.  When critical areas of the macula become swollen with
excess fluid, vision may be so badly blurred that these activities
become difficult or impossible.

Some people with diabetes develop an even more sight-threatening
condition called proliferative retinopathy.  It may occur in
people who have macular edema, but also can develop in those who
don't.  In proliferative retinopathy, abnormal new blood vessels
grow on the surface of the retina.  These fragile new vessels can
easily rupture and bleed into the middle of the eye, blocking
vision.  Scar tissue also may form near the retina, ultimately
detaching it from the back of the eye.  Severe visual loss, even
permanent blindness, may result.  But this happens in only a small
minority of people with diabetes.

How many diabetics are affected?

Approximately 40 percent of all people with diabetes have at least
mild signs of diabetic retinopathy.  About 3 percent have suffered
severe visual loss because of this disease.

In general, the longer one has had diabetes, the greater one's
chances of developing diabetic retinopathy.

What are the symptoms of diabetic retinopathy?

As already indicated, diabetic retinopathy generally causes no
symptoms in its earliest stages.  For the person who develops
macular edema, blurring of vision may provide a clue that
something is wrong.  But proliferative retinopathy can progress a
long way without any warning signs.  That is why a person with
diabetes should make regular visits to an eye specialist, so any
eye problems can be detected and treated if necessary.

How is diabetic retinopathy treated?

Recently, scientists have found that laser treatment can prevent
visual loss in many people with diabetic macular edema.  In this
treatment, called photocoagulation, powerful beams of light from a
laser are aimed at leaking retinal blood vessels in the macula.
The goal of treatment is to seal the vessels and prevent further
leakage.  In many patients, this treatment halts the decline in
vision or even reverses it.

Research also has shown that laser photocoagulation can
dramatically reduce the risk of blindness in people who have
proliferative retinopathy.  For these patients, the laser is used
in a different way: It is not directed at the macula but is aimed
at hundreds of spots in other parts of the retina.  The purpose of
the treatment is to destroy diseased tissue and stop the
retinopathy from getting worse.  In fact, the treatment can reduce
the risk of severe visual loss by 60 percent.

The studies which proved the value of laser treatment for people
with diabetic retinopathy were supported by the National Eye
Institute.  It is part of the National Institutes of Health, a
component of the U.S. Department of Health and Human Services.
These studies also have helped ophthalmologists determine which
diabetic patients need laser treatment and when to begin it.

Who should have laser treatment?

An ophthalmologist (a medical doctor who specializes in the care
of eye conditions) usually will consider laser treatment when a
person with diabetes has proliferative retinopathy or retinal
signs that suggest this condition may soon develop.  Also, people
with a significant degree of macular edema would now be considered
for laser treatment.

When deciding whether to recommend laser treatment to a particular
patient, the ophthalmologist weighs the potential benefits-pre-
venting or delaying severe visual loss against the risk of
unwanted side effects.  These may include some loss of central or
side vision.

Unfortunately, laser treatment cannot restore sight to the person
who has already suffered severe retinal damage from diabetic
retinopathy.  Also, the laser generally is not used when bleeding
inside the eye has made it difficult or impossible for the
ophthalmologist to see the areas that need treatment.


What is vitrectomy?

A few diabetic retinopathy patients-including some who have had
photocoagulation go blind from massive bleeding inside the eye.
Now, ophthalmologists can remove the blood and scar tissue from
the center of the eye.  This procedure is known as vitrectomy.
Following vitrectomy, patients can often see well enough to move
around on their own.  Occasionally, vision in the operated eye
recovers enough for the patient to resume reading or driving.

What research is being done on diabetic retinopathy?

The National Eye Institute is supporting a nationwide study to
determine whether photocoagulation-used alone or in combination
with aspirin-can benefit people who are still in the early stages
of diabetic retinopathy.  Almost 4,000 patients are enrolled in
this S-year clinical trial.  It already has proven the value of
photocoagulation for macular edema (see "How is diabetic retinop-
athy treated?"), and is expected to yield further valuable
findings in the future.

Another clinical trial sponsored by the Institute and Pfizer,
Inc., is evaluating a new drug called sorbinil to see if it can
prevent eye and nerve damage in people with diabetes.

In addition to these clinical trials, the Institute is supporting
an extensive program of research on the causes, detection, and
treatment of diabetic retinopathy.


Who can refer you to an eye care specialist?

If you know you have diabetes, you are probably under the care of
a physician who can refer you to an eye doctor for regular
examinations and treatment, if needed.  You may also request the
name of an appropriate eye doctor from eye care centers affiliated
with academic institutions, from a hospital, or from a diabetes
clinic at a medical center.

You may obtain information on diabetes from the American Diabetes
Association, 1660 Duke Street, Alexandria, Virginia 22314,
telephone [phone]; and the Juvenile Diabetes Foundation,
432 Park Avenue South, 16th Floor, New York, New York 10016,
telephone [phone].  Check your local telephone directory
for their affiliates or chapters near you.

What help is available to the person who has already lost vision
from diabetic retinopathy?

There are many useful devices that can help a partially sighted
person to make the most of his or her remaining vision.  Called
low vision aids, these devices have special lenses or electronic
systems that produce enlarged images of nearby objects.

If you need low vision aids, your eye care specialist can
generally prescribe them.  Often, he or she will be able to
suggest further sources you might contact to get information about
counseling, training and other special services for people with
low vision.  These may include a nearby school of medicine or
optometry.

This brochure was prepared by the National Eye institute, NIH,
Building 31, Room 6A32, Bethesda, MD 20892.  The telephone number
is [phone].

!
*

WHEN YOUR DIAGNOSIS IS CYSTITIS

...it simply means you have an inflammation of the bladder.
(Cystitis comes from "kystis", the Greek word for bladder.)  The
usual cause of this inflammation is a bacterial infection.

This is a very common infection in females...more so in younger
than older females.  It clears rapidly in most uncomplicated
cases with oral medication taken faithfully by the patient.

How did you get it?  The bladder drains through a tube called the
urethra.  The opening is close to the vagina, and not far from
the anus (rectal opening).  The germ involved in many bladder
infections is an erstwhile peaceful and necessary resident of the
human colon which is inadvertently introduced into the bladder
(urethral) opening.  Once in the bladder these germs act like
pathogens (disease-producers) and infect the bladder lining,
producing the characteristic symptoms.

Cystitis can follow or accompany systemic illnesses, excessive
irritation of the tissues in the genital area, such as from
intercourse or repeated trauma as in horseback riding, excessive
generalized dehydration or seeding from infections elsewhere in
the genito-urinary tract (e.g. kidneys).  The symptoms are quite
characteristic -- burning sensation on urination, the often
urgent feeling of having to urinate frequently (though not much
urine may be passed), pressure sensation and distress in the
pelvic area and not infrequently blood in the urine.

Is it serious?  Many cases would gradually go away if untreated,
but a high enough percent of infections become chronic, recur
frequently, spread to other organs such as the kidney, etc. so
that proper medical treatment is by far the wisest course to
pursue.

Treatment is usually simple.  Nearly all cases can be treated
with oral antibacterial medications.  While under treatment,
increasing fluid intake enough so you urinate at least four times
daily, avoiding strenuous physical activity in severe cases,
abstinence from alcohol and sexual intercourse, rest and when the
lower abdominal symptoms are marked, applying heat to the lower
abdomen - all help to gain quicker relief.  Symptoms are usually
relieved within 24-72 hours, BUT THAT DOES NOT MEAN THE INFECTION
IS GONE.  It is important to take all the medication to insure
the best chance for complete eradication of the infection. If you
have persistent symptoms after treatment, return to clinic for
follow-up.

Can cystitis be prevented?  Simple hygiene is important - bathing
and showering.  Incorrect "back to front" or careless wiping
after bowel movements should be avoided - it can smear colon
germs into the urethra.  Sometimes "dry", excessive, or prolonged




sexual intercourse will irritate the tissues enough to "kick off"
cystitis.  (The condition is not, however, considered a venereal
disease under most circumstances.)
Bacteria in the bladder reproduce approximately every twenty
minutes.  One becomes a million in seven hours.  If the bladder
is kept flushed out by adequate fluid input, this rapid bacterial
replication is inhibited and symptomatic infections eliminated.
Since
sexual activity is so commonly linked to bladder infections, it
is a good idea to empty your bladder after sex.  Then drink a
large glass of water.  You may need to get up at 3 AM to urinate,
but when you do you will flush out bacteria which were earlier
introduced.

If you are a sparing consumer of fluids, increase your intake so
that at least once a day the urine is colorless or very pale,
urinating at least four times daily.

So...for best results take your medication faithfully and be sure
to follow-up with the doctor after finishing the course of
treatment.

!
*
End-Stage Renal Disease

Choosing A Treatment That's Right For You

U.S. Department of Health and Human Services
Public Health Service
National Institutes of Health

Contents

Introduction
When Your Kidneys Fail
Treatment Choices
Hemodialysis
Peritoneal Dialysis
Kidney Transplantation
Conclusion
Paying for Treatment
Other Sources of Information

Introduction


This booklet is for people whose kidneys fails to work. This
condition is called end-stage renal disease (ESRD).


Today, there are new and better treatments for ESRD that replace
the work of healthy kidneys. By learning about your treatment
choices, you can work with your doctor to pick the one that's
best for you. No matter which type of treatment you choose, there
will be some changes in your life. But with the help of your
health care team, family, and friends, you may be able to lead a
full, active life.

This booklet describes the choices for treatment: hemodialysis,
peritoneal dialysis, and kidney transplantation. It gives the
pros and cons of each. It also discusses diet and paying for
treatment. It gives tips for working with your doctor, nurses,
and others who make up your health care team. It provides a list
of groups that offer information and services to kidney patients.
It also lists magazines, books, and brochures that you can read
for more information about treatment.

You and your doctor will work together to choose a treatment
what's best for you. This booklet can help you make that choice.

When Your Kidneys Fails


Healthy kidneys clean the blood by filtering out extra water and
wastes. They also make hormones that keep your bones strong and
blood healthy. When both of your kidneys fail, your body holds
fluid. Your blood pressure rises. Harmful wastes build up in your
body. Your body doesn't make enough red blood cells. When this
happens, you need treatment to replace the work of your failed
kidneys.

Treatment Choice

Hemodialysis

Purpose
Hemodialysis is a procedure that cleans and filters your blood.
It rids your body of harmful wastes and extra salt and fluids. It
also controls blood pressure and helps your body keep the proper
balance chemicals such as potassium, sodium, and chloride.






How It Works

Hemodialysis uses a dialyzer, or special filter, to clean your
blood. The dialyzer connects to a machine. During treatment, your
blood travels through tubes into the dialyzer. The dialyzer
filters out wastes and extra fluids. Then the newly cleaned blood
flows through another set of tubes and back into your body.

Getting Ready

Before your first treatment, an access to your bloodstream must
be made. The access provides a way for blood to be carried from
your body to the dialysis machine and then back into your body.
The access can be internal (inside the body --usually under your
skin) or external (outside the body).

Who Performs It

Hemodialysis can be done at home or at a center. At a center,
nurses or trained technicians perform the treatment. At home, you
perform hemodialysis with the help of a family member or friend.
If you decide to do home dialysis, you and your partner will
receive special training.

The Time It Takes

Hemodialysis usually is done three times a week. Each treatment
lasts from 2 to 4 hours. During treatment, you can read, write,
sleep, talk, or watch TV.

Possible Complications

Side effects can be caused by rapid changes in your body's fluid
and chemical balance during treatment. Muscle cramps and
hypotension are two common side effects. Hypotension, a sudden
drop in blood pressure, can make you feel weak, dizzy, or sick to
your stomach.

It usually take a few months to adjust to hemodialysis. You can
avoid many of the side effects if you follow the proper diet and
take your medicines as directed. You should always report side
effects to your doctor. They often can be treated quickly and
easily.



Your Diet

Hemodialysis and a proper diet help reduce the wastes that build
up in your blood. A dietitian can help you plan meals according
to your doctor's orders. When choosing foods, you should remember
to:

■    Eat balanced amounts of foods high in protein such as meat
     and chicken. Animal protein is better used by your body than
     the protein found in vegetables and grains.

■    Watch the amount of potassium you eat. Potassium is a
     mineral found in salt substitutes, some fruits, vegetable,
     milk, chocolate, and nuts. Too much or too little potassium
     can be harmful to your heart.

■    Limit how much you drink. Fluids build up quickly in your
     body when your kidneys aren't working. To much fluid makes
     your tissues swell. It also can cause high blood pressure
     and heart trouble.

■    Avoid salt. Salty foods make you thirsty and cause your body
     to hold water.




■    Limit foods such as milk, cheese, nuts, dried beans, and
     soft drinks. These foods contain the mineral phosphorus. Too
     much phosphorus in your blood causes calcium to be pulled
     from you bones. Calcium helps keep bones strong and healthy.
     To prevent bone problems, your doctor may give you special
     medicines. You must take these medicines everyday as
     directed.

Pros and Cons
Each person responds differently to similar situations. What may
be a negative factor for one person may be positive for another.
However, in general, the following are pros and cons for each
type of hemodialysis.

In-Center Hemodialysis




Pros
■    You have trained professionals with you at all times.
■    You can get to know other patients.

Cons
■    Treatments are scheduled by the center.
■    You must travel to the center for treatment.

Home Hemodialysis

Pros
■    You can do it at the hours you choose.
     (But you still must do it as often as your doctor orders.)
■    You don't have to travel to a center.
■    You gain a sense of independence and control over your
     treatment.

Cons
■    Helping with treatments may be stressful to your family.
■    You need training.
■    You need space for storing the machine and supplies at home.

Working With Your Health Care Team

Questions You May Want To Ask:

■    Is hemodialysis the best treatment choice for me? Why or why
     not?


■    If I am treated at a center, can I go to the center of my
     choice?

■    What does hemodialysis feel like?  Does it hurt?

■    What is self-care dialysis?

■    How long does it take to learn home hemodialysis?  Who will
     train my partner and me?

■    What kind of blood access is best for me?

■    As a hemodialysis patient, will I be able to keep working?
     Can I have treatments at night if I plan to keep working?

■    How much should I exercise?

■    Who will be on my health care team?  How can they help me?

■    Who can I talk with about sexuality, family problems, or
     mane concerns?

■    How/where can I talk to other people who have faced this
     decision?

     Write Other Questions You May Have Here:

Treatment Choice

Peritoneal Dialysis

Purpose
Peritoneal dialysis is another procedure that replaces the work
of your kidneys. It removes extra water, wastes, and chemicals
from your body. This type of dialysis uses the lining of your
abdomen to filter your blood. This lining is called the
peritoneal membrane.

How I Works
A cleansing solution, called dialysate, travels through a special
tube into your abdomen. Fluid, wastes, and chemicals pass from
tiny blood vessels in the peritoneal membrane into the dialysate.
After several hours, the dialysate gets drained from your
abdomen, taking the wastes from your blood with it. Then you fill
your abdomen with fresh dialysate and the cleaning process begins
again.


Getting Ready

Before your first treatment, a surgeon places a small, soft tube
called a catheter into your abdomen. This catheter always stays
there. It helps transport the dialysate to and from your
peritoneal membrane.

Types of Peritoneal Dialysis

There are three types of peritoneal dialysis.

1.   Continuous Ambulatory Peritoneal Dialysis (CAPD)



CAPD is the most common type of peritoneal dialysis. It needs no
machine. It can be done in any clean, well-lip place. With CAPD,
your blood is always being cleaned. The dialysate passes from a
plastic bag through the catheter and into your abdomen. The
dialysate stays in your abdomen with the catheter sealed. After
several hours, you drain the solution back into the bag. Then you
refill your abdomen with fresh solution through the same
catheter. Now the cleaning process begins again. While the
solution is in your body, you may fold the empty plastic bag and
hide it under your clothes, around your waist, or in a pocket.

2.   Continuous Cyclic Peritoneal Dialysis (CCPD)

CCPD is like CAPD except that a machine, which connects to your
catheter, automatically fills and drains the dialysate from your
abdomen. The machine does this at night while you sleep.

3.   Intermittent Peritoneal Dialysis (IPD)

IPD uses the same type of machine as CCPD to add and drain the
dialysate. IPD can be done at home, but it's usually done in the
hospital. IPD treatments take longer than CCPD.


Who Performs It
CAPD is a form of self-treatment. It needs no machine and no
partner. However, with IPD and CCPD, you need a machine and the
help of a partner (family member, friend, or health
professional).

The Time It Takes
With CAPD, the dialysate stays in your abdomen for about 4 to 6
hours. The process of draining the dialysate and replacing fresh
solution takes 30 to 40 minutes. Most people change the solution
four times a day.

With CCPD, treatments are done several times a week, for a total
of 36 to 42 hours per week. Sessions may last up to 24 hours.

Possible Complications
Peritonitis, or infection of the peritoneum, can occur if the
opening where the catheter enters your body gets infected. You
can also get it if there is a problem connecting or disconnecting
the catheter from the bags. Peritonitis can make you feel sick.
It can cause a fever and stomach pain.

To avoid peritonitis, you must be careful to follow the procedure
exactly. You must know the early signs of peritonitis. Look for
reddening or swelling around the catheter. You should also note
if your dialysate looks cloudy. It is important to report these
signs to your doctor so that the peritonitis can be treated
quickly to avoid serious problems.

Your Diet

Diet for peritoneal dialysis is slightly different than diet for
hemodialysis.

■    You may be able to have more salt and fluids.

■    You may eat more protein.

■    You may have different potassium restrictions.

■    You may need to cut back on the number of calories you eat.
     This limitation is because the sugar in the dialysate may
     cause you to gain weight.

Pros and Cons

There are pros and cons to each type of peritoneal dialysis.

Pros
■    You can perform treatment alone

■    You can do it at times you choose.

■    You can do it in many locations.

■    You don't need a machine.

Cons
■    It disrupts your daily schedule.

CCPD

Pros
■    You can do it at night, mainly while you sleep.

Cons
■    You need a machine and help from a partner.

IPD

Pros
■    Health professionals usually perform treatments

Cons
■    You may need to go to a hospital.

■    It takes a lot of time.

■    You need a machine.


Working With Your Health Care Team

Questions You May Want To Ask:

■    Is peritoneal dialysis the best treatment choice for me? Why
     or why not? Which type?

■    How long will it take me to learn peritoneal dialysis?

■    What does peritoneal dialysis feel like? Does it hurt?

■    How will peritoneal dialysis affect my blood pressure?

■    How do I know if I have peritonitis? How is peritonitis
     treated?


■    As a peritoneal dialysis patient, will I be able to continue
     working?

■    How much should I exercise?

■    Who will be on my health care team? How can they help me?

■    Who can I talk with about sexuality, finances, or family
     concerns?

■    How/where can I talk to other people who have faced this
     decision?

     Write Other Questions You May Have Here:


Dialysis Is Not a Cure

Hemodialysis and peritoneal dialysis are treatments that try to
replace your failed kidneys. These treatments help you feel
better and live longer, but they are not cures for ESRD. While
patients with ESRD are now living longer than ever, ESRD can
cause problems over the years. Some problems are bone disease,
high blood pressure, nerve damage, and anemia (having to few red
blood cells). Although these problems won't go away with
dialysis, doctors now have new and better ways to treat or
prevent them. You should discuss these treatments with your
doctor.



Treatment Choice

Kidney Transplantation

Purpose
Kidney transplantation is a procedure that places a health kidney
from another person into your body. This one new kidney does all
the work that your two failed kidneys cannot do.



How It Works
A surgeon places the new kidney inside your body between your
upper thigh and abdomen. The surgeon connects the artery and vein
of the new kidney to your artery and vein. Your blood flows
through the new kidney and makes urine, just like your own
kidneys did when they were healthy. The new kidney may start
working right away or may take up to a few weeks to make urine.
Your own kidneys are left where they are, unless they are causing
infection or high blood pressure.


Getting Ready
You may receive a kidney from a member of your family. This kind
of donor is called a living-related donor. You may receive a
kidney from a person who has recently died. This type of donor is
called a cadaver donor. Sometimes a spouse or very close friend
may donate a kidney. This kind of donor is called a living-
unrelated donor.


It is very important for the donor's blood and tissues to closely
match yours. This match will help prevent your body's immune
system from fighting off, or rejecting, the new kidney. A lab
will do special tests on blood cells to find out if your body
will accept the new kidney.

The Time It Takes
The time it takes to get a kidney varies. There are not enough
cadaver donors for every person who needs a transplant. Because
of this, you must be placed on a waiting list to receive a
cadaver donor kidney. However, if a relative gives you a kidney,
the transplant operation can be done sooner.

The surgery takes from 3 to 6 hours. The usual hospital stay may
last from 10 to 14 days. After you leave the hospital, you will
go to the clinic for regular followup visits.

If a relative or close friend gives you a kidney, he or she will
probably stay in the hospital for one week or less.


Possible Complications
Transplantation is not a cure. There is always a chance that your
body will reject your new kidney, no matter how good the match.
The chance of your body accepting the new kidney depends on your
age, race, and medical condition.

Normally, 75 to 80 percent of transplants from cadaver donors are
working one year after surgery. However, transplants from living
relatives often work better than transplants from cadaver donors.
This fact is because they are usually a closer match.

Your doctor will give you special drugs to help prevent
rejection. These are called immunosuppressants. You will need to
take these drugs every day for the rest of your life. Sometimes
these drugs cannot stop your body from rejecting the new kidney.
If this happens, you will go back to some form of dialysis and
possibly wait for another transplant.

Treatment with these drugs may cause side effects. The most
serious is that they weaken your immune system, making it easier
for you to get infections. Some drugs also cause changes in how
you look. Your face may get fuller. You may gain weight or
develop acne or facial hair. Not all patients have these
problems, and makeup and diet can help.

Some of these drugs may cause problems such as cataracts, extra
stomach acid, and hip disease. In a smaller number of patients,
these drugs also may cause liver or kidney damage when used for a
long period of time.


Your Diet

Diet for transplant patients is less limiting than it is for
dialysis patients. You may still have to cut back on some foods,
though. Your diet probably will change as your medicines, blood
values, weight, and blood pressure change.

■    You may need to count calories. Your medicine may give you a
     bigger appetite and cause you to gain weight.

■    You may have to limit eating salty foods. Your medications
     may cause salt to be held in your body, leading to high
     blood pressure.

■    You may need to eat less protein. Some medications cause a
     higher level of wastes to build up in your bloodstream.

Pros and Cons

There are pros and cons to kidney transplantation.

Pros
■    It works like a normal kidney.

■    It helps you feel healthier.

■    You have fewer diet restrictions.

■    There's no need for dialysis.

Cons
■    It requires major surgery.

■    You may need to wait for a donor.

■    One transplant may not last a lifetime. Your body may reject
     the new kidney.

■    You will have to take drugs for the rest of your life.


Working With Your Health Care Team

Questions You May Want To Ask:

■    Is transplantation the best treatment choice for me? Why or
     why not?

■    What are my chances of having a successful transplant?

■    How do I find out if a family member or friend can donate?

■    What are the risks to a family member or friend if he or she
     donates?

■    If a family member or friend doesn't donate, how do I get
     placed on a waiting list for a kidney? How long will I have
     to wait?

■    What are the symptoms of rejection?

■    Who will be on my health care team? How can they help me?

■    Who can I talk to about sexuality, finances, or family
     concerns?



■    How/where can I talk to other people who have faced this
     decision?

     Write Other Questions You May Have Here:


Conclusion

It's not always easy to decide which type of treatment is best
for you. Your decision depends on your medical condition,
lifestyle, and personal likes and dislikes. Discuss the pros and
cons of each with your health care team. If you start one form of
treatment and decide you'd like to try another, talk it over with
your doctor. The key is to learn as much as you can about your
choices. With that knowledge, you and your doctor will choose a
treatment that suits you best.



Paying for Treatment

Treatment for ESRD is expensive, but the Federal Government helps
pay for much of the cost. Often, private insurance or state
programs pay the rest.

Medicare

Medicare pays for 80 percent of the cost of your dialysis
treatments or transplant, no matter how old you are. To qualify,

■    you must have worked long enough to be insured under Social
     Security (or be the child of someone who has) or

■    you already must be receiving Social Security benefits.

You should apply for Medicare as soon as possible after beginning
dialysis. Often, a social worker at your hospital or dialysis
center will help you apply.

Private Insurance

Private insurance often pays for the entire cost or treatment. Or
it may pay for the 20 percent that Medicare does not cover.
Private insurance also may pay for your prescription drugs.



Medicaid

Medicaid is a state program. Your income must be below a certain
level to receive Medicaid funds. Medicaid may pay for your
treatments if you cannot receive Medicare. In some states, it
also pays the 20 percent that Medicare does not cover. It also
may pay for some of your medicines. To apply for Medicaid, talk
with your social worker or contact your local health department.

Veterans Administration (VA) Benefits

If you are veteran, the VA can help pay for treatment. Contact
your local VA office for more information.

Social Security Income (SSI) and Social Security Disability
Income (SSDI)


These benefits are available from the Social Security
Administration. They assist you with the costs of daily living.
To find out if you qualify, talk to your social worker or call
your local Social Security office.


Organizations That Can Help

There are several groups that offer information and services to
kidney patients. You may wish to contact the following:

■    American Kidney Fund
     Suite 1010
     6110 Executive Boulevard
     Rockville, MD  20852
     [phone]

■    American Association of Kidney Patients
     Suite LL1
     1 Davis Boulevard
     Tampa, FL  33606
     [phone]

■    National Kidney Foundation, Inc.
     30 East 33rd Street
     New York, NY  10016
     [phone]


■    National Kidney and Urologic Diseases
     Information Clearinghouse
     Box NKUDIC
     9000 Rockville  Pike
     Bethesda, MD  20892
     [phone]

Additional Reading

If you would like to learn more about ESRD and its treatment, you
may be interested in reading:






Your New Life With Dialysis--A Patient Guide for Physical and
Psychological Adjustment
Edith T. Oberley, M.A., and Terry D. Oberley, M.D., Ph.D.
Fourth edition, 1991
Charles C. Thomas Publishers
2600 South First Street
Springfield, IL 62794-9265

Understanding Kidney Transplantation
Edith T. Oberley, M.A., and Neal R. Glass, M.D., F.A.C.S.
Charles C. Thomas Publishers, 1987
2600 South First Street
Springfield, IL 62794-9265

Kidney Disease: A Guide for Patients and Their Families
American Kidney Fund
Suite 1010
6610 Executive Boulevard
Rockville, MD  20852

National Kidney Foundation Patient Education Brochures
Includes information on treatment, diet, work, and exercise.
National Kidney Foundation, Inc.
30 East 33rd Street
New York, NY  10016
[phone]

Medicare Coverage of Kidney Dialysis and Kidney Transplant
Services: A Supplement to Your Medicare Handbook
Publication Number HCGA-02183
U.S. Department of Health and Human Services
Health Care Financing Administration
Suite 500
1331 H Street, NW
Washington, DC  20005
[phone]

Renalife Magazine
American Association of Kidney Patients (AAKP)
Suite LL1
1 Davis Boulevard
Tampa, FL  33606
[phone]
Published quarterly.


Family Focus Newsletter
National Kidney Foundation, Inc.
30 East 33rd Street
New York, NY  10016
[phone]

For Patients Only Magazine
Suite 400
20335 Ventura Boulevard
Woodland Hills, CA  91364
[phone]
Published six times per year.

!
*
Diverticulitis

 Although once felt to be similar to left-sided "appendicitis,"
in fact diverticulitis is  probably quite distinct in its
nature and cause.  A diverticulum is generally an  acquired
outpouching of the colon, although there are occasional
congenital  varieties, and some can occur elsewhere in the
digestive tract.  The predisposing  abnormality is
diverticulosis, the occurrence of the puches with no apparent
disease.   This condition is found in over half of all people
over age 60, although very few  (1%) of these develop
diverticulitis.

   It is generally believed that the far reaches of the colon
near the rectum (the  sigmoid colon) are exposed repeatedly to
the very high pressures of contraction in  some individuals.
Since the stool in the area must be propelled forward for
final  removal through a bowel movement, when the fecal matter
is hard or compacted, the  contractions become increasingly
forceful. Ultimately, like the inner tube of an old  tire, the
weaker areas of the colon wall bulge as described, sometimes
forming hundreds of diverticula.

   The factors responsible are believed by many to be dietary.
High fiber foods  generally retain water as they; move through
the colon, leaving the stool soft and  easy to expel. With no
fiber the bolus is hard and inflexible.  Poor fluid intake may
play the same role. Chronic laxatives may cause forceful
contractions as well.

   Diverticulitis is caused when factors weaken the wall of the
diverticulum so much  that the wall microscopically perforates
into the surrounding abdominal cavity.  From  there on, a
process similar to appendicitis may occur.

    Symptoms

   Pain in the left lower abdominal area, often with fever, is
typical.  The patient is  usually older than 50 years. A tender
mass in the abdomen may be felt by the  physician, sometimes
representing an abscess near the site of perforation.

Sometimes, the diverticulum may wear its way into the bladder,
causing urinary  symptoms.

   Differential considerations include those discussed under
appendicitis, but in  addition one must consider cancer of the
colon, and other diseases common to the  older population seen
with this disease.  Certain blood test results, judicious
plain  and barium x-rays, and careful judgment are most
important.

    Treatment

   Unlike appendicitis, diverticulitis only occasionally
requires surgical treatment.  Generally, the digestive system
is placed at total rest with elimination of oral intake,
stomach suction and replacement of fluids by vein.  Antibiotics
are given to counter  the abdominal infection.

   If there is no improvement in 24 to 48 hours (there usually
is), or if things worsen  despite treatment, surgery may be
necessary, with removal of large portions of the  colon
sometimes necessary..  Recurrences may also warrant surgery.
It is essential  that a coexistent tumor be ruled out at some
point, usually with x-rays and a  proctosigmoidoscopy.

   Almost all deaths occur in older patients, under conditions
of emergency surgery.   Thus, when possible, many surgeons
prefer to wait until the patient has stabilized  before
operating, when this is possible.
!
*
                         Dizziness

                   Hope Through Research

      For sale by the Superintendent of Documents, U.S.
                 Government Printing Office
                    Washington, DC 20402

   Prepared by the Office of Scientific and Health Reports
                    National Institute of
                Neurological and Communicative
                     Disorders and Stroke


                 NATIONAL INSTITUTES OF HEALTH
                    Bethesda, Maryland 20892

                   NIH Publication No. 86-76
                        September 1986


                  U.S. DEPARTMENT OF HEALTH
                      AND HUMAN SERVICES
                     Public Health Service
                 National Institutes of Health


Cover:

     Research has given us new ways to diagnose dizziness. Here,
a technician applies electrodes to a  man's face to record eye
movement while the balance system in his inner ear is
stimulated.


Dizziness -

Hope Through Research


     Most of us can remember feeling dizzy--after a roller
coaster ride, maybe, or when looking down from a tall building,
or when, as children, we would step off a spinning
merry-go-round. Even superbly conditioned astronauts have had
temporary trouble with dizziness while in space. In these
situations, dizziness arises naturally from unusual changes that
disrupt our normal feeling of stability.

     But dizziness can also be a sign that there is a
disturbance or a disease in the system that helps people
maintain balance. This system is coordinated by the brain,
which reacts to nerve impulses from the ears, the eyes, the
neck and limb muscles, and the joints of the arms and legs. If
any of these areas fail to function normally or if the brain
fails to coordinate the many nerve impulses it receives, a
person may feel dizzy. The feeling of dizziness varies from
person to person and, to some extent, according to its cause;
it can include a feeling of unsteadiness, imbalance, or even
spinning.

     Disease-related dizziness, whether it takes the form of
unsteadiness or spinning, is fairly common in the older
population. Today, both older and younger people with serious
dizziness problems can be helped by a variety of
techniques--from medication to surgery to balancing exercises.
Such techniques have been developed and improved by scientists
studying dizziness.

     Much of today's research on dizziness is supported by the
National Institute Of Neurological and Communicative Disorders
and Stroke (NINCDS). This Institute is a unit of the National
Institutes of Health. It is the focal point within the Federal
Government for research on the brain and central nervous
system, including studies of the senses through which we
interact with our surroundings.

     With NINCDS support, scientists are searching for better
ways to diagnose and treat dizziness, and are investigating the
mechanisms that help us maintain our normal sense of balance.
These studies, along with basic research on how the ear, the
brain, and the nerves work, hold the best hope for relief for
dizziness sufferers.


A delicate balancing act


     To understand what goes wrong when we feel dizzy, we need
to know about the vestibular system by which we keep a sense of
balance amid all our daily twisting and turning, starting and
stopping, jumping, falling, rolling, and bending.

     The vestibular system is located in the inner ear and
contains the following structures: vestibular labyrinth,
semicircular canals, vestibule, utricle, and saccule. These
structures work in tandem with the vestibular areas of the
brain to help us maintain balance.

[Graphic Omitted]

     The semicircular canals and vestibule of the inner ear
contain a fluid called endolymph that moves in response to
head movement.

     The vestibular labyrinth is located behind the eardrum.
The labyrinth's most striking feature is a group of three
semicircular canals or tubes that arise from a common base. At
the base of the canals is a rounded chamber called the
vestibule. The three canals and the vestibule are hollow and
contain a fluid called endolymph which moves in response to
head movement.

     Within the vestibule and the semicircular canals are
patches of special nerve cells called hair cells. Hair cells
are also found in two fluid-filled sacs, the utricle and
saccule, located within the vestibule These cells are aptly
named: rows of thin, flexible, hairlike fibers project from
them into the endolymph.

     Also located in the inner ear are tiny calcium stones
called otoconia. When you move your head or stand up, the hair
cells are bent by the weight of the otoconia or movement of the
endolymph. The bending of the hair cells transmits an electrical
signal about head movement to the brain. This signal travels
from the inner ear to the brain along the eighth cranial
nerve--the nerve involved in balance and hearing. The brain
recognizes the signal as a particular movement of the head and
is able to use this information to help maintain balance.

[Graphic Omitted]

     In the inner ear, rows of hairlike fibers project from tiny
patches of nerve cells called hair cells.

[Graphic Omitted]

     These tiny calcium stones called otoconia are part of the
inner ear's balance system.

A thorough exam reveals the cause of most dizziness.

     The senses are also important in determining balance.
Sensory input from the eyes as well as from the muscles and
joints is sent to the brain, alerting us that the path we are
following bends to the fight or that our head is tilted as we
bend to pick up a dime. The brain interprets this
information--along with cues from the vestibular system--and
adjusts the muscles so that balance is maintained.

     Dizziness can occur when sensory information is distorted.
Some people feel dizzy at great heights, for instance, partly
because they cannot focus on nearby objects to stabilize
themselves. When one is on the ground, it is normal to sway
slightly while standing. A person maintains balance by
adjusting the body's position to something close by. But when
someone is standing high up, objects are too far away to use to
adjust balance. The result can be confusion, insecurity, and
dizziness, which is sometimes resolved by sitting down.

     Some scientists believe that motion sickness, a malady
that affects sea, car; and even space travelers, occurs when
the brain receives conflicting sensory information about the
body's motion and position. For example, when someone reads
while riding in a car, the inner ear senses the movement of the
vehicle, but the eyes gaze steadily on the book that is not
moving. The resulting sensory conflict may lead to the typical
symptoms of motion sickness: dizziness, nausea, vomiting, and
sweating.

     Another form of dizziness occurs when we turn around in a
circle quickly several times and then stop suddenly. Turning
moves the endolymph. The moving endolymph tells us we are still
rotating but our other senses say we've stopped. We feel dizzy.


Diagnosing the problem


     The dizziness one feels after spinning around in a circle
usually goes away quickly and does not require a medical
evaluation. But when symptoms appear to be caused by an
underlying physical problem, the prudent person will see a
physician for diagnostic tests. According to a study supported
by the National Institute of Neurological and Communicative
Disorders and Stroke, a thorough examination can reveal the
underlying cause of dizziness in about 90 percent of cases.

     A person experiencing dizziness may first go to a general
practitioner or family physician; between 5 and 10 percent of
initial visits to these physicians involve a complaint of
dizziness. The patient may then be referred either to an ear
specialist (otologist) or a nervous system specialist
(neurologist).

[Graphic Omitted]

     As part of a research study, this NINCDS physician tests a
patient's balance.

Tests that make the patient feel dizzy help establish a
diagnosis.

     The patient will be asked to describe the exact nature of
the dizziness, to give a complete history of its occurrence,
and to list any other symptoms or medical problems. Patients
give many descriptions of dizziness-depending to some extent on
its cause. Common complaints are light-headedness, a feeling of
impending faint, a hallucination of movement or motion, or a
loss of balance without any strange feelings in the head. Some
people also report they have vertigo--a form of dizziness in
which one's surroundings appear to be spinning uncontrollably
or one feels the sensation of spinning.

     The physician will try to determine what components of a
patient's nervous system are out of kilter, looking first for
changes in blood pressure, heart rhythm, or vision--all of
which may contribute to the complaints. Sometimes dizziness is
associated with an ear disorder. The patient may have loss of
hearing, discomfort from loud sounds, or constant noise in the
ear, a disorder known as tinnitus. The physician will also look
for other neurological symptoms: difficulty in swallowing or
talking, for example, or double vision.


Tests and scans


     After the initial history-taking and physical examination,
the physician may deliberately try to make the patient feel
dizzy. The patient may be asked to repeat actions or movements
that generally cause dizziness: to walk in one direction and
then turn quickly in the opposite direction, or to
hyperventilate by breathing deeply for 3 minutes.

     In another test, the patient sits upright on an examining
table. The physician tilts the patient's head back and turns it
partway to one side, then gently but quickly pushes the patient
backward to a lying down position. The reaction to this
procedure varies according to the cause of dizziness. Patients
with benign positional vertigo may experience vertigo plus
nystagmus. rapid, uncontrollable back-and-forth movements of
the eyes.

[Graphic Omitted]

     In a new caloric test to diagnose dizziness, air or water
is injected gradually into a patient's ears. The eye movements
that result are recorded.

     One widely used procedure, called the caloric test,
involves electronic monitoring of the patient's eye movements
while one ear at a time is irrigated with warm water or warm
air and then with cold water or cold air. This double stimulus
causes the endolymph to move in a way similar to that produced
by rotation of the head. If the labyrinth is working normally,
nystagmus should result. A missing nystagmus reaction is a
strong argument that the balance organs are not acting
correctly.

     NINCDS-supported scientists at The Johns Hopkins
University in Baltimore observed that not all patients can
tolerate the traditional caloric test. Some become sick when
the ear is irrigated with the standard amount of water or air
before physicians can measure their eye movements. So the
scientists are designing a method of conducting the test more
gradually by slowly adjusting the amount of water or air
reaching the inner ear. Their goal is to reduce patient
discomfort while allowing the test to proceed.

