A Disease Whose Time has Come-- to Die

The month of May is National ALS Awareness Month. Forty thousand people in the USA and Canada have amyotrophic lateral sclerosis (ALS). ALS is also called "Lou Gehrig's disease" after the great New York Yankees first baseman whose career and life were cut short by the disease. There is no cure. It is a fatal illness and most of its victims die within three to five years of diagnosis.

ALS is a motor neuron disease because it attacks the long nerve cells, or neurons, that lead to the voluntary muscles. As the neurons die, the muscles they control atrophy and weaken to the point of uselessness. First symptoms of the disease vary for different people and include difficulty walking, inability to perform tasks like buttoning a shirt, and slurring of speech. As the disease progresses, ALS patients lose their ability to walk, talk, eat, get in and out of bed, and lift their arms. Most die of pneumonia or suffocation because their diaphragm and intercostal (between the ribs) muscles become so weak they cannot cough or breathe. Life can be prolonged by accepting a tracheotomy and mechanical ventilation, although many ALS patients choose instead to receive hospice care and die. If life is extended through artificial means, the deterioration of muscles continues, leading to a locked-in state in which the victim is completely unable to move.

The mind of the ALS patient remains clear and alert. In less than five percent of cases is there any cognitive impairment. Victims of ALS are completely aware of their bodies decaying to immobility and helpless to do anything about it. Their senses of sight, hearing, taste, smell, and touch are unaffected. They feel to a sometimes maddening degree the itch that cannot be scratched and the cramped muscle that cannot be moved. People with ALS are trapped in their bodies until they die from a disease that some of them call "the Beast."

I was diagnosed with ALS in February 2007. Eight months prior to diagnosis, I climbed scaffolding to build a house in post-Katrina Mississippi. Eight months after diagnosis, I started using a walker. Two years prior to diagnosis, I kayaked in the Bahamas. Two years after diagnosis, I moved into a nursing home. I spend my waking hours in a power wheelchair. I get my food and water through a feeding tube into my stomach. I communicate by typing with my eyes on a computer. I cannot hug my daughters, enjoy a meal, or hike along a mountain trail. ALS has not only shortened my life, it has narrowed it.

I grieve for the young parents with ALS whose children will see their lives poured out like water and evaporate before their eyes. I grieve for the young and beautiful women with ALS who will shrivel and die like cut flowers in the hot sun. I grieve for the mothers and fathers and their children who have grown up to be their best friends. They will be separated by a disease that is unmoved by friendship or love but mows people down like a scythe in a field of wheat. Six thousand people will be diagnosed with ALS this year. Six thousand others, having fought their fight, will die from it.

If you or someone you know has ALS, sign up for the national ALS Registry. Contribute to the National ALS Associations in Canada and the United States. Get involved with your local ALS Chapter. Contribute to the ALS Therapy Development Institute. Contribute or get involved with the ALS Guardian Angels. ALS was first described as a distinct illness in 1869 by French doctor Jean-Martin Charcot. We have learned a lot about the disease in the intervening 140 plus years, but ALS continues its destructive rampage among our friends and families. It is time for this disease to die.

(Originally published in the Asheville (NC) Citizen-Times newspaper on 8 May 2011.)