ALS and the End-of-Life Choice

The human body is a marvelous concoction of awareness and action and sensitivity, when all is working well. We suffer sad regrets when things fail, however. People afflicted with amyotrophic lateral sclerosis (ALS, also known as Lou Gehrig’s Disease) suffer a distinctly sorry loss of the action part of the system.

This neurodegenerative disease causes death of the nerve cells that control movement, the motor neurons. Typical early symptoms may include twitches in limbs or tongue, muscle cramping and weakness, slurred speech, or difficulty chewing or swallowing. Muscle weakness and atrophy spread to many body areas as the disease progresses, eventually making controlled movement impossible. Disuse of paralyzed muscles leads to painful cramps; loss of tongue and jaw control interferes with speech, and problems taking in food cause choking and malnutrition.

Prognosis is death by asphyxiation, when the muscles required for respiration succumb to the disease, on average within three years of diagnosis. There is no cure; the only treatment is an expensive drug (Riluzole) that may increase longevity by a few months. Since there is no degeneration of the nerve cells governing sensation or cognition, sufferers of ALS are fully aware of muscle pains and digestive disturbance. It is easy to imagine the misery of sitting or lying immobile for hours on end, incapable of shifting position or moving weight from one area to another. We all feel discomforts of this sort after indefinitely extended sedentary periods; for the ALS sufferer, these can be relieved only by a helper, and then, only if speech is adequate to communicate the problem.

Individuals with ALS face a limited window of opportunity to hasten their deaths to prevent inevitable suffering, before the physical capability to perform the actions needed for self-deliverance is destroyed by inexorable progression of the disease. Missing this opportunity, they face months and years of incapacity, nonexistent autonomy, and daily muscular and digestive and sensory discomfort.

Palliative care offers relief at the cost of energy and mental function. Severe shortness of breath, likened to the sensation of drowning, can lead to repeated panic episodes. Continued life becomes a trade-off between distress and drug-induced unconsciousness. This situation is exacerbated by the full awareness that things will only get worse, until finally relieved by death.

The ALS Association has much useful information about this disease. About 20,000 people in the US have the disease at any time – about 2 out of 100,000 people. Because their life expectancy is only a few years, the rest of us are not likely to meet them.

Of particular significance is this information quoted from the Association web site:

• Once ALS starts, it almost always progresses, eventually taking away the ability to walk, dress, write, speak, swallow, and breathe and shortening the life span. How fast and in what order this occurs is very different from person to person. While the average survival time is 3 years, about twenty percent of people with ALS live five years, 10 percent will survive ten years and five percent will live 20 years or more.

• Progression is not always a straight line in an individual either. It is not uncommon to have periods lasting weeks to months where there is very little or no loss of function. There are even very rare examples in which there is significant improvement and recovery of lost function. These ALS “arrests” and “reversals” are unfortunately usually transient. Less than 1% of patients with ALS will have significant improvement in function lasting 12 months or more.

Perhaps the most well-known person with ALS is theoretical physicist, cosmologist, and author Stephen Hawking, who has a rare early-onset, slow-progressing form of ALS that has gradually paralyzed him beginning at age 21. Hawking is now 75. He has been able to live a productive life with the help of increasingly sophisticated technology that has been adapted to his special needs. Few ALS sufferers have the type of ALS that Hawking has.

Like those with Parkinson’s and early dementia, a person living with ALS usually is not eligible for hospice, since “life” expectancy is beyond six months. Nevertheless, there is a threshold of disease progression beyond which the possibility of self-deliverance vanishes: in dementia, due to loss of mental competency; in Parkinson’s and ALS, due to vanishing capability to perform the actions required. Given this knowledge, sufferers may choose to die before devastating losses occur, on their own terms, while still autonomous and capable, accompanied by loved ones, celebrating life, and sharing a peaceful departure.