What is ALS?

Amyotrophic Lateral Sclerosis

ALS is also known as Lou Gehrig’s disease, or Motor Neuron disease (MND). Lou Gehrig was a famous  baseball player (New York Yankee) in 1920-30’s who had to give up his career because of this disease. Other notable people with ALS or pALS were the physicist Steve Hawking, the actors David Niven (Pink Panther) and Sam Shepard, and the communist dictator Mao Tse Tung.

What it is/does:

  • A progressive disease that affects the brain and spinal cord, ultimately leading to wasting of the muscles that allow a person to walk, move, eat, swallow, talk, and breathe.
  • A rare disease, affecting less than 200,000 people in the nation.
  • Affects ANY person regardless of race, ethnicity, socioeconomic status or gender. It is known to affect more males than females, between 40-70, although a few have been diagnosed as early as the teens and late in life, until the 90’s.
  • Military veterans are approximately twice as likely to develop ALS.
  • People with ALS or pALS usually live 3-5 years although many live beyond. More than half of all people with ALS live more than three years after diagnosis. About 20% of pALS live five years, 10% will survive ten years, and 5% percent will live 20 years or more.
  • Approximately 6,000 people in the U.S. are diagnosed with ALS each year. The incidence of ALS is two per 100,000 people. It is also estimated that 5/100,000 may live with ALS at any one time.
  • The onset of ALS often involves muscle weakness or stiffness. 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 later breathing generally follow. Some pALS also show inappropriate degree of emotions such as crying excessively.
  • According to an analysis done in 2012 from data gathered between 2001-10 reported in a NIH publication, in-home caregiving, assisted ventilation, and hospital care accounted for the most costs in care for a pALS. Eighty-five percent was paid by insurance, 9% by family, and 6% by charitable organizations.

What it is not/does not:

  • It cannot be transferred from one person to another by air such as a cold, or by sexual intercourse such as gonorrhea, or through food such as food poisoning.
  • Reportable, meaning, a doctor or clinic is not required to report the cases of ALS he/she has seen to the Center for Disease Control unlike some diseases (mostly communicable) such as tuberculosis or whooping cough.
  • There is NO KNOWN CAUSE OR CURE. The two medicines approved by FDA for ALS are not cures. Riluzole (Rilutek) extends the life of a pALS for up to 3-5 months. Edaravone (Radicava) delays deterioration of symptoms when given early in the illness.
  • ALS does not present itself the same way in affected people. Its symptoms at onset, course, and history of progression vary greatly from individual to individual. It is not uncommon to have periods lasting weeks to months where there is very little or no loss of function. There are even extremely 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% of patients with ALS will have significant improvement in function lasting 12 months or more.
  • Does not affect sensation: touch, smell, sight, smell, or hearing. It also usually spares the muscles of the eyes, bowel and bladder control, and reproductive functions. Although higher cognitive functions are typically spared, fronto-temporal dementia can be shown in late stage ALS.

ALS in Hawaii

In Hawaii, the more famous pALS were the football player and coach Charlie Wedemeyer and watercolorist Peggy Chun.

Based on the 2016 population statistics, there are 56 people living with ALS at any one time, and 29 new patients are diagnosed every year. As of March 2018, there are 61 known pALS in the state.

There are two national organizations with physical presence and providing services to ALS patients: MDA-Hawaii and ALS Association-Golden West Chapter. Aside from ALS Foundation of Hawaii, there is one other local ALS organization, the ALS Ohana. Veterans with ALS are served by the Veterans Administration. Kaiser-Permanente offers a multi-disciplinary clinic service in addition to other services their members are entitled.

For more information about ALS, please refer to www.alsa.org