DEAR DOCTOR K: Former Massachusetts governor Paul Cellucci recently died from complications of ALS. Can you tell me more about this disease?
DEAR READER: ALS stands for amyotrophic lateral sclerosis. It is also known as Lou Gehrig’s disease, named after the famous baseball player who suffered from it.
There are many different kinds of brain cells. Some do our thinking, some move our muscles (when the ones that think tell them to), and other brain cells do other things (such as see and hear). ALS primarily causes a slow degeneration of the nerve cells that control muscle movements. As a result, people with ALS gradually lose the ability to control their muscles.
ALS generally strikes patients between the ages of 50 and 70. We don’t know what causes ALS. As a result, there is no way to prevent it. Some cases appear to be inherited.
The weakness and wasting (atrophy) of the muscles involves the arms and legs, the breathing muscles, and the muscles of the throat and tongue. The weakness worsens over time. Eventually, people with ALS are trapped in their bodies, but completely alert. They understand what’s going on around them, including people speaking to them. But when the disease progresses to the point where it withers the muscles of their throat and tongue, they cannot answer.
There is no cure for ALS. People with the disease live an average of three to five years after symptoms begin.
Riluzole (Rilutek) is the only drug approved by the Food and Drug Administration for ALS. It can prolong survival in some people. Medications may help to manage symptoms of ALS; pain medications and muscle relaxants may help with painful muscle tightness.
Researchers are making progress in understanding the causes of ALS and in finding treatments that help. Hopefully, the pace of new discovery will speed up and a cure will be found for this terrible disease.