Balance disorders are often accompanied by hearing loss.

     Some patients who cannot tolerate the caloric test are
given a rotatory test. In this procedure, the patient sits in a
rotating chair, head tilted slightly forward. The chair spins
rapidly in one direction, then stops abruptly. Depending on the
cause of dizziness, the patient may experience vertigo after
this rotation.

     In one variation of this test, the chair is placed in a
tent of striped cloth. As the chair rotates, electrodes record
movements of the patient's eyes in response to the stripes. The
physician evaluates these eye movements, a form of nystagmus,
to determine if the patient has a disorder of the balance
system.

     Because disorders of balance are often accompanied by
hearing loss, the physician may order a hearing test.

     Hearing loss and associated dizziness could also be due to
damaged nerve cells in the brain stem, where the hearing and
balance nerve relays signals to the brain. To detect a
malfunction, the physician may order a kind of computerized
brain wave study called a brain stem auditory evoked response
test. In this procedure, electrodes are attached to several
places on the surface of the patient's scalp and a sound is
transmitted to the patient's ear. The electrodes measure the
time it takes nerve signals generated by the sound to travel
from the ear to the brain stem.

[Graphic Omitted]

     This patient will undergo a rotatory test. How her eyes
respond to the stripes as the chair rotates will give otologists
information about her balance system.

[Graphic Omitted]

Patients with dizziness may undergo a computed tomographic (CT)
scan to see if the problem is a tumor or a cyst.

     If there is reason to suspect that the dizziness could
stem from a tumor or cyst, the patient may undergo a computed
tomographic (CT) scan. In a CT scan, x-ray pictures are taken
of the brain from several different angles. These images are
then combined by a computer to give a detailed view that may
reveal the damaging growth.

     Sometimes anxiety and emotional upset cause a person to
feel dizzy. Certain patients may be asked to take a
psychological test, to try to find out whether the dizziness is
caused or intensified by emotional stress.

     The many tests administered by a physician will usually
point to a cause for the patient's dizziness. Disorders
responsible for dizziness can be categorized as:

   * peripheral vestibular, or those involving a disturbance in
     the labyrinth.

   * central vestibular, or those resulting from a problem in
     the brain or its connecting nerves.

   * systemic, or those originating in nerves or organs outside
     the head.


Confused signals


     When someone has vertigo but does not experience faintness
or difficulty in walking, the cause is probably a peripheral
vestibular disorder. In these conditions, nerve cells in the
inner ear send confusing information about body movement to the
brain.

     Ménière's disease. A well-known cause of vertigo is the
peripheral vestibular disorder known as Ménière's disease.
First identified in 1861 by Prosper Ménière, a French
physician, the disease is thought to be caused by too much
endolymph in the semicircular canals and vestibule. Some
scientists think that the excess endolymph may affect the hair
cells so that they do not work correctly. This explanation,
however, is still under study.

     The vertigo of Ménière's disease comes and goes without an
apparent cause; it may be made worse by a change in position
and reduced by being still.

     In addition to vertigo, patients have hearing loss and
tinnitus. Hearing loss is usually restricted at first to one
ear and is often severe. Patients sometimes feel "fullness" or
discomfort in the ear, and diagnostic testing may show unusual
sensitivity to increasingly loud sounds. In 10 to 20 percent of
patients, hearing loss and tinnitus eventually occur in the
second ear.

[Graphic Omitted]

Patients suspected of having Ménière's disease commonly
undergo electronystagmography, an electronic recording of the
caloric test.


Ménière's disease patients are often helped by drugs or surgery.


     Ménière's disease patients may undergo
electronystagmography, an electrical recording of the caloric
test, to determine if their labyrinth is working normally.

     Attacks of Ménière's disease may occur several times a
month or year and can last from a few minutes to many hours.
Some patients experience a spontaneous disappearance of
symptoms while others may have attacks for years.

     Treatment of Ménière's disease includes such drugs as
meclizine hydrochloride and the tranquilizer diazepam to reduce
the feeling of intense motion during vertigo. To control the
buildup of endolymph, the patient may also take a diuretic, a
drug that reduces fluid production. A low-salt diet--which
reduces water retention-is claimed to be an effective treatment
of Ménière's disease.

     When these measures fail to help, surgery may be
considered. In shunt surgery, part of the inner ear is drained
to reestablish normal inner ear fluid or endolymph pressure. In
another operation, called vestibular nerve section, surgeons
expose and cut the vestibular part of the eighth nerve. Both
vestibular nerve section and shunt surgery commonly relieve the
dizziness of Ménière's disease without affecting hearing.

     A more drastic operation, labyrinthectomy, involves total
destruction of the inner ear. This procedure is usually
successful in eliminating dizziness but causes total loss of
hearing in the operated ear--an important consideration since
the second ear may one day be affected.

     Positional vertigo. People with benign positional vertigo
experience vertigo after a position change. Barbara noticed the
first sign of this disorder one morning when she got up out of
bed. She felt the room spinning. Frightened, she quickly
returned to bed and lay down. After about 30 seconds the
vertigo passed. Fearing a stroke, Barbara went to the emergency
room of a hospital for a medical evaluation, which failed to
show a problem. She had no symptoms for several days, then the
problem returned. At this point, Barbara was referred to an
otoneurologist, a physician who specializes in the ear and
related parts of the nervous system.

Viral infections may lead to vestibular neuronitis.

     Like Barbara, most patients with benign positional vertigo
are extremely worried about their symptoms. But the patients
usually feel less threatened once the disorder is diagnosed.

     The cause of benign positional vertigo is not known,
although some patients may recall an incident of head injury.
The condition can strike at any adult age with attacks
occurring periodically throughout a person's life.

     In one type of treatment, the patient practices the
position that provokes dizziness until the balance system
eventually adapts. Rarely, a physician will prescribe
medication to prevent attacks.

     Vestibular neuronitis. In this common vestibular disorder,
the patient has severe vertigo. Jack experienced his first
attack of this problem at 2 a.m. when he rolled over in bed and
suddenly felt the room spinning violently. He started vomiting
but couldn't stand up; finally, he managed to crawl to the
bathroom. When he returned to bed, he lay very still--the only
way to stop the vertigo. Three days later, he was able to walk
without experiencing vertigo, but he still felt unsteady.
Gradually, over the next several weeks, Jack's balance improved,
but it was a year before he was entirely without symptoms.

     Unlike Ménière's disease, vestibular neuronitis is not
associated with hearing loss. Patients with vestibular
neuronitis first experience an acute attack of severe vertigo
lasting for hours or days, just as Jack did, with loss of
balance sometimes lasting for weeks or months. About half of
those who have a single attack have further episodes over a
period of months to years.

     The cause of vestibular neuronitis is uncertain. Since the
first attack often occurs after a viral illness, some
scientists believe the disorder is caused by a viral infection
of the nerve.

     Other labyrinth problems. Inner ear problems with
resulting dizziness can also be caused by certain antibiotics
used to fight life-threatening bacterial infections. Probably
the best-known agent of this group is streptomycin. Problems
usually arise when high doses of these drugs are taken for a
long time, but some patients experience symptoms after short
treatment with low doses, especially.if they have impaired
kidneys.

     The first symptoms of damage to the inner ear caused by
medication are usually hearing loss, tinnitus, or unsteadiness
while walking. Stopping the drug can usually halt further
damage to the balance mechanism, but this is not always
possible: the medicine may have to be continued to treat a
life-threatening infection. Patients sometimes adapt to the
inner ear damage that may-occur after prolonged use of these
antibiotics and recover their balance

     Balance can also be affected by a cholesteatoma, a clump
of cells from the eardrum that grow into the middle ear and
accumulate there These growths are thought to result from
repeated infections such as recurrent otitis media. If
unchecked, a cholesteatoma can enlarge and threaten the inner
ear. But if the growth is detected early, it can be surgically
removed.

[Graphic Omitted]

     This CT scan shows a massive cholesteatoma (arrow), a
tumorlike clump of cells that has grown in the middle ear, and
is a likely cause of this patient's dizziness.


Brain and nerve damage


     The vestibular nerve carries signals from the inner ear to
the brain stem. If either the nerve or the brain stem is
damaged, information about position and movement may be blocked
or incorrectly processed, leading to dizziness. Conditions in
which dizziness results from damage to the brain stem or its
associated nerves are called central causes of dizziness.

     Acoustic neuroma. One central cause of dizziness is a
tumor called an acoustic neuroma. Although the most common sign
of this growth is hearing loss followed by tinnitus, some
patients also experience dizziness.

     An acoustic neuroma usually occurs in the internal
auditory canal, the bony channel through which the vestibular
nerve passes as it leaves the inner ear. The growing tumor
presses on the nerve, sending false messages about position and
movement to the brain.

     The hearing nerve running alongside the vestibular nerve
can also be compressed by the acoustic neuroma, with resulting
tinnitus and hearing loss. Or the tumor may press on other
nearby nerves, producing numbness or weakness of the face. If
the neuroma is allowed to grow, it will eventually reach the
brain and may affect the function of other cranial nerves.

[Graphic Omitted]

     These benign cells from an acoustic neuroma were grown in
tissue culture. If diagnosed early, acoustic neuromas can be
removed completely with no neurological damage.

Early treatment of acoustic neuroma helps patients regain
balance.

     Computed tomography has revolutionized the detection of
acoustic neuromas. If an early diagnosis is made, a surgeon can
remove the tumor. The patient usually regains balance

     Stroke. Dizziness may be a sign of a "small stroke" or
transient ischemic attack (TIA) in the brain stem. TIA's, which
result from a temporary lack of blood supply to the brain, may
also cause transient numbness, tingling, or weakness in a limb
or on one side of the face. Other signs include temporary
blindness and difficulty with speech. These symptoms are
warning signs: one should see a physician immediately for
treatment. If a TIA is ignored, a major stroke may follow.


Systemic diseases: underlying illness


     Dizziness can be a symptom of diseases affecting body
parts other than the-brain and central nervous system. Systemic
conditions like anemia or high blood pressure decrease oxygen
supplies to the brain; a physician eliminates the resulting
dizziness by treating the underlying systemic illness.

     Damaged sensory nerves. We maintain balance by adjusting
to information transmitted along sensory nerves from sensors in
the eyes, muscles, and joints to the spinal cord or brain. When
these sensory nerves are damaged by systemic disease, dizziness
may result.

     Multiple sensory deficits, a systemic disease, is believed
by some physicians to be the chief cause of vaguely described
dizziness in the aged population. In this disorder several
senses or sensory nerves are damaged. The result: faulty
balance.

     People with diabetes, which can damage nerves affecting
vision and touch, may develop multiple sensory deficits. So can
patients with arthritis or cataracts, both of which distort how
sensory information reaches the brain. The first step in
treating multiple sensory deficits is to eliminate symptoms of
specific disorders. Permanent contact lenses can improve vision
in cataract patients, for example, and medication or surgery
may ease pain and stiffness related to arthritis.

     Symptoms of damaged sensory nerves may be relieved by a
collar to eliminate extreme head motion, balancing exercises to
help compensate for sensory losses, or a cane to aid balance.
Some patients are helped by the drug methylphenidate, which can
increase awareness of remaining sensations.

     Systemic neurological disorders such as multiple
sclerosis, Alzheimer's disease, Parkinson's disease, or
Creutzfeldt-Jakob disease may also cause dizziness, primarily
during walking. However, dizziness is rarely the sole symptom
of these nervous system diseases.

     Low blood pressure. One common systemic disease causing
dizziness is postural or orthostatic hypotension. In this
disease, the heart does not move the blood with enough force to
supply the brain adequately. Symptoms include sudden feelings
of faintness, light-headedness, or dizziness when standing up
quickly.

     Because the muscles in aging blood vessels are weak and
the arteries inadequate in helping convey blood to the head,
older people are particularly susceptible to this condition.
Older persons who do not sit or lie down at the first sensation
of dizziness may actually lose consciousness.

[Graphic Omitted]

     Postural hypotension is easily diagnosed. The patient's
blood pressure is checked before standing abruptly and
immediately afterward.

Anxiety can trigger attacks of dizziness.

     People who have undetected anemia or those who are taking
diuretics to eliminate excess water from their body and reduce
high blood pressure are also at risk of developing postural
hypotension.

     A physician can easily diagnose postural hypotension: the
patient's blood pressure is measured before standing abruptly
and immediately afterward. Treatment is designed to eliminate
dizziness by reducing the patient's blood volume.

     A secondary symptom. Dizziness may also be a secondary
symptom in many other diseases. Faintness accompanied by
occasional loss of consciousness can be due to low blood sugar,
especially when the faint feeling persists after the patient
lies down.

     A common cause of mild dizziness--the kind described as
light-headedness--is medicine. A number of major prescription
drugs may produce light-headedness as a side effect. Two types
of drugs that can cause this problem are sedatives, which are
taken to induce sleep, and tranquilizers, which are used to
calm anxiety.


When anxiety strikes


     Tranquilizers may cause a type of dizziness referred to as
light-headedness--but so may anxiety. Cynthia is a young woman
who becomes light-headed under a variety of stressful
circumstances. The light-headedness sometimes is accompanied by
heart palpitations and panic. She can produce these symptoms at
will by breathing rapidly and deeply for a few minutes.

     Cynthia's light-headedness is due to hyperventilation:
rapid, prolonged deep breathing or occasional deep sighing that
upsets the oxygen and carbon dioxide balance in the blood. The
episodes are typically brief and often associated with tingling
and numbness in the fingers and around the mouth.
Hyperventilation is triggered by anxiety or depression in about
60 percent of dizziness patients.

Research offers new insights into the basis of balance.

     Once made aware of the source of the symptoms, a patient
can avoid hyperventilation or abort attacks by breath-holding or
breathing into a paper bag to restore a correct balance of
oxygen and carbon dioxide. If hyperventilation is due to
anxiety, psychological counseling may be recommended.

     Some patients who report dizziness may be suffering from a
psychiatric disorder. Generally these persons will say that
they experience light-headedness or difficulty concentrating;
they may also describe panic states when in crowded places.
Tests of such patients reveal that the inner ear is working
correctly. Treatment may include counseling.


Demystifying dizziness through research


     Scientists are working to understand dizziness and its
sources among the complex interactions of the labyrinth, the
other sense organs, and the brain. The research is offering new
insights into the basis of balance, as well as improvements in
diagnosis, treatment, and prevention of dizziness.

     Innovative surgery. Delicate surgical instruments and
operating microscopes have made possible new methods to help
patients with dizziness. The symptoms of benign positional
vertigo, for example, may be relieved by a microsurgical ear
operation called a singular neurectomy in which a tiny portion
of the vestibular nerve is divided and cut.

     Patients with Ménière's disease may benefit from a
microsurgical operation called the cochleosacculotomy. In this
procedure, a small curette or wire loop is used to reach into
the vestibule of the inner ear and remove the fluid-filled
saccule. An investigator at the Massachusetts Eye and Ear
Institute in Boston has found that this operation relieves
symptoms of vertigo in about 80 percent of patients.

     Space biology. Research also promises to help astronauts
who suffer from dizziness or space sickness. In one study, a
scientist aboard a space shuttle conducted experiments to find
out why half the astronauts who have space sickness at the
start of a flight overcome this problem before the end of the
mission. The investigator, from the Massachusetts Institute of
Technology, found that the space traveler's brain no longer
relies on the gravity-sensitive inner ear structures for
information about position and motion. Instead, the astronaut's
brain realizes that the inner ear is sending false information
and starts to depend more on the eyes to find out about the
body's movements. This finding may enable space biologists to
train astronauts before launch to avoid space sickness.

     During that same space mission, a German scientist
performed experiments that raised questions about the theory
behind the caloric test. According to that theory, alternate
heating and cooling of the endolymph causes the fluid to form
wave-like swirling patterns called convection currents. These
currents make the brain think the head is moving. The result is
nystagmus.

     In space, however, lack of gravity should prevent
convection currents from forming, so the eyes were expected to
remain still. Instead, they moved just as though the test was
being done on Earth in normal gravity. These experiments
indicate there is more than one explanation for why the caloric
test works: when the endolymph is warmed, the fluid expands and
moves the cupula, the top of the cochlear duct. The movement of
the cupula cues the brain that the head is moving and the eyes
respond.

     This research helps scientists interpret methods used to
test vestibular function. It also promises to increase our
understanding of the balance system.

     Currently, scientists at the Johnson Space Center in
Houston and at the Good Samaritan Hospital in Portland are
preparing to study space sickness and vestibular function in a
microgravity (near zero gravity) laboratory. The astronauts'
vestibular function will be analyzed in a series of
experiments, including studies to test whether visual input
becomes more important in maintaining balance as weightlessness
increases. The scientists anticipate that this research will
help all sufferers of motion sickness, not just astronauts.

Scientists are developing better diagnostic tests for dizziness.

     Improved diagnosis. Back on Earth, improvements are being
made in measuring precisely the eye movements of patients
undergoing diagnostic tests for dizziness. Investigators at the
NINCDS-funded research center at the University of California
at Los Angeles have developed a computer-controlled chair in
which a patient is shifted into a variety of body positions to
stimulate the labyrinth. Eye responses are measured with newly
designed computerized instruments. To further stimulate eye
movements, a set of computer-generated visual patterns can be
moved with the chair or independently. of it.

     These instruments will extract much information about a
patient's ability to integrate information from the eyes and
the inner ear, and will help distinguish patients with
different disorders of the balance system.

     Signaling the brain. To understand dizziness, scientists
must find out how stimuli to the labyrinth are translated into
information that the brain can use to maintain balance. How,
for example, is information from the inner ear sent to the
brain and interpreted? Among the scientists studying this
question is an NINCDS grantee at the University of Chicago who
is looking at the different ways hair cells react to the
movement of inner ear fluid. He has identified a characteristic
pattern of electrical response in hair cells. The next step is
to discover how these messages are interpreted by the nerve
cells carrying information to the brain.

[Graphic Omitted]

     An NINCDS grantee at the University of Minnesota adjusts
the controls on an oscilloscope used to monitor patients' eye
movements during vestibular testing.

     Another NINCDS grantee at the University of Minnesota is
studying the activity of the brain when it sends
balance-preserving signals from the sense organs to the
muscles. In one experiment, healthy persons are rotated in the
dark at a constant rate. After a few minutes they no longer
think they are moving. This is because the inner ear only
senses changes in the rate of movement. If the lights are
turned on and both the chair and the room rotate at the same
constant speed, again the person doesn't sense movement. Both
the inner ear and the eyes are fooled into thinking there is no
motion.

     But the investigator found that if the chair and the room
are accelerated, the patient develops what is described as
sensory conflict. The acceleration of the chair tells the inner
ear that there is movement. But the eyes tell the brain that
the body is stationary. How patients react in these conflict
situations reveals how the brain puts together various types of
sensory information to maintain balance. The results of this
and related experiments will help scientists build a
mathematical model of the balance system.


Hope for the future


     For those who are healthy, equilibrium is a sense often
taken. for granted. People can't see their labyrinth, even
though it is as much a sense organ as the ears or the eyes. But
when it is injured, an ability vital to everyday living is
lost.

     Scientists already understand a great deal about the
labyrinth's function and the way the brain maintains balance.
Further research into this complex system should help those who
are incapacitated by dizziness when the balance system goes
awry.


Voluntary health organizations


     The following organizations provide information on
dizziness or on inner ear diseases that cause dizziness:

Acoustic Neuroma Association
P.O. Box 398
Carlisle, PA 17013
[phone]

American Academy of Otolaryngology-Head and
  Neck Surgery
Suite 302
1101 Vermont Avenue, N.W.
Washington, DC 20005
[phone]

Better Hearing Institute
P.O. Box 1840
Washington, DC 20013
[phone]
[phone] (Toll free)

National Hearing Association
721 Enterprise
Oak Brook, IL 60521
[phone]


Human tissue banks


     The study of ear tissue from patients with dizziness and
deafness is invaluable in research. Temporal bones willed by
people with balance or hearing problems and by people with
normal hearing can be used to help research scientists and
physicians training to be otolaryngologists. Physicians in
training study the basic anatomy of the temporal bone and
develop their surgical skills. Scientists use the bones for
research on the inner ear and on congenital disorders that
cause deafness. Middle ear bones (ossicles) and the eardrum are
also used as grafts to surgically correct sound transmission
problems of the middle ear.

     NINCDS supports four temporal bone banks that supply
scientists in every state with tissue from patients who have
dizziness or deafness. The donated temporal bone includes the
eardrum, the entire middle and inner ear, and the nerve tissues
which combine into the brain stem. For information about tissue
donation and collection, write to:

National Temporal Bone Bank
Eastern Center
Massachusetts Eye and Ear Infirmary
243 Charles Street
Boston, MA 02114
[phone], ext. 2711

National Temporal Bone Bank
Midwestern Center
University of Minnesota
Box 396-Mayo
Minneapolis, MN 55455
[phone]

National Temporal Bone Bank
Southern Center
Baylor College of Medicine
Neurosensory Center--Room A523
Houston, TX 77030
[phone]

National Temporal Bone Bank
Western Center
UCLA School of Medicine
31-24 Rehabilitation Center
Los Angeles, CA 90024
[phone]


Some useful definitions:


acoustic neuroma: tumor of the vestibular nerve that may press
on the hearing nerve causing dizziness and hearing loss.

balance system: complex biological system that enables us to
know where our body is in space and to keep the position we
want. Proper balance depends on information from the labyrinth
in the inner ear, from other senses such as sight and touch,
and from muscle movement.

benign positional vertigo: condition in which moving the head
to one side or to a certain position brings on vertigo.

brain stem auditory evoked response (BAER): diagnostic test in
which electrodes are attached to the surface of the scalp to
determine the time it takes inner ear electrical responses. to
sound to travel from the ear to the brain. The test helps
locate the cause of some types of dizziness.

caloric test: diagnostic test in which warm or cold water or
air is put into the ear. If a person experiences certain eye
movements (nystagmus) after this procedure, the labyrinth is
working correctly.

cholesteatoma: a tumorlike accumulation of dead cells in the
middle ear. This growth is thought to result from repeated
middle ear infections.

computed tomography (CT) scan: radiological examination useful
for examining the inside of the ear and head.

diuretic: drug that promotes water loss from the body, through
the urine. Used to treat hypertension, diuretics may bring on
dizziness due to postural hypotension.

dizziness: feeling of physical instability with regard the
outside world.

endolymph: fluid filling part of the labyrinth.

hair cells: specialized nerves found in the semicircular canals
and vestibule. Fibers (hairs) sticking out of one end of the
hair cells move when the head moves and send information to the
brain that is used to maintain balance.

hyperventilation: repetitive deep breathing that reduces the
carbon dioxide content of the blood and brings on dizziness.
Anxiety may cause hyperventilation and dizziness.

inner ear: contains the organs of hearing and balance.

labyrinth: the organ of balance, which is located in the inner
ear. The labyrinth consists of the three semicircular canals
and the vestibule.

Ménière's disease: condition that causes vertigo. The disease
is believed to be caused by too much endolymph in the
labyrinth. Persons with this illness also experience hearing
problems and tinnitus.

middle ear:. the space immediately behind the eardrum. This part
of the ear contains the three bones of heating: the hammer
(malleus), anvil (incus), and stirrup (stapes).

multiple sensory deficits: condition associated with dizziness
in which damage to nerves of the eye and arms or legs reduces
information about balance to the brain.

neurologist: physician who specializes in disorders of the
nervous system.

nystagmus: rapid back-and-forth movements of the eyes. These
reflex movements may occur during the caloric test and are used
in the diagnosis of balance problems.

orthostatic hypotension: see postural hypotension.

otologist: physician who specializes in diseases of the ear.

peripheral vestibulopathy: vestibular disorder in which the
vestibular nerve appears inflamed and paralyzed. Patients may
have one or several attacks of vertigo.

postural hypotension (also called orthostatic hypotension):
sudden dramatic drop in blood pressure when a person rises from
a sitting, kneeling, or lying position. The prime symptom of
postural hypotension, which is sometimes due to low blood
volume, is dizziness or faintness. The condition can be
dangerous in older persons, who may faint and injure
themselves.

semicircular canals: three curved hollow tubes in the inner ear
that are part of the balance organ, the labyrinth. The canals
are joined at their wide ends and are filled with endolymph.

stroke: death of nerve cells due to a loss of blood flow in the
brain. A stroke often results in permanent loss of some
sensation or muscle activity.

TIA: see transient ischemic attack.

tinnitus: noises or ringing in the ear.

transient ischemic attack (TIA): temporary interruption of
blood flow to a part of the brain. Because a TIA may signal the
possibility of a stroke, it requires immediate medical
attention. During a TIA, a person may feel dizzy, have double
vision, or feel tingling in the hands.

vertigo: severe form of dizziness in which one's surroundings
appear to be spinning uncontrollably. Extreme cases of vertigo
may be accompanied by nausea.

vestibular disorders: diseases of the inner ear that cause
dizziness.

vestibular nerve: nerve that carries messages about balance
from the labyrinth in the inner ear to the brain.

vestibular neuronitis: another name for peripheral
vestibulopathy.

vestibule: part of the labyrinth, located at the base of the
semicircular canals. This structure contains the endolymph and
patches of hair cells.

[Graphic Omitted]

While a patient watches a rotating drum, the otoneurologist
checks his eyes for signs of any disturbance in neurological
function that could cause dizziness.


Photograph Credits:

Bill Branson, NIH, cover 1, pages 7, 27.

Howard Bartner, NIH, page 2.

University of California, San Francisco, News Service
Publications, page 3 (upper).

House Ear Institute, Los Angeles, California, page 3 (lower).
Experimental Therapeutics Branch, NINCDS, page 5.

Dr. Bobby R. Alford, Baylor University, page 8.

Dr. Cecil W. Hart, Northwestern University Medical School,
page 10.

Dr. Leonard Proctor, The Johns Hopkins University, page 13.

National Heart, Lung, and Blood Institute, page 16.

Dr. John H. Anderson, University of Minnesota, page 20.
!
*

General Information about Down Syndrome

Fact Sheet Number 4 (FS4), 1993
______________________________________________________________________________

NICHCY
National Information Center for Children and Youth with Disabilities
P.O. Box 1492
Washington, DC 20013
[phone] (Local)
[phone] (TT)
1-800-999-5599 (Toll-free)
______________________________________________________________________________


DEFINITION OF Down Syndrome

        Down syndrome is the most common and readily identifiable chromosomal
        condition associated with mental retardation.  It is caused by a
        chromosomal abnormality:  for some unexplained reason, an accident in
        cell development results in 47 instead of the usual 46 chromosomes.
        This extra chromosome changes the orderly development of the body and
        brain.  In most cases, the diagnosis of Down syndrome is made
        according to results from a chromosome test administered shortly
        after birth.

INCIDENCE

        Approximately 4,000 children with Down syndrome are born in the U.S.
        each year, or about 1 in every 800 to 1,000 live births.  Although
        parents of any age may have child with Down syndrome, the incidence
        is higher for women over 35.  Most common forms of the syndrome do
        not usually occur more than once in a family.

CHARACTERISTICS

        There are over 50 clinical signs of Down syndrome, but it is rare to
        find all or even most of them in one person.  Some common
        characteristics include:

        -       Poor muscle tone;
        -       Slanting eyes with folds of skin at the inner corners (called
                epicanthal folds);
        -       Hyperflexibility (excessive ability to extend the joints);
        -       Short, broad hands with a single crease across the palm on
                one or both hands;
        -       Broad feet with short toes;
        -       Flat bridge of the nose;
        -       Short, low-set ears;
        -       Short neck;
        -       Small head;
        -       Small oral cavity; and/or
        -       Short, high-pitched cries in infancy.

        Individuals with Down syndrome are usually smaller than their non-
        disabled peers, and their physical as well as intellectual development
        is slower.

        Besides having a distinct physical appearance, children with Down
        syndrome frequently have specific health-related problems.  A lowered
        resistance to infection makes these children more prone to
        respiratory problems.  Visual problems such as crossed eyes and far-
        or nearsightedness are higher in those with Down syndrome, as are
        mild to moderate hearing loss and speech difficulty.

        Approximately one third of babies born with Down syndrome have heart
        defects, most of which are now successfully correctable.  Some
        individuals are born with gastrointestinal tract problems that can be
        surgically corrected.

        Some people with Down syndrome also may have a condition known as
        Atlantoaxial Instability, a misalignment of the top two vertebrae of
        the neck.  This condition makes these individuals more prone to
        injury if they participate in activities which overextend or flex the
        neck.  Parents are urged to have their child examined by a physician
        to determine whether or not their child should be restricted from
        sports and activities which place stress on the neck.  Although this
        misalignment is a potentially serious condition, proper diagnosis
        can help prevent serious injury.

        Children with Down syndrome may have a tendency to become obese as
        they grow older.  Besides having negative social implications, this
        weight gain threatens these individuals' health and longevity.  A
        supervised diet and exercise program may help reduce this problem.

EDUCATIONAL AND EMPLOYMENT IMPLICATIONS

        Shortly after a diagnoses of Down syndrome is confirmed, parents
        should be encouraged to enroll their child in an infant
        development/early intervention program. These programs offer parents
        special instruction in teaching their child language, cognitive,
        self-help, and social skills, and specific exercises for gross and
        fine motor development. Research has shown that stimulation during
        early developmental stages improves the child's chances of developing
        to his or her fullest potential.  Continuing education, positive
        public attitudes, and a stimulating home environment have also been
        found to promote the child's overall development.

        Just as in the normal population, there is a wide variation in mental
        abilities, behavior, and developmental progress in individuals with
        Down syndrome.  Their level of retardation may range from mild to
        severe, with the majority functioning in the mild to moderate range.
        Due to these individual differences, it is impossible to predict
        future achievements of children with Down syndrome.

        Because of the range of ability in children with Down syndrome it is
        important for families and all members of the school's education team
        to place few limitations on potential capabilities.  It may be
        effective to emphasize concrete concepts rather than abstract ideas.
        Teaching tasks in a step-by-step manner with frequent reinforcement
        and consistent feedback has been proven successful.  Improved public
        acceptance of persons with disabilities along with increased
        opportunities for adults with disabilities to live and work
        independently in the community, have expanded goals for individuals
        with Down syndrome. Independent Living Centers, group shared and
        supervised apartments and support services in the community have
        proven to be important resources for persons with disabilities.

RESOURCES

Brill, M.T.  (1993).  Keys to parenting a child with Down syndrome.
Hauppauge, NY: Barron's Educational Series.

National Down Syndrome Congress.  (1988).  Down syndrome (revised pamphlet).
(See address below.)

Stray-Gundersen, K.  (1986).  Babies with Down syndrome: A new parent's
guide.  Rockville, MD:  Woodbine House.  [Available from Woodbine House,
1-800-843-7323 (outside DC area) or [phone] (in DC area).]

National Down Syndrome Society. This baby needs you even more.
(See address below.)

ORGANIZATIONS

National Down Syndrome Congress
1605 Chantilly Drive, Suite 250
Atlanta, GA 30324
[phone]
[phone]

National Down Syndrome Society
666 Broadway, Suite 810
New York, NY  10012
[phone]
(1-800) 221-4602 (Toll Free)

The Arc (formerly the Association for Retarded Citizens of the United States)
500 East Border Street, Suite 300
Arlington, TX  76010
[phone]
[phone]
!
*
Title: What Is Dyslexia: 1984 Digest.

Personal Author: Harrie, Roger P.; Weller, Carol

Clearinghouse Number: EC180908

Publication Date: 84

Accession Number: ED262500

Descriptors: *Dyslexia; *Etiology; *Reading Difficulties; *Student
Characteristics

Identifiers: ERIC Digests

Abstract: The digest focuses on the nature of dyslexia and its
characteristics. An initial section notes the variations in definitions
as a function of the discipline. Diagnosis of dyslexia is said to isolate
the specific difficulties associated with the problem and to suggest
appropriate educational intervention. Typical characteristics of dyslexia
are noted, including a family history of reading problems, predominant
occurrence in males, an average or above average IQ and possibly a
proficiency in math, lack of enjoyment of reading as a leisure activity,
problems of letter and word reversal, and difficulty transferring
information from what is heard to what is seen and vice versa. The
contribution of three major factors to dyslexia is addressed: ocular
problems, language problems, and visuo-spatial-motor problems. A list of
references and resources concludes the digest. (CL)

TA- Teachers; Practitioners

Institution Name: ERIC Clearinghouse on Handicapped and Gifted Children,
Reston, Va.

Article Body:

Dyslexia is a term that has been loosely applied to reading disabilities.
Specific definitions for dyslexia vary with disciplines. Those in
medicine define dyslexia as a condition resulting from neurological,
maturational, and genetic causes, while those in psychology relate
dyslexia on the basis of the specific reading problems evidenced and give
no reference to causation. All disciplines would probably agree that
dyslexia is evidenced by persons of otherwise normal intellectual
capacity who have not learned to read despite exposure to adequate
instruction.

HOW IS DYSLEXIA DIAGNOSED?

The diagnosis of dyslexia usually begins with an awareness by parents or
teachers that a problem in reading exists. A physician is often the first
diagnostician to explore the nature of the difficulty. The medical
practitioner should investigate the cause of the reading problem by
conducting a complete physical examination and obtaining a comprehensive
health history. If indicated, the child should be referred for a
neurological examination. If dyslexia is suspected, the physician should
refer the child for further evaluation and treatment by a specialist in
psychoeducational diagnosis.

The major purpose of the diagnostic process is to isolate the specific
difficulties associated with dyslexia and to suggest appropriate
educational intervention. Usually the diagnostician will employ a battery
of assessment instruments that explore the relationship of specific
reading problems to the intellectual, achievement, perceptual, motoric,
linguistic, and adaptive capabilities of the individual. Based on the
results, an intervention plan can be implemented by a special educator or
remedial reading teacher trained in specialized reading techniques. No
one remedial reading method works for all reading disabled students.
Therefore it is important that the teacher have mastery of many different
techniques.

WHAT ARE SOME OF THE CHARACTERISTICS OF DYSLEXIA?

An individual is identified as dyslexic when a significant discrepancy
exists between intellectual ability and reading performance without an
apparent physical, emotional, or cultural cause. Common findings in the
history include, but are not limited to: (1) family history of reading
problems; (2) a predominant occurrence in males (males to females 8:1);
(3) an average or above average IQ and, not uncommonly, a proficiency in
math: (4) no enjoyment of reading as a leisure activity; (5) problems of
letter and word reversal; (6) developmental history of problems in
coordination and left/right dominance; (7) poor visual memory for
language symobols; (8) auditory language difficulties in word finding,
fluency, meaning, or sequence; (9) difficulty transferring information
from what is heard to what is seen and vice versa.

Specific reading problems associated with dyslexia include difficulty in
pronouncing new words, difficulty distinguishing similarities and
differences in words (no for on), and difficulty discriminating
differences in letter sound (pin, pen). Other problems may include
reversal of words and letters, disorganization of word order, poor
reading comprehension, and difficulty applying what has been read to
social or learning situations.

WHAT FACTORS CONTRIBUTE TO DYSLEXIA?

Ocular Problems

Several reliable studies (Helveston 1969; Blika 1982; Keys 1982; Hiatt
1984) have found that dyslexic individuals have no greater incidence of
eye problems than do individuals with normal reading ability. Such
parameters as visual acuity, stereo acuity, ocular alignment and
motility, fusion status (break point amplitude), and refractive error
have not been shown to be significantly different in poor versus normal
readers. Individuals with reading problems should, however, have a
careful eye examination as part of an overall medical examination. There
is no scientific evidence that visual training (including eye muscle
exercises, ocular tracking or pursuit exercises, or glasses with bifocals
or prisms) leads to significant improvement in the performance of
dyslexic individuals.

Language Problems

According to Mattis (1978), the primary contributing factor to dyslexia
is an auditory language deficit. Approximately 86% of the individuals
identified as dyslexic evidence an auditory language disorder that
prevents the individual from linking the spoken form of a word with its
written equivalent. In light of this, any individual with reading
problems should have a careful evaluation of his or her language
capabilities and where indicated, appropriate speech and language
intervention should be provided.

Visuo-Spatial-Motor Problems

In contrast to language problems, visuo-spatial-motor factors of dyslexia
appear less frequently (Robinson and Schwartz 1973). Approximately 5% of
the individuals identified as dyslexic have a visuo-spatial-motor problem
that interferes with sequential organization, scanning, and the
perception of temporal and spatial cues. Although visuo-spatial-motor
confusion is common in young children who are just learning to read,
these problems do not tend to account for severe and persistent reading
difficulties unless the child has missed so much basic reading
instruction that he cannot get caught up. Assessment of visual, spatial,
and motor capacities should be included in the diagnosis of any
coordination or orientation disorder; however, there is no scientific
evidence that interventions such as neurological and sensory
organizational training, laterality training, dominance training, balance
beam, or reflex inhibition will significantly accelerate reading
performance.

Other Factors

The importance of general intelligence in learning to read has been
examined and shown to be a critical factor in both reading and language
abilities. Investigations of the role of dominance in handedness,
eyedness, and mixed laterality have produced no consistent conclusions.
Studies investigating low birth weight, EEG abnormalities, temperamental
attributes, attention deficit disorders, birth order, food additives, and
chemical allergies have yielded mixed results. What is clear is that a
wide range of factors can be associated with reading difficulties but
that these factors work differently in different children. There is no
simple formula for diagnosing and treating a dyslexic child. Each one
requires his or her own individual program.

FOR MORE INFORMATION

Benton, A. L., and D. Pearl. DYSLEXIA. New York: Oxford University Press,
1978.

Blika, S. "Opthalmological Findings in Pupils of a Primary School with
Particular Reference to Reading Difficulties." ACTA OPTHALMOLOGICA 60
(1982):927-934.

Helveston, E. M. "Editorial." THE SIGHT SAVING REVIEW 39 (1969):1.

Hiatt, R. L. "Reading Problems and the Opthalmologist." ANNALS OF
OPTHALMOLOGY 16 (1984):116-122.

Keys, M. P. READING DISABILITIES. AMERICAN ACADEMY OF OPTHALMOLOGY
INSTRUCTION COURSE #117 (1982).

Mattis, S. "Dyslexia Syndromes: A Working Hypothesis." In DYSLEXIA,
edited by A. L. Benton and D. Pearl. New York: Oxford University Press,
1978.

Robinson, M. E., and L. B. Schwartz. "Visuo-motor Skills and Reading
Ability: A Longitudinal Study." DEVELOPMENTAL MEDICINE AND CHILD
NEUROLOGY 15 (1973):281-286.

Rourke, B. P. "Neuropsychological Research in Reading Retardation, A
Review. In DYSLEXIA, edited by A.L. Benton and D. Pearl. New York: Oxford
University Press, 1978.

This Digest was prepared for the ERIC Clearinghouse on Handicapped and
Gifted Children, 1984.

!
*
THE DYSTONIAS

What are the dystonias?

