Amyotrophic Lateral Sclerosis is a deadly, little-known but fairly
common disease of the nervous system.  The cause is not known and
there is no known cure.  Life expectancy is generally considered to
be about 2 to 4 years.

The disease is generally referred to as ALS.  It is also known as
Lou Gehrig's disease after the famous baseball player who died of
it.  It also killed actor David Niven.

ALS can strike anyone.  It generally occurs between the ages of 20
and 90, with incidence normally increasing with age and peaking in
the 60's and 70's.  It is as common as Multiple Sclerosis.

In a study in southwestern Ontario, over a period of years, of all
patients who had died of ALS, the average time from diagnosis to
death was 2.5 years.

There is no treatment for the disease itself.  Some surgical
procedures and some drugs are used to treat symptoms, but the cause
and cure remain medical mysteries.

ALS attacks the motor neurons which convey the brain's message
throughout the body.  When the muscles do not receive these
messages, they wither, lose their strength and become useless.  The
patient is paralyzed.

ALS manifests itself in different ways, depending on which muscles
weaken first.  Some people begin to trip and fall; some lose the
use of their hands and arms; some find it difficult or impossible
to swallow and some have difficulty breathing.  Sometimes
persistent fatigue is the first symptom.  As the disease
progresses, muscle twitching and cramping - sometimes severe -
frequently occurs.

The disease may be present for some time before the symptoms cause
any concern.  This is due to the gradual weakening of the muscles.
Usually this means that many cells have been lost and the disease
is well established before it is identified.  Due to the rather
vague nature of many of the symptoms, such as fatigue, diagnosis is
often difficult.

Because the disease has been taking its toll before it is
identified, almost all victims are already handicapped in some way
when they learn they have ALS.

Eventually, as the disease progresses, all the voluntary muscles
become useless.  The patient cannot move, eat, breathe or
communicate and may choose to be kept alive by life support
systems.  If death does not occur and the disease runs its full
course, the patient becomes completely paralyzed.

Generally, the disease moves quickly although it may proceed slowly
for a number of years.  It may plateau for a period of time.
Usually it proceeds at a fairly even rate which will vary from
patient to patient.

ALS generally strikes people with no history of the disease in
their families.  However, about 5 percent of patients have, or have
had, other family members with ALS.

Fortunately the disease does not affect the mind or the senses -
touch, taste, sight, smell and hearing.  It does not have a direct
effect on the bowel, bladder or sexual functions.

Severe pain is not generally associated with the disease, although
this may be a matter of definition.  As the ALS patient moves into
the later, life-support stages of the disease, the quality of life
deteriorates dramatically.  This subject is explored in depth in
the book, "This Far and No More", the true account of an ALS
patient who finally chooses death through the removal of her
respirator tube.  It is by Andrew Malcom and published by Random
House.

ALS occurs worldwide.  Throughout most of the world, the incidence
is about the same.  In Canada, parts of southwestern Ontario have
an unusually high incidence of ALS.  ALS is not well known but it
is not a rare disease.  It has been recognized for more than 100
years.  The short life span of most ALS patients is the principal
reason for this lack of public awareness.  It is not unusual for a
patient to die within a few months of identification of the
disease.

At any given time, therefore, there are relatively few people alive
who have ALS, and very few indeed in any small community.  In
addition, patients tend to grow weak very quickly and their
families have all they can do to keep the person functioning.
Neither patient nor family have much time for public exposure -
they are not seen, not heard and do not excite the degree of
interest and sympathy generated by many other diseases.