Amyotrophic lateral sclerosis (ALS) is a disease that has a lot of research poured into it, but one that
evades even the most studious of medical professionals. Very little is known of it. What is known of it can basically be explained
in the translation of its name. "Amyotrophic" means "without muscle nourishment"; the muscles are not receiving signals from
the nervous system to move, they are not receiving nourishment. "Lateral" means "to the side"; the side of the spinal cord
is the initial site of damage in an ALS victim's body. "Sclerosis," or "hardened," refers to the hardening of the spinal cord
as a result of the damage inflicted by ALS.
Except for the 5%-10% of cases that involve heredity, no cause of ALS has been found. Without an absolute cause, it's hard
to find a cure (kind of like with cancer).
ALS is a degenerative disease, it deteriorates the motor neurons in the brain and spinal cords. Motor neurons are the little
messengers sent out by the nervous system to tell the muscles to move. ALS attacks these motor neurons, causing the victim
to lose control of his muscles. Every year in America 1-2 people per 100,000 develop ALS; that figures out to about 5,000
new victims per year. Once a person has been diagnosed with ALS, they usually are given a life expectancy of 3-6 years. The
disease can strike anyone at any age, but it most often it strikes in the middle-aged and elderly, more commonly in men.
It's a quirky disease, the truth be told; the quirkiness can be both fascinating and frustrating to victims and their doctors.
For instance, victims cognitive and imaginative brain function is left intact. Though the victim eventually cannot talk or
move, they can still think, remember, compute and daydream - and they are very aware of how "trapped" they are in their own
bodies. Freaky. Therefore depression is a common side effect of ALS.
ALS victims also retain all 5 senses, including touch. Other forms of sclerosis involve numbness or tingling, but ALS does
not. As with other diseases, victims' bodies react to and tolerate ALS in different ways. Some ALS victims do not make it
to the 3-year mark after diagnosis while a small number of victims live for decades (Stephen Hawking, well-known physicist/author,
is perhaps the most incredible example of victim survival; he has lived with ALS since the 1960s). Involuntary muscles, such
as the heart and bowels, are not directly affected by ALS, although they are indirectly affected by various complications
involved with ALS. The only pain a victim usually feels is muscle stiffness and cramping as a result of not moving.
In Lou Gehrig's case, a doctor simply had to look at him to tell he had ALS because the doctor's mother died from the disease.
Other doctors aren't so fortunate to arrive at the difficult diagnosis of ALS quite so easily. It is a particularly challenging
disease to identify. The ALS diagnosis procedure is fundamentally a process of elimination. Because its symptoms (loss of
coordination, difficulty talking, persistent weakness in an appendage) match those of many other diseases, including muscular
dystrophy, the diagnosis process can be rather drawn out, the victim going through test after test.
Though there are a number of treatments for ALS, none of them has been shown to stop the progression of ALS. The best victims
can hope for at this point is a regiment of drugs and devices that help in coping with the symptoms of ALS a bit easier.
Thanks to a drug called Rilutex, muscle function sticks around a little longer in a victim's body, and has been shown to
extend life expectancy about 3 months. Baclofen eases muscle stiffness. To help with the inevitable weight loss, many victims
take branched-chain amino acids (BCAAs) nutritional supplements. Phenytoin eases cramps. Tricyclic antidepressants cut down
the amount of involuntary drooling involved with ALS.
New hope came recently when researchers reported on a new form of treatment referred to "ALS cocktail." The cocktail combines
minocycline (an antibiotic used to treat several brain diseases) with creatine (a dietary supplement also used to treat brain
diseases). In test mice genetically programmed to develop ALS, the ones given the cocktail lived 25% longer than those who
were not. This discovery was a step toward better lives for ALS victims - a small, shaky step, but still a progression.
OTHER FACTS ABOUT ALS:
- In America, the common nickname for ALS is "Lou Gehrigs disease." In other parts of the world, the common name for the
disease is "motor neuron diseas" (leave it to non-Americans to use a more aptly descriptive nickname)
- ALS was nicknamed "Lou Gehrig's Disease" because Lou Gehrig was the first famous person to be publicly diagnosed with
- Eleanor Gehrig, Lou's wife, was one of the leading activist for ALS research for years after his death; today, one of
the leading activists is pitcher Curt Schilling.