WHAT IS ALS/MND? WHAT ARE THE SYMPTOMS OF
ALS/MND? WHAT IS THE AVERAGE LIFE EXPECTANCY?
HOW COMMON IS ALS/MND? HOW IS ALS DIAGNOSED?
WHO IS LIABLE TO GET ALS/MND? WHAT DOES "AMYOTROPHIC
LATERAL SCLEROSIS" MEAN? ARE THERE DIFFERENT
TYPES OF ALS/MND? WHEN WAS ALS FIRST DISCOVERED?
WHAT CAUSES ALS/MND? CAN YOU "CATCH"
ALS/MND? WHAT ABOUT ENVIRONMENTAL CAUSES?
IS THERE ANY TREATMENT AVAILABLE? WHAT CAN
I DO TO HELP? WHERE CAN I LEARN MORE?
WHAT IS ALS/MND? ALS/MND--Amyotrophic Lateral
Sclerosis, aka Motor Neurone Disease--is
a rapidly progressive, fatal neuromuscular
disease. It attacks motor neurons in the
spinal cord and lower brain which transmit
signals from the brain to the voluntary muscles
throughout the body. When motor neurons die
as a result of ALS, the ability of the brain
to control muscle movement is lost. When
muscles fail to receive messages, they weaken,
atrophy, and die. There is no known cure.
ALS/MND is also known as Lou Gehrig's Disease,
and in French, maladie de Charcot.
WHAT ARE THE SYMPTOMS OF ALS/MND? The groups
of muscles affected and the order in which
they are affected varies from one person
to another. Early symptoms usually include
tripping, dropping things, abnormal fatigue
of the arms and/or legs, slurred speech,
muscle cramps and twitches and uncontrollable
periods of laughing or crying. The hands
and feet may be affected first, causing difficulty
in walking or using the hands for the activities
of daily living such as dressing, washing
and buttoning clothes.
About 25% of patients have bulbar (throat)
onset, which means that voice and swallowing
are first affected. About 50% have arm onset,
and 25% leg onset.
The disease frequently takes its toll before
being positively diagnosed: many patients
are significantly debilitated before learning
that they have ALS/MND.
Muscle wasting gradually spreads to the muscles
of the trunk of the body, and the disease
eventually affects swallowing, chewing and
breathing. Complete paralysis eventually
results, usually occurring within two to
five years of diagnosis.
When the diaphragm is attacked, the patient
is unable to breathe for him/herself, and
faces permanent ventilatory support in order
to survive.
ALS/MND attacks only 'motor' neurons. Sight,
touch, hearing, taste, smell and muscles
of the eyes and bladder are generally not
affected. Sexual function and drive are not
affected. The mind is not affected, and remains
sharp despite the progressive degenerating
condition of the body.
Dennis Kaye, a Canadian ALS victim, wrote
an inspirational book on his struggle: "Laugh?
I Thought I'd Die". He said of his like:
"With keen mind and open eyes We watch
ourselves die."
WHAT IS THE AVERAGE LIFE EXPECTANCY? This
is between two and five years for the newly
diagnosed person. Improved medical care is
resulting in longer and more productive lives
for people with ALS/MND. Twenty percent will
live more than five years and up to 10% will
survive more than ten years.
HOW COMMON IS ALS/MND? Once thought rare,
ALS/MND is now fairly common.
In terms of INCIDENCE (how often does it
strike), a normal population produces about
2 new cases per 100,000 every year. (A 1995
Dalhousie University Study found in that
there was a 2.03 per 100,000 population incidence
in Nova Scotia.)
(As ALS/MND is terminal and incurable, death
rates are very close to incidence rates.)
According to the United States National Institute
of Health, some 5,000 people in the U.S.
are newly diagnosed with ALS each year. (That's
about 13 new cases every day!)
Many scientists believe the incidence of
ALS/MND is increasing, though some feel this
may be due to improved understanding of the
illness and better diagnostic techniques.
