Amyotrophic lateral sclerosis,also known as Lou Gehrig’s disease or ALS, is a neurodegenerative disease with no known cure. ALS damages the ability of your nerves to control your muscles while leaving your senses and mental capabilities intact. The disease was first identified in 1869.
The ALS Association states:
“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.”
ALS affects more men than women in a ratio of 3:2 and most often strikes between the ages of 40 and 70. However, ALS can strike anyone at any time and that is part of what makes it such a scary disease. Familial cases are thought to be 5 to 10 percent of all cases, and the incidence of ALS in the U.S. is about 1.5 per 100,00 people. This means approximately 30,000 Americans have an ALS diagnosis at any given time.
While treatment has come a long way in the last 10 years, there is still no cure or truly effective treatment to slow ALS. It is fatal in three to five years in the majority of cases. The one drug approved for treatment of ALS is called riluzole. It has shown a slight improvement in survival time in some patients.
Early symptoms are vague and often limited to weakness without pain in one extremity, making it sometimes difficult to diagnose immediately. Other early symptoms include fatigue even just from talking, muscle atrophy, weakness, and facial fasciculations.
It is best to see an ALS specialist if possible because disease management has changed so much in the past few years. This may mean travel to a large urban area for diagnosis and a treatment plan. After diagnosis and a treatment plan have been established, a local physician can work with you and the specialist on day-to-day symptom management.
One very important part of early planning after diagnosis is determining advanced directives and exactly what the patient wants in terms of treatment when the ALS progresses. A feeding tube may prolong survival, but not everyone wants that. The same issues can apply to intubation and mechanical ventilation. These topics are difficult to discuss initially, but are an extremely important part of the treatment plan. That way when the patient can no longer easily express herself, there are not doubts about what course of treatment to pursue.
ALS Association: About ALS
Medline Plus: Amyotrophic Lateral Sclerosis
Add a Comment3 Comments
The biological mechanisms involved in the creation are just that, mechanisms, and not causes. The what really underlies the development of als in a person's life is factors and patterns that underlie the action of the biological mechanisms.
Further, what this means is that by changing what is going on with the underlying factors and patterns, one can solve als and heal.
So while there may not be any effective way of medically intervening to treat als, there are ways that anyone can use to solve the problem, and many do just that.August 20, 2010 - 4:53pm
Tell me what can I do... please!August 23, 2010 - 2:25pm
The best thing you can do is find a healthcare team with lots of experience in treating ALS patients, and avoid believing all of the mumbo-jumbo crackpot advice you see on the internet.
Good luck and if you need help finding resources, please let me know.August 24, 2010 - 6:29am