The dystonias are movement disorders in which sustained muscle
contractions cause twisting and repetitive movements or abnormal
postures.  The movements, which are involuntary and sometimes
painful, may affect a single muscle; a group of muscles such as
those in the arms, legs or neck; or the entire body.  Diminished
intelligence and emotional imbalance are not usually features of
the dystonias.

What are the symptoms?

Early symptoms may include a deterioration in handwriting after
writing several lines, foot cramps, and a tendency of one foot to
pull up or drag after running or walking some distance.  The neck
may turn or pull involuntarily, especially when the patient is
tired.  Other possible symptoms are tremor and voice or speech
difficulties.  The initial symptoms can be very mild and may be
noticeable only after prolonged exertion, stress or fatigue.  Over
a period of time, the symptoms may become more noticeable and
widespread and be unrelenting; sometimes, however, there is little
or no progression.

How are the dystonias classified?

One way to classify the dystonias is according to the parts of the
body they affect:

     o    Generalized dystonia affects most or all of the body.

     o    Focal dystonia is localized to a specific part of the
          body.

     o    Muftifocal dystonia involves two or more unrelated body
          parts.

     o    Segmental dystonia affects two or more adjacent parts of
          the body.

     o    Hemidystonia involves the arm and leg on the same side of
          the body.  This type of dystonia often results from
          stroke.

Some patterns of dystonia are defined as specific syndromes:

     Torsion dystonia, previously called dystonia musculorum
     deformans, is a rare, generalized dystonia that may be
     inherited, usually begins in childhood, and becomes
     progressively worse.  It can leave individuals seriously
     disabled and confined to a wheelchair.

Spasmodic torticollis, or torticollis, is the most common of the
focal dystonias.  In torticollis, the muscles in the neck that
control the position of the head are affected, causing the head to
twist and turn to one side.  In addition, the head may be pulled
forward or backward.  Torticollis can occur at any age, although
most individuals first experience symptoms in middle age.  It often
begins slowly and usually reaches a plateau.  About 10 to 20
percent of those with torticollis experience a spontaneous
remission, but unfortunately the remission may not be lasting.

Blepharospasm, the second most common focal dystonia, is the
involuntary, forcible closure of the eyelids.  The first symptom
may be uncontrollable blinking.  Only one eye may be affected
initially, but eventually both eyes are usually involved.  The
spasms may leave the eyelids completely closed causing functional
blindness even though the eyes and vision are normal.

Cranial dystonia is a term used to describe dystonia that affects
the muscles of the head, face, and neck.  Oromandibular dystonia
affects the muscles of the jaw, lips, and tongue.  The jaw may be
pulled either open or shut, and speech and swallowing can be
difficult.  Spasmodic dysphonia involves the muscles of the throat
that control speech.  Also called spastic dysphonia or laryngeal
dystonia, it causes strained and difficult speaking or breathy and
effortful speech.  Meige's syndrome is the combination of
blepharospasm and oromandibular dystonia and sometimes spasmodic
dysphonia.  Spasmodic torticollis can be classified as a type of
cranial dystonia.

Writer's cramp is a dystonia that affects the muscles of the hand
and sometimes the forearm, and only occurs during handwriting.
Similar focal dystonias have also been called typist's cramp,
pianist's cramp, musician's cramp, and golfer's cramp.

Dopa-responsive dystonia (DRD), of which Segawa's dystonia is an
important variant, is a condition successfully treated with drugs.
Typically, DRD begins in childhood or adolescence with progressive
difficulty in walking and, in some cases, spasticity.  In Segawa's
dystonia, the symptoms fluctuate during the day from relative
mobility in the morning to increasingly worse disability in the
afternoon and evening as well as after exercise.  Some scientists
feel DRD is not only rare but also rarely diagnosed since it mimics
many of the symptoms of cerebral palsy.

What do scientists know about the dystonias?

Investigators believe that the dystonias result from an abnormality
in an area of the brain called the basal ganglia where the messages
to initiate muscle contractions are believed to originate.
Scientists suspect a defect in the body's ability to process a
group of chemicals called neurotransmitters that help cells in the
brain communicate with each other.  Some of these neurotransmitters
include:

o GABA (gamma-aminobutyric acid), an inhibitory substance that
helps the brain maintain muscle control.

o Dopamine, an inhibitory chemical that influences the brain's
control of complex movement.

o Acetylcholine, an excitatory chemical that helps regulate
dopamine in the brain.  In the body, acetylcholine released at
nerve endings causes muscle contraction.

o Norepinephrine and serotonin, inhibitory chemicals that help the
brain regulate acetylcholine.

Acquired dystonia; also called secondary dystonia, results from
environmental or disease related damage to the basal ganglia.
Birth injury (particularly due to lack of oxygen), certain
infections, reactions to certain drugs, heavy-metal or carbon
monoxide poisoning, trauma, or stroke can cause dystonic symptoms.
Dystonias can also be symptoms of other diseases, some of which may
be hereditary.

About half the cases of dystonia have no connection to disease or
injury and are called primary or idiopathic dystonia.  Of the
primary dystonias, many cases appear to be inherited in a dominant
manner; i.e., only one carrier parent need contribute the dystonia
gene for the disease to occur, each child having a 50150 chance of
being a carrier.  In dystonia, however, there is consider-able
variation in the extent to which symptoms are present in an
individual carrier, and in how frequently symptoms appear in a
specific group of carriers such as a family.  This means that a
carrier may or may not develop a dystonia and that the symptoms may
vary widely even among members of the same family.  The product of
one defective gene appears to be sufficient to cause the chemical
imbalances that may lead to dystonia; but the possibility exists
that another gene or genes and environmental factors may play a
role.

Some cases of primary dystonia may have different types of
hereditary patterns.  Knowing the pattern of inheritance can help
families understand the risk of passing dystonia along to future
generations.

When do symptoms occur?

In some individuals, symptoms of a dystonia appear in childhood
between the ages of 5 and 16, usually in the foot or in the hand.
In generalized dystonia, the involuntary dystonic movements may
progress quickly to involve all limbs and the torso, but the rate
of progression usually slows noticeably after adolescence.

For other individuals, the symptoms emerge in late adolescence or
early adulthood.  In these cases, the dystonia often begins in
upper body parts, with symptoms progressing slowly.  A dystonia
that begins in adulthood is more likely to remain as a focal or
segmental dystonia.

Dystonias often progress through various stages.  Initially,
dystonic movements are intermittent and appear only during
voluntary movements or stress.  Later, individuals may show
dystonic postures and movements while walking and ultimately even
while they are relaxed.  Dystonic motions may lead to permanent
physical deformities by causing tendons to shorten.

In secondary dystonias due to injury or stroke, people often have
abnormal movements of just one side of the body, which may begin at
the time of the brain injury or sometime afterward.  Symptoms
generally plateau and do not usually spread to other parts of the
body.

Are them any treatments?

No one treatment has been found universally effective.  Instead,
physicians use a variety of therapies aimed at reducing or
eliminating muscle spasms and pain.

o Medication.  Several classes of drugs that may help correct
imbalances in neurotransniitters have been found useful.  But
response to drugs varies among patients and even in the same person
over time.  The most effective therapy is often individualized,
with physicians prescribing several types of drugs at different
doses to treat symptoms and produce the fewest side effects.

Frequently, the first drug administered belongs to a group that
reduces the level of the neurotransmitter acetylcholine.  Drugs in
this group include trihcxyphenidyl, benztropine, and procyclidine
HCI.

Drugs that regulate the neurotransmitter GABA may be used in
combination with these drugs or alone in patients with mild
symptoms.  GABA regulating drugs include the muscle relaxants
diazepam, lorazepam, clonazepam, and baclofen.

Other drugs act on dopamine, a neurotransmitter that helps the
brain fine-tune muscle movement.  Some drugs which increase
dopamine include levodopa/carbidopa and bromocriptine.  DRD has
been remarkably responsive to small doses of this dopamine-boosting
treatment.  On the other hand, patients have occasionally
benefitted from drugs that decrease dopamine, such as reserpine or
the investigational drug tetrabenazine.

Anticonvulsants including carbamazepine, usually prescribed to
control epilepsy, have occasionally helped individuals with
dystonia.

o Botulinum toxin.  Minute amounts of this familiar toxin can be
injected into affected muscles to provide temporary relief of focal
dystonias.  First used to treat blepharospasm, such injections have
gained wider acceptance among physicians for treating other focal
dystonias.  The toxin stops muscle spasms by blocking the
excitatory neurotransmitter acetylcholine.  The effect lasts for
several months before the injections have to be repeated.


o Surgery and other treatments.  Surgery may be recommended for
some patients when medication is unsuccessful or the side effects
are too severe.  In selected cases, advanced generalized dystonias
have been helped, at least temporarily, by surgical destruction of
parts of the thalamus, a structure deep in the brain that helps
control movement.  Speech disturbance is a special risk
accompanying this procedure, since the thalamus lies near brain
structures that help control speech.  Surgically cutting or
removing the nerves to the affected muscles has helped some focal
dystonias, including blepharospasm, spasmodic dysphonia and
torticollis.  The benefits of these operations, however, can be
short-lived.  They also carry the risk of disfigurement, can be
unpredictable, and are irreversible.

Some patients with spasmodic dysphonia may benefit from treatment
by a speech-language pathologist.  Physical therapy, splinting,
stress management, and biofeedback may also help individuals with
certain forms of dystonia.

What research is being done?

The ultimate goals of research are to find the cause(s) of the
dystonias so that they can be prevented, and to find ways to cure
or more effectively treat people now affected.  The National
Institute of Neurological Disorders and Stroke (NINDS), a unit of
the Federal government's National Institutes of Health (NIH), is
the agency with primary responsibility for brain and neuromuscular
research.  NINDS sponsors research on dystonia both in its
facilities at the NIH and through grants to medical centers
throughout the country.  Scientists working at the National
Institute on Deafness and Other Communication Disorders, also part
of the NIH, are studying improved treatments for speech and voice
disorders associated with dystonias.  Support for dystonia research
is also provided by privately funded organizations such as the
Dystonia Medical Research Foundation.

Scientists at the NINDS laboratories have conducted detailed
investigations of the pattern of muscle activity in persons with
focal dystonias.

One of the most important characteristics is the failure of
reciprocal inhibition, a normal process in which muscles with
opposite actions work without opposing each other.  In dystonia,
the tightening of muscles is associated with an abnormal pattern of
muscles fighting each other.  Studies at the NINDS have probed the
spinal reflex function and found abnormalities consistent with the
defect in reciprocal inhibition.  Other studies are probing brain
activity and its relation to these observations.

The search for the gene or genes responsible for some forms of
dominantly inherited dystonias has been narrowed to chromosome 9.
The eventual discovery of the actual gene(s) involved should lead
to a laboratory test to diagnose primary dystonia, the
identification of the abnormal gene product, and the rational
development of specific therapies or cures.  Scientists are also
reviewing data on populations thought to inherit the disease in
other ways.

Investigators are asking why the symptoms in primary dystonia vary
so widely in type and severity since such patients show no obvious
cell degenerations or changes in the basal ganglia.  Studies in
this area of the brain are complicated by the large number of
distinct but interrelated neurochemical systems.  Meanwhile,
scientists continue their search for therapies that may help treat
dystonic symptoms.  Investigators are seeking improved methods of
administering botulinum toxin and better surgical techniques to
treat symptoms.

Where can I get more information?

Five privately supported voluntary health agencies supply
information about the dystonias and their own activities and
services:

American Speech-Language-Hearing Association
10801 Rockville Pike
Rockville, Maryland 20852
[phone]
[phone] (toll free, except Alaska, Hawaii
andMaryland)

Benign Essential Blepharospasm Research
Foundation, Inc.
P.O. Box 12468
Beaumont, Tx 77726-2468
[phone]

Dystonia Medical Research Foundation
8383 Wilshire Boulevard, Suite 800
Beverly Hills, California 90210
[phone]

National Foundation for Jewish Genetic
Diseases, Inc.
250 Park Avenue, Suite 1000
New York, New York 10177
[phone]

National Spasmodic Torticollis Association, Inc.
P.O. Box 873
Royal Oak, Michigan 48068-0873
[phone]

For further information, contact:

Office of Scientific and Health Reports
National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, Maryland 20892
[phone]
July 1991

!
*
E. coli, ESCHERICHIA COLI Type O157:H7

CENTERS FOR DISEASE CONTROL AND PREVENTION
Date Last Rev'd: March 9, 1995

FOOD AND WATER BORNE BACTERIAL DISEASES


ESCHERICHIA COLI Type O157:H7
E. coli is a bacteria with many strains, most of which live normally in the
intestines of humans and animals without causing disease.  A newly recognized
strain, E. coli type O157:H7, was first reported to cause bloody diarrhea in
1982, and is now known to cause bloody and non-bloody diarrhea, both
accompanied by abdominal cramps.  The infection it causes can lead to the
hemolytic uremic syndrome (or HUS) (a blood and kidney illness) and
thrombotic thrombocytopenic purpura (or TTP) (a blood and kidney illness that
also affects the nervous system).

E. coli type O157:H7 infections have been linked to eating under-cooked
ground beef and drinking unpasteurized milk. The organism can also spread
easily from person to person, especially in day care centers and nursing
homes. Young children and the elderly are at increased risk for the severe
complications of this infection, occasionally resulting in death.

Dairy cattle appear to be a major reservoir for this type of E. coli.

Diagnosis is usually made by culturing the stool. In many clinical
laboratories, testing for this pathogen is not done routinely.  Requests for
culture should specify using MacConkey-sorbitol agar.  E. coli O157:H7
ferments sorbitol slowly and appears sorbitol negative at 24 hours.

To prevent infection with E. coli O157:H7, beef, especially ground beef,
should be cooked thoroughly and held thereafter at an appropriately hot or
cold temperature.   Undercooked beef should not be served to young children,
the elderly, and the immunocompromised.  No one should drink unpasteurized
milk.  When caring for an infected person, careful hygiene with frequent
handwashing is necessary to prevent person-to-person spread.

Treatment with antibiotics has not proven useful for this acute infection.
Therapy includes replacement of fluids and electrolytes. Hemolytic uremic
syndrome (or HUS) and thrombotic thrombocytopenic purpura (or TTP) require
hospitalization and supportive care.

Due to the relatively recent recognition of E. coli, few statistics are
available on its frequency. At this time it is not reported through the
public health surveillance system, however some states are beginning to
collect data. The data suggest that infections with the organism are
increasing.
!
*
10 Tips To Healthy Eating
April 1994
Cosponsored by:

The American Dietetic Association

---------------------------------------------------------------------------

Experts agree the key to healthy eating is the time-tested advice of

balance, variety and moderation. In short, that means eating a wide variety

of foods without getting too many calories or too much of any one nutrient.

These 10 tips can help you follow that advice while still enjoying the

foods you eat.



  1.  Eat a variety of nutrient-rich foods. You need more than 40 different

     nutrients for good health, and no single food supplies them all. Your

     daily food selection should include bread and other whole-grain

     products; fruits; vegetables; dairy products; and meat, poultry, fish

     and other protein foods. How much you should eat depends on your

     calorie needs. Use the Food Guide Pyramid and the Nutrition Facts

     panel on food labels as handy references.



  2. Enjoy plenty of whole grains, fruits and vegetables. Surveys show most

     Americans don't eat enough of these foods. Do you eat 6-11 servings

     from the bread, rice, cereal and pasta group, 3 of which should be

     whole grains? Do you eat 2-4 servings of fruit and 3-5 servings of

     vegetables? If you don't enjoy some of these at first, give them

     another chance. Look through cookbooks for tasty ways to prepare

     unfamiliar foods.



  3.  Maintain a healthy weight. The weight that's right for you depends on

     many factors including your sex, height, age and heredity. Excess body

     fat increases your chances for high blood pressure, heart disease,

     stroke, diabetes, some types of cancer and other illnesses. But being

     too thin can increase your risk for osteoporosis, menstrual

     irregularities and other health problems. If you're constantly losing

     and regaining weight, a registered dietitian can help you develop

     sensible eating habits for successful weight management. Regular

     exercise is also important to maintaining a healthy weight.



  4. Eat moderate portions. If you keep portion sizes reasonable, it's

     easier to eat the foods you want and stay healthy. Did you know the

     recommended serving of cooked meat is 3 ounces, similar in size to a

     deck of playing cards? A medium piece of fruit is 1 serving and a cup

     of pasta equals 2 servings. A pint of ice cream contains 4 servings.

     Refer to the Food Guide Pyramid for information on recommended serving

     sizes.



  5. Eat regular meals. Skipping meals can lead to out-of-control hunger,

     often resulting in overeating. When you're very hungry, it's also

     tempting to forget about good nutrition. Snacking between meals can

     help curb hunger, but don't eat so much that your snack becomes an

     entire meal.



  6.  Reduce, don't eliminate certain foods. Most people eat for pleasure

     as well as nutrition. If your favorite foods are high in fat, salt or

     sugar, the key is moderating how much of these foods you eat and how

     often you eat them.



     Identify major sources of these ingredients in your diet and make

     changes, if necessary. Adults who eat high-fat meats or whole-milk

     dairy products at every meal are probably eating too much fat. Use the

     Nutrition Facts panel on the food label to help balance your choices.



     Choosing skim or low-fat dairy products and lean cuts of meat such as

     flank steak and beef round can reduce fat intake significantly.



     If you love fried chicken, however, you don't have to give it up. Just

     eat it less often. When dining out, share it with a friend, ask for a

     take-home bag or a smaller portion.



  7. Balance your food choices over time. Not every food has to be

     "perfect." When eating a food high in fat, salt or sugar, select other

     foods that are low in these ingredients. If you miss out on any food

     group one day, make up for it the next. Your food choices over several

     days should fit together into a healthy pattern.



  8.  Know your diet pitfalls. To improve your eating habits, you first

     have to know what's wrong with them. Write down everything you eat for

     three days. Then check your list according to the rest of these tips.

     Do you add a lot of butter, creamy sauces or salad dressings? Rather

     than eliminating these foods, just cut back your portions. Are you

     getting enough fruits and vegetables? If not, you may be missing out

     on vital nutrients.



  9.  Make changes gradually. Just as there are no "superfoods" or easy

     answers to a healthy diet, don't expect to totally revamp your eating

     habits overnight. Changing too much, too fast can get in the way of

     success. Begin to remedy excesses or deficiencies with modest changes

     that can add up to positive, lifelong eating habits. For instance, if

     you don't like the taste of skim milk, try low-fat. Eventually you may

     find you like skim, too.



 10.  Remember, foods are not good or bad. Select foods based on your total

     eating patterns, not whether any individual food is "good" or "bad."

     Don't feel guilty if you love foods such as apple pie, potato chips,

     candy bars or ice cream. Eat them in moderation, and choose other

     foods to provide the balance and variety that are vital to good

     health.



---------------------------------------------------------------------------



Figuring Out Fat



With so much information available about the effects of dietary fat on

health, understanding the role fat plays in a well-balanced diet can be

pretty confusing. To cut through the confusion, it's important to remember

that fat is an essential nutrient that everyone needs to stay healthy.



Fat is a valuable energy source and carries fat-soluble vitamins needed for

proper growth and development. It also contributes important taste and

textural qualities that are part of enjoying food.



Too much fat, however, can increase the risk of heart disease, obesity and

other health problems. When moderating fat intake, it's important to

consider these points:



   *  Health authorities recommend Americans consume 30 percent or less of

     their total daily calories from fat, with 10 percent or less of those

     calories from saturated fat. Remember, the 30 percent refers to your

     total fat intake over time, not single foods or meals. Use the

     following chart to guide your fat intake.



     If you eat this           Total fat     Total saturated

     number of calories         per day        fat per day

     per day:                   (grams)           (grams)



        1,600                 53 or less         18 or less

        2,000                 65 or less         20 or less

        2,200                 73 or less         24 or less

        2,500                 80 or less         25 or less



   *  Use the Nutrition Facts panel on the food label to help determine how

     much fat is in foods. Remember, it's the total fat intake over time

     that's important. A food high in fat can be part of a healthy diet as

     long as it's balanced with other lower-fat food choices.



   *  All fats are a combination of saturated, polyunsaturated and

     monounsaturated fatty acids. Each of these types of fats have

     different effects on the body, but all contain nine calories per gram.



   *  Blood cholesterol levels are influenced by family history, weight,

     age, smoking, physical activity and eating habits. Studies have shown

     that diets which are too high in certain saturated fatty acids and

     dietary cholesterol can raise blood cholesterol.



---------------------------------------------------------------------------



The Food Guide Pyramid



The Food Guide Pyramid is a practical tool to help you make food choices

that are consistent with the Dietary Guidelines for Americans. Using the

Pyramid enables you to eat a variety of foods daily so that you can get the

nutrients you need.



To make the most of the Pyramid, you need to know what counts as a serving.



     Food Group--Serving Size



   * Bread--1 slice bread, 1\2 bagel or English muffin, 1 ounce

     ready-to-eat cereal, 1\2 cup cooked cereal, rice or pasta, or 5-6

     small crackers



   * Vegetable--1 cup raw, leafy vegetables, 1\2 cup cooked or chopped raw

     vegetables or 3\4 cup vegetable juice



   * Fruit--1 medium piece of fruit, 1\2 cup mixed fruit or 3\4 cup fruit

     juice



   * Milk--1 cup milk or yogurt, 11\2 ounces natural cheese or 2 ounces

     process cheese



   * Meat--2 - 3 ounces cooked lean meat, poultry or fish (about the size

     of a deck of cards)



   * Other foods which count as 1 ounce meat: 1\2 cup cooked dry beans, 1

     egg, 2 tablespoons peanut butter or 1\3 cup nuts



---------------------------------------------------------------------------



If You Want To Know More



A nutrition expert can help you develop a personal action plan for

improving your eating habits while keeping the fun in food. A registered

dietitian (R.D.) is an authority on food, nutrition and health, and can

provide valuable information and advice.



To locate a registered dietitian in your area, ask your physician, or call

the consumer nutrition hot line (800/366-1655) of the National Center for

Nutrition and Dietetics, the public education center of The American

Dietetic Association. Registered dietitians are available to answer your

food and nutrition questions Monday through Friday from 9 a.m. to 4 p.m.

central time. In addition, you can listen to nutrition messages in English

and Spanish, Monday through Friday from 8 a.m. to 8 p.m. central time.

---------------------------------------------------------------------------

The American Dietetic Association

National Center for Nutrition and Dietetics

216 West Jackson Boulevard

Chicago, IL 60606-6995



International Food Information Council Foundation

1100 Connecticut Avenue, N.W.

Suite 430

Washington, DC 20036

---------------------------------------------------------------------------



Reprinted from the International Food Information Council Foundation and

the American Dietetic Association, 1994

!
*


                               EMPHYSEMA

           In emphysema, the lungs become less and less
           efficient because of damage to some of the
           millions of alveoli, or air-sacs, at the ends of
           the bronchioles   in the lungs.  It is in the
           alveoli that oxygen and carbon dioxide exchange
           takes place.  Healthy lungs have an elastic
           spongy texture, so they contract and expand
           fully.  If the alveoli become stretched or
           rupture, the elasticity of the lungs is
           gradually destroyed.  This type of damage occurs
           when the alveoli are constantly subjected to
           higher pressure than normal.  This happens to
           people who have long-standing lung disease.

           Chronic bronchitis or asthma, for example,
           cause narrowing of the lung airways.  The
           labored, forceful breathing that results
           strains, weakens and may ultimately damage the
           alveoli.

           WHAT ARE THE SYMPTOMS?

           The main symptom of emphysema is shortness of
           breath, which is likely to become gradually
           worse over a period of years.  If you have
           emphysema, your chest is probably distended
           into a barrel-like shape.  The name of the
           disease comes from the Greek word for
           inflation.  If you also wheeze, cough, and
           bring up phlegm, these are symptoms of other
           kinds of lung trouble, not of emphysema.

           WHAT ARE THE RISKS?

           Emphysema usually occurs in people who have
           bronchitis or asthma.  It is much more common
           in men than in women, and your chances of
           having it increase if you smoke and/or live in
           an area where the air is polluted.  Some people
           are particularly susceptible to emphysema
           because of an inherited defect in the chemical
           make-up of their lungs.  If your job requires
           exceptionally forceful use of lung power, you
           may also be highly susceptible.  Some examples
           of such professions are glass-blowing and
           playing a wind musical instrument.  If you have
           increasing shortness of breath, you risk death
           from eventual respiratory failure.  Emphysema
           also makes you more susceptible to chest
           infections such as pneumonia that can be
           life-threatening.

           There is also a risk of a pneumothorax (lung
           collapse).  In addition, since blood cannot
           flow freely through damaged alveoli, the
           resulting strain on the right side of the
           heart, which pumps blood to the lungs, can lead
           to heart failure.

           WHAT SHOULD BE DONE?

           If you are troubled by breathlessness, you
           should consult your physician.  In the initial
           examination, the physician will probably
           percuss, or finger-tap, your chest, and listen
           to it with a stethoscope.  The doctor may also
           ask you to have a chest X-ray, and to blow hard
           into a peak-flow meter, a machine that measures
           your breathing capacity.  Special breathing
           tests called pulmonary function tests may also
           be necessary.  Because emphysema is usually
           associated with other lung disorders, it is not
           an easy disease to diagnose independently.

           WHAT IS THE TREATMENT?

           If you smoke, stop.  Avoid places with polluted
           air.  Keep away from people who have coughs or
           colds.  Exercise moderately but regularly in
           fresh, clean air.

           Physicians can relieve the symptoms and delay
           the progress of emphysema, but they cannot cure
           it.  If you have bronchitis along with
           emphysema, you may be told to inhale
           bronchodilator drugs, which widen the airways
           and help prevent further damage to the alveoli.
           Since bronchitis and lung infections of any
           kind aggravate emphysema, the best way to help
           control the disease is to prevent respiratory
           infection.  Thus your physician may prescribe
           antibiotics as a preventive measure.

!
*
General Information about EPILEPSY

Fact Sheet Number 6 (FS6), 1993
______________________________________________________________________________

NICHCY
National Information Center for Children and Youth with Disabilities
P.O. Box 1492
Washington, DC 20013
[phone] (Local)
[phone] (TT)
1-800-999-5599 (Toll-free)
______________________________________________________________________________

DEFINITION OF EPILEPSY

        According to the Epilepsy Foundation of America, epilepsy is a
        physical condition that occurs when there is a sudden, brief change
        in how the brain works.  When brain cells are not working properly,
        a person' consciousness, movement, or actions may be altered for a
        short time.  These physical changes are called epileptic seizures.
        Epilepsy is therefore sometimes called a seizure disorder.  Epilepsy
        affects people in all nations and of all races.

        Some people can experience a seizure and not have epilepsy.  For
        example, many young children have convulsions from fevers.  These
        febrile convulsions are one type of seizure.  Other types of seizures
        not classified as epilepsy include those caused by an imbalance of
        body fluids or chemicals or by alcohol or drug withdrawal.  A single
        seizure does not mean that the person has epilepsy.

INCIDENCE

        About two million Americans have epilepsy; of the 125,000 new cases
        that develop each year, up to 50% are in children and adolescents.

CHARACTERISTICS

        Although the symptoms listed below are not necessarily indicators of
        epilepsy, it is wise to  consult a doctor if you or a member of your
        family experiences one or more of them:

        -       "Blackouts" or periods of confused memory;
        -       Episodes of staring or unexplained periods of
                unresponsiveness;
        -       Involuntary movement of arms and legs;
        -       "Fainting spells" with incontinence or followed by excessive
                fatigue; or
        -       Odd sounds, distorted perceptions, episodic feelings of fear
                that cannot be explained.

        Seizures can be generalized, meaning that all brain cells are
        involved.  One type of generalized seizure consists of a convulsion
        with a complete loss of consciousness.  Another type looks like a
        brief period of fixed staring.

        Seizures are partial when those brain cells not working properly are
        limited to one part of the brain.  Such partial seizures may cause
        periods of ╥automatic behavior╙ and altered consciousness.  This is
        typified by purposeful- looking behavior, such as buttoning or
        unbuttoning a shirt. Such behavior, however, is unconscious, may be
        repetitive, and is usually not recalled.

EDUCATIONAL IMPLICATIONS

        Students with epilepsy or seizure disorders are eligible for special
        education and related services under the Individuals with
        Disabilities Education Act (IDEA), formerly the Education of the
        Handicapped Act (Public Law 94-142).  Epilepsy is classified as
        "other health impaired" and an Individualized Education Program (IEP)
        would be developed to specify appropriate services.  Some students
        may have additional conditions such as learning disabilities along
        with the seizure disorders.

        Seizures may interfere with the child's ability to learn.  If the
        student has the type of seizure characterized by a brief period of
        fixed staring, he or she may be missing parts of what the teacher is
        saying.  It is important that the teacher observe and document these
        episodes and report them promptly to parents and to school nurses.

        Depending on the type of seizure or how often they occur, some
        children may need additional assistance to help them keep up with
        classmates.  Assistance can include adaptations in classroom
        instruction, first aid instruction on seizure management to the
        student's teachers, and counseling, all of which should be written
        in the IEP.

        It is important that the teachers and school staff be informed about
        the child's condition, possible effects of medication, and what to
        do in case a seizure occurs at school. Most parents find that a
        friendly conversation with the teacher(s) at the beginning of the
        school year is the best way to handle the situation.  Even if a child
        has seizures that are largely controlled by medication, it is still
        best to notify the school staff about the condition.

        School personnel and the family should work together to monitor the
        effectiveness of medication as well as any side effects.  If a
        child's physical or intellectual skills seem to change, it is
        important to tell the doctor.  There may also be associated hearing
        or perception problems caused by the brain changes.  Written
        observations of both the family and school staff will be helpful in
        discussions with the child's doctor.


        Children and youth with epilepsy must also deal with the
        psychological and social aspects of the condition.  These include
        public misperceptions and fear of seizures, uncertain occurrence,
        loss of self control during the seizure episode, and compliance with
        medications. To help children feel more confident about themselves
        and accept their epilepsy, the school can assist by providing
        epilepsy education programs for staff and students, including
        information on seizure recognition and first aid.

        Students can benefit the most when both the family and school are
        working together. There are many materials available for families
        and teachers so that they can understand how to work most effectively
        as a team.

RESOURCES

Epilepsy Foundation of America.  (1987).  Epilepsy:  You and Your Child,
Information for Parents.  Landover, MD: Epilepsy Foundation of America.
(Telephone: [phone] for publications.)

Freeman, John M., Vining, Eileen, Pillas, Diana J. (1993). Seizures and
Epilepsy in Childhood: A Guide for Parents (rev. ed.). Baltimore, MD: The
Johns Hopkins University Press. (Telephone: [phone].)

Kobrin, Eileen R. (1991). Issues and Answers: A Guide for Parents of Teens
and Young Adults with Epilepsy.  Landover, MD: Epilepsy Foundation of America.
(Telephone: [phone] for publications.)

Reisner, H.  (Ed.). (1988).  Children with Epilepsy: A Parent's Guide.
Kensington, MD: Woodbine House. (Telephone: [phone] (in D.C.);
800-843-7323.)


ORGANIZATIONS

Epilepsy Foundation of America (EFA)
4351 Garden City Drive, Suite 406
Landover, MD  20785
[phone]; (800) EFA-1000 (Toll Free)
[phone] for publications

National Institute of Neurological Disorders and Stroke (NINDS)
National Institutes of Health
Building 31, Room 8A06
9000 Rockville Pike
Bethesda, MD  20892
[phone]
!
*
How to Choose a Doctor and Hospital

àIf You Have Epilepsy That Is Difficult to Control

Hospitals and doctors are not all alikeà

àthey vary in quality due to differences in their training, experience and
services. These differences in quality become greater and matter more when
you need sophisticated medical care for a complex condition.

A surgeon, for example, who performs a complex procedure often, has better
success with it than a surgeon who does the same procedure only
occasionally. The complication rate and the death rate for the same
procedure may be many times higher at one hospital than another.

Clearly, the doctor and hospital that you choose have a direct impact on
how well you do ù especially when you need treatment for a condition such
as epilepsy, a chronic recurrence of seizures due to brain abnormalities.
Epilepsy is not rare. It affects nearly one in every 100 Americans, or more
than 2 million of us.

Seizures can be controlled with medications in about 75 percent of people.
When epilepsy can't be controlled with medications it is called
"intractable." This means that disabling seizures continue to occur even
though the patient has received the maximum medical treatment. Intractable
epilepsy may be helped by surgery. In the past 10 years, major advances
have been made in the accuracy of diagnosing epilepsy, in drug and surgical
treatment, and in understanding the daily problems people with epilepsy
face. As a result of advances in surgery, each year 2,000 to 5,000 new
patients could benefit from surgery, whereas only 500 people are helped
currently.

But taking advantage of these advances means making some difficult and
important decisions such as choosing a doctor and a hospital. No one has
more at stake than you; it's one of the most important decisions of your
life.

This guide deals with difficult-to-control epilepsy. You may be reading it
because you or a family member have just been told you have epilepsy,
because your epilepsy is not being controlled by medications, or because
you are considering epilepsy surgery. By making the comparisons we talk
about, you will be prepared should the need for surgery arise.

How Do You Judge Quality?

Most of us do more research when we buy a car or television set than when
we choose a doctor and hospital. That may be because we don't know what
questions to ask or what to base our evaluation on. There is no consumer
magazine that rates doctors and hospitals the way Consumer Reports rates
air conditioners.

There are many different ways to measure quality care, and there is no
universal agreement on which should be used. However, at The Cleveland
Clinic Foundation, we believe that you can use the following six points, or
quality indicators, to compare health care providers:

-Credentials

-Experience

-Range of services

-Participation in research and education

-Patient satisfaction

-Outcome

Choosing a doctor or hospital is often influenced by values. You may want a
hospital that is close to home. You may want a hospital with a specific
religious affiliation. But when you need specialized medical care for
difficult-to-control epilepsy, it is essential that you also include in
your decision a doctor's qualifications and a hospital's track record.
These quality indicators will help you with that kind of evaluation should
you require surgery for epilepsy.

Sidebar

A Step-by-Step Guide

This guide helps you choose a doctor and hospital by:

-explaining intractable epilepsy and how it's diagnosed

-describing treatment for difficult-to-control epilepsy ù
seizure-preventing drug therapy, monitoring and epilepsy surgery;

-describing how patients are monitored and evaluated for surgery

-explaining six points that indicate quality; and

-providing questions and answers from The Cleveland Clinic Foundation that
you can use to compare doctors and hospitals.

Epilepsy

Epilepsy is a neurological disease, a disease of the central nervous
system. Seizures occur when discharges of electrical energy from brain
cells become uncontrolled. A sudden medical problem can trigger a seizure,
but this doesn't mean the diagnosis is epilepsy. Epilepsy involves seizures
that continue to occur.

Seizures that involve all of the brain are called generalized. Those that
are limited to one part of the brain are called partial, or focal,
seizures. This guide deals mainly with the most common form of focal
seizure ù the anterior temporal lobe seizure.

It's important for people with epilepsy to be treated at the appropriate
level of care, according to the National Association of Epilepsy Centers
(NAEC). Patients whose epilepsy is hard to control should be referred to
specialized centers, according to the NAEC, while those whose epilepsy can
be more easily controlled ordinarily need not be referred to such centers.

If you have a seizure, your family doctor may start treatment. If seizures
can't be controlled within three months, "a referral to a general
neurologist is indicated," according to the NAEC. If the seizures can't be
controlled within nine months after that, a referral to a specialty
epilepsy center is the next step. And if a person is likely to need
epilepsy surgery, the NAEC recommends referral to a fourth-level surgical
epilepsy center. See the box for more information.

Sidebar

What Is a Fourth-Level Surgical Epilepsy Center?

This is the highest level of specialty epilepsy center described by the
NAEC.

At this level, the center should be capable of conducting complete
preoperative evaluation and monitoring. Its staff should have the expertise
to perform a broad range of surgical procedures for epilepsy.

How is it treated?

Evaluation. A thorough evaluation to find the cause of seizures or localize
their source includes a detailed medical history as well as a complete
physical and neurological examination. In addition, most patients require
further testing such as blood chemistry and urine tests;
electroencephalograms (EEGs); and skull x-rays and brain scans such as
computerized tomography (CT scans), magnetic resonance imaging (MRI) and
positron emission tomography (PET) scans.

Medication. Most seizures can be controlled by medication. But
anti-convulsive drug therapy is individualized. The drug selected depends
upon the type of seizure being treated. Even in patients with the same
seizure type, the effective dose will vary with age, weight, sex and other
factors. The majority of people may be treated with a single drug. However,
if that fails, various drug combinations may be tried. The process of
finding the most effective drug, drug dose, or drug combination can take
weeks or months. It also involves taking systematic measurements to monitor
the amount of drugs present in the blood.

When seizures can't be controlled by drugs, surgery is considered. In the
past, it could take more than 10 years to determine that drugs couldn't
control seizures. That now can be determined within two years.

Monitoring for Surgery. Patients must be tested and monitored to determine
if surgery is the right choice. This involves making sure that the seizures
really are caused by epilepsy and that the seizures can't be controlled ù
even by drugs given in the highest possible doses. Finally, intensive
monitoring is essential in determining the location of the seizures, which
dictates the type of surgery necessary.

This process generally begins with prolonged video-EEG and monitoring. With
electrodes attached to the scalp, EEG equipment and video cameras are used
to record seizures and monitor the brain's activity. If doctors can find
the seizure source using this technique, surgery is considered. If more
information is needed, the brain itself is monitored. This involves placing
electrodes or grids inside the skull over a specific region of the brain.

Testing also includes the intracarotid amobarbital test (Wada Test), which
is used prior to surgery to delineate areas of the brain that control
speech and memory function.

Surgery. Different types of seizures require different surgeries.

-Anterior temporal lobe resections: Patients who have focal seizures
originating in the anterior temporal lobe can benefit the most from
surgery. That is because their seizures are restricted to an area of the
brain that can be removed without damaging vital functions such as speech,
memory and movement. The operation that is performed is called resection,
or removal, of a specific area, or section, of the brain. If seizures
originate in the anterior temporal lobe, the appropriate operation is
called anterior temporal lobe resection. Other areas of brain tissue can be
removed depending upon the seizure source.

-Corpus callosotomy is done to reduce the frequency of disabling seizures
for patients who have intractable generalized seizures which result in
frequent falls and injuries to the body.

-Hemispherectomy, which involves removing one of the brain's hemispheres,
is done to stop seizures for patients who have hemiplegia (paralysis of one
side of the body).

After surgery, some patients may be completely free of seizures; others may
find their seizures reduced by varying degrees. Some patients may have to
continue to take medication, but their seizures are better controlled.
Surgery may not be successful for some people, and a second operation may
be needed.

