medical condition known as Lou Gehrig's disease. The paper presents pertinent data about the disease both from the literature available and from a personal position of testimony. Also, this paper reviews the technologies that are used to relive patients who suffer from the disease, and delves into the problems associated with attempts to mitigate the debilitating effects of Lou Gehrig's disease.
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), those cells that normally control the muscles that are voluntary, according to the National Institute of Neurological Disorders and Stroke (NINDS). The ALS sufferer is taken through the painful reality of 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, in the spinal cord and in the brainstem; they are, as NINDS describes, the "vital communication links between the nervous system and the muscles in the body that are voluntary. The voluntary muscles in the body are those that the individual controls. The heart is an involuntary muscle, because it doesn't require messages from the brain to keep working. Muscles are "bundles of elongated cells" that allow a human's thoughts to be turned in to action (NRPT). A baseball pitcher winds up and throws the ball because his motor neurons communicate to the muscles that the pitch is ready to be thrown. If a person has something to say, he or she can say it "loud," which would be a matter of asking your larynx, mouth and tongue to go into action, and also your breathing would have to controlled so the air could move "through the larynx in the first place" (NRPT).
When ALS strikes a person, that communication from the nervous system and the voluntary muscles is interrupted, the NINDS explains. Both the "…upper motor neurons and the lower motor neurons degenerate or die, ceasing to send messages to muscles," NINDS continues. Basically, the ability of the brain to launch any voluntary movement of the muscles is gone.
There are other disabilities associated with ALS; in fact in time a patient with ALS loses most or all of his or her arm, leg, and body functions, plunging the person into a very serious dilemma. And when the muscles in the human diaphragm and chest do not respond to messages from the motor neurons in the brain and spinal column, patients are put on a ventilation system just to keep alive (NINDS). In fact, "Most people with ALS die from respiratory failure," the NINDS fact sheet explains, and that is usually within a period of 3 to 5 years (albeit, a small number of ALS patients live up to ten years).
Does ALS cause the mind / intelligence to deteriorate?
ALS does not have a negative impact on a person's "…mind or intelligence" although some patients do become depressed for obvious reasons that are not related to the attacks on their bodies that leave their voluntary muscles impotent (NINDS). The ability to see, to taste, to hear, to smell and to recognize touch are not affected. ALS patients also retain control (in most cases) of the eye muscles and of the bowel and bladder functions, NINDS continues. About 25,000 to 30,000 Americans have ALS at any one given time, and each year roughly 5,000 people are diagnosed with ALS; all ethnic backgrounds and races can be hit with ALS, but age makes a difference, according to the NINDS. In fact individuals between the ages of 40 to 50 years are most apt to be diagnosed with ALS. Men are diagnosed with ALS more often than women are.
Machines related to 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 thought of staying alive superficially is not an appealing alternative to some patients. In my own experience, my father had ALS. He was offered the thought that he would only live three to five years at best. When he was first diagnosed with ALS he had great difficulty just walking and keeping his balance.
There were times when he simply fell backwards for no apparent reason other than he had a vertigo related to the motor neurons' failure to send the right messages to his muscles. About a year after he was diagnosed with ALS, he was using a wheelchair and he needed to be assisted just to get in and out of his wheelchair and the automobile. About eighteen months after he was diagnosed he could not feed himself anymore. As to his need to use the toilet, he had to be physically lifted up and carried to the toilet, and had to be held while he was having a bowel movement. Indeed he also needed to be wiped by his caregiver as well. It was a hopeless, painful and embarrassing situation for my father.
As to the wheelchair, my father made the decision that a wheelchair would be his only way of getting around, and of course someone had to push and steer the wheelchair for him. He also made the decision not to buy any expensive machines to help extend his life. He told his family that it was a gift to us to just let the disease progress as it would naturally and hence no machines for breathing or for other services were employed by my father. He was sedated and remained in our home with his family around him. It was horrible to witness his demise, and of course it was also very stressful to lose him.
But knowing we only had a limited amount of time to be with him was acceptable to our family in the sense that he had a good attitude about this whole process. Why prolong the disease that was going to take his life anyway? That was his attitude. We made the most out of his last years.
Juxtaposed with our father's situation -- in which he insisted on just letting the disease play through its cycle -- a friend of the family now suffering from ALS is using technologies to prolong her life. What makes her bout with ALS different is that she has gone the machine route. Caregivers are with her around the clock -- and her insurance policy does not cover that service. Moreover, the sound of all the machines and having people moving in and out of the house constantly has taken a toll on her family. You can feel the tension and stress in that family; not only that, her husband has been paying out of his savings for three years and is completely stressed out.
The husband is concerned that if he doesn't keep paying for all the machines and the caregivers, he may be perceived as not doing enough for her; in fact he fears he may be perceived as wishing for her death. But even though this is not true, the family is under tremendous stress; there is anger, there are a lot of tears and one can detect animosity in the house.
My point is this: my father took the more humane route by just letting the ALS run its course rather than prolonging the pain by hooking him up to machines.
What are the machines and what do they do?
The NINDS informational materials explain that mechanical ventilation (respirators) works to help the lungs become inflated and deflated. A tube is passed from the nose or the mouth to the trachea for short-term ventilation. For longer term ventilation, surgeons actually perform a tracheotomy, "…in which a plastic breathing tube is inserted directly in the patient's windpipe through an opening in the neck" (NINDS, p. 5).
The ALS Association (ALSA) describes machines that are used for ALS patients. The NPPV (Bi-PAP) -- which stands for "bi-level positive air pressure" -- is a "non-invasive respiratory device with two levels of air pressure." There is a higher air pressure when the patient breathes in and the air pressure is lowered when the patient breathes out. Sometimes a mask is fitted over the nose, the mouth, "…or both mouse and nose together, or through nasal pillows that fit just your nostrils" (ALSA).
Because breathing while sleeping is difficult the Bi-PAP is useful in many cases. The muscles are allowed to rest and the patient is given the ability to breathe "more efficiently during the day," ALSA explains. The buildup of carbon dioxide is also avoided -- a buildup can change breathing patterns -- and also the ability to "cough effectively" can be enhanced by the Bi-PAP.
The need for an In-Exsufflator -- also known as a cough assist device -- is expressed by some doctors treating ALS patients. This helps patients to clear…