What is ALS?

ALS is a fatal, neurodegenerative disease. It is a progressive disorder that occurs when motor nerve cells in the nervous system cease functioning and die. Once these motor nerve cells are destroyed, the patient is left without the ability to control voluntary muscles.

What are the different forms of ALS?

  • Sporadic – the most common form of the disease, and 90% of all cases do not have a genetic predisposition.
  • Familial – one form linked to chromosome 21 SOD1 gene.

5,600 people in the United States are diagnosed with ALS each year. 60% of the people diagnosed are men and 93% of the people are Caucasian. Most cases occur in patients between the ages of forty and seventy. 90% of diagnosed cases have no hereditary cause and are just an isolated case.

Symptoms

  • Earliest symptoms of twitching, cramping, or stiffness may be overlooked.
  • The part of body affected first depends on which muscles are degenerated first.
  • Rate of progression varies in different individuals. However, all patients eventually develop severe weakness of limb muscles causing quadriplegia, pharyngeal muscles causing impaired ability to eat, and respiratory muscles causing inability to breath unassisted.

Statistics

  • The annual incidence (number of new cases per year) is about 6,000 people in the U.S. every year.
  • The annual mortality rate is about 6,000 people in the U.S. each year.
  • Life expectancy for an ALS patient is two to five years.
  • Someone dies from ALS every 90 minutes.
  • ALS does not discriminate; it can strike anyone without regard to race, sex, or socioeconomic circumstances.
  • The average medical expenses for an ALS patient are around $200,000 per year.
  • There is no known cause or cure for sporadic ALS.

Treatment

There is no known cure for ALS, only remedies to prolong life or to improve the quality of life. Drugs such as Rilutek are used to maintain function of muscles. It is important to remember that ALS does not affect the mind so treatments to help improve the quality of life are critical.