Controlling seizures is only the first step in treating the whole person.
Educational, social and psychological counseling are all part of the total
treatment plan.

Sidebar

How Can You Judge Monitoring Facilities?

The NAEC recommends that these facilities include the following:

-An inpatient recording suite with access to full resuscitative
capabilities.

-A dedicated unit with a nursing staff whose sole function is to care for
individuals with epilepsy. The unit's design and furnishings should
minimize risk of injury to patients subject to seizures and falls.

-On-site, 24-hour medical coverage.

-Availability of the full spectrum of imaging services on-site.

-Fourth-level surgical programs that perform monitoring of patients with
indwelling electrodes must ensure electrical safety and must meet the
American EEG Society's recommendations for intensive neuro-diagnostic
monitoring.

Where Do You Begin?

Measuring quality in ways that are useful to consumers is a new idea in
health care. Because of that, if may not be possible to get complete
information for each of these quality indicators. But when providers are
willing to give you as much information as possible, it's a good sign. It
shows that they are dedicated to maintaining and improving their quality,
responsive to patients, and confident of their capability.

If you are told that you have epilepsy, if your seizures cannot currently
be controlled, or if you may need epilepsy surgery, talk to your family
doctor or the neurologist who is treating you. Ask about having an epilepsy
specialist review your diagnosis and treatment plan. Epilepsy specialists
have training and experience in epilepsy treatment and devote a major
portion of their practice and research to epilepsy. Get the names of
several doctors and hospitals with the most experience. Ask the questions
we suggest. Make comparisons. Then make your decision. Be an informed
consumer for yourself and your family.

How to Use Quality Indicators

How can you use these indicators to judge if one doctor or hospital is
better for you than another? By combining information from more than one
quality indicator, according to a report "The Quality of Medical Care:
Information for Consumers" produced by the U.S. Congress, Office of
Technology Assessment.

The report states that patients about to have surgery can be confident if
the hospital performs a high number of relevant procedures, if it has a low
mortality (death) rate, and if the surgeon has extensive training and
experience in the procedure.

On the other hand, the report states: "...if a hospital had a high
mortality rate and a low volume of procedures, the patient might wish to
question the surgeon about that hospital and about alternatives, even if
other hospitals required longer travel."

1. Credentials

Do the doctor and hospital measure up?

Credentials have been set by nationally recognized medical professional
organizations to verify that doctors and hospitals meet certain standards
in the delivery of health care.

Doctors:

Board certification, or an international equivalent, is a sign that doctors
are highly trained in their field. Doctors who specialize should be board
certified in the specialty in which they are practicing. Each specialty has
a national board which is responsible for setting standards doctors must
meet in order to be certified. Doctors who are board certified in their
specialty have completed the amount of training that the specialty board
requires, have practiced for a specified number of years in that specialty,
and have passed a difficult examination in their specialty area. Some
excellent doctors are not board certified. Board certification, however, is
generally a good indication of competence and experience.

Doctors who specialize in epilepsy are board certified in the specialty of
neurology or neurosurgery.

Has the doctor been board certified in neurology?

All 8 Cleveland Clinic epilepsy specialists are board certified in
neurology by the American Board of Psychiatry and Neurology. All have
completed epilepsy and/or neurophysiology fellowships.

Our 6 child neurologists are board certified in neurology with special
qualification in child neurology by the American Board of Psychiatry and
Neurology. All are board certified in pediatrics by the American Board of
Pediatrics.

Does the doctor who monitors and interprets the EEG tracings have board
certification and special expertise in monitoring?

Seven of 8 Cleveland Clinic epilepsy specialists who monitor and interpret
EEG data are board certified in clinical neurophysiology by the American
Board of Clinical Neurophysiology.

Is the epilepsy surgeon board certified in neurological surgery?

All 4 epilepsy surgeons in the epilepsy program are board certified in
neurological surgery by the American Board of Neurological Surgery.

Hospitals:

The Joint Commission on Accreditation of Healthcare Organizations (JCAHO)
is the nationwide authority that surveys hospitals. The JCAHO decides
whether a hospital gets, keeps, or loses accreditation based on its meeting
certain criteria for staffing, equipment and facility safety requirements.
Although accreditation is voluntary, most hospitals go through the process.
If the hospital that you are considering is not accredited, it is important
to know why.

Although no organization exists to accredit epilepsy centers, the National
Association of Epilepsy Centers has recommended some guidelines for
services offered as well as staffing.

Hospitals that do measure up are often in the public spotlight for their
medical advances and the quality of their care. Information about a
hospital's reputation is available through the mass media, books such as
The Best Hospitals in America, the government, and consumer groups.

Sidebar

For information about a hospital's status, call the Joint Commission on
Accreditation of Healthcare Organizations at 708/916-5800.

Is the hospital accredited by the JCAHO?

Yes.

Does the facility meet guidelines for services and staff for fourth-level
specialized epilepsy centers as recommended by the National Association of
Epilepsy Centers?

Yes. The Cleveland Clinic's Epilepsy Center meets and exceeds the
guidelines for both fourth-level medical and fourth-level surgical epilepsy
centers. These include the categories of personnel (doctors, nurses,
technologists) that staff the program; their credentials; tests that should
be performed; and equipment that should be available for accurate
evaluation and monitoring.

Has the hospital been positively and consistently recognized for medical
excellence and leadership by the news media, consumer advocates and
government agencies?

Yes. Most recently, the Cleveland Clinic was recognized as one of the six
best neurology centers in America, based on the responses of 400 medical
specialists, in the August 5, 1991 issue of U.S. News & World Report. A
previous physician survey in the April 30, 1990 issue of the magazine also
recognized the Cleveland Clinic as a leader in neurology.

For two years in a row, those same issues of U.S. News have named the
Clinic one of "America's Best Hospitals." The Cleveland Clinic has also
been singled out for excellence in "The Best in Medicine: Where to Get the
Finest Health Care For You and Your Family" (Crown, 1990).

2. Experience

Does practice make perfect?

In the case of complex, specialized medical and surgical care for epilepsy,
the more experience the doctor and hospital have with the necessary
procedures, the better the results usually will be.

Board-certified neurologists and neurosurgeons gain experience by devoting
a major portion of their practice and research to epilepsy and often
receive further training when available. Neurologists may complete one- or
two-year fellowships in epilepsy. Expertise in epilepsy surgery comes from
the number, variety and complexity of cases neurosurgeons and epilepsy
centers perform over a period of years.

The small number of epilepsy centers that perform 75 or more resections a
year have developed expert, specialized teams that handle epilepsy surgery
on a routine basis, according to The National Association of Epilepsy
Centers (NAEC). Centers that perform 25 to 50 resections a year can be
quite expert as well.

"One procedure every other week appears to be the minimum number necessary
for a team to maintain its skills and do complex procedures on a routine
basis," according to NAEC guidelines.

However, many U.S. hospitals do only five to 10 of these surgeries a year.
And, while this low volume is to be expected in new programs, it is not
enough to maintain skills necessary for the proper surgical treatment of
patients with epilepsy.

It may be important to ask whether the doctor performs all procedures at
one hospital or several. If the doctor performs procedures at more than one
hospital, this increases the volume but means the doctor is working with
different teams. The teams, therefore, don't have as much experience
working together as they would if the doctor were working with the same
team at the same hospital all the time.

How many patients are treated at the hospital each year?

We provide follow-up care to more than 1,000 patients with epilepsy,
approximately half of whom are infants, children or adolescents.

How many patients undergo epilepsy monitoring at the facility yearly?

The Cleveland Clinic monitors approximately 300 patients per year.

How long has the hospital been performing epilepsy surgery?

Our program began in 1978. Since that time, we have performed more than 400
epilepsy surgeries.

How many epilepsy surgeries are performed each year?

The Cleveland Clinic currently performs more than 100 epilepsy surgeries
yearly. Approximately one-third of these surgeries are done to help
infants, children or adolescents who have intractable epilepsy.

How many of these surgeries are resections?

The Cleveland Clinic performs approximately 75 resections each year.

Were all procedures performed by the doctor at one hospital or several?

Our physicians perform all procedures exclusively at the Cleveland Clinic.

Have the doctors established a special expertise in epilepsy and epilepsy
surgery?

Our neurologists and neurosurgeons have developed special expertise in
epilepsy by devoting a major portion of their training, practice and
research to this field.

Sidebar

Volume Guidelines:

Some organizations have suggested volume guidelines for the number of times
a procedure should be performed in order for it to be done competently.

-Surgical procedures for epilepsy should only be performed in centers doing
at least 25 resective procedures for epilepsy a year, according to the
National Association of Epilepsy Centers.

3. Range of Services

What services are available?

Hospitals with a broad range of services can treat more complex medical
conditions and better handle complications that may occur. If complications
arise, you want the best care available, and you want it immediately.

Range of specialty departments:

Medical conditions don't always exist in isolation. Related conditions
could endanger the health of other organs. Therefore, immediate access to a
full range of specialty departments within a facility is critical.

Range of diagnostic and treatment options:

Surgery for epilepsy is not the only treatment option. Even when surgery is
required, it usually follows a long period of medical treatment. It's
important, therefore, to go to a facility that can treat epilepsy in a
variety of ways. That way you will get the most effective, appropriate and
cost-effective treatment available.

Fourth-level epilepsy centers, as defined by the NAEC, are facilities that
provide comprehensive diagnostic and treatment services designed for
patients with intractable seizures. These centers may have separate medical
and surgical programs or one combined medical and surgical program.

A fourth-level surgical epilepsy center, according to the NAEC, should be
able to do complete surgical evaluations and should have staff with the
expertise to perform a broad range of epilepsy surgeries.

The range of electrodiagnostic, surgical and imaging services that should
be provided, according to the NAEC, are presented in the box* to the right.

Is help available from a full range of specialty departments should
complications arise due to related conditions?

The Cleveland Clinic's 500 physicians ù who are all on staff full-time ù
provide care in 100 specialties and subspecialties. Because all of these
specialties, including pediatrics, are represented at one facility, prompt
consultation, diagnosis and treatment are available.

Does the hospital offer a wide variety of options for diagnosing and
treating epilepsy?

The Cleveland Clinic' s combined medical and surgical epilepsy program
meets and exceeds the requirements of a fourth-level epilepsy surgery
center suggested by NAEC in the box* to the left. The Cleveland Clinic's
comprehensive range of services includes:

-A testing site for experimental anti-convulsants; comprehensive programs
for monitoring blood-drug levels

-On-site radiology and nuclear medicine facilities with the full range of
imaging services for epilepsy, including PET, SPECT, computerized
tomography (CT), magnetic resonance imaging (MRI) and angiography of the
brain

-Two, four-bed epilepsy and seizure monitoring units

-The full range of electrodiagnostic services, including 24-hour video EEG
monitoring with surface and sphenoidal electrodes

-Invasive 24-hour recording with three different types of invasive
electrodes

-Intracarotid amobarbital test (Wada Test)

-A wide range of epilepsy surgeries: anterior temporal lobe resection;
cortical resections of extra-temporal areas of brain tissue; corpus
callosotomy; hemispherectomy; and the use of stereotactic and microsurgical
techniques

-Awake surgery

-A nursing staff whose sole function is to care for people with epilepsy

-Psychological, rehabilitation and social services

Sidebar

Fourth-Level Epilepsy Center Services*

Electrodiagnostic

-24-hour video/EEG with surface and sphenoidal electrodes

-Invasive 24-hour recording with subdural electrodes, depth electrodes

-Intracarotid amobarbital (Wada) testing

-Functional cortical mapping utilizing subdural electrodes or
intraoperative stimulation

-Evoked potential recording, capable of being used safely with implanted
electrodes

-Electrocorticography

Epilepsy surgery

-Emergency neurosurgery

-Complication management

-Open biopsy

-Stereotactic biopsy

-Lesional excision

-Intracranial electrodes and cortical resection

-Corpus callosotomy

-Cortical resection, including hemispherectomy

Imaging

-Magnetic resonance imaging

-Computerized axial tomography

-Cerebral angiography

Pharmacological expertise

-Quality-assured anti-epileptic drug levels

-24-hour anti-epileptic drug level service

-Pharmacokinetic consultative services

4. Participation in Research and Education

What type of hospital is it?

There are many advantages to selecting a hospital that combines patient
care with research and education.

Ideally, the individuals engaged in patient care, research and teaching are
organized around a given disease or class of patients, facilitating the
sharing of knowledge, research and clinical findings. This approach results
in the most rapid transfer of basic scientific knowledge from the
laboratory to care delivered at the patient's bedside.

Those individuals on the staff of such a hospital are exposed to an
important interchange of ideas. They are also exposed to the newest
treatments and forms of technology. At teaching hospitals, physicians are
available 24 hours a day.

There may be other advantages to choosing a teaching hospital. Private,
not-for-profit, teaching hospitals had lower mortality rates than other
types of hospitals, a study in the December 1989 issue of the New England
Journal of Medicine suggested.

Sidebar

Private, not-for-profit, teaching hospitals had lower mortality rates than
other types of hospitalsà

Is the hospital associated with a teaching program?

Yes. Incorporated in 1935, The Cleveland Clinic Educational Foundation
sponsors one of the nation's largest physician postgraduate training
programs.

Does the hospital conduct research or clinical trials related to epilepsy?

Basic research in the laboratory is being conducted into the causes of
epilepsy.

Epilepsy Monitoring Unit staff are researching the fundamental anatomy and
mechanisms that cause epilepsy, using data from long-term implantation of
subdural electrodes in epilepsy patients.

Clinical research is being conducted into improving and individualizing
intensive presurgical monitoring, modifying surgical techniques, memory
function in patients before and after surgery, brain tumors and epilepsy,
and epilepsy seizure classification.

Research into the natural history of how and why seizures recur following
epilepsy surgery is ongoing.

The effectiveness of experimental anti-convulsants along with various drug
combinations is being studied. This gives patients, whose seizures the new
drugs are meant to control, access to drugs that will not be available to
the public for many years.

Cleveland Clinic neurologists are studying ways to streamline and
accelerate epilepsy therapy to allow patients û adults and children û to
change drug regimens in only five days instead of the usual weeks to
months.

Does the hospital have residency training programs in neurology and
neurosurgery?

The departments of Neurology and Neurological Surgery train five to 10
fellows each year in one- and two-year fellowship programs and five
residents each year who are interested in advanced work in epilepsy and
clinical neurophysiology. The Cleveland Clinic offers an EEG training
program.

5. Patient Satisfaction

Is everybody happy?

If you ask one person about his or her experience with a doctor or
hospital, you get one person's point of view. Patient satisfaction surveys
allow you to judge quality based on the experience of many previous
patients. This provides you with a more objective measure to use.

Most hospitals routinely use surveys to learn if patients are satisfied
with their medical experience. They can use these results to improve their
services.

Patient satisfaction often reflects the personal side of care. Surveys ask
questions such as, How willing are the doctors and nurses to listen? Do
they answer questions and explain treatments? How much time does the doctor
spend with the patient? Is the hospital clean? Is the food good?

Patient satisfaction information can predict what your experience in a
particular hospital is likely to be.

How satisfied are hospitalized patients with their experience at this
facility?

87% of patients who are hospitalized at the Cleveland Clinic for a
neurological condition such as epilepsy are satisfied with their hospital
care.

94% say they would return.

How satisfied are outpatients with their experience at this facility?

91% of patients with a neurological problem such as epilepsy who come to
the Cleveland Clinic for outpatient services are satisfied with their
experience.

90% say they would return.

Is there a program to help patients and their families with the
difficulties that may arise during a hospital stay?

Cleveland Clinic Foundation patients may call an ombudsman û another name
for a patient-relations representative û if they have concerns about their
care.

Patients in the Cleveland Clinic hospital may dial a 24-hour Helpline from
their hospital room if they have any problems, questions, suggestions or
concerns related to service.

6. Outcome Indicators

How did the patient do?

Many outcome indicators can be used to measure the success of treatment.
The importance of each varies with the treatment. For procedures used to
treat life-threatening conditions, death rates and complication rates are
important.

For epilepsy surgery, however, death and complications are uncommon. The
way outcome is measured for epilepsy surgery û including anterior temporal
lobe surgery which this guide emphasizes û is in terms of the operation's
impact on seizure control.

Successful epilepsy surgery will result in eliminating or significantly
reducing the number of seizures. Seizure-free patients may still experience
auras û warning feelings that may occur before a seizure û and take
medication. However, while seizures couldn't be controlled with drugs
before surgery, they can be controlled after surgery.

Although success can't be guaranteed, especially for people who are
seriously ill, chances for a good outcome are increased by choosing a
hospital with good outcomes for the treatment that you are undergoing. If
you can't get information about outcome from a doctor or hospital, look at
alternatives. Try to compare results for patients like yourself to learn
what your risks or chances of success really are.

However, comparing epilepsy centers on the basis of outcomes is not easy.
Centers define improvement differently (see the box on the next page).
You're comparing apples and oranges when one hospital defines success as
reducing seizures by 90 percent or more and another defines success as
reducing seizures by 75 percent or more.

Seizure patterns change over time. Some people are seizure-free right after
surgery and then have a seizure. Others have seizures after surgery that
decrease in frequency and eventually stop. This means success rates change
depending on whether they are reported at six months or two years.

Success also depends on the type of patient you are. The more localized, or
confined, the source of your seizure, the better the chances that surgery
can make you seizure-free. In general, following anterior temporal
lobectomy, 60 percent of people are seizure-free and 80 percent show
worthwhile improvement. This is according to outcomes reported by our
center and other large centers. Specific outcome information is presented
on the opposite page.

What is the hospital's success rate for anterior temporal lobe resection?

Overall, 70 percent of patients continued to be seizure- free or to have
the frequency of their seizures reduced by 90 percent or more one year
after surgery. These results are from our most recently published study in
the Journal of Epilepsy.

Within what range can you generally expect success rates for anterior
temporal resection to be?

Anterior temporal resection has resulted in success rates of 55 to 70
percent, according to a National Institutes of Health Consensus Development
Conference on Surgery for Epilepsy held in 1990. Success by this panel was
defined as no seizures for five years after surgery, although some auras
may still be present and some patients may still be taking medication.

Sidebar

How Are Outcomes Reported?

Outcomes are reported by the degree to which seizures are reduced following
surgery.

Results are expressed by first telling what percentage of patients were
seizure-free.

Next, there is a group of patients who have a significant reduction in the
number of their seizures. This is where centers differ in the way they
classify successful outcomes:

-Some define success as reducing seizures by 90 percent or more.

-Others define success as reducing seizures by 75 percent or more.

A center's results will be higher if it defines success as reducing
seizures by 75 percent or more. Its results will be lower if it defines
success as reducing seizures by 90 percent or more.

At the Cleveland Clinic, we consider surgery successful when it makes a
patient seizure-free or reduces that patient's seizures by 90 percent or
more.

The Epilepsy Program

The Cleveland Clinic's epilepsy team is a national and international
pacesetter in treating epilepsy, both medically and surgically. The
Cleveland Clinic is one of only a few U.S. medical centers with the
technology and experience necessary to map the brain's electrical activity,
localize the seizure source, and then, if advisable, remove it.

-The sections of Epilepsy and Sleep Disorders are staffed by nine
physicians, including child neurologists, adult neurologists and a
psychiatrist. Approximately 25 technologists and five nurses are involved
in patient care activities. Working closely with this group are specialists
from neurosurgery, psychiatry, nuclear medicine, radiology and
neuropathology.

-The Section of Epilepsy Surgery includes four staff surgeons. These
surgeons perform more than 150 epilepsy surgeries of all types each year.
These include implantation of stereotactic depth electrodes, and epidural
peg and foramen ovale electrodes; microsurgical temporal lobectomy;
extratemporal resection; hemispherectomy; and corpus callosotomy.

-Epilepsy specialists utilize three different types of invasive recording
devices to monitor patients being considered for surgery, whereas most
centers employ only one of the techniques. With all of these options
available, the epilepsy team can individualize the screening and mapping of
each patient prior to surgery and operate with more precision.

-The Cleveland Clinic has developed a program to train personnel from the
Bodelschwinghsche Clinics in Bethel, Germany, in state-of-the-art brain
mapping and surgical techniques.

-Since 1988, the Cleveland Clinic has sponsored three international
conferences on epilepsy-related topics.

-The Cleveland Clinic is one of only a handful of epilepsy centers
currently performing second operations for people whose first operation has
not succeeded in controlling seizures. Nearly half of 15 patients in a
recently published study have remained seizure-free for periods of up to 82
months, giving selected patients a second chance at seizure control.

Pioneering Contributions

Epilepsy surgeons must precisely distinguish between tissue that is causing
seizures from that which is not so that they can remove as much of the
seizure source as possible without disturbing normal brain tissue. The
Cleveland Clinic's team has contributed greatly to this process:

-Development of sophisticated brain mapping techniques in 1980 for patients
with intractable epilepsy, allowing surgeons to more precisely delineate
the source of seizures and enabling them to remove more tissue without
damaging vital functions. This increases the likelihood of eliminating
seizures.

-Pioneering of a microsurgical technique, for use in anterior temporal lobe
surgery, that allows removal of more of the anterior temporal lobe without
unnecessarily removing adjacent tissue.

-Development of a new approach to evaluating the effects of extent of
resection on surgical outcome and complications. This approach, using
postoperative MRI, may allow comparisons of surgical techniques practiced
at different institutions.

-Development of a computerized, paperless EEG system to collect and store
data. This permits sorting, manipulation and analysis of data on screen,
making the most critical highlights of the data rapidly accessible.

-Development of a program in collaboration with the Cleveland Clinic's
Neurological Computing Section that permits on-line calculation of
single-dose pharmacokinetic studies.

-Development of the epidural peg electrode, which may be necessary to
accurately localize the seizure source during monitoring.

-Development of a technique that uses the epidural peg with the foramen
ovale electrode to allow some patients to avoid more invasive procedures.

-Adaptation of electrodes and other surgical techniques to be used in the
presence of scarring that exists in difficult second operations for
patients whose seizures recur.

Sidebar



For Information

If you need more information or want to make an appointment with a
Cleveland Clinic epilepsy specialist, please call one of the numbers below:

216/444-8919

(in Cleveland)

800/545-7718

!
*

Exstrophy of the Bladder is a rare developmental abnormality that is present at birth (congenital) and is characterized by the absence of a portion of the lower abdominal wall and the front part of the bladder wall (anterior vesical wall).  The rear portion of the bladder wall (posterior vesical wall) turns outward  (exstrophy) through the opening in the abdominal wall and urine is excreted through this opening.  The exact cause of Exstrophy of the Bladder is not known.

The physical characteristics of this disorder are the result of developmental
abnormalities during embryonic or fetal growth.

Symptoms
--------

Exstrophy of the Bladder is a rare birth defect characterized by the absence of  part of the lower abdominal wall as well as the front portion of the bladder  wall.  The rear wall of the bladder turns outward (exstrophy) through the  opening in the abdominal wall.  Exstrophy of the Bladder results in the inability to control the release of urine (incontinence).

The physical characteristics of Exstrophy of the Bladder include:  a highly
arched pubic bone; a wide separation between the bones that form the hips (ischia); and unusual fibrous tissue growth between the separated ischia.  The  connection between the kidneys and the tubes that carry urine from the kidneys to the bladder (ureters) may be abnormally narrow.  The ureters may also be wider than normal (dilated).  In males with Exstrophy of the Bladder, the scrotum is usually very broad and the testes are typically retracted.  The penis may be unusually short and displaced to one side.  In females with Exstrophy of the Bladder, the halves of the clitoris and labia are usually widely separated.  The opening to the vagina is typically misplaced and, in some cases, unusually narrow.

If Exstrophy of the Bladder is not surgically corrected, urine can flow back
into the kidneys from the ureters (reflux).  The kidneys may become overloaded with urine (hydronephrosis), leading to acute infection of the kidneys (pyelonephritis) and eventual kidney failure.
Affected Population
-------------------

Exstrophy of the Bladder is a rare disorder that affects males 3 times more
frequently than females.  The physical characteristics of this developmental defect are obvious at birth (congenital).

Related Disorders
-----------------

Symptoms of the following disorders can be similar to those of Exstrophy of the Bladder.  Comparisons may be useful for a differential diagnosis:

Exstrophy of the Cloaca Sequence is a rare developmental defect that is obvious at birth.  Infants with this disorder are missing a portion of the lower
abdominal wall as well as the front portion of the bladder wall (anterior
vesical wall).  This birth defect is more severe than Exstrophy of the Bladder.  The rear portion of the bladder wall is also exposed through the abdomen.  A  part of the intestinal lining (mucosa) may also protrude through the abdominal wall.  The opening of the rectum to the outside of the body is usually missing or abnormally small (stenotic).  Most infants with Exstrophy of the Cloaca Sequence also have a sac on the outside of the abdomen that contains portions of the intestines (omphalocele).


Therapies:  Standard
--------------------

The diagnosis of Exstrophy of the Bladder may be made before birth (prenatally).  Ultrasound studies of the developing fetus can reveal specific features that are characteristic of this abnormality (e.g., protrusion of the bladder wall through  the abdomen).  Early prenatal diagnosis can lead to surgical intervention  shortly after birth.

The treatment of Exstrophy of the Bladder consists of corrective surgery to
reunite the walls of the bladder and to close the opening in the lower abdomen.  During surgery, the lower abdominal wall is surgically closed and the bladder is  reconstructed using a segment of the colon to form the front and upper portions  of the bladder wall.  Approximately 70 percent of infants with Exstrophy of the  Bladder are successfully treated with this surgical procedure.

If this surgery is not successful in establishing continence, an alternative but
rarely used procedure may be performed.  During the surgery, the ureters are  implanted into a portion of the large intestine (ureterosigmoidostomy) and an opening between the colon and the surface of the abdominal wall is created  (colostomy).  Yearly follow-up with ultrasound studies and laproscopic studies is recommended for people who have had this type of surgery.  They may be at slightly higher risk for abnormal cellular changes of the colon at the site of  the diversion.

In another corrective procedure used to treat infants who have Exstrophy of the  Bladder, urinary flow is surgically diverted by connecting the ureters to the  lower portion of the small intestine (ileal loop diversion) or to part of the  large intestine (colon loop diversion).

In some cases, infants with Exstrophy of the Bladder may also require a series  of surgical procedures to reconstruct the external genitalia.  These surgeries  are usually performed before the age of 2 years.
The outlook for maintaining normal kidney function after surgical correction and  reconstruction is good.  However, some individuals with this disorder may experience long-term urinary problems such as kidney stones, kidney infections,  and varying degrees of urinary incontinence.  Other treatment is symptomatic and  supportive.

Therapies:  Investigational
---------------------------

Research on birth defects and their causes is ongoing.  The National Institutes  of Health (NIH) is sponsoring the Human Genome Project which is aimed at mapping every gene in the human body and learning why they sometimes malfunction.  It is hoped that this new knowledge will lead to prevention and treatment of birth defects in the future.

This disease entry is based upon medical information available through October  1994.  Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check  with the agencies listed in the Resources section for the most current  information about this disorder.
Resources
---------

For more information on Exstrophy of the Bladder, please contact:

National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT  06812-1783
[phone]
[phone] (TDD for the hearing impaired)

National Support Group for Exstrophy of the Bladder
5075 Medhurst St.
Solon, OH  44139
[phone]

NIH/National Kidney and Urologic Diseases Information Clearinghouse (NKUDIC)
9000 Rockville Pike
Bethesda, MD  20892
[phone]

Simon Foundation
P.O. Box 815
Wilmette, IL  60091
[phone]

Help for Incontinent People (HIP)
P.O. Box 544
Union, SC  27379
[phone]
[phone]

March of Dimes Birth Defects Foundation
1275 Mamaroneck Ave.
White Plains, NY  10605
[phone]
!
*
Facts for Consumers from the Federal Trade Commission
Eye Wear -- May 1994

Produced in cooperation with the American Academy of
Ophthalmology, the National Association of Optometrists and
Opticians, and the Opticians Association of America

If you, like many Americans, wear eyeglasses or contact lenses,
you probably know that comparison shopping can help you find
quality eye wear that meets your budget. In fact, your ability to
comparison shop for eyeglasses is aided by a Federal Trade
Commission Regulation.  Under federal law, you have a right to a
copy of your eyeglass prescription so that you can shop for the
best value in eye wear. If you are buying contact lenses,
comparison shopping also can help you find an eye care specialist
who offers products and services suited for you.

This fact sheet explains what rights you have under the law and
gives you information about various types of eye care
professionals. It also gives some suggestions about selecting an
eye care specialist and shopping for eye exams, eyeglasses, and
contact lenses.

Your Legal Rights

Eyeglasses

The Federal Trade Commission's (FTC) "Prescription Release Rule"
requires eye doctors to give you your eyeglass prescription, at
no extra cost, immediately after an eye exam that includes a
refraction (a test that determines the prescription needed to
correct your vision).  Your eye doctor may withhold your eyeglass
prescription until you have paid for your eye exam, but only if
your eye doctor requires immediate payment whether or not a
visual correction is needed.

With prescription in hand, you can shop for eyeglasses just as
you would for other health-related products and services, looking
for the best quality at the best price. You have a legal right to
your eyeglass prescription, so request it if it is not provided
immediately after an eye examination in which a refraction is
performed.

Contact lenses

Although not required by federal law, many eye care specialists
will give you a copy of your contact lens specifications. Some
states require eye care specialists to give you a copy of your
contact lens specifications. It is important to have a copy of
these specifications if you want to buy your contact lenses,
especially replacement or duplicate lenses, from a different
specialist. Otherwise, you may have to pay for another lens
fitting exam.

Selecting An Eye Care Specialist

Price is not your only consideration in choosing someone to
examine your eyes, fill your prescription, or fit you for contact
lenses. You also might be concerned about: the type of eye care
specialist you wish to visit; the quality of eye care and eye
wear you may receive; and the service that is promised,
especially if some adjustments or modifications prove necessary.

Eye Professionals

Before selecting an eye care specialist, you should know the
difference among the three types of eye care specialists _
ophthalmologists, optometrists, and opticians _ and the services
each is qualified to perform.  Only ophthalmologists and
optometrists may issue eyeglass and contact lens prescriptions.
Ophthalmologists are physicians who are either medical doctors
(M.D.'s) or osteopathic physicians (D.O.'s). They  specialize in
diagnosing and treating diseases of the eyes. They can prescribe
drugs, perform examinations and eye surgery, and dispense
eyeglasses and contact lenses.

Optometrists have doctor of optometry degrees (O.D.'s).  Though
they are not medical doctors, they can examine eyes for vision
problems and eye diseases and dispense eyeglasses and contact
lenses. State law determines the extent to which optometrists may
diagnose and treat eye diseases and prescribe drugs, and laws
vary from state to state. In states where optometrists are not
permitted to provide certain treatments, they will normally refer
you to an ophthalmologist or other appropriate medical
practitioner for such treatments.

Opticians fill prescriptions for eye wear written by
ophthalmologists and optometrists. They may not examine eyes or
prescribe lenses. They dispense eyeglasses and in some states are
permitted to fit and dispense contact lenses. About half the
states require opticians to be licensed.

Quality of Eye Care and Eye Wear

It may be difficult to predict the quality of the eye care, eye
wear, and related services you will receive. Studies show that
price and the type of practitioner are not necessarily
indications of quality.

To help ensure quality care, you might ask for recommendations
from your friends. You also might want to check with local
consumer affairs offices and consumer organizations to see if any
complaints have been lodged against the eye care specialist you
are considering.

Services

Investigate what kind of service eye care providers will give
you, especially if you have a problem. You may want to consider
the following  information.

Eye Exams: A thorough eye examination includes a refraction,
tests for other vision conditions, and an eye health exam. An eye
exam also can reveal whether you are a good candidate for contact
lenses. Remember, only M.D.'s, D.O.'s and O.D.'s may perform eye
exams.

Eyeglasses: If your eye exam shows you need a visual correction,
you may decide you want eyeglasses.  Ask about the delivery time
for eyeglasses, any refund policy, and who pays for replacement
lenses or frames if there are problems with either the lenses or
the frames.

Contact Lenses: Contact lenses are important health care devices
that require proper fitting and care.  Thus, if you decide you
want to buy contact lenses, additional steps are necessary beyond
the basic eye exam -_ the contact lens evaluation and the
fitting. They include measuring the curvature of your eye and
determining which lens is best for you. The evaluation and
fitting may be performed by ophthalmologists, optometrists, and,
in some states, opticians.

Comparison Shopping For Contacts

Because buying contact lenses is more complicated than buying
eyeglasses, here are some questions you might ask eye care
specialists.

What do you charge for an eye exam, lenses, a contact lens
evaluation, fittings, a lens care kit, follow-up visits, and
"insurance" service agreements?

These items may be priced individually or sold as a package. Some
advertisements for contact lenses quote bargain prices for the
contact lenses alone.  But bargain-priced contact lenses may not
be the best purchase if the other essential goods and services
are not included in the price.

If you are a first-time wearer of contact lenses, you will need
services like fittings and follow-up visits in addition to
lenses. Even experienced contact wearers may require several
appointments before they get a proper fit.

So before you select an eye care specialist, ask about the total
cost of care, including what tests are included in the eye exam.
A reasonably-priced package may be a better deal than
bargain-priced goods that do not include free follow-up visits.

What is your refund policy?

Not everyone who wants to wear contacts is able to adapt to them.
With a good refund policy, you will not lose your entire
investment if you cannot wear the contacts.

How many types and brands of contacts do you sell?

It may take several visits to find the right contact lenses for
your eyes. If the specialist carries a large selection of contact
lenses, it can increase your chances of getting a good fit.

How much do you charge for replacement lenses?

It is possible to lose or damage contact lenses. Find out how
much you will be charged and how long you will have to wait if
you need a replacement lens. Many providers offer service
agreements, also known as "insurance" arrangements, that will
cover the full or partial cost of replacing lost or damaged
lenses. Find out whether such an agreement is available, what it
covers, and how much it costs compared to the cost of replacement
lenses.

What is your policy on the release of my contact lens
specifications?

Under the FTC Rule, the eye care specialist must provide you with
your eyeglass prescription, but not your contact lens
specifications.  Without these measurements, comparison shopping
for contact lenses is difficult, if not impossible. Ask about the
doctor's policy before you make an appointment for an eye exam.
If you want a copy of your contact lens specifications, shop for
an eye care provider who will release it to you.

For More Information

If you have questions about your rights under the FTC's Rule
governing eyeglasses, write: Correspondence Branch, Federal Trade
Commission, Washington, DC 20580. Although the FTC generally does
not intervene in individual disputes, the information you provide
helps in its law enforcement efforts.

For information about other consumer issues, send for a list of
more than 100 free FTC publications. Write: Best Sellers, Public
Reference, Federal Trade Commission, Washington, DC 20580.

Eyeglasses: 8/78;11/80;6/83;4/86;12/87.
Looking for Contact Lenses: 10/82;1/85;4/86.
!
*
Fever

     Fever is a symptom experienced by almost everyone at some time.  The
body's normal temperature is not necessarily 98.6 degrees Fahrenheit or 37.7
degrees Celsius.  Rather, there is a "range of normal" varying from 96.8 to 100
degrees orally.  A rectal temperature is one degree higher than oral; an
axillary or underarm temperature is one degree lower.  For any individual there
is a temperature cycle of two to three degrees during a 24 hour period, with
the highest temperature usually occuring in late afternoon, and the lowest in
the early morning hours.
     Factors such as external cold or warmth, exercise, and activity also
influence body temperature, especially in infants and children.  The reading of
an oral thermometer is also influenced by the recent intake of cold or hot food
or fluids, by smoking, or by inability to hold the mouth closed.
     The normal range of temperature is maintained by a balance between heat
PRODUCED in the body and heat LOST by the body.
     When this balance of heat production and heat loss is upset, the
temperature rises and the person experiences a fever, defined as an oral
temperature of more than 100 degrees Fahrenheit, or 37.7 degrees Celsius.  It
is important to know that a fever is a symptom and not a disease.
     There are some body signs and symptoms that are typical effects of the
rise in temperature.  A person with fever appears and feels flushed.  The pulse
and breath rate increase and perspiration may be heavy.  There may be a general
feeling of chilling.  Shivering and shaking result, often followed by a rise in
the temperature.  One feels generally "achey", tired, or weak; a headache or
sore eyes may also be present.  A feeling of being very thirsty results from
the dehydration that occurs when fever is present.  As a result, urination may
be less frequent and the urine a darker color, the lips, mouth, and tongue may
feel dry and "coated".
     It is a very rare occurrence for a fever to go beyond 105-106 degrees
Fahrenheit or 40.5-41.1 degrees Celsius.
     Now - what to do about this symptom of fever?  Identifying the cause of
the fever is important.  Some fever causing illnesses such as ear infections or
kidney infections respond to direct treatment.  However, fever also commonly
accompanies "flu" or cold symptoms, mono, or other virus infections for which
there is no direct cure.  In such illnesses, treatment is aimed at relieving
symptoms.
     Treatment of the fever involves the following points:
     1. Let heat evaporate from the skin - do not overdress.  Wear light
clothing indoors and use only what covering is needed to keep from feeling
chilled.  Sponge, bathe, or shower with lukewarm water if you can do so safely.
Do not use ice packs or very cold water, as extreme cold constricts blood
vessels and prevents the loss of heat.
     2. Drink 2-3 quarts of liquid each 24 hours to replace fluid lost because
of the fever.  Soups, juices, gelatin, fruit drinks, soda pop, popsicles and
ice cream are suggestions for fluids to use.  It makes sense to avoid alcohol
or excess caffeine such as in coffee, as those liquids excessively increase the
output of urine.
     3. Take aspirin, acetaminophen or ibuprofen.  These medications are fever
reducers and pain relievers.  Consult the bottle label or your health care
provider for the usual dosage.  You should avoid ibuprofen if you are allergic
to aspirin.  Aspirin should NOT be used for fever in children or teens up to 19
years old because  t's use has been associated with the development of Reye
Syndrome (a rare childhood disease that causes coma, liver damage and death).
     4. Since heat is produced by action of skeletal muscles, it is sensible to
avoid strenuous activity even though you may feel well enough to be active.
     It is expected that a fever will be present early in the course of a viral
illness such as a cold or other respiratory illness.  It usually does not
exceed 102.5 degrees Fahrenheit or 39.1 degrees Celsius in adults and goes down
over a period of 2-3 days.  Seek medical advice if adequate doses of aspirin or
aspirin substitutes and sponging do not temporarily reduce the fever, or if the
fever continues to be 102 degrees or above for over 48 hours.  If the fever has
returned to normal and then suddenly rises again a few days later, seek
attention for a possible secondary infection.
     Fever in the absence of other symptoms is also significant, so if a fever
continues without obvious reason, seek medical advice.
     Medical evaluation of the significance of a fever is made easier if you
can answer some of the following questions:  How high is the fever?  What is
the pattern to the highs and lows?  Do you have shaking chills or periods of
sweating?  What have you done to bring the fever down and has this helped?
What other symptoms are present?
!
*
                Fibromyalgia -- a guide for patients

                              David A. Nye MD





    What is fibromyalgia?