In terms of PREVALENCE (how many are affected
at any one time), ALS/MND affects about seven
to eight people out of every 100,000.
It is estimated that as many as 30,000 Americans
have the disease at any given time, and that
some 300,000 Americans who are alive and
apparently well today will die with ALS.
In Canada, from a population of about 30
million, two to three Canadians die every
day of ALS/MND. Over 2,500 Canadians currently
have ALS. Some 20,000 to 25,000 Canadians
who are alive and apparently in good health
will die of ALS/MND.
HOW IS ALS/MND DIAGNOSED? At present there
is no definitive means of diagnosis of ALS/MND.
Most diagnoses are made by eliminating all
other possibilities--ailments whose symptoms
resemble those of ALS/MND. Neurologists use
a number of clinical tests to establish a
profile, including blood testing, EMG, MRI,
etc.
WHO IS LIABLE TO GET ALS/MND? Anyone can
get ALS/MND. Most who develop ALS/MND are
between the ages of 40 and 70. There are,
however, cases of the disease attacking persons
in their twenties and thirties. Generally
though, ALS/MND occurs in greater percentages
as men and women grow older.
ALS/MND strikes men slightly more frequently
than women. About one in 800 adult men, and
one in 1,200 adult women die of ALS/MND.
ALS/MND occurs throughout the world with
no racial, ethnic or socioeconomic boundaries.
90% of ALS/MND cases are people with no family
history of the disease. Ten percent of the
cases are classified as familial or inherited
ALS/MND.
WHAT DOES "AMYOTROPHIC LATERAL SCLEROSIS"
MEAN? "A-myo-trophic" comes from
the Greek language. "A" means no
or negative. "Myo" refers to muscle,
and "Trophic" means nourishment---"No
muscle nourishment." When a muscle has
no nourishment, it "atrophies"
or wastes away. "Lateral" identifies
the areas in a person's spinal cord where
portions of the nerve cells that nourish
the muscles are located. As this area degenerates
it leads to scarring or hardening--"sclerosis"--in
the region.
WHY IS IT ALSO KNOWN AS "LOU GEHRIG'S
DISEASE"? Lou Gehrig, "The Iron
Horse", was a baseball superstar in
the late 20's and 30's, one of the "Golden
Ages" of baseball. After 14 glory-filled
seasons, Lou Gehrig's career, and his life,
were cut short by ALS/MND.
New York born-and-bred Lou Gehrig had brief
but impressive call-ups with the New York
Yankees in 1923 and '24, and made the big
club for good in 1925. On the first of June,
manager Miller Huggins called on Gehrig to
start at first base for the injured Wally
Pipp. So began one of sport's truly amazing
feats. On May 2, 1939, an ailing Lou Gehrig
would take himself out of the lineup. In
between, Gehrig appeared in every one of
the Yankees' 2,130 regular-season games.
Though today Lou Gehrig's name may bring
"the illness" and "the streak"
to mind, he was in his day a true superstar.
His accomplishments will forever keep him
among the legends of the game. Gehrig hit
for power, hit for average, and was a consistent
run producer. He was twice named the American
League's Most Valuable Player. In his 14
full seasons the Yankees won seven World
Series championships. The Wall Street Journal
recently ran an article which stated that
statistically, as measured by "contribution
to team success" Lou Gehrig was the
greatest player of all time, and that his
compensation in today's market would exceed
$10 million per year.
Many call the Yankees "Murderers' Row"
team of 1927 the greatest team in history.
In that year, Gehrig's teammate the legendary
Babe Ruth hit 60 home runs, a mark only once
equalled (by Roger Maris with 61 in 1961,
in a longer season). And in that year Lou
Gehrig batted .375, hit 47 home runs with
52 doubles and 18 triples. He drove in 175
runs, and was named League MVP. He repeated
as MVP 1936. In 1934, Gehrig won the Triple
Crown for leading the league in batting average,
home runs, and runs batted in: .363, 49,
and 165.
