ALS Awareness Day on the MP Blog

Tis the season for the walks. Webster HS is doing the Jim Schoemehl walk and the ALS association is doing their Walk to D'Feet in Forest Park (for which I'm on the committee). If you stumble upon here and wonder, what they hey is ALS? I'm here to tell you. I will admit, this is not my writing, this is taken from www.alsa.org

Just what is ALS? Amyotrophic Lateral Sclerosis (ALS) is a motor neuron disease, first described in 1869 by the noted French neurologist Jean-Martin Charcot. Although the cause of ALS is not completely understood, the last decade has brought a wealth of new scientific understanding about the disease that provides hope for the future. Lou Gehrig first brought national and international attention to the disease in 1939 when he abruptly retired from baseball after being diagnosed with ALS. Most commonly, the disease strikes people between the ages of 40 and 70, and as many as 30,000 Americans have the disease at any given time. ALS has cut short the lives of other such notable and courageous individuals as Hall of Fame pitcher Jim "Catfish" Hunter, Senator Jacob Javits, actors Michael Zaslow and David Niven, creator of Sesame Street Jon Stone, boxing champion Ezzard Charles, NBA Hall of Fame basketball player George Yardley, pro football player Glenn Montgomery, golfer Jeff Julian, golf caddie Bruce Edwards, British soccer player Jimmy Johnstone, musician Lead Belly (Huddie Ledbetter), photographer Eddie Adams, entertainer Dennis Day, jazz musician Charles Mingus, composer Dimitri Shostakovich and U.S. Army General Maxwell Taylor.ALS is a neurodegenerative disease that usually attacks both upper and lower motor neurons and causes degeneration throughout the brain and spinal cord. A common first symptom is a painless weakness in a hand, foot, arm or leg, which occurs in more than half of all cases. Other early symptoms include speech swallowing or walking difficulty.The biological mechanisms that cause ALS are only partially understood. The only known cause of ALS is a mutation of a specific gene: the SOD1 gene. This mutation is believed to make a defective protein that is toxic to motor nerve cells. The SOD1 mutation, however, accounts for only 1 or 2 percent of ALS cases, or 20 percent of the familial (inherited) cases.Familial ALS represents between five to 10 percent of all cases. The rest arise spontaneously and mysteriously, making seemingly random attacks on previously healthy adults. ALS can strike anyone, anytime.Physicians have limited choices for treating ALS, and the options that do exist have come into use within the last 10 years. Studies suggest that patients' length of survival and quality of life are enhanced by night-time breathing assistance early in the course of the disease and by aggressive application of alternate feeding options to assure good nutrition once swallowing becomes difficult. At this time, Riluzole® is the only drug that has been approved by the FDA for treatment of ALS. In clinical trials, Riluzole® has shown a slight benefit in modestly increasing survival time.Stem cell and gene therapy are promising areas of research. In a variety of studies, ALS mouse models are being used to develop treatments that may someday lead to similar human clinical trials. Gene therapy is one field of research where The ALS Association is concentrating support for more study.More significant advances of research into ALS has occurred in the last decade than all of the time since Charcot identified the disease. Advances in technology and the genetic revolution are aiding researchers in unlocking the ALS mystery. As more scientists focus on this perplexing disease, the outlook for new understanding brightens each day.

Facts You Should Know About ALS
The onset of ALS is insidious with muscle weakness or stiffness as early symptoms. Progression of weakness, wasting and paralysis of the muscles of the limbs and trunk as well as those that control vital functions such as speech, swallowing and later breathing generally follows.
In most cases, mental faculties are not affected.
ALS is not contagious.
It is estimated that ALS is responsible for nearly two deaths per hundred thousand population annually. More people die every year of ALS than of Huntington's disease or multiple sclerosis and it occurs two-thirds as frequently as multiple sclerosis.
Approximately 5,600 people in the U.S. are diagnosed with ALS each year. The incidence of ALS (two per 100,000 people) is five times higher than Huntington's disease and about equal to multiple sclerosis. It is estimated that as many as 30,000 Americans may have the disease at any given time.
Although the life expectancy of an ALS patient averages about two to five years from the time of diagnosis, this disease is variable and many people live with quality for five years and more. More than half of all patients live more than three years after diagnosis.
About twenty percent of people with ALS live five years or more and up to ten percent will survive more than ten years and five percent will live 20 years. There are people in whom ALS has stopped progressing and a small number of people in whom the symptoms of ALS reversed.
ALS occurs throughout the world with no racial, ethnic or socioeconomic boundaries.
ALS can strike anyone.
Present treatment of ALS includes one drug, riluzole (Rilutek©) and is aimed at symptomatic relief, prevention of complications and maintenance of maximum optimal function and optimal quality of life. Most of this, in the later stages, requires substantial physical caregiving. Click here for more information on Rilutek.
In 1991 a team of ALSA-funded researchers linked familial ALS to chromosome 21. In 1993 the research team identified a defective SOD1 gene on chromosome 21 as responsible for many cases of familial ALS. Further study indicated over 60 mutations (structural defects) in the SOD (superoxide dismutase) enzyme which alters the enzyme's ability to protect against free radical damage to motor neurons. These studies open possibilities for future therapies or strategies to effectively mediate both familial and sporadic ALS. But much more research on the SOD enzyme is needed. Also, researchers have not ruled out other gene involvement (on other chromosomes) in ALS.
There can be significant costs for medical care, equipment and home health caregiving later in the disease. It is important to be knowledgeable about your health plan coverage and other programs for which your may be eligible, including SSA, Medicare, Medical and Veteran Affairs benefits.
Rilutek®, the first treatment to alter the course of ALS, was approved by the FDA in late 1995. This antiglutamate drug was shown scientifically to prolong the life of persons with ALS by at least a few months. More recent studies suggest Rilutek® slows the progress of ALS, allowing the patient more time in the higher functioning states when their function is less affected by ALS. Rilutek® is manufactured by Aventis Pharmaceuticals. There is a patient Assistance Program that helps patients who qualify to receive the drug without charge. Many private health plans cover the cost of Rilutek®. Contact your local ALSA Chapter or the National Office for the Patient Assistance Program resource and more information about access to Rilutek®.Reports from three separate patient databases described long range experience with Rilutek®. All three reports suggest a trend of increasing survival with Rilutek® over time. More studies that are double blind and controlled are needed to confirm these database observations. The trend appears to indicate that longer periods of time than those used in the Rilutek® clinical trials may be needed to see the long-term survival advantage of the drug. An interesting observation was that despite the fact that the Irish government provides Rilutek® free of charge to people in Ireland with ALS, only two-thirds of the patients registered in the Ireland national ALS database reported taking Rilutek.

September 8th, 2001 my friend was diagnosed with ALS, he passed away July 25th, 2003. The image is with his wife Alicia, daughter Bradie, mom Barb and dad Jim. This was when he first had to use a wheelchair.