        Fibromyalgia is a common and disabling disorder affecting 2-4%

        of the population, women more often than men.  Despite the

        condition's frequency, the diagnosis is often missed.  Patients

        with fibromyalgia usually ache all over, sleep poorly, are stiff

        on waking, and are tired all day.  They are prone to headaches,

        memory and concentration problems, dizziness, numbness and

        tingling, itching, fluid retention, crampy abdominal or pelvic

        pain and diarrhea, and several other symptoms.



        There are no diagnostic lab or x-ray abnormalities, but a

        physician can confirm the diagnosis by finding multiple tender

        points in characteristic locations.  Fibromyalgia often runs in

        families, suggesting an inherited predisposition.  It may lie

        dormant until triggered by an injury, stress, or sleep

        disturbance.  It is closely related to the chronic fatigue and

        irritable bowel syndromes.  Some have suggested that these are

        all just different facets of the same underlying disorder.





    What causes it?



        Fibromyalgia has mistakenly been thought to be either an

        inflammatory or a psychiatric condition.  However, no evidence

        of inflammation or arthritis has been found, and patients with

        fibromyalgia are now known to be no more depressed or anxious

        than those with other chronic, painful, debilitating conditions.

        It is now believed that depression and anxiety when present are

        more often the result than the cause of fibromyalgia.



        There is some evidence that fibromyalgia may be due to an

        abnormality of deep sleep.  Abnormal brain waveforms have been

        found in deep sleep in many patients with fibromyalgia.

        Fibromyalgia-like symptoms can be produced in normal volunteers

        by depriving them of deep sleep for a few days.  Low levels of

        somatostatin, a hormone important in maintaining good muscle and

        other soft tissue health, have been found in patients with

        fibromyalgia.  This hormone is produced almost exclusively in

        deep sleep, and it's production is increased by exercise.



        I should point out though that while this is my personal

        favorite among the theories of the cause of fibromyalgia, there

        are several others, and at this time there is probably not a

        majority of fibromyalgia researchers that supports any one

        theory.





    How is it treated?



        Fibromyalgia is difficult to treat, not because treatment isn't

        usually successful (it is), but because it will take a lot of

        work, education, and involvement on your part for it to be

        successful.  Simply starting the right medication will have

        little effect.  Successful treatment of fibromyalgia requires:



        1) Regular sleep hours and an adequate amount of sleep.



        2) Medication to improve deep sleep.



        3) Daily gentle aerobic exercise.



        4) Avoidance of physical and emotional stress.



        5) Treatment of any coexisting sleep disorders.



        If any of these five are omitted, significant improvement is

        unlikely.





    Regular sleep



        Patients with FMS must get to bed by the same time every night

        and sleep as long as they need to.  Staying up just one hour

        late may precipitate an exacerbation that lasts for several

        days.  Many patients with fibromyalgia have exacerbations

        triggered by the change over to or from Daylight Savings time.

        Try to make the switch in fifteen minute increments every few

        days instead of by one hour overnight.  I have had no success

        getting patients truly feeling well who work off shifts that

        prevent them from having a consistent bedtime.





    Medications



        Amitriptyline (Elavil), a medication commonly used to treat

        depression, also helps fibromyalgia, probably by improving the

        quality and depth of deep sleep rather than by any effect on

        mood.  When sleep is normalized, the symptoms of fibromyalgia

        begin to improve.  Patients taking amitriptyline usually don't

        note significant improvement until they are on enough to make

        them sleep through the night and have a dry mouth throughout the

        day.  A few patients with fibromyalgia experience no trouble

        with sleep an night, but still seem to respond in the same way

        to amitriptyline.  It is suspected that even though these

        patients are asleep, sleep function is not normal, accounting

        for their feeling that sleep is not refreshing.



        Some morning grogginess should be expected when amitriptyline is

        begun.  Starting at a low dose and increasing gradually helps

        minimize any initial side effects.  If you are having lots of

        trouble with side effects, go up more slowly.  By the end of two

        weeks, most patients find that the side effects are settling

        down and the fibromyalgia symptoms are starting to improve.  It

        usually takes a lot of fiddling with the dose to get it exactly

        right.  The dose that is initially effective may become less

        effective after a period of time and it may need to be increased

        slightly.  Most patients will need to continue on medication

        indefinitely.



        Almost everyone on enough amitriptyline to help fibromyalgia

        gets a dry mouth.  Some patients have a paradoxical stimulant

        effect from it, with rapid heart beat and some trouble falling

        asleep.  If these side effects are severe, other medications can

        be added to block them.  Amitriptyline may also cause a craving

        for sweets and weight gain.  I recommend you avoid sweets

        entirely while on amitriptyline.  Exercise will also help to

        keep your weight down, as will eating a low-fat diet such as the

        Weight Watchers one.  Some patients report that such a diet also

        helps their fibromyalgia symptoms.  Constipation is another

        frequent side effect.  Magnesium supplements are often helpful

        for constipation and may also help fibromyalgia symptoms in some

        patients.



        Several other medications have been used to treat fibromyalgia,

        although none seems to work quite as often as amitriptyline.

        These include cyclobenzaprine (Flexeril), diphenhydramine

        (Benadryl), and alprazolam (Xanax).  These have many of the same

        side effects as amitriptyline but they also have shorter

        durations of action so side effects tend not to last into the

        day as much.  None of these other medications cause weight gain

        the way amitriptyline does.  There are several herbal and other

        "alternative" remedies that some patients feel are helpful.

        While I can't recommend them simply because they haven't been

        adequately studied for efficacy or long term harm, I don't

        discourage patients from using them if they find them helpful.





    Exercise



        Daily gentle aerobic exercise is very important.  While patients

        who do too much exercise too soon or of the wrong kind will make

        themselves temporarily worse, most patients who don't begin a

        daily aerobic exercise regimen will notice little improvement in

        their fibromyalgia symptoms.  Aerobic exercise is defined as

        exercise that gets your heart rate up to a target heart rate for

        the duration of the exercise period.  Heart rates are measured

        in beats per minute.  It is accurate enough for our purposes

        just to take your pulse for 6 seconds and multiply by 10.  The

        aerobic target heart rate is calculated from the following

        formula:



            (220 - age - rhr) x .6 + rhr



        where age is your age in years and rhr your resting heart rate,

        determined by taking your pulse when you wake up but before

        getting out of bed.  A good place to feel your pulse is at the

        wrist turned palm up, next to the large bone on the thumb side

        at the end of your forearm.  If you are exercising hard enough

        you should be able to feel your heart beating and can just count

        that.  For most people, the aerobic target heart rate is at

        about the point where they can no longer sing but can still talk

        comfortably.



        The kind of exercise is unimportant.  Just make sure to pick

        something that doesn't make you hurt worse.  It may take trying

        several different kinds before finding one or more types that

        agree with you.  Popular kinds include walking, regular or

        exercise bicycles, ski simulators, rowing machines, rebounders,

        swimming, and *gentle* aerobic dance.  Jogging, vigorous aerobic

        dance, and weight lifting tend not to very good choices.  If

        your pain is mainly in your legs or back, consider exercising

        just your arms with a Thighmaster or similar equipment.



        While many patients insist that they get plenty of exercise at

        work, doing housework, or in their yard, this is rarely the

        case.  These types of exercise are rarely helpful, as they don't

        result in a sustained elevation of the heart rate, and often

        increase pain and make patients feel worse.  You need to set

        aside a time specifically for daily excercise.



        Particularly if you are out of shape, start out with just 3-5

        minutes of exercise and gradually increase as tolerated,

        shooting for twenty to thirty minutes.  Take a few minutes to

        stretch your muscles, then start out slowly, increasing to full

        speed after a minute or two.  Slow down again for the last

        minute or two and repeat the stretches.  There are five

        recommended stretches, each done for 20 seconds a side.  They

        should be gentle and painless.  Hold onto a tree or post for

        support for #s 3-5:



        1) Shrug your shoulders in a circular motion.



        2) Reach your arm over your head and bend to the opposite side.



        3) Bend forward with your legs straight.



        4) Pull your foot towards your buttock while standing on the

        other leg.



        5) With your feet flat on the ground and one foot ahead of the

        other, lean forward, bending just the front knee.



        Exercise is more effective if done in the late afternoon or

        evening.  If you absolutely can't do it then, exercising earlier

        in the day is better than not exercising at all, but you will

        probably need to exercise longer for the same effect.  Some

        patients find that exercise provides an immediate benefit,

        making them feel more alert and comfortable for several hours.

        If you experience this effect, you may want to try exercising

        three times a day instead of just once.  Patients who can do

        this are the ones most likely to eventually be able to get off

        medication.



        Exercise seems not to work through conditioning of muscles but

        rather through a direct, possibly hormonal effect on pain and

        sleep.  Patients who have been exercising regularly and then

        miss a day usually find that their fibromyalgia symptoms are

        significantly worse the next day.





    Avoid physical and emotional stress



        Exercise is an indispensible component of successful treatment,

        but too much physical activity of the wrong kind can precipitate

        a relapse.  Rather than doing housecleaning, yard work, or other

        physical activity all on one day, break up the task so that you

        do a half hour or an hour every day until it is done.  While it

        is difficult to learn to do this, it is essential that you be

        able to sense when you have reached your limit and stop.  You

        need to be able to say no to family and friends when you are not

        up to some outing or other activity.  Don't take on extra

        stressful responsibilities if you don't have to.  If you have

        ongoing problems with depression or anxiety, consider seeking

        help for them from your family doctor or psychiatrist in an

        attempt to lower your overall stress level.  Relaxation

        techniques or a chronic pain program can also help lower your

        stress level.  Fibromyalgia patients must learn to manage their

        physical and emotional resources.





    Treat other sleep disorders



        Several other sleep disorders besides insomnia may aggrevate

        fibromyalgia.  Almost half of men with fibromyalgia and some

        women have obstructive sleep apnea.  In this condition the

        patient snores loudly and has periodic pauses in breathing after

        which he starts breathing again with a snort.  Periodic limb

        movements of sleep is a condition in which patients twitch every

        30 to 90 seconds for long periods during the night.  Patients

        may be completely unaware of either of these conditions until

        the spouse complains.  Not only will it be difficult to get

        fibromyalgia symptoms to improve without treating these

        disorders, but if sleep apnea is left untreated it may lead to

        accidental death or injury as well as early strokes or heart

        attacks.



        Other common sources of repeated sleep disturbance are a

        spouse's snoring and young children.  If the spouse drinks

        alcohol in the evenings or is overweight, then avoidance of

        alcohol after supper or weight may eliminate snoring.  Sleeping

        propped up on the side will often help.  At the very least, the

        patient can wear earplugs.  Children are harder to put off but

        fortunately most soon outgrow their need for care at night.



        It is important to avoid prescription tranquilizers and sleeping

        medications of the benzodiazepine group.  While these may help

        you get to sleep, they supress deep sleep and therefore often

        make fibromyalgia worse.  Alcohol and narcotic pain medications

        taken in the evenings have the same effect on deep sleep and

        should be avoided.  Try not to exercise just before bed, as this

        may make it harder to fall asleep.  Patients with fibromyalgia

        should probably give up caffeine completely as even one cup in

        the morning can sometimes disrupt sleep at night and may also

        directly increase muscle pain and headaches.  If you are

        drinking more than a cup a day you should gradually taper

        yourself off caffeine-containing beverages over two weeks or so

        to minimize caffeine withdrawal symptoms such as headaches.





    Support and education



        For best results, you need to be actively involved in your

        treatment and to have as clear an understanding of this

        complicated disorder as possible.  I recommend that you keep

        this handy and re-read it periodically.  Patients with

        fibromyalgia often elicit less sympathy and support from family,

        friends, and employers than they deserve because of the lack of

        outward evidence of disease.  Many patients have been told by

        other physicians that there is nothing wrong with them or that

        it is "all in your head" which can be very demoralizing.  For

        these reasons, and just because it is good to know that you are

        not alone, I strongly encourage attending support group

        meetings.  There are local chapters in most areas now of the

        Fibromyalgia Network.  This organization produces an excellent

        newsletter which is well worth getting.  Here's how to contact

        them:



          Fibromyalgia Network

          5700 Stockdale Hwy, Suite 100

          Bakersfield, CA 93309

          info line: 1-805-631-1950 from 10am-2pm Pacific Time

          publication: FM Newsletter (quarterly); $15/yr USA, $17/yr Canada





    Conclusion



        With a little work, most patients can make it to the point where

        they feel good most of the time.  Even with good results from

        treatment however brief relapses are common, perhaps caused by

        staying up as little as one hour late one evening, skipping

        exercise, a disruption in your routine, increased stress, a

        storm front moving in, or often for no apparent reason.  You

        will do best if you "give in to it" when this happens and try to

        get extra rest.  Ibuprofen or naproxen and hot baths may help at

        these times.  If at all possible, try not to stop exercising

        when this happens, even if you have to back off on the amount a

        little.  Once you have had a period of feeling relatively well,

        it should always be possible to get you back to that point again

        by trying to identify what derailed you and correcting the

        problem.

-----



David Nye, MD * Neurology Dept., Midelfort Clinic, Eau Claire, Wisconsin



Your comments and suggestions are always welcome!

Write to the MARRTC Arthritis Resource Center developer Janet Elizabeth

Horton, BGS at the Fibromyalgia Resource Center.



                          Last Modified: 5/24/95
!
*


                  DEAR DOCTOR COLUMNS ARE WRITTEN BY UAB
               UNIVERSITY OF ALABAMA AT BIRMINGHAM) EXPERTS
                AND PUBLISHED WEEKLY IN THE BIRMINGHAM NEWS

FOOT PAIN

Question:

   For years I've had pain in my right foot, which started with a
   sharp pain like pins sticking in the top of my toes.  It
   started after I stepped on a sharp stick, which didn't go
   through my shoe but caused a sharp pain.  It's gotten worse
   over the years and is severe at times, along with burning and
   numbness.  The doctor biopsied the nerve in my ankle but found
   no cause for the pain.  He said my vitamin B-12 levels were
   low and gives me shots, but they don't help the pain.

Answer:

     Vitamin B-12 can be low for a number of reasons, but one
that can result in pain and numbness in the feet is pernicious
anemia.  Pernicious anemia causes abnormal red blood cells and
also damages nerves, which can result in numbness, tingling, and
pain in the feet and legs as well as the arms and hands.
   Pernicious anemia is caused by failure to absorb B-12 from the
intestinal tract because an essential chemical called intrinsic
factor is not present in the stomach. If the cause of your foot
pain were pernicious anemia, it would most likely cause pain in
both feet rather than one, and B-12 injections would probably
have relieved the pain.
   UAB neurologists, physicians who specialize in problems
related to nerves and the nervous system, note that because the
pain is limited to the toes of the right foot, it may be due to
the injury you mention or to some other local problem that is
irritating or pressing on the nerve to the toe.
   To determine whether the problem is a localized one or whether
it is related to an underlying medical problem, you may want to
ask your doctor about tests called nerve conduction studies.
These tests are done in conjunction with a complete neurological
examination.  They are designed to determine how well the nerves
function in the extremities, which often can tell the neurologist
whether the pain is related to a local problem with the nerve or
related to a more general medical problem.
!
*
Frostbite


THIS IS A HEALTH-LINE MESSAGE FROM THE UNIVERSITY OF WISCONSIN-MADISON.
     Take steps to avoid frostbite!  If you expect to be outside in the cold
wear suitable clothing and keep dry.  Since water is a better conductor of heat
than air, you will become chilled rapidly if  your clothing is wet.  Low
temperatures and wind combine to produce the chill factor which determines how
long a body part can be exposed before freezing begins.  Our toes, fingers,
cheeks, nose, and ears are particularly susceptible to frostbite, If you are
not moving actively, circulation to your skin slows, and freezing  may occur
more rapidly.  Dressing appropriately, keeping active, staying dry, and
warming-up often are positive steps you can take to prevent frostbite.  Don't
touch metal with your bare hands or allow gasoline or fuel stored outside to
come in contact with the skin.  In both cases, freezing is almost a certainty.
      How do you know if there is frostbite?  The early signs of frostbite are
whiteness and pain of the area followed by numbness.  The skin feels cold,
hard, and there is little or no sensation when you run your finger over the
area.
     Frostbite is freezing of the skin and underlying tissues causing swelling
and clotting in the small blood vessels.  If the injury from freezing is
extreme, there may be complete death of the tissue resulting in gangrene.
     Treatment should be directed toward rapid rewarming of the affected area
and prevention of further injury.  The involved tissue should never be rubbed
or messaged.  Small areas such as nose, ears or fingers can be warmed by the
hands.  Fingers and hands can be warmed by blowing warm breath on them or
tucking them under your armpit.  Larger areas should be warmed by the use of
continuous warm compresses, or, ideally, by soaking in a water bath.  With
either method, the temperature of the water should be about 104 degrees
Fahrenheit, which is just above body temperature and feels comfortably warm,
but, not hot.  Continue rewarming until a pink color returns to the tip of the
frozen part.  This usually takes about 30 minutes.  Treat the thawing or thawed
part very gently as the damaged tissues are very prone to injury.
     Typically, persons with frostbite complain of aching pain as the injured
parts become warmer and are more comfortable when the parts are cool.  Despite
this, frostbitten areas must be warmed up to body temperature to avoid further
tissue damage.  After thawing, avoid exposure to the frostbitten area to
further cold or injury.
     If you suspect possible frostbite injury, consult you physician or other
health professional.  Thank you.

!
*
Fact Sheet    Gas in the Digestive Tract

National Institutes of Health
National Institute of Diabetes & Digestive & Kidney Disease

DD Clearinghouse

Gas in the digestive tract is not a subject that most people like
to talk about, but the truth is that all of us have it and must
get rid of it in some way. Normally, the gas passes out through
the rectum or is belched through the mouth. These are both
necessary functions of the body that allow us to eliminate gas.

When gas does not pass out of the body easily, it can collect in
some part of the digestive tract, causing bloating and
discomfort. Even normal amounts of gas in the body can bother
people who are sensitive to this pressure. Although gas usually
is not a sign of a medical problem, it can be. So if you have
persistent or extreme gassiness (flatulence), mention it to your
doctor when you have a checkup.

What Causes Gas?

A common source of upper intestinal gas is swallowed air. Each
time we swallow, small amounts of air enter the stomach. This gas
in the stomach is usually passed into the small intestine where
part of it is absorbed. The rest travels into the colon (large
intestine) to be passed out through the rectum.

In some people, part of the gas is belched out instead of being
passed from the stomach into the intestine. This happens for
several reasons. People under a lot of stress often swallow large
amounts of air. Some people swallow air frequently because they
have postnasal drip, chew gum, or smoke. Rapid eating or poorly
fitting dentures also may cause too much air to be swallowed.
Also, drinking beverages that contain carbonated water may
increase gas in the digestive tract. These drinks contain carbon
dioxide, which can produce large amounts of gas when warmed in
the stomach. People with a gas problem should avoid carbonated or
"sparkling" drinks.

What Causes Repetitive Belching?

Some people experience frequent belching. This might occur after
a person has swallowed air without realizing it. Sometimes
belching accompanies movement of stomach material back up
(reflux) into the esophagus (swallowing tube). To clear material
from the esophagus, a person may swallow frequently, which leads
to more intake of air and further belching.

Another cause of repeated belching is gastritis (inflammation of
the stomach). There are many causes of acute or chronic
gastritis, but the most common cause is infection with a
bacterium called Helicobacter pylori. When this organism gets
into the stomach, it can produce bloating. This condition usually
can be diagnosed by a specialist in digestive diseases
(gastroenterologist). The doctor may detect the infection with a
breath test or a blood test. The doctor also may take a sample of
tissue (biopsy) from the stomach, using a lighted, flexible tube
(endoscope) that is inserted through the mouth. Helicobacter
pylori infection usually is treated with antibiotics and a
bismuth preparation.

Do Any Foods Cause Gas?

The foods we eat can be a factor in the production of gas in the
lower intestine. Some foods such as cauliflower, brussels
sprouts, dried beans, broccoli, cabbage, and bran are not
completely digested in the small intestine. When the undigested
bits of food reach the colon, they are fermented by the bacteria
that live in the colon, causing gas.

Today many people are trying to improve their nutrition and
health by eating more fiber. However, some people discover that
adding large amounts of fiber to their diets causes gassiness.
This can happen when someone begins eating more whole grain
cereals such as whole bran, oatmeal, or oat bran, or whole grain
breads or fresh fruits, and vegetables. They get a feeling of
being bloated when they first begin the high-fiber diet, but
within 3 weeks or so, they may adapt to it. Some people, however,
don't adapt, and the bloating from eating a lot of fiber can be a
permanent problem.

A common cause of excess lower intestinal gas is that a person's
body may not have enough lactase, and enzyme normally found in
the small intestine. Lactase is needed to digest lactose, the
sugar found in milk and other dairy products. When this sugar
passes undigested into the colon, it is fermented by bacteria,
and gas forms. This can be a cause of excessive flatulence.

If lactase deficiency is suspected of causing your gas, your
doctor may tell you to stop eating dairy products for a while to
see if you will have less gas. The doctor also may give you a
blood test or a breath test to find out if you are lactose
intolerant. The breath test detects hydrogen that is released by
the bacteria as the undigested lactose ferments in the colon.

How Much Gas Does the Body Produce?

The amounts of gas that people produce vary. Most people produce
between a pint and a half gallon of gas each day. Oxygen, carbon
dioxide, and nitrogen from swallowed air make up a large part of
flatus. Fermenting foods in the colon produce hydrogen and
methane as well as carbon dioxide and oxygen. All of these
components of flatus (gas) are odorless. The unpleasant odor of
some flatus is the result of trace gases, such as hydrogen
sulfide, indole, and skatole, which are produced when foods
decompose in the colon.

What Causes the Abdominal Pain and Bloating?

Eating a lot of fatty food can cause bloating and discomfort
because that fat delays stomach emptying, allowing gas to build
up there. This problem can avoided by eating less fatty meals.
The feeling of bloating in the abdomen may increase during the
day and become most severe after a large meal. Many people think
the bloated feeling after eating is caused by large amounts of
gas. Researchers, however, have not found any connection between
this symptoms and the total amount of gas in the abdomen. Studies
show that in some people even modest amounts (1 ounce to 1/2
pint) of gas in the intestine can cause spasms there, especially
after eating.

Gas in the upper abdomen often is relieved by belching. Sometimes
people try to swallow air to make themselves belch. This doesn't
work, however, because it only adds to the amount of gas in the
stomach and does not reduce the discomfort.

Gas can collect anywhere in the lower intestine. Often if
collects on the left side of the colon, and the pain can be
confused with heart disease. When gas collects on the right side
of the colon, the pain can be like that caused by gallbladder
disease or even appendicitis.

A bloated feeling is probably not anything to be concerned about,
but it can be a symptom of a more serious problem,m such as an
intestinal obstruction. If your problem is chronic, or if you are
experiencing a severe increase in gassiness, you should talk to
your doctor.

Is Gassiness Caused by a Disease?

If excess gas is your only symptom, it is probably not caused by
a disease. The problem may occur simply because you swallow air
or digest food incompletely. It could be that your intestines
have the kind of bacteria that produce a lot of gas. You could
have a sluggish bowel that does not get rid of air readily. You
might have an irritable bowel, often called spastic colon, which
means that you cannot tolerate gas accumulating inside of the
intestines, so even small amounts of air feel uncomfortable.

Do Over-the-Counter Drugs Relieve Gas?

Many claims are made for over-the-counter drugs intended to
relieve gassiness. Often people find that these drugs do not help
much, but some of them do help some people. Simethicone,
activated charcoal, and digestive enzymes, such as the lactose
supplement lactase, are among those doctors often recommend.
Sometimes doctors prescribe drugs called gastrointestinal
stimulants that help move gas through the intestines more readily
and that may help gassiness in some cases.

Some Suggestions on How To Reduce Gas in the Digestive Tract

If you are bothered by excessive belching or flatus, and your
physician has determined that you have no serious disease, the
following suggestions may be helpful:

■    Check with a dentist to make sure dentures fit properly.

■    Avoid chewing gum or sucking on hard candies (especially
     sugarless gum or dietetic candies that contain sorbitol).

■    Eliminate carbonated beverages from your diet.

■    Avoid milk and milk products if you have lactose
     intolerance.

■    Eat fewer gas-producing food such as cauliflower, brussels
     sprouts, bran, beans, broccoli, and cabbage.

■    Walking, jogging, calisthenics, and other exercised help to
     stimulate the passage of gas through the digestive tract.

If your symptoms persist or worsen, see your doctor to make sure
that the condition is not caused by abnormalities in your
digestive tract.

Additional Readings

Clayman, CB, ed. The American Medical Association Encyclopedia of
Medicine. New York: Random House, 1989. Reference guide with
sections on digestive tract gas and other digestive problems.
Available in libraries and bookstores.

Jain, NK, Vela, JS, Pitchumoni, CS. Intestinal gas: insights into
an ancient malady. Practical Gastroenterology 1987; 11(6): 32-44.
Review article for physicians discusses the causes and treatments
of gas. Available in medical libraries.

Levitt, MD. Intestinal gas: what do we offer the patient?
Endoscopy Review 1991; 9(5): 43-46. Article for physicians
discusses the causes, evaluations, and treatments for gas.
Available in medical libraries.

Larson, DE, Editor-in-chief. Mayo Clinic Family Health Book. New
York: William Morrow and Company, Inc., 1990. General medical
guide with section on intestinal gas. Available in libraries and
bookstores.

National Digestive Diseases Information Clearinghouse

Box NDDIC
9000 Rockville Pike
Bethesda, MD  20892
[phone]


The National Digestive Diseases Information Clearinghouse is a
service of the National Institute of Diabetes and Digestive and
Kidney Diseases, part of the National Institutes of Health, under
the U.S. Public Health Service. The clearinghouse was authorized
by Congress to focus a national effort on providing information
to the public, patients and their families, and doctors and other
health care professionals. The clearinghouse works with
organizations to educate people about digestive health and
disease. The clearinghouse answers inquires; develops, reviews,
and distributes publications; and coordinates informational
resources about digestive diseases.

Publications produced by the clearinghouse are reviewed carefully
for scientific accuracy, appropriateness of content, and
readability. Publications produced by sources other than the
clearinghouse also are viewed for scientific accuracy and are
used, along with clearinghouse publications, to answer requests.

This publication is not copyrighted. The clearinghouse urges
users of this fact sheet to duplicate and distribute as many
copies as desired.

U.S. Department of Health and Human Services
Public Health Service
National Institutes of Health

!
*
GAUCHER DISEASE: CURRENT ISSUES IN DIAGNOSIS AND TREATMENT

                   National Institutes of Health
            Technology Assessment Conference Statement
                      February 27-March 1, 1995

This statement will be published as:
     Gaucher Disease: Current Issues in Diagnosis and Treatment. NIH
     Technol Assess Statement 1995 Feb 27-Mar1:1-__.
For making reference to technology assessment statement no. 16 in the
electronic form displayed here, it is recommended that the following format be
used:
     Gaucher Disease: Current Issues in Diagnosis and Treatment. NIH
     Technol Assess Statement Online 1995 Feb 27-Mar 1 [cited year month
     day]:1-__.

Introduction

Gaucher disease is a rare inherited enzyme deficiency, which researchers
estimate may be present in 10,000-20,000 Americans. It is a panethnic disorder,
with highest prevalence in the Ashkenazi Jewish population.

During the past decade, much progress has been made in understanding the
molecular biology of the disease and in the ability to treat patients with the
disorder. However, many issues regarding diagnosis, population screening, and
therapy for patients with Gaucher disease are controversial.

Gaucher disease is characterized by a remarkable degree of variability in its
clinical signs and symptoms, ranging from severely affected infants to
asymptomatic adults. Many patients suffer from anemia, bone damage, and
enlarged livers and spleens; a few develop severe central nervous system
damage. Gaucher disease is a potentially lethal disorder. All patients with
Gaucher disease have a genetic defect in the enzyme glucocerebrosidase, which
results in the accumulation of the lipid glucocerebroside within intracellular
structures known as lysosomes.

Patients with Gaucher disease have been classified into three major types on
the basis of clinical signs and symptoms: type 1, non-neuronopathic (adult);
type 2, acute neuronopathic (infantile); and type 3, subacute neuronopathic
(juvenile). All types of Gaucher disease can be diagnosed by demonstrating a
deficiency of glucocerebrosidase activity.

The most striking differences among the three types are the presence or absence
of neurologic manifestations and the rate of their progression. However, people
with the same type of the disorder may differ in their clinical presentation.
For example, certain patients with type 1 Gaucher disease, which is by far the
most common type, may display some combination of anemia, low blood platelet
levels, massively enlarged livers and spleens, and extensive skeletal disease.
In contrast, other type 1 patients may have no symptoms and can be identified
only by screening or during evaluation for other diseases.

The gene for glucocerebrosidase, which is located on chromosome 1q21, has been
characterized and sequenced. Many mutations in the glucocerebrosidase gene have
been identified in DNA from different patients; several of these mutations are
frequent. Although some patients with the same DNA mutations have similar
clinical courses, other patients with the same mutations have very different
clinical manifestations. It is still not clear to what extent a person's
clinical features (phenotype) or prognosis can be accurately predicted through
current mutation analysis. Furthermore, although the molecular techniques can
be used for early prenatal diagnosis, detection of individuals carrying the
disease gene, and population screening, the appropriate clinical application of
these molecular techniques remains unresolved.

Gaucher disease has been traditionally managed by supportive therapy including
total or partial removal of the spleen, blood transfusions, orthopedic
procedures, and occasionally bone marrow transplantation. More recently, enzyme
replacement therapy has become available and has proven effective in many
patients. Enzyme replacement therapy has successfully reversed many of the
manifestations of the disorder, including abnormal blood counts, increased
liver and spleen size, and some skeletal abnormalities. The therapy is very
costly, however, ranging from $100,000 to $400,000 annually for each patient.

The purpose of this Technology Assessment Conference was to evaluate current
concepts concerning diagnosis, screening, genetic counseling, and management of
Gaucher disease. In this effort, the National Institute of Mental Health,
together with the Office of Medical Applications of Research of the National
Institutes of Health, convened a Technology Assessment Conference, Gaucher
Disease: Current Issues in Diagnosis and Treatment. The conference was
cosponsored by the National Institute of Child Health and Human Development,
the National Institute of Diabetes and Digestive and Kidney Diseases, the
National Institute of Neurological Diseases and Stroke, the National Center for
Research Resources, the National Center for Human Genome Research, and the
Office of Rare Disease Research.

Following 1-1/2 days of presentations by experts in the relevant fields and
discussions with the audience, an independent panel composed of specialists and
generalists from the medical and other related scientific disciplines, as well
as a public representative, considered the evidence and formulated a consensus
statement in response to the following six previously stated questions:

   * What is the natural history of Gaucher disease, and what is the
     appropriate technology to assess the severity and to predict the
     progression of this disorder?
   * What are the roles of current molecular and enzymatic assays for
     ascertaining affected individuals and carriers in various populations?
   * What are the indications for treatment of patients with Gaucher disease,
     and what are the appropriate modes of therapy?
   * What are the goals for and consequences of treatment, and how can the
     therapeutic interventions be assessed?
   *  Under what circumstances could genotype/phenotype correlations be used
     for patient care and counseling?
   *  What are appropriate directions for future research and other relevant
     issues that should be pursued?

What Is the Natural History of Gaucher Disease, and What Is the Appropriate
Technology to Assess the Severity and to Predict the Progression of This
Disorder?

The natural history of Gaucher disease is incompletely documented. The
progression and outcome are well understood only in type 2 disease (infantile
form). Type 3 disease (juvenile form) has a more variable course. The type 1
(adult) form is most common, especially variable, and least well characterized.
Furthermore splenectomy, orthopedic intervention, and enzyme replacement
therapy for type 1 Gaucher disease alter its course, natural progression, and
outcome. Thus, it is important to standardize the reporting of the effects of
these interventions.

Type 1 disease typically presents after infancy and often not until adult life.
Indeed, some genotypically affected individuals may never come to medical
attention, and their number is unknown. With DNA analysis of family members,
many such individuals will be diagnosed. Current technologies may unmask and
identify organ-specific manifestations in these asymptomatic individuals.
Simple hematologic and biochemical assays and imaging techniques can be used to
assess disease progression. Skeletal disease is especially difficult to assess.
Mutation analysis provides precise diagnosis but may not give information
concerning the severity or progression of the disease. In addition, there are
considerable differences in the degree to which organ systems are affected.
Furthermore, there are reports of intrafamilial variation. Differences in
disease severity have been demonstrated even in identical twins. Thus, other
genetic and nongenetic factors appear to be involved in the expression of the
disease.

Prenatal diagnosis now affords an opportunity to assess the natural progression
of the disease from before birth. Such information may be critical in choosing
appropriate technology for prognosis and therapy. Appropriate systematic and
quantitative description of the disease is essential to understand its natural
course. Patient characterization requires clarification of the terminology used
to describe patients, which at this time is confused (e.g., "asymptomatic"
versus "asymptomatic but with physical signs and laboratory evidence of
disease").

What Are the Roles of Current Molecular and Enzymatic Assays for Ascertaining
Affected Individuals and Carriers in Various Populations?

Enzyme analysis of leukocyte or fibroblast extracts is appropriate to confirm
or exclude the diagnosis of Gaucher disease. Several methodologies for
enzymatic diagnosis are currently available and are reliable in experienced
hands. No consensus has yet been reached on a single most appropriate method,
which makes it essential that each laboratory have rigid internal quality
assurance and quality control of the method it uses.

The prognosis for patients with type 1 disease cannot be predicted from the
residual enzyme activity measured in tissues. Enzymatic analysis cannot be used
to detect carriers reliably.

Analysis of DNA for mutations by molecular methods (genotyping) is appropriate
in all individuals with glucocerebrosidase deficiency. Genotyping of siblings
and parents of affected individuals is important to ascertain other potentially
affected individuals who may be asymptomatic and to identify carriers for
genetic counseling. Enzyme analysis of parents of affected individuals is also
valuable to exclude the possibility of asymptomatic glucocerebrosidase
deficiency in a parent with two mutant alleles, only one of which was
identified by genotyping. Although current genotype/phenotype correlations are
imperfect, genotyping may indicate that neurologic complications are unlikely.
It has less value in predicting the likelihood of other complications.

Molecular methods can provide accurate carrier detection, particularly in
defined populations. For example, in the Ashkenazi Jewish population, screening
for five mutations allows detection of approximately 95 percent of heterozygous
individuals. The greater variety of mutations in non-Jewish populations makes
carrier detection in these populations more challenging with currently
available technology. Analysis of some mutations by DNA amplification can be
complicated by the presence of a highly homologous pseudogene that is located
nearby. Quality control of the molecular techniques is important, as is
awareness of the complexities in interpreting data produced by these
amplification methods.

Widespread application of genetic screening to detect either presymptomatic
patients with Gaucher disease or heterozygous carriers is not appropriate at
this time. The medical value of presymptomatic diagnosis of patients with
Gaucher disease and carrier testing has not been established. For this reason,
pilot studies examining the potential benefits and/or harms of such screening
programs should be encouraged. Ideally, the target community should be involved
in the implementation and evaluation of such pilot studies.

What Are the Indications for Treatment of Patients With Gaucher Disease, and
What Are the Appropriate Modes of Therapy?

The clinical features of type 1 glucocerebrosidase deficiency are highly
variable, ranging from serious multisystem involvement to the absence of signs
or symptoms. In addition, the age of onset of clinical features in those who
develop symptoms is variable. This degree of variability raises several
important issues that must be considered before initiating treatment. First,
the characteristic signs of the disorder, which include anemia,
thrombocytopenia without bleeding, hepatosplenomegaly without pain or
discomfort, and radiologic changes without evidence of fractures or bone pain,
must be differentiated from the symptoms of the disorder, such as bleeding,
somatic pain, bone crises, and fractures. Second, knowledge is inadequate on
the effect of treatment for patients who display signs but no symptoms of the
disease. There is a reasonable consensus to treat those who exhibit symptoms;
however, no agreement exists on the clinical criteria for initiating treatment.
No consistent guidelines are available at this time because of the lack of
sufficient information about the natural history of the disease.

In addition, a group of individuals, of unknown number, have the enzyme
deficiency but have not developed signs or symptoms. Because we cannot predict
whether these individuals will ever become symptomatic, the appropriateness of
prophylactic therapy has not been determined.

A systematic evaluation of enzyme-deficient individuals to define the natural
history of the disease is lacking. For symptomatic patients, there should be
sufficient extant data given the number of patients who have already been
identified, treated, and extensively followed. For asymptomatic individuals, it
is necessary to develop protocols for longitudinal evaluation.

Conservative therapy has a role in Gaucher disease, such as hydration,
analgesics, and narcotics for pain in bone crises and orthopedic surgical
intervention for fractures. The use of vitamin D, calcium, and bisphosphonate
in bone crises and for bone growth requires further study.

Although bone marrow transplantation is an effective form of therapy, the risk
of mortality and morbidity makes this mode of treatment less desirable.

In type 1 disease, there is good evidence that enzyme replacement therapy with
mannose-terminated placental or recombinant glucocerebrosidase is beneficial in
reducing hepatosplenomegaly, improving hematologic parameters, and, to a lesser
extent, in alleviating bone disease. Enzyme therapy appears to obviate the need
for splenectomy in most cases.

Several patients with type 2 disease are reported to have been treated with
enzyme replacement therapy, and there was no substantial improvement in their
neurologic problems. With current technology, enzyme replacement therapy is
unlikely to prove efficacious for patients with type 2 disease. The efficacy of
enzyme replacement for neurologic abnormalities in type 3 disease remains to be
established.

For individuals with type 1 disease, controversies continue over aspects of
enzyme replacement therapy, such as dosage, methods and frequency of
administration of the enzyme, and cost. The most contentious issue, and
potentially the most difficult for patients and their physicians, is enzyme
dosage. Clinical successes have been observed with both the "high" and "low"
dosage regimens (described as amount of enzyme administered during a 4-week
internval for purposes of comparison, independent of dosage schedule): the 120
U/kg/4 weeks and the 30 U/kg/4 weeks, respectively. Inadequate clinical
responses were also reported for all dosage regimens tested. The debate about
dosage is complicated by the failure to compare data adequately and by the
diversity of protocols. Review of the data indicates two salient points. First,
patients vary considerably and unpredictably in their responses. Second, many
patients do well on lower dosage regimens. The use of low-dosage regimens for
such patients would markedly reduce costs. Debates focusing on minimal
differences in degrees and rate of improvement have detracted from the
appreciation of the treatment's value.

