ASK DR. BAUGHAN February 20, 1998
LOU GEHRIG, STEPHEN HAWKING AND SANDY
“Please write about the causes, symptoms and treatment for ALS.”
Few types of disease are more frustrating and tragic than diseases that cause progressive loss of nervous system function. Amyotrophic Lateral Sclerosis (ALS) is one of those diseases. It was first made “famous” when the great first baseman for the New York Yankees, Lou Gehrig, developed the disease. Gehrig held the record for about 60 years for consecutive games played in major league baseball and was known as the “Ironman” for his strength and health. The disease reduced him to a man who needed help standing and could not even lift a baseball bat. His mind remained clear and calm enough to produce the dramatic quote of being “the luckiest man alive” when honored at Yankee Stadium by his fans. Stephen Hawking is perhaps the most brilliant physicist since Einstein and is the author of the book (made into PBS series), A Brief History of Time. This genius can move only one finger in his specialized wheelchair to move himself and “type” out his computer-generated voice.
Both men typify the physical consequences of ALS. The nerves to the muscles are damaged, causing progressive weakness to the point of paralysis. Prior to paralysis, there can be twitching and spasms. Eventually the muscles wither from disuse. The nerves that control swallowing, speech and breathing can be affected. The patient can be at risk for choking or aspirating food or fluids into the lungs during swallowing. One unusual symptom can happen when a part of the brainstem is affected and results in sudden uncontrollable laughing, crying, or garbled speech or sudden inability to make a vocal sound. All of this occurs with the patients fully aware that their bodies are betraying them. Some patients die in a year; some live for decades.
We do not know the cause of ALS. Current theories are pursuing the idea that an amino acid (the building block of proteins) may accumulate and have a toxic effect on certain nerves. Why only certain nerves are affected is a mystery. In 5-10% of cases there may be a hereditary pattern, but in the rest there is not. Could this mean a gene goes awry in the 1 in 100,000 people who develop ALS? Maybe. We know so little it is extremely frustrating for patients, families and their physicians. The frustration and desperation is compounded by the lack of any proven treatment for ALS. The best neurologists in the world feel very helpless dealing with ALS.
In 1994, the first reports of a new hopeful treatment for ALS were released in the New England Journal of Medicine. The medication, riluzole, was used in 77 patients and the results compared to 78 patients given a placebo. No patients improved. There was a statistical debate over whether it made a significant difference in prolonging life or prolonging the time needed before a patient needed a ventilator to help him/her breathe. Even the researchers acknowledged that the patients needed to be studied longer. One year in the course of a disease that might cause progressive disability just may not be meaningful. This disease produces ethical dilemmas in research on when is treatment worthwhile. When does it prolong “quality of life” or when does it prolong suffering? An editorial that accompanied the article expressed caution about getting too hopeful about it because in the past decade, hopes had been raised, then dashed, about possible treatments with other substances, such as thyrotropin-releasing hormone, threonine, and branched-chain amino acids. Physical therapy, occupational therapy, and medical devices can help maintain some functional abilities, but they do not affect the diseases effect on the nerves.
Sandy McReel is a resident of our community with ALS. A benefit including auction items and raffle prizes to raise money to help him and his family with medical expenses is being organized by his friends and will be held at the Mt. Cranmore Pub on Monday, February 23, 1998. For more information, call