Amyotrophic lateral sclerosis; often called "Lou Gehrig's Disease". A "degenerative disease of the nervous system, affecting the brain cells (motor neurons) that carry impulses from the brain and spinal cord to the muscles". Some high-profile victims: senator Jacob Javits, Catfish Hunter, Dmitri Shostakovich, actor Michael Zaslow, Leadbelly, actor David Niven (he lost his voice; Rich Little had to dub it for a film), and Charles Mingus, who kept composing, after the loss of motor skills.

A progressive degenrative disease that attacks nerve cells in the brain and spinal cord. These cells are prevented from receiving proper nourishment, and begin to atrophy. As the motor neurons that control muscle ability in the body die off, the brain begins to lose control of the muscles, until the victim is completely paralyzed. When control for vital functions is lost, the ALS sufferer dies. Most people still retain full functionality of their brain through the full scope of the disease.

www.alsa.org says the cause of ALS is not completely understood, but I seem to recall a discovery of the gene responsible for the disease in 1994 or 1995, shortly after my uncle died of ALS. ALS cannot be halted or reversed, but its progress can be slowed with the drug Rilutek. Other drugs for ALS are still in clinical trials.

Stephen Hawking also has ALS. It is a disease that affects the nervous system. As you can see in Stephen Hawking that it does not effect you enough that you cannot use your brain. Stephen Hawking has a special wheelchair that allows him to do what he does. As of yet there is no cure.

Sometime in the mid-1990's, researchers discovered a gene for familial ALS--the autosomal dominant form of ALS that can be inherited and passed from generation to generation. Unfortunately, only about 5% of ALS cases are familial--and this gene isn't implicated in all familial cases--so this particular discovery doesn't really help people like bitter_engineer's uncle, Stephen Hawking, or a friend of my family who died recently. The gene is thought to produce superoxide dismutase, an enzyme that's involved in the metabolism of free radicals.

As the disease progresses, motor neurons slowly degenerate, but sensory neurons are unimpaired, meaning that the patient can still feel pain, discomfort, heat, cold, etc. Bowel and bladder function are preserved, as are most autonomic functions; so are mental abilities (as bitter_engineer says). The patient often dies from a problem with swallowing or breathing; pneumonia is common.

ALS belongs with Alzheimer's disease on top of the list of "Most Horrible Diseases Known to Man."

In a year, my aunt will be dead and in the ground.

This is a story about my family vacation. Every night we sat down for dinner at their house. I could not have thought up a more unpleasant ordeal.

Let's get something straight right off the bat. The matriarch has two sons, my father and my uncle. They are both doctors. We are the children of doctors. We know medical terminology. "Aspirate" means that she coughs food and drink into her trachea while her paralyzed throat muscles struggle with the peristalsis even babies can manage. "Pneumonia" is what you get when chunks of comestibles coat the upper respiratory tract with lovely growth medium for colonies of unfriendly bacteria.

When they said "motor neuron atrophy", they meant that she would fracture her ankle walking in the living room of her own home.

When they said "degenerative", they meant her speech would come out as an unintelligible howl. Like a fucking zombie from a horror movie after four packs of cigarettes. It's not as funny as it sounds.

When they say "not completely understood", they mean "helpless".

I hope you can understand me when I say that the self-help books on this disease don't quite do it justice.

We're sitting at dinner, having some good conversations considering the circumstances. My aunt is using her finger to push chunks of potato down her throat. I stare at my glass of water. We pause uncomfortably, silently, every time she starts choking. She doesn't really drink anymore. She squirts fluid down her throat with a bottle and hopes for the best. Every night, I brought my laptop and excused myself early. Every night, closed-ear headphones were my friend.

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