Lou Gehrig's Disease: ALS Technologies and Personal Impact

What Is Lou Gehrig's Disease?

Lou Gehrig's disease — also known by its medical name, Amyotrophic Lateral Sclerosis (ALS) — is a "rapidly progressive, invariably fatal neurological disease" that attacks an individual's nerve cells (neurons), particularly those that control voluntary muscles, according to the National Institute of Neurological Disorders and Stroke (NINDS). The ALS sufferer experiences this disease gradually, as the motor neurons degenerate slowly and take away the patient's ability to move muscles as he or she once did.

The motor neurons are the nerve cells in the brain, spinal cord, and brainstem. As NINDS describes them, they are the "vital communication links between the nervous system and the voluntary muscles of the body." Voluntary muscles are those that a person consciously controls. The heart, by contrast, is an involuntary muscle — it does not require messages from the brain to keep beating. Muscles are "bundles of elongated cells" that allow a human's thoughts to be turned into action (NRPT). A baseball pitcher winds up and throws the ball because his motor neurons communicate to his muscles that the pitch is ready to be thrown. If a person wants to speak loudly, his or her larynx, mouth, and tongue must all go into action, and breathing must be controlled so that air can move "through the larynx in the first place" (NRPT).

When ALS strikes, that communication between the nervous system and the voluntary muscles is interrupted. Both the "upper motor neurons and the lower motor neurons degenerate or die, ceasing to send messages to muscles," NINDS explains. Essentially, the brain loses the ability to initiate any voluntary movement of the muscles.

Other disabilities are associated with ALS as well. In time, a patient loses most or all arm, leg, and body function, placing the person in a profoundly serious situation. When the muscles of the diaphragm and chest no longer respond to messages from the motor neurons, patients must be placed on a ventilation system just to stay alive (NINDS). "Most people with ALS die from respiratory failure," the NINDS fact sheet notes, typically within three to five years of diagnosis, although a small number of ALS patients live up to ten years.

Effects on the Mind and Who Is Affected

ALS does not negatively affect a person's mind or intelligence, although some patients do become depressed — for reasons related to their circumstances rather than to any neurological attack on cognition (NINDS). The ability to see, taste, hear, smell, and recognize touch is not affected. In most cases, ALS patients also retain control of their eye muscles and their bowel and bladder functions (NINDS).

Approximately 25,000 to 30,000 Americans have ALS at any given time, and roughly 5,000 new cases are diagnosed each year. All ethnic backgrounds and races can be affected, though age is a significant factor — individuals between 40 and 50 years of age are most likely to be diagnosed. Men are diagnosed with ALS more often than women.

Machines and ALS: A Personal Reflection

Why are machines not always the best answer for an ALS sufferer? For one thing, they are very expensive, and the prospect of remaining alive with significant mechanical support is not an appealing alternative for some patients. In my own experience, my father had ALS. He was told that he would likely live only three to five years. When he was first diagnosed, he had great difficulty walking and maintaining his balance.

There were times when he simply fell backwards for no apparent reason other than a vertigo related to his motor neurons' failure to send the correct messages to his muscles. About a year after his diagnosis, he was using a wheelchair and needed assistance getting in and out of it as well as in and out of the automobile. About eighteen months after his diagnosis, he could no longer feed himself. He also had to be physically lifted and carried to the toilet, and required assistance throughout. It was a hopeless, painful, and deeply embarrassing situation for him.

Regarding the wheelchair, my father made the decision that it would be his only means of mobility — and of course someone had to push and steer it for him. He also made the decision not to purchase expensive machines to extend his life. He told his family that it was a gift to us to simply let the disease progress naturally. No machines for breathing or other life-sustaining purposes were employed. He was sedated and remained at home, surrounded by his family. It was painful to witness his decline, and the grief of losing him was immense.

Knowing that we had only a limited time with him was nonetheless something our family found acceptable, in the sense that he faced this process with a good attitude. His perspective was simple: why prolong a disease that was going to take his life anyway? We made the most of his final years.

Juxtaposed with our father's situation — in which he chose to let the disease run its natural course — a family friend now suffering from ALS has taken a different path, using technology to prolong her life. Caregivers are with her around the clock, and her insurance does not cover that service. Moreover, the constant noise of machinery and the steady flow of people through the house have taken a considerable toll on her family. The tension and stress in that household are palpable. Her husband has been drawing on his savings for three years and is completely overwhelmed.

He worries that if he stops paying for the machines and caregivers, others will perceive him as not doing enough — or even as wishing for her death. Although this is not true, the family is under enormous strain: there is anger, there are tears, and there is detectable animosity in the home.

The contrast illustrates a larger point: my father chose the more humane route by allowing ALS to run its course rather than prolonging the suffering by connecting him to machines.

Conclusion: Technology and the Choice to Accept ALS

The person with ALS faces a profound choice: to use machines to sustain life while the body inevitably deteriorates, or to let the disease run its course and accept its progression without the stress of constant noise, enormous expense, and the other burdens that technology in this context can impose. Having witnessed the strain created by machines that are only temporarily effective, and having also seen what a more peaceful acceptance of the disease can look like, it is my view that accepting the inevitable may, for many patients and families, be the better path. As research into ALS treatments continues to advance, that calculus may one day change — but for now, the human cost of machine-prolonged survival deserves careful, honest consideration.

Works Cited

ALS Association (ALSA). (2008). Respiratory Care. Retrieved November 7, 2012, from http://web.alsa.org.

DynaVox Technologies. (2011). DynaVox EyeMax Accessory for DynaVox Vmax. Retrieved November 7, 2012, from http://www.spectronicsinoz.com.

Focus on ALS. (2009). BiPAP. Retrieved November 7, 2012, from

Madsen, Amy. (2011). NeuRx Diaphragm Pacing System Approved for ALS. MDA/ALS Newsmagazine. Retrieved November 7, 2012, from http://alsn.mda.org.

National Institute of Neurological Disorders and Stroke. (2009). Retrieved November 7, 2012, from http://www.ninds.nih.gov/disorders/amyotrophiclateralsclerosis/detail_ALS.htm.

National Register of Personal Trainers (NRPT). (2010). Expand Your Knowledge / Muscle Anatomy / Voluntary Muscles. Retrieved November 7, 2012, from http://www.nrpt.co.uk.

Pfiumm, Michelle. (2011). DPS Sleep. ALSTDI (ALS Therapy Development Institute). Retrieved November 7, 2012, from http://blogs.als.net/post/2011/12/06/DPS-sleep.aspx.