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The onset of ALS is insidious with muscle weakness or stiffness as early symptoms. Inevitable progression of wasting and paralysis of the muscles of the limbs and trunk as well as those that control vital functions such as speech, swallowing and breathing follows.
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In most cases, mental faculties are not affected. Also, ALS is not contagious.
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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.
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A little over 5,000 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.
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The life expectancy of an ALS patient averages about two to five years from the time of diagnosis. Half of all affected live more than three years after diagnosis.
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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.
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ALS occurs throughout the world with no racial, ethnic or socioeconomic boundaries.
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ALS can strike anyone. Someone you know or love will die from ALS unless a cure or prevention is found.
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Present treatment of ALS 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 nursing management of a patient who is alert but functionally quadriplegic with intact sensory function, bedridden and aware he or she is going to die.
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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.
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The financial cost to families of persons with ALS is exceedingly high. It is estimated that in the advanced stages, care can cost an average of $200,000 a year. Patients' and relatives' entire savings are quickly depleted because of the extraordinary cost involved in the care of ALS patients.
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Riluzole, the first treatment to alter the course of ALS, was approved by the FDA in late 1995. This antiglutamate drug appears to prolong the life of persons with ALS by at least a few months and more recent studies suggest iluzole slows the progress of ALS, allowing the patient more time in the higher functioning states.