Lou Gehrig batted .295 in his rookie year
and his last full year, 1938; the only full
seasons he hit under .300--in 1930 he hit
.379. Over his career he hit 23 grand slams,
a record to this day. He *averaged* an astonishing
147 RBI's per season. As the 1939 season
opened, Lou Gehrig was an obviously sick
man. He would play in only eight more games.
In Washington on April 30, he singled for
career hit #2721. That hit was his last.
Two days later, at Detroit's Briggs' Stadium,
he took himself out. He played in an exhibition
game against the Yankee farm club in Kansas
City, then flew to Rochester for examination
at the Mayo Clinic. On June 19, his 36th
birthday, he was finally diagnosed with Amyotrophic
Lateral Sclerosis. (The NY Times called the
illness "a form of infantile paralysis.")
On the fourth of July, 1939, 60,000 packed
Yankee Stadium to honour a hero. And on June
2, 1941, just short of his 38th birthday,
Lou Gehrig died.
Lou Gehrig's consecutive game streak, one
of the greatest records in the history of
sport, was never so much as approached until
Baltimore Orioles' shortstop Cal Ripken Jr.
surpassed it in 1995. The occasion generated
a great deal of much-needed publicity of
the illness, and was marked by the founding
of a research fund in Mr. Ripken's name.
(Nathan Brown has a terrific tribute page
to this great man at http://www.nmia.com/~browns/gehrig.htm
)
ALS/MND claimed the life of actor David Niven,
US Senator Jacob Javits, and afflicts physicsist
Stephen Hawking. Jon Stone, co-creator of
Sesame Street, died in 1997 of ALS/MND. Michael
Zaslow, popular American daytime drama actor,
was diagnosed with ALS in 1997.
ARE THERE DIFFERENT TYPES OF ALS/MND? There
are three common classifications, and numerous
sub-categories: Sporadic: the most common
form of ALS/MND Familial: less than 10% of
ALS/MND cases suggest genetic inheritance
Guamanian: a high number of cases of ALS/MND
occur in Guam and the Trust Territories of
the Pacific
WHEN WAS ALS/MND FIRST DISCOVERED? ALS/MND
was first described in 1869 by Jean-Martin
Charcot, a trail- blazing French neurologist.
WHAT CAUSES ALS/MND? The cause of ALS/MND
is not yet known. While many theories are
being researched, at present neither a cure
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/MND
patients have this genetic predisposition,
there is no evidence of a clinical difference
between the familial and the sporadic forms
of the illness.
A currently favoured theory combines genetics
and toxicity (poisons): that sporadic ALS/MND
occurs in those with a genetic predisposition
to motor neuron degeneration, and that the
illness is then triggered by environmental
insult. In Nov 1996, Dr. Jeffrey Rothstein
of Johns Hopkins University found that 42%
of sporadic ALS/MND patients have a defect
in the gene that controls the protein EAAT2,
a protein which helps to regulate the brain's
glutamate levels, well known to be a factor
in motor neuron degeneration.
WHAT ABOUT ENVIRONMENTAL CAUSES? The very
high incidence of ALS/MND 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/MND
symptoms.
CAN YOU "CATCH" ALS/MND? ALS/MND
cannot be "caught" -- it is not
contagious. Though some scientists believe
it is possible that ALS/MND is caused by
a slow-acting or latent "virus",
there is absolutely no fear that it is contagious:
there is no increased incidence among medical
personnel who deal with ALS/MND patients.
IS THERE ANY TREATMENT AVAILABLE? While no
drug has been found which has been proven
to prevent, reverse, or stop the progression
of ALS, a drug which may slow the progression
is now available. Rilutek has been shown
to extend life expectancy by approximately
three months. Earlier use of Rilutek or use
of Rilutek in combination with other drugs
may prove even more effective. Information
on Rilutek is available from your doctor
or from several websites including this one
from its manufacturer:
http://www.alsinfo.com/rilutek_facts_care
Note -- This info is tailored for US customers.