Current studies are evaluating regimens with dosages even lower than 30 U/kg/4
weeks. The patients in these studies may respond well, but some respond more
slowly. Initial and maintenance therapy should be directed at achieving
sustained benefit with the lowest possible dosage. The choice of dosage and
frequency of enzyme administration will have to be adjusted individually while
each patient's progress is monitored. Response may be slow regardless of
dosage.

Given the limited number of patients, the treatment strategies, including
criteria for intake, dosage, and periodic reevaluation, should be standardized
to ensure that data from multiple centers can be pooled to evaluate the
proposed treatment regimens. The resolution of these treatment issues can be
addressed best through carefully designed, cooperative, clinical trials. The
questions to be answered by such trials will be refined if existing data sets
are pooled and analyzed without preconceived constraints. In addition, further
studies should include the development of more efficient cellular targeting and
uptake of the enzyme. The clinical and ethical ramifications of enzyme therapy
and the funding of clinical trials must be considered.

Studies to evaluate alternative forms of enzyme replacement therapy and
alternative approaches, such as the use of inhibitors of sphingolipid
biosynthesis, should be encouraged. Moreover, Gaucher disease is an excellent
candidate for gene therapy, and continued research on this modality, including
the use of animals models, is therefore indicated.

What Are the Goals for and Consequences of Treatment, and How Can the
Therapeutic Interventions Be Assessed?

The goals of treatment are the amelioration of the manifestations of Gaucher
disease and the overall improvement of the health and quality of life of
patients.

Although alglucerase has been shown to ameliorate many of the manifestations of
type 1 Gaucher disease, the major current concerns are the proper indications
to begin treatment, the most appropriate treatment regimens, and cost.
Answering the many questions concerning the management of Gaucher disease will
require a cooperative effort of considerable scale. For this cooperative effort
to have its intended impact, the organizer of the cooperative effort must be
free of real or perceived bias. The National Institutes of Health should take
the initiative and foster the establishment of a cooperative group of
investigators involved in the diagnosis and treatment of patients with Gaucher
disease. Three phases in the operation of the proposed group are (1)
establishment of a patient registry, (2) analysis of the existing data on
natural history and response to therapy, and (3) design and conduct of clinical
trials to address unanswered questions.

It would be advantageous to enter all patients with Gaucher disease into a
registry. Such a registry would provide a valuable resource for increasing our
knowledge of the natural history of the disease, help to identify predictors of
response, and facilitate clinical trials to answer specific questions about
therapy.

Clinical trials will be most informative if recruitment numbers are adequate to
answer the questions addressed, if individuals are stratified for the most
relevant variables (e.g., genotypes or baseline levels) to ensure comparability
of the various subject groups, and if patients are randomized to treatment arms
where appropriate.

A high priority for an early clinical trial is comparison of the dosage and
frequency of enzyme administration to symptomatic patients. Outcomes to be
assessed should include not only hemoglobin concentration, platelet count,
spleen and liver size, and bone integrity but also the patients' functional
state, convenience, satisfaction, quality of life, impact on the family, and
cost.

A second priority for a clinical trial is to assess the need for enzymatic
treatment of asymptomatic patients. Such a trial might initially be confined to
high-risk, asymptomatic patients to increase the likelihood of observing a
preventive effect of treatment.

Under What Circumstances Could Genotype/Phenotype Correlations Be Used for
Patient Care and Counseling?

More than 50 mutations of the glucocerebrosidase gene have been identified.
Investigators are using disparate nomenclature for mutations with reference to
genomic DNA, cDNA, the exon involved, and the amino acid alteration in the
enzyme. For consistency and ease of communication, mutations should be
described using both the amino acid sequence and cDNA, when appropriate.

Most investigators have categorized their study populations into Ashkenazi
Jewish and non-Jewish cohorts. Four mutations are reported to account for 89-94
percent of type 1 Gaucher alleles in the Ashkenazi Jewish population and 60-75
percent of type 1 Gaucher alleles in non-Jewish populations. However, these
studies do not provide an unbiased estimate of the allele frequencies.
Therefore, accurate calculations of the number of individuals who carry the
disease genotype are not possible.

Concordance between the genotype and phenotype in Gaucher disease is imperfect.
Families with multiple affected members with different clinical presentations
and families having discordant identical twins further demonstrate the
imprecision of genotype/phenotype correlation. However, the following general
conclusions can be drawn based on current data.

  1. 1. Homozygosity for N370S (1226G) precludes neuronopathic involvement;
     that is, it produces type 1 disease only. Nevertheless, this genotype is
     present in individuals with considerable variability in expression,
     ranging from absence of signs and symptoms, to mild to moderate disease,
     to, less commonly, severe type 1 Gaucher disease.
  2. 2. Compound heterozygotes with one N370S (1226G) allele have non-
     neuronopathic Gaucher disease (the one exception a child with oculomotor
     involvement). These individuals generally have more severe type 1 disease
     than do N370S (1226G) homozygotes.
  3. 3. Homozygotes for L444P (1448C) in the Swedish Norrbottnian population
     generally present with neuronopathic type 3 disease of variable severity.
     This same genotype in the Japanese population is associated with
     non-neuronopathic disease, indicating that genotype/phenotype
     correlations, to the extent that they exist, may vary with genetic
     background.

The lack of predictability of phenotype from genotype suggests other genetic
and/or nongenetic effects on the phenotype. The opportunity exists to study
ethnic isolates, such as the Swedish Norrbottnian and Israeli Jenin Arab
populations, each with a single Gaucher genotype and variable expression, to
determine the nature of these other genetic and nongenetic factors.

This imperfect agreement between genotype and phenotype limits the ability to
establish the prognosis for individual patients and also restricts the
usefulness of genotyping for population screening and prenatal diagnosis. The
failure of genotype to predict phenotype complicates genetic counseling for
newly diagnosed patients and their families, and for prenatal diagnosis.

Testing has been conducted for affected individuals, for carriers, and for
prenatal diagnosis. Another reason for genotyping is to estimate carrier and
affected frequencies in various populations. Genotyping of anonymous unselected
populations is recommended to determine allele frequencies.

The benefits of general population screening for affected individuals are not
clear, because treatment of individuals with glucocerebrosidase deficiency who
do not have signs or symptoms has not yet been demonstrated to be necessary or
efficacious. In addition, the genotype offers limited prognostic information
for the individual. Extensive education of health care providers about Gaucher
disease should be initiated to ensure accurate and early diagnosis of
symptomatic patients.

Carrier screening has most commonly been conducted to provide reproductive
counseling and options to couples. This requires (1) a simple, accurate, and
relatively inexpensive test to identify most of the carriers (>95 percent
sensitivity) with few false positives (high specificity); (2) a disorder of
significant clinical severity; (3) a defined population for screening; (4) a
test that allows accurate prediction of the clinical course of the disease; (5)
a public and professional education program; and (6) informed consent for
screening. Although genotyping for Gaucher disease meets the test criteria,
genetic counseling can provide only a risk assessment of passing on the gene
but not a specific prognosis for future affected children. The uncertainty of
disease severity in each affected individual and the lack of public and
professional awareness of Gaucher disease argue against carrier screening in
the general population at this time. Cultural mores within specific communities
should be considered and may justify carrier screening. Carrier testing for
family members of affected individuals is appropriate. In addition,
peer-reviewed pilot studies of carrier screening programs may be of value.

Prenatal diagnosis for Gaucher disease is possible and allows couples at risk
to make informed reproductive decisions. Because of the considerable
variability in disease severity, personal experience with Gaucher disease in a
family member will influence the genetic counseling process and ultimate
decision. Genetic counseling is confounded by the inability to predict clinical
prognosis uniformly, by the heightened anxiety engendered by facing
probabilistic information, and by the availability of an encouraging but
extremely expensive enzyme replacement therapy. The description of the illness,
its manifestations, and its potential response to therapy all influence the
decisions made by couples, individuals, and families.

What Are Appropriate Directions for Future Research and Other Relevant Issues
That Should Be Pursued?

Nomenclature must be standardized throughout the field, from the clinical
setting to the molecular genetic laboratory. For example, a term as basic as
"asymptomatic" is used by workers in the field in very different ways. It is
recommended that the term asymptomatic be reserved for individuals who are
truly without symptoms. The mutation designation also needs to be standardized
for improved ease of communication.

A uniform clinical severity score for type 1 Gaucher disease must be developed
and formally validated to permit effective communication regarding the efficacy
of treatment. This clinical rating scale should be sensitive to both the signs
and the symptoms of the disorder.

A nationwide clinical database should be established that is independent of any
corporate entity, particularly those involved in the screening, diagnosis,
management, or treatment of Gaucher disease. This will facilitate research to
elucidate the natural history of the disease, identify key prognostic factors
for disease progression, and determine the influence of various therapies. The
database should accumulate extant information and collect missing data on
extant patients to permit evaluation of enzyme targeting, uptake efficiency,
optimal dosage and schedule, and clinical outcome. In the event of prenatal
diagnosis, the neonatal, childhood, adolescent, and adult course of the disease
should be carefully documented. Molecular genetic data should be determined and
correlated with the clinical information to refine the limits of
genotype/phenotype correlations. Such a database will permit the use of
mathematical modeling to investigate these questions. The information in the
database should be made available to investigators after formal review of their
research proposals. Within the database, it should be recognized that multiplex
families and identical twins represent unique groups for the study of other
factors influencing phenotype.

Nationwide, cooperative, controlled clinical trials should be conducted to
establish optimal regimens and to determine their efficacy for asymptomatic
individuals with minimal clinical signs. These clinical trials should be free
of influence from any commercial entity.

The available technology is not yet appropriate for large-scale,
population-based screening for Gaucher disease. However, we recommend that
additional pilot projects be supported, independent of corporate influence, to
identify carriers and affected individuals. Examples of potential projects
include estimation of the true prevalence of the various mutant alleles in
defined or mixed populations by anonymous testing; further evaluation of
educational needs within screened populations; and elucidation of the value of
enzyme delivery to asymptomatic, enzyme-deficient individuals with varying
clinical signs. Laboratories providing screening or diagnostic testing should
participate in national quality assurance/quality control programs.

Studies to better understand the basic biochemistry and cell biology of the
enzyme are needed. Identifying and developing animal models should be
encouraged. They provide opportunities to improve the understanding of the
pathogenesis and to test experimental strategies including gene therapy.

Enzyme replacement therapy clearly has improved the health and quality of life
of individuals with Gaucher disease. This treatment has been made possible
through the efforts of investigators at the National Institutes of Health and
other researchers nationally and internationally. The cooperative efforts of
government and industry have also proven effective. The contributions of the
patients and their families, who have participated so willingly in many
therapeutic trials, have been especially important.

The experience with enzyme replacement therapy to date has led to new
understanding about the disease and its treatment. At the same time, this
experience has raised concerns about the development of costly therapies for
disorders of very low prevalence. These concerns include the following:

   * Should the rights to exclusive marketing of an orphan drug be coupled to
     Federal approval of the price charged for the drug, and should uniform
     accounting practices be required?
   * Can such price regulation of orphan drugs be imposed without deterring the
     development of effective drugs for uncommon diseases?
   * How can it be ensured that the price citizens pay for a drug reflects the
     Federal contribution to its development?
   * Should society be informed of corporate and other relationships between
     entities engaged in screening and the manufacturers of therapeutic agents?
   * Should pharmaceutical companies and their representatives have direct
     access to patients?
   * Given the potentially extraordinary costs of treatment, how should the
     benefits to affected individuals and their families be balanced with the
     other health care needs of society?
   * Regarding costly treatments such as alglucerase, what are the implications
     for the future health care of patients and their families when insurance
     becomes exhausted and/or when coverage is provided by managed care systems
     employing fixed capitation limits?

Conclusions

The success of enzyme replacement therapy for Gaucher disease is a credit to
the investigators, the National Institutes of Health, the pharmaceutical
manufacturer, and the many patients and their families. Evidence presented at
this Technology Assessment Conference leads to the following conclusions:

   * Despite the success of enzyme therapy, treatment is limited by the cost of
     the agent.
   * The cost of the treatment makes it imperative to determine the lowest
     effective initial and maintenance dosages, to define the appropriate
     clinical indications for treatment, and to establish uniform methods in
     order to optimize outcome assessment.
   * The value of treatment for asymptomatic individuals has not been
     determined.
   *  General population screening for affected individuals and for carriers is
     not appropriate at this time.
   * As a prototype for all rare diseases, the plight of the patients with
     Gaucher disease raises difficult financial and ethical issues that we as a
     society must address.
!
*
                   Don't lose sight of glaucoma

                        [Graphic Omitted]

                  Information for people at risk

                      National Eye Institute

                   National Institutes of Health

                        Public Health Service

              U.S. Department of Health and Human Services

                     NIH Publication No. 91-3251


1. What is glaucoma ?


     Glaucoma is an eye disease in which the normal fluid
pressure inside the eyes slowly rises, leading to vision
loss-or even blindness. This brochure is about open-angle
glaucoma, the most common form of the disease.


2. What causes it?


     At the front of the eye, there is a small space called the
anterior chamber. Clear fluid flows in and out of the chamber
to bathe and nourish nearby tissues. In glaucoma, for still
unknown reasons, the fluid drains too slowly out of the eye. As
the fluid builds up, the pressure inside the eye rises. Unless
this pressure is controlled, it may cause damage to the optic
nerve and other parts of the eye and loss of vision.

[Graphic Omitted]

3. Who is most likely to get it?


     Nearly 3 million people have glaucoma, a leading cause of
blindness in the United States. Although anyone can get
glaucoma, some people are at higher risk. They include:

[Graphic Omitted]

   * Blacks over the age of 40.

   * Anyone over the age of 60.

   * People with a family history of glaucoma.

   * Among Blacks, studies show that glaucoma is:

   * Five times more likely to occur in Blacks than in Whites.

   * About four times more likely to cause blindness in Blacks
     than in Whites.

   * Fifteen times more likely to cause blindness in Blacks
     between the ages of 45-64 than in Whites of the same age
     group.


4. What are the symptoms?


     At first, there are no symptoms. Vision stays normal, and
there is no pain. However, as the disease progress, a person
with glaucoma may notice his or her side vision gradually
failing. That is, objects in front may still be seen clearly,
but objects to the side may be missed. As the disease worsens,
the field of vision narrows and blindness results.


5. How is it detected?


     Many people may know of the "air puff" test or other tests
used to measure eye pressure in an eye examination. But, this
test alone cannot detect glaucoma. Glaucoma is found most often
during an eye examination through dilated pupils. This means
drops are put into the eyes during the exam to enlarge the
pupils. This allows the eye care professional to see more of
the inside of the eye to check for signs of glaucoma.


6. How can it be treated?


     Although open-angle glaucoma cannot be cured, it can
usually be controlled. The most common treatments are:


Medications


     These may be either in the form of eyedrops or pills. Some
drugs are designed to reduce pressure by slowing the flow of
fluid into the eye. Others help to improve fluid drainage.

     For most people with glaucoma, regular use of medications
will control the increased fluid pressure. But, these drugs may
stop working over time. Or, they may cause side effects.

     If a problem occurs, the eye care professional may select
other drugs, change the dose, or suggest other ways to deal
with the problem.


Laser Surgery


     During laser surgery, a strong beam of light is focused on
the part of the anterior chamber where the fluid leaves the
eye. This results in a series of small changes, which makes it
easier for fluid to exit the eye. Over time, the effect of
laser surgery may wear off. Patients who have this form of
surgery may need to keep taking glaucoma drugs.


Surgery


     Surgery can also help fluid escape from the eye and
thereby reduce the pressure. However, surgery is usually
reserved for patients whose pressure cannot be controlled with
eyedrops, pills, or laser surgery.


7. What research is being done?


     A large amount of research is being done in the U.S. to
learn what causes glaucoma and to improve its diagnosis and
treatment. For instance, the National Eye Institute (NEI) is
funding a number of studies to find out what causes fluid
pressure to increase in the eye. By learning more about this
process, doctors may be able to learn the exact cause of the
disease and learn better how to prevent and treat it. The NEI
also supports clinical trials of new drugs and surgical
techniques that show promise against glaucoma.


8. What can you do to protect your vision


     Studies have shown that early detection and treatment of
glaucoma, before it causes major vision loss, is the best way
to control the disease. So, if you fall into one of the
high-risk groups for the disease, make sure to have your eyes
examined through dilated pupils every two years by an eye care
professional.


To learn more about glaucoma, write:

National Eye Health Education Program,
Box 20/20, Bethesda, MD 20892.

[Graphic Omitted]

NATIONAL EYE HEALTH EDUCATION PROGRAM
!
*
Primary Open Angle Glaucoma

Glaucoma is is one of the leading causes of irreversable blindness in America
and effects one in every hundred people.  It is a disease where the optic nerve
is damaged which results in loss of vision.  If untreated, it often leads to
blindness, but in the majority of cases, treatment can be effective in stopping
the progression of vision loss.

Unfortunately, in most cases, we cannot restore vision that has been lost, but
we are relatively effective with both medical and surgical techniques in
preventing further visual loss.

Glaucoma occurs in all races of people.  However it is much more common in
African Americans than in whites.   Primary open angle glaucoma is unusual in
people under 40 years of age but the risk increases with increasing years.  At
40 - 50 years the risk is about one in one hundred increasing to about one in
ten by age 80.  It is also more common in diabetics, hypertensives, the near
sighted and those who have had trauma to their eyes (often forgotten because
the trauma may have occurred many years before onset).  A family history of
glaucoma increases the risk fourfold.

The eye is a closed system where a nutritional fluid (known as aquous humor)
is made by the eye.  There is also a drain, called the trabecular meshwork
which allows the fluid to exit.  In glaucoma, as one gets older or due to some
other causes, the drain of the eye does not work as it should, the fluid backs
up and the pressure in the eye rises.  If one considers the eye to be like a ball,
as the pressure rises, increased pressure is placed on the wall of the eye.
There is only one weak spot in the wall where the optic nerve is located.  The
optic nerve is like a bundle of wires originating in the retina going to the brain.
In order to exit the eye, there has to be a weak spot in the wall and this is the
sight of damage due to increased eye pressure from glaucoma.  As in a ball
with a weak spot, it bulges.   When this bulge occurs, there is a kinking of
these "wires" that compress the optic nerve.  This causes them to be damaged.
For both good and bad, the damage tends to affect your peripheral vision first.
This damage usually does not occur very rapidly, and often it is not noticed by
the patient.  That is the reason we need to do specific tests to see if any
damage has occurred to the peripheral vision.  If the pressure remains
elevated, continued damage will occur until finally the center of vision (which
contains the ability to read) is finally affected.  The eye pressure at which this
damage occurs is different in all people, but in general the more damage that
has occurred, like a snowball rolling down a hill, the easier it is for more
damage to occur and lower pressures are necessary.  If untreated, glaucoma
does lead to blindness.
!
*
GONORRHEA

Nearly 750,000 cases of gonorrhea are reported annually in the
United States.  Another 750,000 unreported cases, mostly among
teenagers and young adults, are believed to occur each year. The
annual cost of gonorrhea and its complications is estimated at
close to $1 billion.

Gonorrhea is caused by the gonococcus, a bacterium that grows and
multiplies quickly in moist, warm areas of the body such as the
cervix, urethra, mouth, or rectum.  In women, the cervix is the
most common site of infection.  However, the disease can spread
to the uterus (womb) and fallopian tubes, resulting in pelvic
inflammatory disease (PID); this can cause infertility and
ectopic (tubal) pregnancy.

Gonorrhea is most commonly spread during genital contact, but it
can also be passed from the genitals of one partner to the throat
of the other during oral sex (pharyngeal gonorrhea).  Gonorrhea
of the rectum can occur in people who practice anal intercourse
and may also occur in women due to spread of the infection from
the vaginal area.

Gonorrhea can be passed from an infected woman to her newborn
infant during delivery.  When the infection occurs in children,
it is most commonly due to sexual abuse.

Symptoms

The early symptoms of gonorrhea often are mild, and most women
who are infected have no symptoms of the disease.  If symptoms of
gonorrhea develop, they usually appear within 2 to 10 days after
sexual contact with an infected partner, although a small
percentage of patients may be infected for several months without
showing symptoms.  The initial symptoms in women include a
painful or burning sensation when urinating or an abnormal
vaginal discharge.  More advanced symptoms, which indicate
progression to PID, include abdominal pain, bleeding between
menstrual periods, vomiting, or fever.  Men usually have a
discharge from the penis and a burning sensation during urination
that may be severe.  Symptoms of rectal infection include
discharge, anal itching, and sometimes painful bowel movements.
Diagnosis

Two techniques, gram stain and culture, are generally used to
diagnose gonorrhea.  Many doctors prefer to use both tests to
increase the chance of an accurate diagnosis.  The gram stain is
quite accurate for men but less so for women.  The test involves
placing a smear of the discharge on a slide that has been stained
with a dye and then examining it under a microscope for the
presence of the gonococcus.  Test results usually can be given to
the patient at the time of an office or clinic visit.

The culture test, the preferred method of diagnosing gonorrhea in
women, involves placing a sample of the discharge onto a culture
plate and incubating it for up to 2 days to allow the bacteria to
multiply.  The accuracy of this test depends on the site from
which the sample is taken.  Cervical samples are accurate, for
example, approximately 90 percent of the time.  Throat cultures
can also be done to detect pharyngeal gonorrhea.

Treatment

Ampicillin, amoxicillin, or some type of penicillin used to be
recommended for the treatment of gonorrhea.  Because penicillin-
resistant gonorrhea is increasing, other antibiotics that are
given by injection such as ceftriaxone or spectinomycin now are
used to treat most gonococcal infections.  Other new antibiotics
can be taken by mouth.

Gonorrhea often occurs together with chlamydial infection,
another common sexually transmitted disease (STD).  Therefore,
doctors usually prescribe a combination of antibiotics, such as
ceftriaxone and doxycycline, to treat both diseases.  Women who
are pregnant should not take doxycycline and are usually given an
alternative antibiotic such as erythromycin.

Regardless of what drug is prescribed, it is important that the
patient take the full course of medication and that he or she
return to the doctor's office or clinic for followup.  All sex
partners of a person with gonorrhea should be tested and treated
appropriately even if they do not have symptoms of infection.

Complications

If gonorrhea is not treated, the bacteria can spread to the
bloodstream and infect the joints, heart valves, or the brain.
The most common consequence of gonorrhea, however, is PID, a
serious infection of the female reproductive organs, that occurs
in an estimated 1 million American women each year.  PID can scar
or damage cells lining the fallopian tubes, resulting in
infertility in as many as 10 percent of women affected.  In
others, the damage prevents the proper passage of the fertilized
egg into the uterus.  If this happens, the egg may implant in the
tube; this is called an ectopic or tubal pregnancy and is life-
threatening to the mother if not detected early.

An infected woman who is pregnant may give the infection to her
infant as the baby passes through the birth canal during
delivery.  Most states require that the eyes of newborns be
treated with silver nitrate or other medication immediately after
birth to prevent gonococcal infection of the eyes, which can lead
to blindness.  Because of the risk of gonococcal infection to
both mother and child, doctors recommend that a pregnant woman
have at least one test for gonorrhea during her pregnancy.

Prevention

Because gonorrhea is highly contagious and yet may cause no
symptoms, all men and women who have sexual contact with more
than one partner should be tested regularly for the disease.
Using condoms (rubbers) during sexual intercourse is very
effective in preventing the spread of infection.  Diaphragms may
also reduce the risk of transmission.  Constant awareness and
precautions are necessary because a person who has once
contracted the disease does not become immune--many people
acquire gonorrhea more than once.

Research

Scientists supported by NIAID are continuing to learn more about
the organism that causes gonorrhea and are working on better
methods to prevent, diagnose, and treat it.  The dramatic rise of
antibiotic-resistant strains of the gonococcus underscores the
need for a means of preventing gonorrhea.  A method has been
developed to detect these resistant strains, which helps the
physician select an appropriate treatment.

An effective vaccine against gonorrhea remains a key research
priority for NIAID- supported scientists.  This work is
complicated by the fact that the gonococcus is a complex organism
that infects only humans.  It has a remarkable ability to protect
itself by changing or mutating.  The gonococcus also uses a
protein on its surface that "tricks" the immune system into
protecting the invading organism.  Excluding this protein from
any future vaccine will be critical.  Several experimental
vaccines are being developed by NIAID-supported investigators.

By using protective measures during sexual activity, by being
tested for gonorrhea whether or not symptoms are present, by
taking medication promptly and properly if infection occurs, and
by abstaining from intercourse until the infection is cured,
sexually active people can reduce their risk of gonorrhea and its
complications.


Prepared by:
Office of Communications
National Institute of Allergy and Infectious Diseases
National Institutes of Health
Bethesda, Maryland 20892

U.S. DEPARTMENT OF HEALTH AND HUMAN SERVICES Public Health
Service, August 1992
!
*
Gout
Symptoms
--------

Pseudogout can appear in a chronic or acute form, or a
combination of these two types.

About one-fourth of patients with Pseudogout experience one or
more acute (sudden) attacks characterized by rapid swelling and
pain in a joint, stiffness and occasional redness and heat over
the affected area.  An attack usually reaches its peak within
12 to 36 hours and can last several days to weeks.  In most
people the symptoms of acute attacks will disappear eventually
even without treatment.  The most commonly affected joint is
the knee, although any joint may be affected.

The milder non-acute forms of the disorder are more likely to
occur in several joints such as the wrists and fingers, as well
as the knee.  Pain and swelling occur, not as severe as in a
sudden attack, but they tend to last longer. People with
chronic Pseudogout find that pain becomes worse with activity.
Some experience acute attacks as well as the chronic form,
while others with the chronic form may never experience an
acute attack.

General Discussion
------------------

Pseudogout is a disorder resembling gout, characterized by
deposits of calcium pyrophosphate dihydrate crystals in a
single joint or multiple joints which causes mild chronic
inflammation of those joints.

Causes
------

The cause of Pseudogout is unknown.  Its frequent association
with other conditions such as osteoarthritis, diabetes
mellitus, hyperparathyroidism, gout or hemochromatosis suggests
that the deposits of the CPPD crystals in the cartilage are
secondary to degenerative changes in the joints.  A familial
pattern of incidence has been observed in several countries.


Related Disorders
-----------------

Gout is a recurrent acute arthritis of peripheral joints which
results from deposition, in and about the joints and tendons,
of monosodium urate crystals from supersaturated hyperuricemic
body fluids.  This disorder may become chronic and deforming,
but in most cases it is responsive to drugs and diet therapy.

Therapies:  Standard
--------------------

Acute attacks of Pseudogout are treated by removing the excess
fluid and CPPD crystals from the affected joint.  The drugs
used most often for treatment of Pseudogout are aspirin and the
other non-steroidal anti-inflammatory drugs such as those
commonly prescribed for many types of arthritis.  These drugs
reduce the pain and swelling caused by inflammation.  Stomach
upset is a common side effect of these drugs which may be
avoided if they are taken with milk or food.

If an acute attack has occurred in only one joint, a
corticosteroid may be injected into the joint.

During an attack of Pseudogout the affected joint may need
rest. Splints, canes and other devices for protecting joints
may be fitted.  Once the episode subsides, or in cases of the
milder chronic form, rest should be balanced with appropriate
exercise prescribed by a doctor or a physical therapist.

In a very few people with Pseudogout, surgery may be necessary
to correct a joint that is badly damaged, very painful or
unstable.  Surgery can be performed either to repair a joint or
to replace it entirely with an artificial joint. Surgery can be
effective for reducing pain, improving movement and correcting
disability.

Therapies:  Investigational
---------------------------

Researchers at the Mayo Clinic in Rochester, MN have tested the
experimental anti-inflammatory drug colchicine as a treatment
in severe cases of Pseudogout when symptoms did not respond to
any other treatment.  More research is needed before this drug
can be recommended for treatment of Pseudogout.

This disease entry is based upon medical information available
through June 1988.

Resources
---------

For more information on Pseudogout, please contact:

National Organization for Rare Disorders (NORD)
P.O. Box  8923
New Fairfield, CT  06812-1783
[phone]
[phone] (TDD for the hearing impaired)

The Arthritis Foundation
1314 Spring St., NW
Atlanta, GA  30309
[phone]
[phone]

NIH/National Arthritis and Musculoskeletal and Skin Diseases (NIAMS) Information
Clearinghouse
9000 Rockville Pike
Bethesda, MD  20892
[phone]


References
----------

Pseudogout.  Schumacher: Arthritis Medical Information Series (1983).

THE MERCK MANUAL 15th ed:  R. Berkow, et al: eds; Merck, Sharp
& Dohme Research Laboratories, 1987.  P. 1266.

CECIL TEXTBOOK OF MEDICINE, 18th ed.:  James B. Wyngaarden, and
Lloyd H. Smith, Jr., Eds.:  W. B. Saunders Co., 1988.  P. 2037.
!
*
Guillain-Barre syndrome


Definition:
Inflammation of nerves (neuritis) associated with fever.


Description:
Guillain-Barre' syndrome (GBS) is also known as acute
demyelinating polyneuropathy because of its adverse effect on
myelin, a sheath that protects normal nerve tissue.

This acute illness is frequently severe and fulminant and
occurs at a rate of one case per million population per month.
This represents approximately 3,500 cases per year in the
United States and Canada.  In over two-thirds of cases GBS a
viral infection precedes the onset of the disease by 1-3 weeks.

Herpes infections, cytomegalovirus, or Epstein-Barr virus
(causative agent in mononucleosis) account for a large
percentage of viral-triggered cases. Approximately 5-10% of GBS
cases can occur within 1-4 weeks of a surgical procedure or
associated with lymphoma, Hodgkin's disease, or systemic lupus
erythematosus. GBS is thought to be somehow immune-mediated but
the exact pathogenesis of the disease remains unknown.

The signs and symptoms of GBS include absent reflexes and
progressive limb paralysis.  There may also be some mild
disturbances in sensation.  Eventual involvement of the
respiratory muscles is also seen.

Most patients with GBS will require hospitalization and about
one-quarter will require respiratory support on a mechanical
ventilator.  The prognosis is good with approximately 85% of
patients making a complete recovery.  The mortality rate is 3-
4%.  Management is largely supportive, but a technique of
filtering the blood known as plasmapheresis has shown to be
effective.  Treatment with steroids has been used in the past
but a beneficial role has yet to be proven.


___________________________________________________________
                ADDITIONAL REFERENCES
___________________________________________________________


InfoTrac * Health Reference Center ■ Dec '91 - Dec '94

  THE MATERIAL CONTAINED IN HEALTH REFERENCE CENTER IS PROVIDED
  ONLY FOR INFORMATIONAL PURPOSES AND SHOULD NOT BE CONSTRUED AS
  MEDICAL ADVICE OR INSTRUCTION.  CONSULT YOUR HEALTH PROFESSIONAL
  FOR ADVICE RELATING TO A MEDICAL PROBLEM OR CONDITION.

 Heading:   GUILLAIN-BARRE SYNDROME
             ■ Dictionary definition
     DEFINITION (15 lines)
       Mosby's Medical, Nursing, and Allied Health Dictionary, 4th Edition
             COPYRIGHT 1994 by Mosby-Year Book, Inc.
    Guillain-Barre syndrome
    ------------------------------------------------------------------------
    an idiopathic, peripheral polyneuritis occurring between 1 and 3 weeks
    after a mild episode of fever associated with a viral infection or with
    immunization. Symmetric pain and weakness affect the extremities, and
    paralysis may develop. The neuritis may spread, ascending to the trunk
    and involving the face, arms, and thoracic muscles. The course of the
    disease is variable; some people may have minimal symptoms, whereas
    others may have symptoms severe enough to require critical nursing care,
    including respiratory assistance and a CircOlectric  bed. The disease
    resolves itself completely in a few weeks or a few months. There is no
    treatment other than supportive care. Also called acute febrile
    polyneuritis, acute idiopathic polyneuritis, infectious polyneuritis.
     -END-

InfoTrac * Health Reference Center ■ Dec '91 - Dec '94

  THE MATERIAL CONTAINED IN HEALTH REFERENCE CENTER IS PROVIDED
  ONLY FOR INFORMATIONAL PURPOSES AND SHOULD NOT BE CONSTRUED AS
  MEDICAL ADVICE OR INSTRUCTION.  CONSULT YOUR HEALTH PROFESSIONAL
  FOR ADVICE RELATING TO A MEDICAL PROBLEM OR CONDITION.

 Heading:   POLYRADICULONEURITIS
    12.    Guillain-Barre syndrome. v16 Medical Update June
       '93 p6(1)
        TEXT / HEADINGS
     TEXT (16 lines)
    COPYRIGHT 1993 Medical Education and Research Foundation
      Guillain-Barre syndrome (GBS) is characterized by muscular weakness
    and mild sensory loss. Although the cause is unknown, usually the
    syndrome begins shortly after a person experiences an infection,
    surgery, or an immunization.
      Weakness usually begins in the legs and progresses to the arms. Ninety
    percent of GBS patients reach their worst state of the syndrome in the
    second or third week after symptoms start.
      Some patients may have symptoms of unsteadiness in walking and severe
    loss of tactile sensation. About 10 percent of patients relapse after
    initial improvement and enter a chronic state. However, the patient
    normally experiences considerable improvement over a period of months.
      Convalescence  may  last  for months or years and should include
    physical therapy. Although 30 percent of adults with the syndrome still
    have residual weakness that may require retraining, use of orthopedic
    appliances, or operations, most make an almost complete recovery.
     -END-

InfoTrac * Health Reference Center ■ Dec '91 - Dec '94

 Heading:   POLYRADICULONEURITIS
    21.    The Guillain-Barre syndrome. (Review
       Article)(Current Concepts) by Allan H. Ropper il v326
       The New England Journal of Medicine April 23 '92
       p1130(7)
        ABSTRACT / HEADINGS / IN LIBRARY
     ABSTRACT (22 lines)
        The neurological disorder Guillain-Barre syndrome
    is the most common cause of generalized paralysis.
    Patients with Guillain-Barre syndrome experience
    muscle weakness, pain and paralysis. Extreme cases can
    affect breathing, eye movement and the autonomic
    nervous system; about 3% to 8% of patients die from
    complications. For these reasons, patients with
    Guillain-Barre syndrome should be watched closely in
    the hospital. Diagnosis of Guillain-Barre syndrome can
    be confirmed by evaluation of nerve conduction and
    examination of cerebrospinal fluid. Approximately two-
    thirds of Guillain-Barre patients develop the disorder
    after a viral infection, and some have an underlying
    condition such as cancer. Most Guillain-Barre patients
    recover within weeks or months, but have long-lasting,
    usually minor complications. Activated T-cells, or
    cells of the immune system, may be responsible for the
    inflammation that occurs in the protective sheath that
    surrounds nerve cells. Research indicates that plasma
    exchange and infusion of gamma globulin may be more
    effective therapy than the no-longer-used
    corticosteroid treatment.
     -END-

InfoTrac * Health Reference Center ■ Dec '91 - Dec '94

  THE MATERIAL CONTAINED IN HEALTH REFERENCE CENTER IS PROVIDED
  ONLY FOR INFORMATIONAL PURPOSES AND SHOULD NOT BE CONSTRUED AS
  MEDICAL ADVICE OR INSTRUCTION.  CONSULT YOUR HEALTH PROFESSIONAL
  FOR ADVICE RELATING TO A MEDICAL PROBLEM OR CONDITION.

 Heading:   POLYRADICULONEURITIS
     1.    Guillain-Barre syndrome. (Brain, Nerve, and Muscle
       Disorders) by Timothy A. Pedley The Columbia Univ.
       Coll. of Physicians & Surgeons Complete Home Medical
       Guide Edition 2 '89 p639(1)
        TEXT / HEADINGS
     TEXT (22 lines)
    COPYRIGHT 1989 Crown Publishers Inc.
      Guillain-Barre, Syndrome
      The Guillain-Barre syndrome is an illness that usually occurs one to
    two weeks after a mild viral infection such as a sore throat,
    bronchitis, or flu. Symmetric weakness of the limbs develops over a few
    days, sometimes progressing to complete paralysis. The face muscles may
    be paralyzed as well, making it impossible to swallow normally. In
    severe cases, paralysis of respiratory muscles requires tracheostomy and
    artificial ventilation. With intensive medical treatment and support,
    the majority of patients recover, but about one-third are left with some
    residual weakness.
      The illness results from inflammation and destruction of myelin
    similar to that seen in multiple sclerosis. The main difference,
    however, is that multiple sclerosis attacks the central nervous system,
    whereas in the Guillain-Barre syndrome it is the peripheral nerves that
    are affected. Another difference is that Guillain-Barre syndrome does
    not recur except in rare instances. The nerve damage is thought to be
    the result of an abnormal immune reaction directed against the myelin of
    the peripheral nervous system. Diagnosis is made by the clinical
    features, characteristic changes in cerebrospinal fluid, and electrical
    studies of the peripheral nerves and muscles, a procedure known as
    electromyography (EMG).
     -END-
!
*
FACT SHEET: What is Guillain-Barre Syndrome?

Guillain-Barre (ghee-yan bah-ray) syndrome is a disorder in which
the body's immune system attacks part of the nervous system.  The
first symptoms of this disorder include varying degrees of weakness
or tingling sensations in the legs.  In many instances the weakness
and abnormal sensations spread to the arms and upper body.  These
symptoms can increase in intensity until the muscles cannot be used
at all and the patient is almost totally paralyzed.  In these cases
the disorder is life threatening - potentially interfering with
blood pressure, heart rate, and breathing - and is considered a
medical emergency.  The patient is often put on a respirator to
assist with breathing and is watched closely for problems such as
an abnormal heart beat, infections, blood clots, and high or low
blood pressure.  Most patients, however, recover from even the most
severe cases of Guillain-Barre syndrome, although some continue to
have minor problems.

Guillain-Barre syndrome can affect anybody.  It can strike at any
age and both sexes are equally prone to the disorder.  The syndrome
is rare, however, afflicting only about one person in 100,000.
Usually Guillain-Barre occurs a few days or weeks after the patient
has had symptoms of a respiratory or gastrointestinal viral
infection.  Occasionally pregnancy, surgery, or vaccinations will
trigger the syndrome.  The disorder can develop over the course of
hours or days, or it may take up to 3 to 4 weeks.  Most people
reach the stage of greatest weakness within the first 2 weeks after
symptoms appear, and by the third week of the illness 90 percent of
all patients are at their weakest.

What causes Guillain-Barre syndrome?

