Lou Gehrig's Disease-A Death Sentence
Imagine, if you will, being in the prime of life. You are established in your career, you have your home, your family, and your friends. Everything seems to be going your way, and like most of us, your life is to full to waste time contemplating your demise. Then like out of the blue, you awake one morning to find you are having weakness in your muscles and you just don’t feel right. Not being too concerned, you go to see your doctor to find out what the minor problem may be. Unfortunately your problem is not minor, but instead is very major. To your astonishment and disbelief you are told you have Lou Gehrig’s Disease, and in all reality you have been given a death sentence.
To most people the name Lou Gehrig’s Disease is familiar, but not many people know what it is. Lou Gehrig’s Disease or Amyotrophic Lateral Sclerosis (ALS) "is a fatal disease of the motor neurons (nerve cells) that control the skeletal muscles of the body" ("Amyotrophic Lateral Sclerosis," Microsoft). ALS first received its medical name in 1874 from the French neurologist Jean Martin Charot, who came upon the name, because he observed the wasting away of a patient’s muscles (known medically as amyotrophy) and the scarring and hardening, or sclerosis, of the bundles of motor neurons running down each side (laterally) through the spinal cord ("ALS," Microsoft).
The more popular name, Lou Gehrig’s Disease comes from the legendary baseball player, Lou Gehrig, "who succumbed from ALS in 1941" ("ALS," Microsoft). Throughout the many years of research, it has been found that with ALS, "the nerve cells that reach from the brain to the spinal cord (upper motor neurons) and the spinal cord to the peripheral nerves (lower motor neurons) for some unknown reason die," which leads to a progressive loss of the ability to move virtually any muscle in the body (Robinson 139).
In essence this means that all "voluntary" muscles, those controlled by conscious thought, such as the arm, leg or trunk muscles, are affected by ALS. By contrast, ALS does not affect sensation, thought processes, the heart muscle, and other internal organs. Especially painful to imagine is that "most patients with ALS do not lose the control of their eye muscles or their ability to think," which essentially traps them in a body that will not function for them anymore (139).
ALS affects approximately 30,000 people in the United States, with about 5,000 new cases each year (Robinson 139). There are two major forms of ALS known, these are familial and sporadic. Familial ALS accounts for about 10% of all ALS cases (139). As the name implies, familial ALS is believed to be caused by the inheritance of one or more faulty genes. Therefore, ALS could be passed from one generation to another in a particular family with the faulty gene. Sporadic ALS has no known cause. There has been some suggestion of environmental toxins as causes, "but to date no research has confirmed any of the candidates investigated, such as aluminum or metal dental fillings" (139). Less known than the other two, there is a third type called "Western Pacific ALS," which occurs in Guam and other Pacific islands (139). This form combines the symptoms of both ALS and Parkinson’s disease.
The disease ALS usually begins between the ages of 40 and 70, although younger onset is possible, and men are slightly more likely then women to develop it ("Amyotrophic Lateral Sclerosis" Encyclopedia Americana). Some of the earliest signs of ALS are muscle weakness and slurred speech. Most often a weakness of the legs begins by being more pronounced on one side than on the other. Leg weakness may first become apparent by an increased frequency of stumbling on uneven pavement, or an unexplained difficulty climbing stairs. Loss of leg function is "usually more pronounced among people with familial ALS" (Robinson 139). Another early sign of muscle weakness is in the arms. This may lead to difficulty grasping and holding a cup, for instance, or loss of dexterity in the fingers. This tends to be "more prevalent in people with sporadic ALS" (139). Less often, the earliest sign of ALS is weakness in the "bulbar" muscles, those muscles in the mouth and throat that control chewing, swallowing, and speaking ("Amyotrophic Lateral Sclerosis" Textbook). A person with "bulbar" weakness usually becomes hoarse or tired after speaking at length, or their speech may become slurred.
While muscle weakness comes in the earliest stages, ALS almost always progresses rapidly to involve virtually all the voluntary muscle groups in a patient’s body. As the disease progresses, symptoms will include "loss of the ability to walk, to use the arms and hands, to speak clearly or sometimes not at all, to swallow, and finally to hold the head up" ("ALS" Textbook). With the progression of weakness of the respiratory muscles, difficulty in breathing and coughing, and poor swallowing control increases the likelihood of inhalation of food or saliva (aspiration). Aspiration increases the likelihood of lung infection, "which is often the cause of death" ("ALS" Textbook).
Although medical technology is rapidly developing worldwide, there is still no known cure for ALS, and no treatment that can significantly alter its course. There are many things which can be done, however, "to help maintain quality of life and to retain functional ability" to a certain extinct (Robinson 139). Usually treatment focuses on the management of the symptoms, which involves a combination of "physical, occupational, speech, and respiratory therapies" ("ALS" Microsoft). As the disease progresses and the muscles degenerate, various devices may provide support, such as ankle braces, neck collars, reclining chairs, wheelchairs, and hospital beds. Mechanical ventilation may be used when breathing becomes too difficult ("ALS" Microsoft).
Even though, modern mechanical ventilators are small and portable, "fewer than 10 percent of the 30,000 Americans choose to use this option. Interestingly, in Japan the figure is 50 percent" (O’Reilley 15). This may be due, although, to the fact that this option is "extremely costly when added to the various other equipment that ALS patients need" (15).
The progressive nature of ALS means that most patients will eventually require full-time nursing care. This care is often provided by the spouse of a patient or other family members. Sometimes the physical and emotional burden of care can be overwhelming, and the "caregivers need to recognize and provide for their own needs as well as those of the ALS patient, so that they can prevent depression, burnout, and bitter feelings" (Robinson 139).
According to the ALS Association, "50 percent of patients survive from three to five years after diagnosis, and in about 20 percent of cases, patients will live longer than five years, while only 10 percent may live as long as ten years or more after diagnosis." Therefore it is understandable that support groups are sponsored nationwide "to give important psychological aid to the patient, as well as the caregiver so they may come to terms with the losses ALS inflicts" (139).
With the medical advances that we have today, it is hard to understand how a disease could still be so mystifying. Not only do researchers not know the specific cause of ALS, but they have still not found anything to even slow the progression. There is no known way to prevent ALS or to even alter its course. Most patients, no doubt, will feel they have been given a "death sentence" when the diagnosis of ALS is made. Until more evidence and research into this mysterious disease is done, patients will continue to feel they are left with few options for their remaining lifetime. Through God’s will, hopefully, there will be a cure found soon, so that no more people will have to face an unsure future with a diagnosis of ALS and families won’t have to see the devastation that their loved ones go through.
Works Cited
"Amyotrophic Lateral Sclerosis." The Encyclopedia Americana. 1990
"Amyotrophic Lateral Sclerosis." Microsoft Encarta Online Encyclopedia. 2001. Mar.15,
2001.<http://encarta.msn.com>
"Amyotrophic Lateral Sclerosis." The Textbook of Medicine, Ed. James B. Wyngaarden, M.D. 2 vols. Philadelphia: W.B. Saunders, Co., 1982
O’ Reilley, Dale. "Fear of Death, Love of Life." New York Times. 25 June 2000, late ed.: 15
Robinson, Richard. Gale Encyclopedia of Medicine. Edition 1. 1999. p.139.
By Sheila Howell English 102 online, Spring 2001
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