Information for other countries is available
but limited to contact phone numbers. To
access them, go to: http://www.rp-rorer.com/
Other drugs being developed include the Sanofi
drug (SR57746A) and various nerve growth
factors. These drugs remain in the research
stage.
Other areas of on-going research focus on
finding the cause rather specific treatment.
This includes investigation into a possible
viral link, the role of calcium binding proteins,
coenzyme Q10, enzyme SOD1, and glutamate
toxicity.
Efforts also continue to identify and develop
tests for the presence of additional autoantibodies
in patients with Motor Neuron Diseases. The
presence of antibodies suggest the patient
may benefit from immunosuppressive treatments.
The discovery of some of these antibodies
and successful treatment of patients has
resulted in a revision of the diagnostic
criteria for ALS. Similarly, there is new
emphasis on the possible misdiagnosis of
some patients with Lyme disease as having
ALS.
Drugs such as Neurontin, Baclofen, snake
venom, etc. were developed for other diseases
and continue to mentioned as possibly helpful
in ALS. At this time none of these drugs
have been proven effective in ALS although
some continue to be tested. Because these
drugs are already approved for other uses
by regulating agencies they can be prescribed
by physicians and are therefore sometimes
used by individual doctors outside of a formal
research program. This may be in response
to a specific request by a patient, as an
attempt to control a specific symptom in
a particular patient, or simply the individual
doctor's belief that he has seen some positive
effect from use.
While conventional medicine cannot offer
a cure or treatment that slows progression
substantially, it does offer supportive care
such as medication to ease muscle spasms,
techniques to reduce the risk of choking,
varying levels of respiratory support, therapy
to maximize mobility and flexibility, and
equipment to assist with activities of daily
living.
Alternative medicine also offers a variety
of treatments. The value of these treatments
varies considerably. Some, such as use of
dietary supplements containing anti-oxidants,
creatine, Vit. E, are possible solutions
suggested by medical research. Many of these
products are now being evaluated in controlled
tests, but as "natural" products
they are readily available over the counter
and so are being widely used by individual
ALS patients. Some of these may have benefit,
others may turn out to be just the latest
fad in ALS treatment.
Other treatments such as removal of dental
mercury filling have been tested and rejected
by the medical community but continue to
have strong supporters and create controversy.
Still others continue to be promoted despite
a lack of any scientific basis. In general,
ALS patients should be highly suspicious
of treatments supported only by testimonial
evidence (recommendations from supposed users),
those purported to be effective in a large
number of unrelated diseases, those which
have been available for many years but never
subjected to controlled research, and those
offered by only a single doctor or clinic.
WHAT CAN I DO TO HELP? ALS/MND takes a tremendous
toll on the physical, financial, and emotional
resources of its victims and their families.
ALS/MND societies and support groups around
the world do much to help, but there's always
need for more.
If you know someone with ALS/MND, offer your
time to relieve the family members of the
constant strain. Or just to talk, to listen,
to be a friend.
Spread the word. We must continue to raise
public awareness of this devestating killer.
Get involved, find out, write, phone, talk,
shout about it.
Give money. Patients often need expensive
home renovations, specialized equipment,
therapy, and nursing care, for an open-ended
time frame. And science needs funding to
intensify the search for cause and cure,
so we can eliminate this nightmare.
WHERE CAN I LEARN MORE? The Internet and
World Wide Web contain a tremendous amount
of information on ALS/MND. Support groups,
universities, medical and scientific associations,
drug companies, and many individuals are
represented, most with links to other sites.
A separate FAQ on ALS Internet Resources
will be posted regularly along with this
FAQ.
We will not be hidden and left to die ever again!!
Get used to it."... Kyle George Hahn (PALS dx 1995)