No one yet knows why Guillain-Barre strikes some people and not
others.  Nor does anyone know exactly what sets the disease in
motion.  What scientists do know is that the body's immune system
begins to attack the body itself, causing what is known as an
autoimmune disease.   Usually the cells of the immune system attack
only foreign material and invading organisms.  In Guillain-Barre
syndrome, however, the immune system starts to destroy the myelin
sheath that surrounds the axons of many nerve cells, or even the
axons themselves (axons are long, thin extensions of the nerve
cells; they carry nerve signals).  The myelin sheath surrounding
the axon speeds up the transmission of nerve signals and allows the
transmission of signals over long distances.

In diseases in which the nerve cells' myelin sheaths are injured or
degraded, the nerves cannot transmit signals efficiently.  That is
why the muscles begin to lose their ability to respond to the
brain's commands, commands that must be carried through the nerve
network.  The brain also receives fewer sensory signals from the
rest of the body, resulting in an inability to feel textures, heat,
pain, and other sensations.  Alternately, the brain may receive
inappropriate signals that result in tingling, "crawling-skin," or
painful sensations.  Because the signals to and from the arms and
legs must travel the longest distances they are most vulnerable to
interruption.  Therefore, muscle weakness and tingling sensations
usually first appear in the hands and feet.

When Guillain-Barre is preceded by a viral infection, it is
possible that the virus has changed the nature of cells in the
nervous system so that the immune system treats them as foreign
cells.  It is also possible that the virus makes the immune system
itself less discriminating about what cells it attacks.  Scientists
are investigating these possibilities and others to find why the
immune system goes awry in Guillain-Barre syndrome and other
autoimmune diseases.  The cause and course of Guillain-Barre
syndrome is an active area of neurological investigation,
incorporating the cooperative efforts of neurological scientists,
immunologists, and virologists.

How is Guillain-Barre syndrome diagnosed?

Guillain-Barre is called a syndrome rather than a disease because
it is not clear that a specific disease-causing agent is involved.
A syndrome is a medical condition characterized by a collection of
symptoms (what the patient feels) and signs (what a doctor can
observe or measure).  Because the signs and symptoms of the
syndrome can be quite varied, doctors may find it difficult to
diagnose Guillain-Barre in its earliest stages.

Several disorders have symptoms similar to those found in Guillain-
Barre, so doctors examine and question patients carefully before
making a diagnosis.  Collectively, the signs and symptoms form a
certain pattern that helps doctors differentiate Guillain-Barre
from other disorders.  For example, physicians will note whether
the symptoms appear on both sides of the body (most common in
Guillain-Barre) and the quickness with which the symptoms appear
(in other disorders muscle weakness may progress over months rather
than days or weeks).  In Guillain Barre, reflexes such as knee
jerks are usually lost.  Because the signals traveling along the
nerve are slower, a nerve conduction velocity (NCV) test can give
a doctor clues to aid the diagnosis.  In Guillain-Barre patients,
the cerebrospinal fluid that bathes the spinal cord and brain
contains more protein than usual.  Therefore a physician may decide
to perform a spinal tap, a procedure in which the doctor inserts a
needle into the patient's lower back to draw cerebrospinal fluid
from the spinal column.  Laboratory scientists, working with
clinical neurologists, are conducting research that may help
provide physicians with more precise and reliable diagnostic tests
for this disorder.

How is Guillain-Barre treated?

There is no known cure for Guillain-Barre syndrome.  However, there
are therapies that lessen the severity of the illness in most
patients, and there are a number of ways to treat the complications
of the disease.

Currently, plasmapheresis and high-dose immunoglobulin therapy are
used in the more serious cases of Guillain-Barre syndrome.
Plasmapheresis is a method by which whole blood is removed from the
body and processed so that the red and white blood cells are
separated from the plasma, or liquid portion of the blood.  The
blood cells are then returned to the patient without the plasma,
which the body quickly replaces.  Scientists still don't know
exactly why plasmapheresis works, but the technique seems to reduce
the severity and duration of the Guillain Barre episode.  This may
be because the plasma portion of the blood contains elements that
the immune system needs to function.  When these elements are
removed along with the plasma, the immune system is not able to
attack the nervous system as effectively.

In high-dose immunoglobulin therapy, doctors give intravenous
injections of the proteins that the immune system uses to attack
invading organisms.  Investigators have found that these
immunoglobulins, when given to Guillain-Barre patients, can lessen
the immune attack on the nervous system.  Investigators don't know
why this is, but some suggest that the immunoglobulins may
overwhelm the immune system and keep it from attacking the nerve
cells and their myelin sheaths.

The use of steroid hon-nones has also been tried as a way to reduce
the severity of Guillain-Barre, but controlled clinical trials have
not demonstrated that this treatment is effective.

Much of the treatment for this syndrome consists of keeping the
patient's body functioning during recovery of the nervous system.
This can sometimes require placing the patient on a respirator, a
heart monitor, or other machines that assist body function.  The
need for this sophisticated machinery is one reason why Guillain-
Barre syndrome patients are usually treated in hospitals, often in
an intensive care ward.  In the hospital, doctors can also look for
and treat the many problems that can afflict any paralyzed patient-
complications such as pneumonia or bed sores.

Often, even before recovery begins, caregivers may be instructed to
manually move the patient's limbs to help keep the muscles flexible
and strong.  Later, as the patient begins to recover limb control,
physical therapy begins.  Carefully planned clinical trials of new
and experimental therapies are the key to improving the treatment
of patients with Guillain-Barre syndrome.  Such clinical trials
begin with the research of basic and clinical scientists who,
working with clinicians, identify new approaches to treating
patients with the disease.

What is the long-term outlook for those with Guillain-Barre
syndrome?

Guillain-Barre syndrome can be a devastating disorder because of
its sudden and unexpected onset.  In addition, recovery is not
necessarily quick.  As noted previously, patients usually reach the
point of greatest weakness or paralysis days or weeks after the
first symptoms occur.  Symptoms then stabilize at this level for a
period of days, weeks, or, sometimes, months.  The recovery period
may be as little as a few weeks or as long as a few years.  About
30 percent of those with Guillain-Barre still feel a residual
weakness after 3 years.  About 3 to 5 percent may suffer a relapse
of muscle weakness and tingling sensations many years after the
initial attack.

Guillain-Barre syndrome patients face not only physical
difficulties, but emotionally painful periods as well.  It is often
extremely difficult for patients to adjust to sudden paralysis and
dependence on others for help with routine daily activities.
Patients sometimes need psychological counseling to help them
adapt.

What research is being done?

Scientists are concentrating on finding new treatments and refining
existing ones.  Scientists are also looking at the workings of the
immune system to find which cells are responsible for beginning and
carrying out the attack on the nervous system.  The fact that so
many cases of Guillain-Barre begin after a viral infection suggests
that certain characteristics of these viruses may activate the
immune system inappropriately.  Investigators are searching for
those characteristics.  As noted previously, neurological
scientists, immunologists, virologists, and pharmacologists are all
working collaboratively to learn how to prevent this disorder and
to make better therapies available when it strikes.

Where can I go for more information?

The National Institute of Neurological Disorders and Stroke
conducts and supports a wide range of research on neurological
disorders, including Guillain-Barre syndrome.  For more information
on this or other neurological disorders, or on the Institute and
its research programs, contact:

Office of Scientific and Health Reports
Neurological Institute
P.O. Box 5801
Bethesda, MD 20824
[phone]
[phone]


The organization listed below provides printed information and
assistance to Guillain-Barre patients and other interested parties.

Guillain-Barre Syndrome Foundation
International
P.O. Box 262
Wynnewood, PA 19096
[phone]

September 1992

Prepared by
Office of Scientific and Health Reports
National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, Maryland 20892

!
*
HANTAVIRUS ILLNESS IN THE UNITED STATES

CENTERS FOR DISEASE CONTROL AND PREVENTION
Date Last Rev'd: March 9, 1995

HANTAVIRUS ILLNESS IN THE UNITED STATES


An  outbreak of unexplained illness occurred in the Southwestern part of  the
United  States  in  1993.  Laboratory findings from the Centers  for  Disease
Control and Prevention indicate that the illness is caused by a hantavirus.
The   newly   recognized  hantavirus-associated  disease,  called  Hantavirus
Pulmonary  Syndrome (HPS), begins with one or more symptoms including  fever,
severe  muscle  aches, headache, and cough which progress rapidly  to  severe
lung disease, often requiring intensive care treatment.

Since the hantavirus was discovered in 1993, less than 100 cases of HPS  have
been identified in 20 states.  These states encompass the western half of the
United States and most recently a few eastern states as well.  Over half  the
people who get HPS die from the illness.  In 1994, about two dozen cases have
been  confirmed.   It is predicted that less than 50 cases  will  occur  this
year.   So far the earliest known case dates back to 1975.  Almost all  cases
have had evidence of close contact with rodents (deer mice or cotton rats).

Rodents are the primary reservoir host of the recognized hantaviruses.   Each
hantavirus  appears to have a preferred rodent host, but other small  mammals
can  be infected as well.  Data strongly suggests that the deer mouse is  the
primary  carrier  of the hantavirus seen in all parts of  the  United  States
except the  Eastern Coast and the Southeast. In the Southeast, the cotton rat
has  been  identified as a carrier for the virus causing  HPS.   Evidence  of
infection  has  also  been  found  in pinon  mice,  brush  mice  and  western
chipmunks.   The  deer mouse is highly adaptable and is  found  in  different
habitats,  including  human  residences in rural  and  semirural  areas,  but
generally not in urban centers.

Hantaviruses  do  not cause obvious illness in their rodent hosts.   Infected
rodents  shed  virus  in saliva, urine, and feces for  many  weeks,  but  the
duration and period of maximum infectivity are unknown.

Human  infection may occur when infective saliva or excreta  are  inhaled  as
aerosols produced directly from the animal.  Transmission may also occur when
fresh  or  dried  materials  contaminated by rodent  excreta  are  disturbed,
directly introduced into broken skin, introduced into the eyes, or, possibly,
ingested  in  contaminated food or water.  Persons have also become  infected
after being bitten by rodents.

Ticks, fleas, mosquitos and other biting insects are not known to have a role
in  the transmission of hantaviruses.  Person-to-person transmission has  not
been  associated with any of the previously identified hantaviruses  or  with
the  recent outbreak in the Southwest.  Cats and dogs are not known to  be  a
reservoir host of hantaviruses in the United States.  However, these domestic
animals may bring infected rodents into contact with humans.

Hantavirus  pulmonary syndrome does not appear to be limited to a  particular
age, race, ethnic group, or gender.  The chance of exposure to hantavirus  is
greatest when individuals work, play or live in closed spaces where there  is
an active rodent infestation.  It is important to be aware of possible rodent
exposure,  for  example,  when working in crawl spaces,  opening  phone  line
stations or using air condition equipment after winter storage.

Travel  to and within all areas where hantavirus infection has been  reported
is  safe.  The possibility of exposure to hantavirus for campers, hikers, and
tourists  is  very small and reduced even more if steps are taken  to  reduce
rodent contact.

Cleaning of areas with small numbers of rodents should include wearing  latex
or  rubber  gloves  and  wetting down affected areas with  general  household
disinfectant solutions such as Lysol or bleach and water or Amonia.  Cleaning
of  areas  with  large numbers of rodents includes wearing  latex  or  rubber
gloves,  goggles,  HEPAfilter mask and wetting  the  area  with  disinfectant
solutions or bleach and water.

Remember  that the chances of getting HPS are very low.  However, if  you  do
get the disease, it can be very serious.


RECOMMENDATIONS FOR PREVENTION AND CONTROL

Summary
   This report provides interim recommendations for prevention and control of
hantavirus  infections  associated with rodents in  the  southwestern  United
States.  It  is  based  on  principles of rodent and  infection  control  and
contains  specific  recommendations for  reducing  rodent  shelter  and  food
sources  in  and  around  the home, recommendations for  eliminating  rodents
inside  the home and preventing them from entering the home, precautions  for
preventing  hantavirus infection while rodent-contaminated  areas  are  being
cleaned up, prevention measures for persons who have occupational exposure to
wild rodents, and precautions for campers and hikers.

INTRODUCTION
    The recently recognized hantavirus-associated disease among residents  of
the  southwestern  United States and the identification of rodent  reservoirs
for  the virus in the affected areas warrant recommendations to minimize  the
risk   of  exposure  to  rodents  for  both  residents  and  visitors.  While
information is being gathered about the causative virus and its epidemiology,
provisional  recommendations  can be made on the  basis  of  knowledge  about
related   hantaviruses.   These  recommendations   are   based   on   current
understanding of the epidemiologic features of hantavirus infections  in  the
Southwest;  they  will  be  periodically  evaluated  and  modified  as   more
information becomes available.
    Rodents are the primary reservoir hosts of recognized hantaviruses.  Each
hantavirus appears to have preferential rodent hosts, but other small mammals
can  be infected as well. Available data strongly suggest that the deer mouse
(Peromyscus  maniculatus) is the primary reservoir of  the  newly  recognized
hantavirus in the southwestern United States. Serologic evidence of infection
has  also been found in pi±on mice (P. truei), brush mice (P. boylii), cotton
rats (Sigmodon hispidus), and western chipmunks (Tamias spp.). P. maniculatus
is  highly  adaptable  and  is found in different habitats,  including  human
residences in rural and semirural areas, but generally not in urban centers.
    Hantaviruses  do  not  cause apparent illness in their  reservoir  hosts.
Infected  rodents shed virus in saliva, urine, and feces for many weeks,  but
the  duration and period of maximum infectivity are unknown. The demonstrated
presence  of  infectious virus in saliva of infected rodents and  the  marked
sensitivity  of these animals to hantaviruses following inoculation  suggests
that biting may be an important mode of transmission among rodents.
    Human infection may occur when infective saliva or excreta are inhaled as
aerosols  produced  directly from the animal. Persons  visiting  laboratories
where  infected  rodents  were housed have been infected  after  only  a  few
minutes of exposure to animal holding areas. Transmission may also occur when
dried  or  fresh  materials  contaminated by rodent  excreta  are  disturbed,
directly  introduced into broken skin, introduced onto the conjunctivae,  or,
possibly,  ingested in contaminated food or water. Persons have  also  become
infected after being bitten by rodents.
    Arthropod  vectors  are not known to have a role in the  transmission  of
hantaviruses. Person-to-person transmission has not been associated with  any
of  the previously identified hantaviruses or with the recent outbreak in the
Southwest.  Cats and dogs are not known to be reservoir hosts of hantaviruses
in  the  United  States. However, these domestic animals may  bring  infected
rodents into contact with humans.
    Known  hantavirus infections of humans occur primarily in adults and  are
associated  with  domestic, occupational, or leisure  activities  that  bring
humans  into  contact  with infected rodents, usually  in  a  rural  setting.
Patterns  of seasonal occurrence differ, depending on the virus,  species  of
rodent   host,   and   patterns   of  human   behavior,   cases   have   been
epidemiologically associated with the following situations:
ƒ   planting or harvesting field crops;
ƒ   occupying previously vacant cabins or other dwellings;
ƒ   cleaning barns and other outbuildings;
ƒ   disturbing rodent-infested areas while hiking or camping;
ƒ   inhabiting dwellings with indoor rodent populations;
ƒ   residing in or visiting areas in which the rodent population has shown an
   increase in density.

     Hantaviruses  have  lipid  envelopes  that  are  susceptible   to   most
disinfectants (e.g., dilute hypochlorite solutions, detergents, ethyl alcohol
[70%],  or  most  general-purpose household disinfectants).  How  long  these
viruses survive after being shed in the environment is uncertain.
    The  reservoir hosts of the hantavirus in the southwestern United  States
also  act as hosts for the bacterium Yersinia pestis, the etiologic agent  of
plague. Although fleas and other ectoparasites are not known to play  a  role
in  hantavirus epidemiology, rodent fleas transmit plague. Control of rodents
without concurrent control of fleas may increase the risk of human plague  as
the rodent fleas seek an alternative food source.
    Eradicating  the reservoir hosts of hantaviruses is neither feasible  nor
desirable.  The  best  currently available approach for disease  control  and
prevention  is  risk reduction through environmental hygiene  practices  that
deter rodents from colonizing the home and work environment.

GENERAL HOUSEHOLD PRECAUTIONS IN AFFECTED AREAS
    Although  epidemiologic studies are being conducted to identify  specific
behaviors  that may increase the risk for hantavirus infection in  humans  in
the United States, rodent control in and around the home will continue to  be
the  primary prevention strategy (Box 1). CDC has issued recommendations  for
rodent-proofing urban and suburban dwellings and reducing rodent  populations
through habitat modification and sanitation.

Box 1. General precautions for residents of affected areas
Eliminate  rodents  and reduce the availability of food sources  and  nesting
sites used by rodents inside the home.
ƒ    Follow the recommendations in the section on Eliminating Rodents  Inside
   the Home.
ƒ    Keep  food (including pet food) and water covered and stored in  rodent-
   proof metal or thick plastic containers with tight-fitting lids.
ƒ    Store  garbage  inside  homes in rodent-proof  metal  or  thick  plastic
   containers with tight-fitting lids.
ƒ    Wash  dishes and cooking utensils immediately after use and  remove  all
   spilled food.
ƒ   Dispose of trash and clutter.
ƒ   Use spring-loaded rodent traps in the home continuously.
ƒ    As  an  adjunct to traps, use rodenticide with bait under a  plywood  or
   plastic  shelter  (covered bait station) on an ongoing  basis  inside  the
   house.

Note:   Environmental  Protection  Agency  (EPA)-approved  rodenticides   are
commercially  available.  Instructions  on  product  use  should  always   be
followed. Products that are used outdoors should be specifically approved for
exterior  use.  Any  use of a rodenticide should be preceded  by  use  of  an
insecticide to reduce the risk of plague transmission. Insecticide sprays  or
powders  can  be used in place of aerosols if they are appropriately  labeled
for flea control.

Prevent  rodents from entering the home. Specific measures should be  adapted
to local circumstances.
ƒ   Use steel wool or cement to seal, screen, or otherwise cover all openings
   into the home that have a diameter greater than or equal to 1/4 inch.
ƒ    Place metal roof flashing as a rodent barrier around the base of wooden,
   earthen, or adobe dwellings up to a height of 12 inches and buried in  the
   soil to a depth of 6 inches.
ƒ   Place 3 inches of gravel under the base of homes or under mobile homes to
   discourage rodent burrowing.

Reduce rodent shelter and food sources within 100 feet of the home.
ƒ    Use  raised  cement  foundations in new construction  of  sheds,  barns,
   outbuildings, or woodpiles.
ƒ    When  possible,  place woodpiles 100 feet or more from  the  house,  and
   elevate wood at least 12 inches off the ground.
ƒ   Store grains and animal feed in rodent-proof containers.
ƒ    Near buildings, remove food sources that might attract rodents, or store
   food and water in rodent-proof containers.
ƒ    Store hay on pallets, and use traps or rodenticide continuously to  keep
   hay free of rodents.
ƒ   Do not leave pet food in feeding dishes.
ƒ   Dispose of garbage and trash in rodent-proof containers that are elevated
   at least 12 inches off the ground.
ƒ    Haul  away  trash, abandoned vehicles, discarded tires, and other  items
   that may serve as rodent nesting sites.
ƒ   Cut grass, brush, and dense shrubbery within 100 feet of the home.
ƒ    Place  spring-loaded  rodent traps at likely spots  for  rodent  shelter
   within 100 feet around the home, and use continuously.
ƒ    Use  an  EPA-registered rodenticide approved for outside use in  covered
   bait  stations at places likely to shelter rodents within 100 feet of  the
   home.

NOTE:  Follow  the recommendations specified in the section  on  Clean-Up  of
Rodent-Contaminated  Areas  if  rodent  nests  are  encountered  while  these
measures are being carried out.

ELIMINATING RODENTS INSIDE THE HOME AND REDUCING RODENT ACCESS TO THE HOME
    Rodent infestation can be determined by direct observation of animals  or
inferred  from the presence of feces in closets or cabinets or on  floors  or
from  evidence that rodents have been gnawing at food. If rodent  infestation
is detected inside the home or outbuildings, rodent abatement measures should
be  completed  (Box 2). The directions in the section on Special  Precautions
should  be followed if evidence of heavy rodent infestation (e.g.,  piles  of
feces  or  numerous dead animals) is present or if a structure is  associated
with a confirmed case of hantavirus disease.

Box  2.  Eliminating rodent infestation: Guidance for residents  of  affected
areas
ƒ    Before  rodent elimination work is begun, ventilate closed buildings  or
   areas  inside  buildings  by opening doors and windows  for  at  least  30
   minutes.  Use an exhaust fan or cross ventilation if possible.  Leave  the
   area  until the airing-out period is finished. This airing may help remove
   any aerosolized virus inside the closed-in structure.
ƒ    Second, seal, screen, or otherwise cover all openings into the home that
   have  a  diameter of greater than or equal to │1/4 inch. Then  set  rodent
   traps  inside  the  house, using peanut butter as bait. Use  only  spring-
   loaded traps that kill rodents.
ƒ   Next, treat the interior of the structure with an insecticide labeled for
   flea  control; follow specific label instructions. Insecticide  sprays  or
   powders  can  be  used  in  place of aerosols if  they  are  appropriately
   labeled  for  flea  control.  Rodenticides may  also  be  used  while  the
   interior is being treated, as outlined below.
ƒ    Remove  captured rodents from the traps. Wear rubber or  plastic  gloves
   while handling rodents. Place the carcasses in a plastic bag containing  a
   sufficient   amount  of  a  general-purpose  household   disinfectant   to
   thoroughly  wet  the carcasses. Seal the bag and then  dispose  of  it  by
   burying  in a 2- to 3-foot-deep hole or by burning. If burying or  burning
   are  not  feasible,  contact your local or state health  department  about
   other appropriate disposal methods. Rebait and reset all sprung traps.
ƒ    Before  removing  the gloves, wash gloved hands in a  general  household
   disinfectant and then in soap and water. A hypochlorite solution  prepared
   by  mixing 3 tablespoons of household bleach in 1 gallon of water  may  be
   used  in  place  of  a  commercial disinfectant. When using  the  chlorine
   solution, avoid spilling the mixture on clothing or other items  that  may
   be  damaged. Thoroughly wash hands with soap and water after removing  the
   gloves.
ƒ   Leave several baited spring-loaded traps inside the house at all times as
   a  further  precaution  against rodent reinfestation.  Examine  the  traps
   regularly.  Disinfect  traps no longer in use  by  washing  in  a  general
   household  disinfectant or the hypochlorite solution. Disinfect  and  wash
   gloves  as described above, and wash hands thoroughly with soap and  water
   before beginning other activities.

NOTE:  EPA-approved rodenticides are commercially available. Instructions  on
product use should always be followed. Products that are used outdoors should
be specifically approved for exterior use. Any use of a rodenticide should be
preceded  by use of an insecticide to reduce the risk of plague transmission.
Insecticide  sprays or powders can be used in place of aerosols if  they  are
appropriately labeled for flea control.

CLEAN-UP OF RODENT-CONTAMINATED AREAS
    Areas  with  evidence  of  rodent activity (e.g.,  dead  rodents,  rodent
excreta) should be thoroughly cleaned to reduce the likelihood of exposure to
hantavirus-infected materials. Clean-up procedures must  be  performed  in  a
manner that limits the potential for aerosolization of dirt or dust from  all
potentially contaminated surfaces and household goods (Box 3).

Box  3.  Clean-up  of rodent-contaminated areas: Guidance  for  residents  of
affected areas
ƒ   Persons involved in the clean-up should wear rubber or plastic gloves.
ƒ   Spray dead rodents, rodent nests, droppings, or foods or other items that
   have   been   tainted   by   rodents  with  a  general-purpose   household
   disinfectant.  Soak the material thoroughly, and place in a  plastic  bag.
   When  clean-up is complete (or when the bag is full), seal the  bag,  then
   place  it  into  a  second plastic bag and seal.  Dispose  of  the  bagged
   material  by burying in a 2- to 3-foot-deep hole or by burning.  If  these
   alternatives  are  not  feasible,  contact  the  local  or  state   health
   department concerning other appropriate disposal methods.
ƒ    After  the above items have been removed, mop floors with a solution  of
   water,  detergent, and disinfectant. Spray dirt floors with a disinfectant
   solution.  A  second mopping or spraying of floors with a  general-purpose
   household   disinfectant   is  optional.  Carpets   can   be   effectively
   disinfected  with  household disinfectants or  by  commercial-grade  steam
   cleaning   or  shampooing.  To  avoid  generating  potentially  infectious
   aerosols, do not vacuum or sweep dry surfaces before mopping.
ƒ    Disinfect countertops, cabinets, drawers, and other durable surfaces  by
   washing  them  with  a  solution of detergent,  water,  and  disinfectant,
   followed  by  an  optional  wiping-down with a  general-purpose  household
   disinfectant.
ƒ    Rugs and upholstered furniture should be steam cleaned or shampooed.  If
   rodents have nested inside furniture and the nests are not accessible  for
   decontamination, the furniture should be removed and burned.
ƒ    Launder potentially contaminated bedding and clothing with hot water and
   detergent. (Use rubber or plastic gloves when handling the dirty  laundry;
   then  wash and disinfect gloves as described in the section on Eliminating
   Rodents  Inside the Home.) Machine-dry laundry on a high setting  or  hang
   it to air dry in the sun.

SPECIAL  PRECAUTIONS FOR HOMES OF PERSONS WITH CONFIRMED HANTAVIRUS INFECTION
OR BUILDINGS WITH HEAVY RODENT INFESTATIONS

   Special precautions are indicated in the affected areas for cleaning homes
or buildings with heavy rodent infestations (Box 4). Persons conducting these
activities  should  contact the responsible local, state, or  federal  public
health  agency  for  guidance. These precautions may  also  apply  to  vacant
dwellings  that  have attracted numbers of rodents while  unoccupied  and  to
dwellings  and  other  structures that have been  occupied  by  persons  with
confirmed hantavirus infection. Workers who are either hired specifically  to
perform  the  clean-up  or asked to do so as part of  their  work  activities
should  receive  a  thorough orientation from the responsible  health  agency
about  hantavirus transmission and should be trained to perform the  required
activities safely.

Box  4.  Special precautions for clean-up in homes of persons with hantavirus
infection or buildings with heavy rodent infestation
ƒ    A  baseline serum sample, preferably drawn at the time these  activities
   are  initiated, should be available for all persons conducting the  clean-
   up  of  homes or buildings with heavy rodent infestation. The serum sample
   should be stored at -20 C.
ƒ    Persons  involved in the clean-up should wear coveralls  (disposable  if
   possible),  rubber  boots  or disposable shoe covers,  rubber  or  plastic
   gloves,  protective  goggles,  and an appropriate  respiratory  protection
   device,   such   as   a  half-mask  air-purifying  (or  negative-pressure)
   respirator  with  a  high-efficiency particulate air (HEPA)  filter  or  a
   powered  air-purifying  respirator (PAPR) with HEPA  filters.  Respirators
   (including  positive-pressure  types) are  not  considered  protective  if
   facial  hair  interferes with the face seal, since proper  fit  cannot  be
   assured.  Respirator practices should follow a comprehensive user  program
   and be supervised by a knowledgeable person.
ƒ   Personal protective gear should be decontaminated upon removal at the end
   of  the day. If the coveralls are not disposable, they should be laundered
   on  site. If no laundry facilities are available, the coveralls should  be
   immersed in liquid disinfectant until they can be washed.
ƒ    All  potentially infective waste material (including respirator filters)
   from  clean-up  operations that cannot be burned or deep  buried  on  site
   should  be double bagged in appropriate plastic bags. The bagged  material
   should  then  be  labeled as infectious (if it is to be  transported)  and
   disposed of in accordance with local requirements for infectious waste.
ƒ   Workers who develop symptoms suggestive of HPS within 45 days of the last
   potential   exposure  should  immediately  seek  medical  attention.   The
   physician  should contact local health authorities promptly if hantavirus-
   associated  illness is suspected. A blood sample should  be  obtained  and
   forwarded  with the baseline serum through the state health department  to
   CDC for hantavirus antibody testing.

PRECAUTIONS  FOR  WORKERS  IN AFFECTED AREAS WHO  ARE  REGULARLY  EXPOSED  TO
RODENTS
     Persons  who  frequently  handle  or  are  exposed  to  rodents   (e.g.,
mammalogists,  pest-control workers) in the affected  area  are  probably  at
higher risk for hantavirus infection than the general public because of their
frequency  of  exposure.  Therefore, enhanced precautions  are  warranted  to
protect them against hantavirus infection (Box 5).

Box 5. Precautions for workers in affected areas who are exposed to rodents
ƒ    A  baseline  serum sample, preferably drawn at the time  of  employment,
   should  be  available for all persons whose occupations  involve  frequent
   rodent contact. The serum sample should be stored at -20C.
ƒ    Workers  in potentially high-risk settings should be informed about  the
   symptoms  of  the  disease and be given detailed  guidance  on  prevention
   measures.
ƒ    Workers who develop a febrile or respiratory illness within 45  days  of
   the  last potential exposure should immediately seek medical attention and
   inform  the  attending  physician of the potential  occupational  risk  of
   hantavirus   infection.  The  physician  should   contact   local   health
   authorities  promptly  if hantavirus-associated illness  is  suspected.  A
   blood  sample  should be obtained and forwarded with  the  baseline  serum
   through  the  state  health  department to  CDC  for  hantavirus  antibody
   testing.
ƒ    Workers  should  wear  a half-face air-purifying (or  negative-pressure)
   respirator  or PAPR equipped with HEPA filters when removing rodents  from
   traps  or  handling  rodents in the affected area. Respirators  (including
   positive-pressure  types)  are not considered protective  if  facial  hair
   interferes  with  the  face  seal, since proper  fit  cannot  be  assured.
   Respirator  use  practices should be in accord with a  comprehensive  user
   program and should be supervised by a knowledgeable person.
ƒ    Workers  should wear rubber or plastic gloves when handling  rodents  or
   handling   traps  containing  rodents.  Gloves  should   be   washed   and
   disinfected before removing them, as described above.
ƒ    Traps  contaminated by rodent urine or feces or in which  a  rodent  was
   captured  should be disinfected with a commercial disinfectant  or  bleach
   solution.  Dispose  of  dead  rodents  as  described  in  the  section  on
   Eliminating Rodents inside the Home.
ƒ   Persons removing organs or obtaining blood from rodents in affected areas
   should  contact  the  Special  Pathogens Branch,  Division  of  Viral  and
   Rickettsial  Diseases,  National Center for Infectious  Diseases,  Centers
   for  Disease  Control  and  Prevention,  [telephone  (404)  639-1115]  for
   detailed safety precautions.

PRECAUTIONS FOR OTHER OCCUPATIONAL GROUPS WHO HAVE POTENTIAL RODENT CONTACT
    Insufficient  information  is available at this  time  to  allow  general
recommendations  regarding risks or precautions for persons in  the  affected
areas  who work in occupations with unpredictable or incidental contact  with
rodents  or their habitations. Examples of such occupations include telephone
installers,   maintenance  workers,  plumbers,  electricians,   and   certain
construction  workers.  Workers  in these jobs  may  have  to  enter  various
buildings,  crawl  spaces,  or  other sites  that  may  be  rodent  infested.
Recommendations  for such circumstances must be made on a case-by-case  basis
after  the specific working environment has been assessed and state or  local
health departments have been consulted.

PRECAUTIONS FOR CAMPERS AND HIKERS IN THE AFFECTED AREAS
   There is no evidence to suggest that travel into the affected areas should
be  restricted.  Most usual tourist activities pose little or  no  risk  that
travelers  will  be  exposed  to rodents or their excreta.  However,  persons
engaged  in  outdoor  activities  such  as  camping  or  hiking  should  take
precautions  to  reduce  the  likelihood of  their  exposure  to  potentially
infectious materials (Box 6).

Box 6. Reducing risk of hantavirus infection: Guidance for hikers and campers
ƒ    Avoid  coming into contact with rodents and rodent burrows or disturbing
   dens (such as pack rat nests).
ƒ    Do  not  use cabins or other enclosed shelters that are rodent  infested
   until they have been appropriately cleaned and disinfected.
ƒ   Do not pitch tents or place sleeping bags in areas in proximity to rodent
   feces or burrows or near possible rodent shelters (e.g., garbage dumps  or
   woodpiles).
ƒ   If possible, do not sleep on the bare ground. Use a cot with the sleeping
   surface at least 12 inches above the ground. Use tents with floors.
ƒ   Keep food in rodent-proof containers.
ƒ     Promptly  bury  (or--preferably--burn  followed  by  burying,  when  in
   accordance  with local requirements) all garbage and trash, or discard  in
   covered trash containers.
ƒ    Use only bottled water or water that has been disinfected by filtration,
   boiling,  chlorination,  or  iodination  for  drinking,  cooking,  washing
   dishes, and brushing teeth.

CONCLUSION
    The  control  and  prevention recommendations in  this  report  represent
general  measures to minimize the likelihood of human exposure to hantavirus-
infected rodents in areas of the southwestern United States affected  by  the
outbreak   of   hantavirus-associated  respiratory  illness.  Many   of   the
recommendations may not be applicable or necessary in unaffected locales. The
impact  and  utility  of the recommendations will be  assessed  as  they  are
implemented  and will be continually reviewed by CDC and the  involved  state
and  local  health agencies as additional epidemiologic and  laboratory  data
related  to the outbreak become available. These recommendations (which  were
developed in July) will be supplemented or modified in the future.

To  receive  additional  recommendations for the prevention  and  control  of
hantavirus infections associated with rodents in the United States, you  will
need to call 404-332-4565 and follow the prompts. The following documents  on
Hantavirus  are  available: 310031, Hantavirus Illness in the United  States;
310032,  Hantavirus  Illness Prevention Information; 310033,  Guidelines  for
Removing  Organs  or Obtaining Blood from Rodents Potentially  Infected  with
Hantavirus;   310034,  Laboratory  Management  of  Agents   Associated   with
Hantavirus  Pulmonary Syndrome: Interim Biosafety Guidelines;  310035,  State
Contacts for Hantavirus Information
!
*
HAY FEVER (ALLERGIC RHINITIS)

Hay fever (the common term for allergic rhinitis) is the body's
response to normally harmless foreign particles inhaled into the
nose and throat.  The reaction is often caused by inhaling
pollen, such as ragweed, tree or grass pollen, but may also
result from mold spores generated in dead or decaying organic
matter, as well as dust, animal dander and feathers.  Allergic
rhinitis affects approximately 20 million Americans.

The term "hay fever" dates back to a time when harvesting and
haying in late summer widely led to discomfort in farm workers.
However, the most common symptoms of allergic rhinitis - itching
and tearing eyes, a runny nose, uncontrollable sneezing and a
scratchy, sore throat - may occur at any time of the year during
a particular pollen season (seasonal allergic rhinitis) or
throughout the year, depending on what someone is allergic to
(perennial allergic rhinitis).  Despite the name, fever is not a
sign of such conditions, and allergic rhinitis is not contagious.

For most people allergens, the substances that cause allergies,
do not threaten general health.  When people breathe in foreign
substances, their immunological system produces antibodies, which
resist and defend the body against disease and thus fend off any
harmful effects.  In people who suffer from allergic rhinitis,
however, the body produces allergic antibodies that react with
the allergens, and the familiar symptoms of allergies appear.
Allergies seem to run in families, although different family
members may be allergic to different factors.

The mechanism of allergy

Allergic symptoms result from a chain of events.  First,
allergens (such as pollens) reach the mucous membranes of the
nose, eyes and throat after they are inhaled.  Allergic
antibodies (IgE) are manufactured by the body of allergic
individuals; these antibodies then attach to cells known as mast
cells in the mucous membranes.

When allergic individuals are reexposed to these allergens, the
IgE attached to the mast cells combines with the offending
allergens.  The mast cells then release chemicals such as
histamine into the bloodstream.  As a result of this release, a
variety of allergic symptoms occur, such as itching, swelling of
the mucous membranes lining the nose and throat caused by a
widening of blood vessels, and a release of fluids caused by an
increase in the permeability ("leakiness") of small blood
vessels.

Skin testing

When the cause of an allergic reaction is difficult to identify
or important to confirm, skin tests are the easiest way to find
out what substances are responsible for the symptoms.  Usually,
more than one substance plays a part in allergies.  In these
tests a very small, dilute, highly purified amount of a
particular allergen is placed on a scratched area of the skin or
injected just under the skin.  When the substance causes the skin
to become red and swollen at the scratched or injected area, the
allergen has probably been pinpointed.  These tests are usually
performed by an allergist.


Drug therapy

Fortunately, there are many medications available that
effectively reduce and, in some cases, practically eliminate
symptoms in most hay fever sufferers.  Different drugs work in
different ways to interfere with and prevent the chemical
reactions leading to allergic attacks.  The best known of these
drugs, antihistamines, compete with histamine for receptors on
the mucous membranes of the nose, eyes and throat.  Through this
blocking action, histamine never reaches its target, thus
preventing and relieving the symptoms of hay fever.

Different people vary in their response to the same
antihistamine.  Therefore, it may take experimentation with
several medications before the best antihistamine is found.
Drowsiness is a typical side effect of most, but not all,
antihistamines; if you find that you become very tired after
taking an antihistamine, you should avoid driving or doing
anything else that requires you to be fully alert.  Also, it is
sensible to avoid alcohol or tranquilizers when taking
antihistamines, since these substances can cause added
drowsiness.  Some over-the-counter and prescription allergy
medicines contain a combination of an antihistamine with a
decongestant.  Nose drops or a nasal decongestant spray may be
used occasionally if sinus pressure is particularly troublesome.
However, too frequent use of these direct- acting preparations
may make your condition worse by building tolerance to the drug
contained in them.  Prescription eye drops may be used when
itching, burning, and weeping eyes interfere with normal
activities.

Cromolyn sodium is another very effective agent that prevents
allergic attacks.  This drug is available as a nasal spray and as
eye drops.  Instead of blocking histamine from reaching its
target (like antihistamines do), cromolyn prevents the release of
histamine and other substances from mast cells altogether.

Corticosteroids, another group of drugs, may be tried in severe
cases of allergic rhinitis.  These drugs may be used alone or in
combination with other drugs when alternative forms of therapy
are ineffective and/or when the person has other medical
conditions requiring special treatment.  They help provide relief
by decreasing the swelling of the mucous membranes lining the
nose and throat and by preventing the release of substances from
the mast cells which cause allergic reactions.  Nasal steroids
are often used for hay fever because they are less likely than
oral steroids to affect other parts of the body.

Immunotherapy

When antihistamines or other medications provide insufficient
relief, many allergens are involved, or a particular allergy
season is prolonged, immunotherapy may be the answer for some
allergy sufferers.  Immunotherapy is particularly effective
against ragweed, grass and tree pollens and mites and reportedly
relieves two-thirds of all patients of their symptoms.

