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What is ALS?
Why is it known as Lou Gehrig's disease?
Lou Gehrig, a famous baseball player in the U.S. during the 1930's,
became afflicted with ALS. He was known as baseball's "Ironman".
Strength, agility, excellent health - Lou Gehrig had everything it took
to become a baseball legend. But Lou Gehrig had something else. At the
peak of his career, he was diagnosed as having Amyotrophic Lateral
Sclerosis (ALS). He died at
the age of 38.
When was ALS first discovered?
ALS was first described in 1869 by Jean-Martin Charcot, a French
neurologist. Since that time a number of theories about the cause of
ALS have been developed. Some scientists believe it is possible that
ALS is caused by a slow-acting or latent "virus". If it is caused by an
organism, there is absolutely no fear that it is contagious. There is
no increased incidence among medical personnel who deal with ALS
patients. Work has also been done on the possible role of the thyroid
gland and trauma.
What causes ALS?
The cause is not yet known although several theories are now being
researched. At present neither a cure for ALS nor a means of prevention
is known. In 1993, scientists announced in a paper published in the
British journal "Nature" that they had isolated the gene associated
with about 20% of the cases
of the inherited form of the disease. While only 10% of ALS patients
have
this genetic predisposition, there is no evidence of a clinical
difference
between the familial and the sporadic forms of the illness.
What about environmental causes?
The very high incidence of ALS on the island of Guam, in Western New
Guinea and on Kii peninsula of Japan may provide some clues about
environmental influences. Heavy metals such as lead and mercury are
suspected causes, as is aluminium, which can poison the body and cause
ALS symptoms. Some people may have a genetic makeup which makes them
susceptible to an environmental cause of ALS.
What parts of the body does it affect?
Because it attacks only motor neurons, ALS does not affect the mind.
The person with ALS remains mentally sharp and in full possession of
the
senses of sight, hearing, taste, smell and touch. Bladder and bowel
muscles
are generally not affected by ALS. ALS seldom causes pain, although
some
people do have cramps and secondary discomfort from lengthy sitting or
lying
down.
Is sexual function affected by the disease?
No.
Are there different types of ALS?
There are three classifications:
· Sporadic
(which is the most common form of ALS)
· Familial (a
small number of cases suggest genetic inheritance of ALS)
· Guamanian (a
high number of cases of ALS occur in Guam and the Trust Territories of
the Pacific)
What are the early symptoms of ALS?
ALS usually becomes apparent either in the throat or upper chest area
or in the arms and legs. Some people begin to trip and fall; some lose
the use of their hands and arms; some find it hard to swallow and some
slur
their speech.
Can you "catch" ALS? And what does it do?
ALS cannot be "caught" - it is not contagious. In 90% of ALS cases, it
strikes people with no family history of the disease. Ten percent of
the cases are classified as familial or inherited ALS. It may occur at
any
age, with the likelihood increasing as people grow older. However, many
are struck down in the prime of life. ALS occurs equally in men and
women.
Because the disease frequently takes its toll before being positively diagnosed, many patients are debilitated before learning they have contracted ALS. The disease does not affect the senses of taste, touch, sight, smell and hearing, or the mind.
ALS wreaks a devastating effect on patients as well as their families. As they struggle to cope with the prospect of advancing disability and death, it consumes their financial and emotional reserves. It is a costly disease in its later stages, demanding both extensive nursing care and expensive equipment.
Is there hope for people with ALS?
Yes, certainly. Based on recent medical discoveries, drug trials are
now underway. Advances in our knowledge about other neurological
diseases
may also continue to shed light on the cause of ALS and help us find a
cure.
What is the incidence of ALS? How many people in Canada are
affected?
It is not a rare disease, anyone can get it. It affects about six or
seven people out of every 100,000. Over 3,000 Canadians currently have
ALS. Two to three Canadians die every day of ALS. Most people with ALS
are between the ages of 50 and 75 though there are cases of teenagers
with
the disease. In about 5-10% of cases of ALS there is a hereditary
pattern
About 90-95% of cases are "sporadic" ALS: anyone can be affected.
How long until complete paralysis?
This can occur at anytime within two to five years of diagnosis.
What is the average life expectancy?
This is between two and three years for the newly diagnosed person.
However, it is important to understand that improved medical care is
resulting in longer and more productive lives for people with ALS.
Twenty percent will live more than five years and up to 10% will
survive more than ten years.
Drug therapy
Potential new drug therapy for ALS
· Riluzole
· IGF-1
· Neurotin
· Sanofi
SR57746A
· GDNF
Riluzole—Rhône-Poulenc Rorer are in the process of filing a second appeal with the Federal Government. It is expected that this process may take up to six months before the company can be heard.
IGF-1 (myotrophin) is a growth factor administered subcutaneously. At the present time, about 200 people (drawn from 2000 names) are receiving it in the U.S. It is not available in Canada.
Neurotin (Gabapentin) is an oral drug produced by Parke Davis. It does not show many side effects and can slow down the disease by 10 to 15 percent. This drug is expensive - $250 per month - but most insurance plans in most provinces cover 80 percent of the cost.
Sanofi SR57746A is a compound which exhibits neurotrophic (nerve sustaining) and neuroprotective (preventing nerve death) effect. Developed by Sanofi Recherche of France, the trials began in May 1997 in approximately 60 centres in Europe and North America. There are currently two studies using SR57746A. One (EFC 1923) will include about 1200 volunteers who will already be taking Rilutek and who, in addition, will receive SR57746A or placebo. The second study (EFC 2941) will include about 800 volunteers who are not taking Rilutek and will compare SR57764A against the placebo. Canada has eight centres participating, including Vancouver General Hospital. Approximately 50 patients in Canada are enrolled to date with Vancouver accounting for 34 of the 50 people involved in the study. Although the mechanism is not fully understood, SR57746A appears to mimic the activity, or to stimulate the natural production of neurotrophic factors in the body. Only those who are referred by their physicians or neurologists to the principal investigators at one of the participating centres could have been part of this trial. The drug will be administered in capsule form. Two doses are being tested: 1 mg. per day and 2 mg. per day. No serious drug-related side effects were detected in the preliminary study.
GDNF can be administered by a catheter inserted in the brain in which the drug is injected. It shows interesting results in animal testing in the U.S. and Edmonton, Alberta.
For additional information on the above, please contact the Amyotrophic Lateral Sclerosis Society of Canada at (416) 362-0269 or 1-800-267-4257.
This information was developed by the Amyotrophic Lateral Sclerosis Society of Canada and is herewith used with permission.
Amyotrophic Lateral Sclerosis Society of Canada. What is ALS? Available at: http://www.als.ca/alsWHAT.htm. Accessed October 21, 1999.
The information in this document is for general educational purposes only. It is not intended to substitute for personalized professional advice. Although the information was obtained from sources believed to be reliable, Arbor Publishing Corp, its representatives, and the providers of the information do not guarantee its accuracy and disclaim responsibility for adverse consequences resulting from its use. For further information, consult a physician and the organization referred to herein.