We will survive
Focus on ALS
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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 respiration follows. Mental faculties are not affected. Also, ALS is not contagious.
It is estimated that ALS is responsible for nearly two deaths per hundred thousand populations annually. More people die every year of ALS than of Huntington's disease or multiple sclerosis.
Nearly 5,000 people in the U.S. are newly 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. The prevalence of ALS is six to eight per 100,000.
The life expectancy of an ALS patient averages about two to five years from the time of diagnosis. There are encouraging new drugs and reason for HOPE. With recent advances in research and improved medical care, people are able to manage or cope with this disease easier than in Lou Gehrig's era.
About twenty percent live five years or more and up to ten percent will survive more than ten years.
ALS is not a rare disease and occurs throughout the world with no racial, ethnic or socioeconomic boundaries.
ALS can strike anyone, and it is projected that of the U.S. population living today, in excess of 300,000 Americans will die from ALS unless a cure or prevention is found.
ALS selectively destroys the motor neurons in the nervous system. Therefore, consideration as to its cause has been given to agents with selective properties such as toxic agents and genetic factors. In a family lineage approximately five to ten percent of ALS is familial, occurring more than once
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 structural defects in the SOD (superoxide dismutase) enzyme which reduces the enzyme's ability to protect against free radical damage to motor neurons. These studies open possibilities for possible therapies or strategies to effectively mediate ALS. But much more research on the SOD enzyme is needed. Also, researchers have not ruled out other gene involvement (perhaps on other chromosomes) in ALS.
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.
The financial cost to families of persons with ALS is exceedingly high. In the advanced stages care can cost up to $200,000 a year. Entire savings of relatives of patients are quickly depleted because of the extraordinary cost involved in the care of ALS patients.
Rilutek®, the first ever treatment to alter the course of ALS, was approved by the FDA in late 1995. This anti glutamate appears to prolong the life of persons with ALS by at least a few months. Rhône-Poulenc Rorer manufactures Rilutek.
Source: The ALS Association (ALSA) is a national not-for-profit organization dedicated solely to the fight against ALS