After skin testing is done to pinpoint the particular allergens
one is allergic to, immunotherapy is begun.  It consists of a
series of injections with increasingly stronger concentrations of
an allergen extract on a regular schedule.  These allergen
extracts produce allergen-specific immunoglobulin-G (IgG)
antibodies, which block the combination of an allergen with IgE
antibodies and thereby prevent the release of histamine and other
substances from mast cells.  Booster shots are given at longer
intervals throughout the season.  This procedure is more
expensive than drug therapy and must be maintained without
interruption during one or more pollen seasons; it is usually
undertaken by people with a prior condition that has ruled out
other forms of therapy or whose symptoms are too severe to be
relieved by any other method.

Avoiding allergens

The severity of seasonal allergic rhinitis symptoms often is
directly related to the weather.  The pollen count is highest on
breezy, dry summer afternoons; rides in an open car increase
exposure to the allergens.  The pollen count falls dramatically
when the weather is cold or humid and when heavy rains occur in
the morning.  Whatever the climate, the hay fever sufferer will
almost certainly be more comfortable in the city than in the
country and indoors rather than outdoors, especially if the
interior is air-conditioned.  If exposure to the outdoors during
the height of a pollen season cannot be avoided, wearing a face
mask to avoid inhalation of pollen granules may be helpful.
Rarely, moving to an area that has a low pollen count, at least
during the pollen season, may be the only alternative to
immunotherapy.

If the cause of the symptoms is known to be mold spores, damp
woods should be avoided; newspapers should be discarded promptly,
and bed pillows (avoid those with feathers and down) should be
aired regularly in the sun.  Raking leaves, spreading mulch, or
other interactions with decaying vegetation should be completely
avoided, since mold spores flourish in such materials; if such
exposure is absolutely necessary, a face mask can be worn.
Dehumidifiers placed in damp, moist areas of the home (such as
bathrooms and laundry rooms) or use of antimildew sprays and
mold-resistant paint will limit mold growth.

For allergies to animal dander, the only source of relief may be
elimination of pets from the home; if this is not possible, then
the pet should be kept outside.  Again, a face mask may be
helpful, especially for people who work with animals.
Elimination of feather pillows and down quilts and jackets may
also help if an allergy to feathers is diagnosed; pillows and
mattresses with hypoallergenic fillings, such as foam, are widely
available.  Dust is more difficult to deal with, but it, too, can
be limited.  For example, mattresses, boxsprings and pillows
should be covered with vinyl or synthetic waterproof coverings
that can be sponged clean periodically.  Upholstered furniture,
stuffed toys, wall hangings, wall-to-wall carpeting, and curtains
are all dust collectors; vinyl-covered furniture, venetian
blinds, and hardwood, linoleum, and tile floors are kept clean
easily.  The area should be vacuumed and wet mopped frequently to
catch loose balls of dust.  Another good strategy is to maintain
clean, adequate filters for heating and air-conditioning systems
so that allergens are not dispersed throughout the home; many
people have sneezing attacks during the winter because dust
hidden in heating ducts is discharged with warm air from the
ducts.
!
*

                                        Writer:
                                        Mary Beth Howley



SUGGESTIONS, WARNINGS
FOR HAY FEVER SUFFERERS



     BIRMINGHAM, Ala...With the prime time for allergies almost
upon us, Dr. James R. Bonner, director of the University of Alabama
at Birmingham (UAB) allergy clinic, offers some suggestions and
warnings for those people who will be doing a lot of sneezing and
sniffling in the months ahead.
     Nearly 20 percent of the population suffers from allergies,
according to Bonner, and for those people suffering from hay fever,
late summer is a prime time for their allergies to "act up."
Ragweed pollen counts are particularly high beginning in mid-August
until mid-October.
     "If a person is pollen allergic, there is not a whole lot they
can do to avoid these symptoms except stay indoors, keep the
windows shut and run the air conditioner," Bonner says.
     But as most people have to leave the confines of their homes
at some point, Bonner recommends drug intervention.  Common
symptoms of hay fever include itching, sneezing, drainage and
blockage of the nose, sore throat and sometimes severe
cases of asthma.  Most people, whose hay fever is nothing more than
a minor nuisance, can alleviate their symptoms with over-the-
counter antihistamines and decongestants.  Antihistamines help
symptoms such as drainage, sneezing and itching.  Symptoms such as
blockage and stuffy nose need a decongestant.  Bonner cautions
people that one of the major side effects of the over-the-counter
antihistamines is drowsiness and should not be combined with other
sedating medications or with alcohol.
     Decongestants tend to make some people jittery and may cause
sleepless nights.  People with high blood pressure should be
careful when taking decongestants as those drugs cause a slight
increase in blood pressure.  Bonner says a physician's intervention
may be needed for those whose hay fever allergies become
incapacitating.
     In that instance, Bonner says he recommends prescription
antihistamines, decongestants and allergy shots.  The prescription
drugs do not cause side effects associated with the over-the-
counter brands.  He also prescribes nasal inhalers which are very
effective with pollens and work best if taken before the season
starts.
     For more information call the allergy information service at
934-9777 or 1-800-822-4413.
!
*

HEADACHES


An estimated 40 million Americans experience chronic headaches.
For at least half of these people, the problem is severe and
sometimes disabling. It can also be costly: headache sufferers
make over 8 million visits a year to doctors' offices, according
to the National Institutes of Health. Migraine sufferers alone
lose over 64 million workdays because of headache pain.

Several areas of the head can hurt during a headache, including a
network of nerves which extends over the scalp and nerves in the
face, mouth, and throat. Also sensitive to pain, because they
contain delicate nerve fibers, are the muscles of the head and
blood vessels found along the surface and at the base of the
brain.

The bones of the skull and tissues of the brain itself, however,
never hurt, because they lack pain-sensitive nerve fibers.

The ends of these pain-sensitive nerves, called nociceptors, can
be stimulated by stress, muscular tension, dilated blood vessels,
and other triggers of headache.

Once stimulated, a nociceptor sends a message up the length of
the nerve fiber to the nerve cells in the brain, signaling that a
part of the body hurts. The message is determined by the location
of the nociceptor. A person who suddenly realizes "My toe hurts,"
is responding to nociceptors in the foot that have been
stimulated.

A number of chemicals help transmit pain-related information to
the brain. Some of these chemicals are natural painkilling
proteins called endorphins. One theory suggests that people who
suffer from severe headache and other types of chronic pain have
lower levels of endorphins than people who are generally pain
free.

Should You Call or See a Physician?

Not all headaches require medical attention. Some headaches can
be caused by missed meals or occasional muscle tension and are
easily remedied through lifestyle modifications. Some headaches,
however, may be signals of more serious disorders such as head
injury that call for prompt medical care. According to the
National Institutes of Health, these include:

1. Sudden, severe headache
2. Headache associated with convulsions
3. Headache accompanied by confusion or loss of consciousness
4. Headache following a blow on the head
5. Headache associated with pain in the eye or ear
6. Persistent headache in a person who was previously headache
free
7. Recurring headache in children
8. Headache associated with fever

When professional medical care is needed, headache sufferers are
advised to first visit a primary care or family practitioner.
Sometimes, individuals are referred to specialists, such as
internists, neurologists or dentists for additional diagnosis and
treatment options. Sometimes, too, referrals to psychologists are
recommended.

How Are Headaches Diagnosed?

Experts agree that a detailed medical history, and
question-and-answer session with a headache sufferers can often
produce enough information for a diagnosis. Many types of
headaches have clear-cut symptoms which fall into an easily
recognizable pattern.

Among the questions are:
1. How often do you have headaches?
2. Where is the pain?
3. How long do the headaches last?
4. When did you first develop headaches?
5. What, if any, over-the-counter or prescription medications do
you use?
6. What is your medical history with regard to head trauma and
surgery?

The individual's sleep and lifestyle habits, as well as work and
family situations, may also be probed.

Blood tests are sometimes used to rule out thyroid disease,
anemia or infections which might be the cause of the headache.
Sometimes, x-rays are recommended to screen for the possibility
of a brain tumor or blood clot.

Sometimes, although rarely, a test called an electroencephalogram
(EEG) may be recommended to measure brain activity. While EEG's
can indicate a malfunction in the brain, they cannot usually
pinpoint a problem that might be causing a headache.

In unusual instances, it may be recommended that a headache
sufferer undergo a computed tomographic (CT) scan. The CT scan
produces images of the brain that show variations in the density
of different types of tissue. The scan enables the physician to
distinguish, for example between a bleeding blood vessel in the
brain and a brain tumor.

The CT scan is an important diagnostic tool in cases of headache
associated with brain lesions or other serious disease. Experts
generally agree, however, that this sophisticated and expensive
technology is not required to diagnose simple or periodic
headache.

An eye exam is usually performed to check for weakness in the eye
muscle or unequal pupil size. Both of these symptoms are evidence
of a possible aneurysm--an abnormal ballooning of a blood vessel.
A physician who suspects that a headache patient has an aneurysm
may also recommend an angiogram.

In this test, a special fluid which can be seen on an X ray is
injected into the patient and carried in the bloodstream to the
brain to reveal any abnormalities in the blood vessels there.

Thermography, an experimental technique for diagnosing headache,
may become a useful clinical tool. In thermography, an infrared
camera converts skin temperature into a color picture or
thermogram with different degrees of heat appearing as different
colors.

Skin temperature is affected primarily by blood flow. Research
scientists have found that thermograms of headache patients show
strikingly different heat patterns from those of people who never
or rarely get headaches.

Types of Headaches

Headaches are classified as one or more of the following types:
1. Vascular
2. Muscle contraction
3. Traction, or
4. Inflammatory

Vascular headaches -- a group that includes the well-known
migraine -- are so named because they are thought to involve
abnormal function of the brain's blood vessels or vascular
system. Muscle contraction headaches appear to involve the
tightening or tensing of facial and neck muscles. Traction and
inflammatory headaches are symptoms of other disorders, ranging
from stroke to sinus infection. Some people have more than one
type of headache.

Migraine Headaches

The most common type of vascular headache is migraine. Migraine
headaches are usually characterized by severe pain on one or both
sides of the head, an upset stomach, and at times disturbed
vision.  There are two primary types of migraine-caused headache:
classic and common.

The major difference between the two types is the appearance of
neurological symptoms 10 minutes to 30 minutes before a classic
migraine attack. These symptoms are called an aura. The person
may see flashing lights or zigzag lines, or may temporarily lose
vision. Other classic symptoms include speech difficulty,
weakness of an arm or leg, tingling of the face or hands, and
confusion.

The pain of a classic migraine headache is described as intense,
throbbing, or pounding and is felt in the forehead, temple, ear,
jaw, or around the eye. Classic migraine starts on one side of
the head but may eventually spread to the other side. An attack
lasts 1 to 2 days.

The common migraine -- a term that reflects the disorder's
greater occurrence in the general population -- is not preceded
by an aura. But some people experience a variety of vague
symptoms beforehand, including mental fuzziness, mood changes,
fatigue, and unusual retention of fluids.

During the headache phase of a common migraine, a person may have
diarrhea and increased urination, as well as nausea and vomiting.
Common migraine pain can last 3 or 4 days.

Both classic and common migraine can strike as often as several
times a week, or as rarely as once every few years. Both types
can occur at any time. Some people, however, experience migraines
at predictable times-- near the days of menstruation or every
Saturday morning after a stressful week of work.

What Causes a Migraine Headache?

Research scientists are unclear about the precise cause of
migraine headaches. There seems to be general agreement, however,
that a key element is blood flow changes in the brain. People who
get migraine headaches appear to have blood vessels that
overreact to various triggers.

Scientists have devised one theory of migraine which explains
these blood flow changes and also certain biochemical changes
that may be involved in the headache process.

According to this theory, the nervous system responds to a
trigger such as stress by creating a spasm in the nerve-rich
arteries at the base of the brain. The spasm closes down or
constricts several arteries supplying blood to the brain,
including the scalp artery and the carotid or neck arteries.

As these arteries constrict, the flow of blood to the brain is
reduced. At the same time, blood-clotting particles called
platelets clump together--a process which is believed to release
a chemical called serotonin. Serotonin acts as a powerful
constrictor of arteries, further reducing the blood supply to the
brain.

Reduced blood flow decreases the brain's supply of oxygen.
Symptoms signaling a headache, such as distorted vision or
speech, may then result, similar to symptoms of stroke.

Reacting to the reduced oxygen supply, certain arteries within
the brain open wider to meet the brain's energy needs. This
widening or dilation spreads, finally affecting the neck and
scalp arteries. The dilation of these arteries triggers the
release of pain-producing substances called prostaglandins from
various tissues and blood cells.

Chemicals which cause inflammation and swelling, and substances
which increase sensitivity to pain are also released. The
circulation of these chemicals and the dilation of the scalp
arteries stimulate the pain-sensitive nociceptors. The result,
according to this theory: a throbbing pain in the head.

Women and Migraines

Although males and females seem to be equally affected by
migraine, the condition appears to be more common in adult women
than in men. Both sexes may develop migraine in infancy, but most
often the disorder begins between the ages of 5 and 35.

The relationship between female hormones and migraine is still
unclear. Women may have "menstrual migraine"--headaches around
the time of their menstrual period--which may disappear during
pregnancy. Other women develop migraine for the first time when
they are pregnant. Some are first affected after menopause.

The effect of oral contraceptives on headaches is perplexing.
Scientists report that some migrainous women who take birth
control pills experience more frequent and severe attacks.
However, a small percentage of women have fewer and less severe
migraine headaches when they take birth control pills.

And normal women who do not suffer from headaches may develop mi-
graines as a side effect when they use oral contraceptives.
Investigators around the world are studying hormonal changes in
migrainous women in the hope of identifying the specific ways
these naturally occurring chemicals may cause headaches.

Migraine "Triggers" and the Migraine "Personality"

The existence of a migraine personality is a controversial theory
which suggests that migraine patients are compulsive, rigid, and
perfectionistic.  However, not all migraine patients have these
traits and that not all individuals with these personality
characteristics have migraines.

Rather than focusing on character traits, scientists are turning
to heredity to identify the causes of migraines.  It is possible
that the abnormal regulation of blood vessels which leads to
migraines is an inherited trait.  Many migrained sufferers have a
family history of migraine, but the exact hereditary nature of
this condition is still unknown.

Factors believed to "trigger" migraines include:
1. Stress
2. Emotions
3. Biological conditions
4. Environmental conditions
5. Fatigue
6. Glaring or flickering lights
7. The weather
8. Certain foods

It may seem hard to believe that eating such seemingly harmless
foods as yogurt, nuts, and lima beans can result in a painful
migraine headache. However, some scientists believe that these
foods and several others contain chemical substances such as
tyramine which constrict arteries--the first step of the migraine
process. Other scientists believe that foods cause headaches by
setting off an allergic reaction in susceptible people.

While a food-triggered migraine usually occurs soon after eating,
other triggers may not cause immediate pain. Scientists report
that people can develop migraine not only during a period of
stress but also afterwards when their vascular systems are still
reacting.

Other Forms of Migraine

In addition to classic and common, migraine headache can take
several other forms including:
1. Hemiplegic migraine
2. Opthalmoplegic migraine
3. Basilar artery migraine
4. Benign exertional headache
5. Status migrainosus, and
6. Headache-free migraine

Individuals with hemiplegic migraine have temporary paralysis on
one side of the body, a condition known as hemiplegia. Some
people may experience vision problems and vertigo--a feeling that
the world is spinning. These symptoms begin 10 minutes to 90
minutes before the onset of headache pain.

In ophthalmoplegic migraine, the pain is around the eye and is
associated with a droopy eyelid, double vision, and other sight
problems.

Basilar artery migraine involves a disturbance of a major brain
artery. Preheadache symptoms include vertigo, double vision, and
poor muscular coordination. This type of migraine occurs
primarily in adolescent and young adult women and is often
associated with the menstrual cycle.

Benign exertional headache is brought on by running, lifting,
coughing, sneezing, or bending. The headache begins at the onset
of activity, and pain rarely lasts more than several minutes.

Status migrainosus is a rare and severe type of migraine that can
last 72 hours or longer. The pain and nausea are so intense that
people who have this type of headache are often hospitalized. The
use of certain drugs can trigger status migrainosus. Neurologists
report that many of their status migrainosus patients were
depressed and anxious before they experienced headache attacks.

Headache-free migraine is characterized by such migraine symptoms
as visual problems, nausea, vomiting, constipation, or diarrhea.
Individuals, however, do not experience head pain. Headache
specialists have suggested that unexplained pain in a particular
part of the body, fever, and dizziness could also be possible
types of headache-free migraine.

Treatment Options For Migraine Headache

Medications, biofeedback, stress reduction, and elimination of
certain foods from the diet are the most common methods of
preventing and controlling migraine and other vascular headaches.


Regular exercise, such as swimming or vigorous walking, can also
reduce the frequency and severity of migraine headaches.

During a migraine headache, temporary relief can sometimes be
obtained by using cold packs or by pressing on the artery found
in front of the ear on the painful side of the head.

Medications

There are two ways that medications are used for migraines:
1. To prevent the attacks
2. To relieve symptoms after the headache occurs

For infrequent migraine, medication is sometimes taken at the
first sign of a headache in order to stop it or to ease the pain.
People who get occasional mild migraine may benefit by taking
aspirin or acetaminophen at the start of an attack. Aspirin
raises a person's tolerance to pain and also discourages clumping
of blood platelets. Small amounts of caffeine may be useful if
taken in the early stages of migraine.

For migraine sufferers who get moderate to severe headaches,
stronger drugs may be recommended to control the pain.

One of the most commonly used drugs for the relief of classic and
common migraine symptoms is ergotamine tartrate, a
vasoconstrictor which helps counteract the painful dilation stage
of the headache. For optimal benefit, the drug is taken during
the early stages of an attack. If a migraine has been in progress
for about an hour and has passed into the final throbbing stage,
ergotamine tartrate will probably not help.

Because ergotamine tartrate can cause nausea and vomiting, it may
be combined with antinausea drugs. Research scientists caution
that ergotamine tartrate should not be taken in excess or by
people who have angina pectoris, severe hypertension, or
vascular, liver, or kidney disease.

Patients who are unable or who choose not to take ergotamine
tartrate may benefit from other drugs that constrict dilated
blood vessels or help reduce blood vessel inflammation.

For headaches that occur three or more times a month, preventive
treatment is often recommended. Medications used to prevent
classic and common migraine include methysergide maleate, which
counteracts blood vessel constriction, propranolol, which stops
blood vessel dilation, and amitriptyline, an antidepressant.

According to the National Institutes of Health, in a study of
propranolol, amitriptyline, and biofeedback conducted by the
Houston Headache Clinic, scientists found that migraine patients
improved most on a combination of propranolol and biofeedback.
Patients who had mixed migraine and muscle-contraction headaches
received the greatest benefit from a combination of propranolol,
amitriptyline, and biofeedback.

Another study showed that propranolol may continue to prevent
migraine headaches even after patients have stopped taking the
drug. The scientists who conducted the study speculate that
long-term therapy with propranolol may have a lasting effect on
blood vessels, training them to react less than usual to the
triggers of migraine.

Antidepressants called MAO inhibitors also prevent migraine.
These drugs block an enzyme called monoamine oxidase which
normally helps nerve cells absorb the artery-constricting
chemical, serotonin.

MAO inhibitors can have potentially serious side effects --
particularly if taken while ingesting foods or beverages that
contain tyramine, a substance that closes down arteries.

Several new drugs for the prevention of migraine have been
developed in recent years, including papaverine hydrochloride,
which produces blood vessel dilation, and cyproheptadine, which
counteracts serotonin.

All these antimigraine drugs can have adverse side effects. To
avoid long-term side effects of preventive medications, headache
specialists advise patients to reduce the dosage of these drugs
and then to stop taking them as soon as possible.

All medications, including such over-the-counter drugs as
aspirin, carry risks.  Only the individual migraine sufferer can
assess the best treatment for him- or herself.  Individuals
should always question their physicians about the benefits, risks
and costs of medications and treatment alternatives.

Biofeedback and Relaxation Training

The use of medications for the prevention or treatment of
migraine is often combined with biofeedback and relaxation
training.

Biofeedback--a technique once considered "fringe medicine" by
mainstream medical practitioners--is now an acceptable and safe
way to allow individuals to better control such body function
indicators as blood pressure, heart rate, temperature, muscle
tension, and brain waves.

Thermal biofeedback allows a patient to consciously raise hand
temperature. Some patients who are able to increase hand
temperature can reduce the number and intensity of migraines. The
mechanism of this hand-warming effect is being studied by
research scientists.

An individual learning thermal biofeedback wears a device which
transmits the temperature of an index finger or hand to a
monitor. While the individual tries to warm his hands, the
monitor provides feedback either on a gauge that shows the
temperature reading or by emitting a sound or beep that increases
in intensity as the temperature increases. The patient is not
told how to raise hand temperature, but is given suggestions such
as "Imagine that your hands feel very warm and heavy."

In another type of biofeedback called electromyographic or EMG
training, the patient learns to control muscle tension in the
face, neck, and shoulders.

Either kind of biofeedback may be combined with relaxation
training, during which patients learn to relax the mind and body.

Biofeedback can be practiced at home with a portable monitor. But
the ultimate goal of treatment is to wean the individual from the
machine. The machine is used primarily to provide feedback to the
individual.  Overtime, with practice and commitment, the
individual can do biofeedback without the machine's direct
feedback.

The Antimigraine Diet

Scientists estimate that a small percentage of migraine sufferers
will benefit from a treatment program focused solely on
eliminating headache provoking foods and beverages.

Other migraine patients may be helped by a diet to prevent low
blood sugar. Low blood sugar, or hypoglycemia, can cause dilation
of the blood vessels in the head. This condition can occur after
a period without food: overnight, for example, or when a meal is
skipped. People who wake up in the morning with a headache may be
reacting to the low blood sugar caused by the lack of food
overnight.

Treatment for headaches caused by low blood sugar consists of
scheduling smaller, more frequent meals for the individual. A
special diet designed to stabilize the body's sugar-regulating
system is sometimes recommended.

For the same reason, many specialists also recommend that
migraine patients avoid oversleeping on weekends. Sleeping late
can change the body's normal blood sugar level and lead to a
headache.

Other Vascular Headaches

After migraine, the most common type of vascular headache is the
toxic headache produced by fever. Pneumonia, measles, mumps, and
tonsillitis are among the diseases that can cause severe toxic
vascular headaches. Toxic headaches can also result from the
presence of foreign chemicals in the body.

Other kinds of vascular headaches include "clusters," which cause
repeated episodes of intense pain, and headaches resulting from a
rise in blood pressure.

The Impact of Chemicals

Repeated exposure to nitrite compounds can result in a dull,
pounding headache that may be accompanied by a flushed face.
Nitrite, which dilates blood vessels, is found in such products
as heart medicine and dynamite. Hot dogs and other meats
containing sodium nitrite can also cause headaches.

"Chinese restaurant headache" can occur when a susceptible
individual eats foods prepared with monosodium glutamate (MSG)--a
staple in many Oriental kitchens. Soy sauce, meat tenderizer, and
a variety of packaged foods contain this chemical which is touted
as a flavor enhancer.

Vascular headache can also result from exposure to poisons, even
common household varieties like insecticides, carbon
tetrachloride, and lead.

Children who eat flakes of lead paint may develop headaches. So
may anyone who has contact with lead batteries or lead glazed
pottery.

Painters, printmakers, and other artists may experience headaches
after exposure to art materials that contain chemicals called
solvents. Solvents, like benzene, are found in turpentine, spray
adhesives, rubber cement, and inks.

Drugs such as amphetamines can cause headaches as a side effect.

Another type of drug-related headache occurs during withdrawal
from long-term therapy with the antimigraine drug ergotamine
tartrate.

According to some, "the hangover headache" may be reduced by
taking honey, which speeds alcohol metabolism, or caffeine, a
constrictor of dilated arteries. Caffeine, however, can cause
headaches as well as cure them. Heavy coffee drinkers often get
headaches when they try to break the caffeine habit.

Cluster Headaches

Cluster headaches, named for their repeated occurrence in groups
or clusters, begin as a minor pain around one eye, eventually
spreading to that side of the face. The pain quickly intensifies,
compelling the individual to pace the floor or rock in a chair.

Other symptoms include a stuffed and runny nose and a droopy
eyelid over a red and tearing eye.

Cluster headaches last between 30 minutes and 45 minutes. But the
relief people feel at the end of an attack is usually mixed with
dread as they await a recurrence. Clusters can strike several
times a day or night for several weeks or months. Then,
mysteriously, they may disappear for months or years. Many people
have cluster bouts during the spring and fall. At their worst,
chronic cluster headaches can last continuously for years.

Cluster attacks can strike at any age but usually start between
the ages of 20 and 40. Unlike migraine, cluster headaches are
more common in men and do not run in families. Research
scientists have observed certain physical similarities among
people who experience cluster headache.

People who get cluster headaches tend to be heavy smokers and
drinkers. Paradoxically, both nicotine, which constricts
arteries, and alcohol, which dilates them, trigger cluster
headaches. The exact connection between these substances and
cluster attacks is not known.

Despite a cluster headache's distinguishing characteristics, its
relative infrequency and similarity to such disorders as
sinusitis can lead to misdiagnosis. Some cluster patients have
had tooth extractions, sinus surgery, or psychiatric treatment in
a futile effort to cure their pain.

Research studies have turned up several clues as to the cause of
cluster headache, but no answers. One clue is found in the
thermograms of untreated cluster patients, which show a "cold
spot" of reduced blood flow above the eye.

The sudden start and brief duration of cluster headaches can make
them difficult to treat. By the time medicine is absorbed into
the body, the attack is often over. However, research scientists
have identified several effective drugs for these headaches. The
antimigraine drug ergotamine tartrate can subdue a cluster, if
taken at the first sign of an attack. Injections of
dihydroergotamine, a form of ergotamine tartrate, are sometimes
used to treat clusters.

Some cluster patients can prevent attacks by taking propranolol
or methysergide. Investigators have also discovered that mild
solutions of cocaine hydrochloride applied inside the nose can
quickly stop cluster headaches in most patients. This treatment
may work because it both blocks pain impulses and it constricts
blood vessels.

Another option that works for some cluster patients is rapid
inhalation of pure oxygen through a mask for 5 minutes to 15
minutes. The oxygen seems to ease the pain of cluster headache by
reducing blood flow to the brain.

In chronic cases of cluster headache, certain facial nerves may
be surgically cut or destroyed to provide relief. However, these
procedures have had limited success. Some cluster patients have
had facial nerves cut only to have them regenerate years later.

Chronic high blood pressure can cause headache, as can rapid
rises in blood pressure like those experienced during anger,
vigorous exercise, or sexual excitement.

Muscle-Contraction Headaches

Tension headache is named not only for the role of stress in
triggering the pain, but also for the contraction of neck, face,
and scalp muscles brought on by stressful events. Tension
headache is a severe but temporary form of muscle-contraction
headache. The pain is mild to moderate and feels like pressure is
being applied to the head or neck. The headache usually
disappears after the period of stress is over.

By contrast, chronic muscle-contraction headaches can last for
weeks, months, and sometimes years. The pain of these headaches
is often described as a tight band around the head or a feeling
that the head and neck are in a cast. The pain is steady, and is
usually felt on both sides of the head. Chronic
muscle-contraction headaches can cause sore scalps -- even
combing one's hair can be painful.

Many scientists believe that the primary cause of the pain of
muscle-contraction headache is sustained muscle tension. Other
studies suggest that restricted blood flow may cause or
contribute to the pain.

Occasionally, muscle-contraction headaches will be accompanied by
nausea, vomiting, and blurred vision, but there is no preheadache
syndrome as with migraine. Muscle-contraction headaches have not
been linked to hormones or foods, as has migraine, nor is there a
strong hereditary connection.

Research has shown that for many people, chronic
muscle-contraction headaches are caused by depression and
anxiety. These people tend to get their headaches in the early
morning or evening when conflicts in the office or home are
anticipated.

Emotional factors are not the only triggers of muscle contraction
headaches. Certain physical postures--such as holding one's chin
down while reading--can lead to head and neck pain. Tensing head
and neck muscles during sexual excitement can also cause
headache. So can prolonged writing under poor light, or holding a
phone between the shoulder and ear, or even gum-chewing.

More serious problems that can cause muscle contraction headaches
include degenerative arthritis of the neck and temporomandibular
joint dysfunction, or TMJ. TMJ is a disorder of the joint between
the temporal bone (above the ear) and the mandible or lower jaw
bone. The disorder results from poor bite and jaw clenching.

Treatment for Muscle-Contraction Headaches

Treatment for muscle-contraction headache varies. The first
consideration is to treat any specific disorder or disease that
may be causing the headache. For example, arthritis of the neck
is treated with anti-infammatory medication and temporomandibular
joint dysfunction may be helped by corrective devices for the
mouth and jaw.

Acute tension headaches not associated with a disease are treated
with muscle relaxants and analgesics like aspirin and
acetaminophen. Stronger analgesics, such as propoxyphene and
codeine, are sometimes prescribed. As prolonged use of these
drugs can lead to dependence patients taking them should have
periodic medical check ups and follow their physicians'
instructions carefully.  All medications carry risks.
Individuals should weigh the benefits, risks and costs of
treatment alternatives.

Nondrug therapy for chronic muscle-contraction headaches includes
biofeedback, relaxation training, and counseling. A technique
called cognitive restructuring teaches people to change their
attitudes and responses to stress. Individuals might be
encouraged, for example, to imagine that they are coping
successfully with a stressful situation.

In progressive relaxation therapy, people are taught to first
tense and then relax individual muscle groups. Finally, the
individual tries to relax his or her whole body. Many people
imagine a peaceful scene or memory. Passive relaxation does not
involve tensing of muscles. Instead, individuals are encouraged
to focus on different muscles, suggesting that they relax.

Individuals with chronic muscle-contraction headaches may also be
helped by taking antidepressants or MAO inhibitors. Mixed
muscle-contraction and migraine headaches are sometimes treated
with barbiturate compounds, which slow down nerve function in the
brain and spinal cord.

Some individuals who suffer infrequent muscle-contraction
headaches benefit from hot showers or moist heat applied to the
back of the neck. Cervical collars are sometimes recommended as
an aid to good posture. Physical therapy, massage, and gentle
exercise of the neck may also be helpful.

When Is Headache a Warning for More Serious Disorders?

Like other types of pain, headaches can serve as warning signals
of more serious disorders. This is particularly true for
headaches caused by traction or inflammation.

Traction headaches can occur if the pain-sensitive parts of the
head are pulled, stretched, or displaced, as for example, when
eye muscles are tensed to compensate for eyestrain.

Headaches caused by inflammation include those related to
meningitis as well as those resulting from diseases of the
sinuses, spine, neck, ears, and teeth.  Ear and tooth infections
and glaucoma can cause headaches.  In oral and dental disorders,
headache may be experienced as pain in the entire head, including
the
face.

Traction and inflammatory headaches are treated by curing the
underlying problem. This may involve surgery, antibiotics or
other drugs.

Characteristics of the various types of traction and inflammatory
headaches vary by disorder:

Brain tumors: Brain tumors are diagnosed in about 11,000 people
every year. As they grow, these tumors sometimes cause headache
by pushing on the outer layer of nerve tissue that covers the
brain or by pressing against pain-sensitive blood vessel walls.
Headache resulting from a brain tumor may be periodic or contin-
uous. Typically, it feels like a strong pressure is being applied
to the head. The pain is relieved when the tumor is destroyed by
surgery, radiation, or chemotherapy.

Stroke: Headache may accompany several conditions that can lead
to stroke, including hypertension or high blood pressure,
arteriosclerosis, and heart disease. Headaches are also
associated with completed stroke, the latter occurs when brain
cells die from lack of sufficient oxygen.

Many stroke-related headaches can be prevented by careful
management of the person's condition through diet, exercise, and
medication.

Mild to moderate headaches are associated with transient ischemic
attacks (TIA's), which result from a temporary lack of blood
supply to the brain. The head pain occurs near the clot or lesion
that blocks blood flow.

The similarity between migraine and symptoms of TIA can make
diagnosis difficult. The rare person under age 40 who suffers a
TIA may be misdiagnosed as having migraine; similarly, TlA-prone
older patients who suffer migraine may be misdiagnosed as having
stroke-related headaches.

Spinal Tap: About one-fourth of the people who undergo a lumbar
puncture or spinal tap develop a headache. Many scientists
believe tnese headaches result from leakage of the cerebrospinal
fluid that flows through pain-sensitive membranes around the
brain and down to the spinal cord.

The fluid, they suggest, drains through the tiny hole created by
the spinal tap needle, causing the membranes to rub painfully
against the bony skull. Since headache pain occurs only when the
patient stands up, the "cure" is to remain lying down until the
headache runs its course--anywhere from a few hours to several
days.

Head trauma: Headaches may develop after a blow to the head,
either immediately or months later. There is little relationship
between the severity of the trauma and the intensity of headache
pain. One cause of trauma headache is scar formation in the
scalp.

Another is ruptured blood vessels which result in an accumulation
of blood called a hematoma. This mass of blood can displace brain
tissue and cause headaches as well as weakness, confusion, memory
loss, and seizures. Hematomas can be drained to produce rapid
relief of symptoms.

Arteritis and meningitis: Arteritis, an inflammation of certain
arteries in the head, primarily affects people over age 50.
Symptoms include throbbing headache, fever, and loss of appetite.
Some patients experience blurring or loss of vision. Prompt
treatment with corticosteroid drugs helps to relieve symptoms.
Headaches are also caused by infections of meninges, the brain's
outer covering, and phlebitis, a vein inflammation.

Tic douloureux: Tic douloureux, or trigeminal neuralgia, results
from a disorder of the trigeminal nerve. This nerve supplies the
face, teeth, mouth, and nasal cavity with feeling and also
enables the mouth muscles to chew. Symptoms are headache and
intense facial pain that comes in short, excruciating jabs set
off by the slightest touch to or movement of trigger points in
the face or mouth.

People with tic douloureux often fear brushing their teeth or
chewing on the side of the mouth that is affected. Many tic
douloureux patients are controlled with drugs, including
carbamazepine. Patients who do not respond to drugs may be helped
by surgery on the trigeminal nerve.

Sinus infection: In a condition called acute sinusitis, a viral
or bacterial infection of the upper respiratory tract spreads to
the membrane which lines the sinus cavities. When one or all four
of these cavities are filled with bacterial or viral fluid, they
become inflamed, causing pain and sometimes headache. Treatment
of acute sinusitis may include antibiotics, analgesics, or
decongestants.

Chronic sinusitis may be caused by an allergy to such irritants
as dust, ragweed, animal hair, and smoke. Research scientists
disagree about whether chronic sinusitis triggers headache.

A Childhood Problem

Like adults, children experience the infections, trauma, and
stresses that can lead to headaches. In fact, research shows that
as young people enter adolescence and encounter the stresses of
puberty and secondary school, the frequency of headache
increases.

Migraine headaches often begin in childhood or adolescence. The
National Institutes of Health report that over 1 million children
age 16 and under experience migraine and other vascular
headaches.

Children with migraine often have nausea and excessive vomiting.
Some children have periodic vomiting, but no headache--the
so-called "abdominal migraine." Research scientists have found
that these children usually develop headaches when they are
older.

Phenobarbital, cypropheptadine, and certain anticonvulsant drugs
are used to treat migraines in children. A diet may be prescribed
to protect the child from foods that trigger headache. Sometimes
psychological counseling or other psychiatric treatments for the
child and the parents are recommended.

Studies have shown that thermal biofeedback can help children
with migraines control their headaches.

Childhood headache can be a sign of depression. Parents should
alert the family pediatrician if a child develops headaches along
with other symptoms such as a change in mood or sleep habits.

Antidepressant medication and psychotherapy may be effective
treatments for childhood depression and related headache.  All
medications, however, carry risks.  Parents should carefully
weigh the benefits, risks and costs of treatment alternatives for
their children.

Source: Adapted from Headache: Hope Through Research
U.S. Department of Health and Human Services, National Institutes
of Health
!
*
Headaches

                           HEADACHES


                       TYPES OF HEADACHES

All of us suffer from an occasional headache; in fact, 20 million
Americans see their doctors each year because of headaches.
Although headaches can be very uncomfortable and temporarily
disabling, most are not associated with serious illness and can be
relieved by resting in a quiet room or by taking a nonprescription
painkiller, such as aspirin or acetaminophen.  Some types of headaches,
however, may require stronger prescription medications, and others are
warning signs to seek immediate medical attention.  These warning
signs include:

  * Severe, sudden headaches that seem to come on like a
    "bolt out of the blue."
  * Headaches accompanied by loss of consciousness, alertness
    or sensation; confusion; visual blurring, or other
    neurological changes.
  * Recurrent headaches affecting one particular area, such
    as an eye, temple, etc.
  * Recurrent headaches of increasing intensity or frequency.
  * Headaches accompanied by neck stiffness and fever.
  * Headaches that wake you up.
  * Any unexplained change in the nature or frequency of
    headaches.


TENSION HEADACHES

The most common headaches are those associated with
tension or muscle contractions and are directly related to
stress.  The pain tends to be steady and dull rather than
throbbing.  It is usually felt in the temples, forehead, neck or
back of the head.  Sometimes, the pain seems to encircle
the head like a tight band.  Tension headaches may occur
at any time, but are most commonly experienced during
periods of stress or worry.

Treatment involves relieving the tension through massage,
heat, a hot shower, relaxation techniques or any activity that
puts aside the worries of the moment.  Nonprescription
painkillers, such as aspirin or acetaminophen, may also help.
For severe muscle tension headaches, other slightly more
potent drugs may be prescribed.  These drugs may cause
drowsiness and slow reflexes and should be taken with
caution by people who work with machinery or drive.  Most
doctors also recommend that such medication be used for
only short periods of time (not more than a few days).


MIGRAINE HEADACHES

Migraine headaches vary from person to person, but
typically they are throbbing headaches affecting one side of
the head.  They are often accompanied by a number of
other symptoms, which also vary according to individual
cases.  Some people have very little head pain but suffer
from distorted vision and hearing or feelings of intense
anxiety.  Others may suffer from incapacitating pain lasting
for several days.  Most migraine patients fall between these
two extremes.

Many people are warned of an impending migraine attack by
bizarre distortions of size, position, time and place -- the so-
called "Alice-in-Wonderland syndrome."  Others see flashing
lights or bright colors in unusual shapes.  Nausea, vomiting,
chills, fever, dizziness, diarrhea; abdominal, arm or leg pain;
and sensitivity to light are still other symptoms that may
accompany a migraine.

Regardless of the differing symptoms, all migraine
headaches are related to changes in the blood vessels of
the head and neck. This is why they are often referred to as
vascular headaches.  Early warning symptoms of a migraine
are thought to be caused by a narrowing of these blood
vessels, while the head pain is believed to be a result of the
subsequent expanding or dilating of the vessels.  What
causes these changes is unknown, although  many
researchers now belie