ALS Ice Bucket Challenge Progress

 


Who Gets ALS?

ALS is a progressive neurodegenerative disease that affects nerve cells in the brain and spinal cord. There is currently no cure for the disease.

Based on U.S. population studies, a little over 5,000 people in the U.S. are diagnosed with ALS each year. (That's 15 new cases a day.) Every 90 minutes, someone is diagnosed with the disease and someone passes away from it.

It is estimated that at least 16,000 have the disease at any given time.

Most people who develop ALS are between the ages of 40 and 70, with an average age of 55 at the time of diagnosis. However, cases of the disease do occur in persons in their twenties and thirties.

ALS is 20 percent more common in men than in women. However, with increasing age, the incidence of ALS is more equal between men and women.

About 90 percent of ALS cases occur without family history. The remaining 10 percent of ALS cases are inherited through a mutated gene. On average, it takes about one year before a final ALS diagnosis is made.

There are several research studies – past and present – investigating possible risk factors that may be associated with ALS. More work is needed to conclusively determine what genetics and/or environment factors contribute to developing ALS. It is known, however, that military veterans are approximately twice as likely to develop ALS.

Half of all people affected with ALS live at least three or more years after diagnosis. Twenty percent live five years or more. Up to 10 percent will live more than 10 years.

Only four drugs are currently FDA-approved to treat ALS: Riluzole, Nuedexta, Radicava, and Tiglutik. Nuedexta is approved to treat pseudobulbar affect, difficulty with emotional control, including inappropriate laughing and crying, which impacts some people with ALS.

The estimated cost to develop a drug to slow or stop the progression of ALS from an idea to an approved drug is between $2 billion and $3 billion.

There is some evidence that people with ALS are living longer, at least partially due to clinical management interventions and possibly other compounds and drugs under investigation.

Facts You Should Know

  • ALS is not contagious.
  • Currently, no cure for ALS exists.
  • Although the life expectancy of a person with ALS averages about two to five years from the time of diagnosis, the disease is variable. Many people can live with the disease for five years or longer. In fact, more than half of all people with ALS live more than three years after diagnosis.
  • Once ALS starts, it almost always progresses, eventually taking away the ability to walk, dress, write, speak, swallow, and breathe, and shortening the life span. How fast and in what order this occurs is very different from person to person. While the average survival time is three years, about 20 percent of people with ALS live five years, 10 percent will survive 10 years and 5 percent will live 20 years or longer.
  • Progression is not always a straight line in an individual, either. It is not uncommon to have periods lasting weeks to months where there is very little or no loss of function. There are even very rare examples in which there is significant improvement and recovery of lost function. These ALS "arrests" and "reversals" are, unfortunately, usually transient. Less than 1 percent of people with ALS will have significant improvement in function lasting 12 months or longer.
  • Approximately 5,000 people in the U.S. are diagnosed with ALS each year. The incidence of ALS is two per 100,000 people, and it is estimated that at least 16,000 Americans may be living with ALS at any given time.
  • About 90 percent of ALS cases occur without family history. The remaining 10 percent of ALS cases are inherited through a mutated gene.
  • On average, it takes about one year before a final ALS diagnosis is made.
  • ALS occurs throughout the world with no racial, ethnic, or socioeconomic boundaries. The disease can affect anyone.
  • Military veterans are approximately twice as likely to develop ALS.
  • The onset of ALS often involves 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 breathing, generally follows.
  • There can be significant costs for medical care, equipment, and home health care later in the disease. It is important to be knowledgeable about your health plan coverage and other programs for which you may be eligible, including Social Security Disability Insurance (SSDI), Medicare, Medicaid, and Veteran Affairs (VA) benefits. Only four drugs are currently FDA-approved to treat ALS: Riluzole, Nuedexta, Radicava, and Tiglutik. Nuedexta is approved to treat pseudobulbar affect, difficulty with emotional control, including inappropriate laughing and crying, which impacts some people with ALS.
  • The estimated cost to develop a drug to slow or stop the progression of ALS is between $2 billion and $3 billion from an idea to an approved drug.

Last Revised May 2019