Seal Cove man battles ALS by Laurie Schreiber, from The Bar Harbor Times

TREMONT - Al Butler is doing his best to keep his spirits up in the face of the devastating illness he was diagnosed with in December - amyotrophic lateral sclerosis, popularly known as Lou Gehrig's disease. Mr. Butler is one of about 30,000 people in the United States who suffer from ALS. 

         Born and raised in Seal Cove, he is, at 46, a man of many interests - a master carpenter, cook and caterer. His lobster cookers are a center of attention at the Stewart estate's Fourth of July fireworks party. His symptoms first occurred last July, when he was working in the kitchen at Southwest Harbor's Top of the Hill Restaurant. He was pulling heavy bread pans out of the oven when he noticed he had a problem lifting them. Later in the summer, using his hammer, his thumb and forearm felt weak. He thought it was carpal tunnel syndrome. A visit to his local physician led to a consultation with an orthopedic surgeon and then a neurosurgeon in Bangor. He underwent a CAT scan and, because both his mother and father died, several years apart, of cerebral hemorrhage, Mr. Butler also requested a magnetic resonance imaging test to check for cerebral clotting. These tests showed nothing wrong. It wasn't until he had an electromyogram, performed to study the health of the body's nerves and muscles and involving electrodes inserted into muscles to measure electrical signals, that doctors were able to reach a diagnosis. 
        Still thinking that maybe he was just suffering from Lyme disease, which he says has symptoms that mimic ALS, he went for a second opinion to Boston. Test results were the same. "That's when it actually hit me," he says. "It put me in shock." 
        ALS is a progressive disease that attacks specialized nerve cells called motor neurons, which control the movement of voluntary muscles. ALS causes the motor neurons to gradually disintegrate, weakening the body's voluntary muscles and eventually resulting in total paralysis. When lower motor neurons in the bottom of the brain are affected, the muscles responsible for speech, chewing and swallowing weaken. When the lower motor neurons in the spinal cord are involved, function is lost in the muscles of the limbs, neck and trunk. Upper motor neuron damage, in the top of the brain, results in weakness, muscle stiffness and exaggerated reflexes. Involuntary muscles are not affected and the voluntary muscles that move the eyes usually aren't affected. Control of bladder and bowel function remains intact, as does sexual function. The senses, including vision, hearing and touch, are not affected, nor is the intellect. Pain is not associated with the disease, which generally strikes adults between the ages of 35 and 65, at any stage. 
        The Muscular Dystrophy Association is one of the world's leading sponsors of ALS research and medical services, and has established eight research and clinical care centers whose primary mission is to find the cause of and a cure for ALS. Several lines of research are focusing on a flawed gene which appears to be associated with ALS; a neurotransmitter which may cause damage to motor neurons because there may be too much of it; and the possibility that ALS is caused by a faulty immune system. Researchers are investigating reports of possible risk factors that may set off the disease. For a small percentage of patients, there is a hereditary factor. There is no cure, but some drugs can control involuntary muscle twitching and other symptoms, respirators can help patients who have trouble breathing as the disease progresses, and other treatments have shown some indication of influencing the course of the disease. 
        Several months after the diagnosis, Mr. Butler is coping with fatigue, muscle weakness and occasional slurred speech. His tongue is swollen at times, his thumbs are weak, and it can take 10 minutes some mornings to button his shirt. It's an effort to write. "The brain is telling your muscles to do it, but they're not actually doing it," he says. 
        Most of what he is trying to write lately has to do with the couple's massive financial difficulties, as they struggle to pay their usual monthly bills on the house, utilities, food, transportation and taxes, as well as the health insurance they must keep up in order to cover medical costs. The dining room table is covered with the paperwork involved in understanding the illness, dealing with creditors, and navigating Social Security, expected to be a process of a half year or more before disability benefits are granted. At the same time, they must figure out how to keep Gloria working as Al become increasingly debilitated, and where to get the money for hiring a caregiver, making the house handicap-accessible, and buying a wheelchair and handicap-accessible van. Travel expenses are mounting, too, as they continue to work with specialists in Boston. Even when the disability comes through, that and Gloria's salary will not cover expenses. "They call this a rich man's illness," she says. "It costs so much to stay alive." "And the worst thing is, your brain doesn't go," Al says. "When you're in a situation like this, you're better off to just die. At least your spouse doesn't have to be stressed out." 
        Al and Gloria are looking for any treatments and test protocols out there. They tried to get him into one experimental protocol but his illness had not progressed enough to qualify. Another medication they are looking at is said to be a muscle builder. Holistic medicine is another avenue they're exploring. One Worcester, Mass., practitioner is focusing on boosting his immune system. "I figure their theories are just as good as any doctor who prescribes medicine," he says - especially considering the drugs often have debilitating side effects. The idea from the holistic standpoint is that the immune system is affected by toxins in the body, so the toxins must be eliminated. About 300 types of food were tested and, although Mr. Butler misses his morning coffee and doughnut, he faithfully keeps away from foods that failed the test, and takes herbs and supplements. "Hopefully, this will get rid of the fatigue and strengthen me," he says. "But the bottom line is, there's nothing you can do, and nobody knows anything about it."
        How to help Friends and family of Al and Gloria Butler are organizing a Community Support Fund and request contributions. Harbor House will accept contributions and administer the fund. Patricia Tierney is fund coordinator. Irving and Nancy Silverman are chairmen, and Fran Martin is secretary and may be reached at 244-4085. The Butlers are taking steps in other directions, as well. They ask people to request members of Congress to support ALS legislation, HR 353, "the Amyotrophic Lateral Sclerosis Treatment and Assistance Amendments of 1999." The bill would waive the Social Security Act's 24-month waiting period for Medicare coverage of ALS patients, and provide Medicare coverage of ALS drugs. Letters should be addressed: The Honorable (name of representative), U.S. House of Representatives, Washington, DC 20515; and The Honorable (name of senator), U.S. Senate, Washington, DC 20510. Maine's delegation already supports the legislation. The Butlers request the community to have friends and relatives in other states contact their members of Congress. They are looking into setting up fund-raising events toward research, and have included Al's photo in the ALS March of Faces, April 17, when a Boston Marathon runner hopes to raise $1 million toward research. Contributions of $5 or more per ALS Awareness Ribbon Lapel Pin, or a donation, may be made by check or money order to: ALS March of Faces, 402 Sal Blvd., Suite B, Trenton, OH 45067. Or order a pin on the Internet at: http://members.aol.com/alsmof2/ribbons.html.