The chilly Ice Bucket Challenge is lighting up social media and raising funds and awareness about amyotrophic lateral sclerosis (ALS), or Lou Gehrig’s disease, in honor of the baseball great who contracted ALS. So far, the challenge has raised more than $41 million for the ALS Association.
ALS is a fatal progressive neuromuscular disease that affects motor neurons ““ specialized cells in the brain and spinal cord that control voluntary muscle movements. As the disease progresses over time, motor neurons die off and can no longer send the messages that control muscle movement, leading to muscle weakness, involuntary twitching and eventually, paralysis. People with ALS lose the ability to walk, to speak and even to breathe. They may eventually become completely paralyzed, according to the ALS Association.
Worldwide, the disease affects about 5 out of every 100,000 people, according to the National Library of Medicine. In the U.S., about 30,000 Americans have the disease at any given time. About 1 in 10 of those cases is caused by a genetic defect. The rest of the time, doctors don’t know what caused the disease.
Most people with ALS start showing symptoms between the ages of 40 and 70, but sometimes symptoms begin earlier. On average, a person survives for three to five years after being diagnosed with ALS, but everyone’s experience is different. About 20 percent of people with ALS can live five years or more after diagnosis, and 5 percent can live 20 years. Symptoms include:
- Difficulty breathing
- Difficulty swallowing, including choking easily, drooling or gagging
- Head drop due to weakness of the neck muscles
- Muscle cramps
- Muscle contractions
- Muscle weakness that slowly gets worse. Often, the weakness will affect one part of the body first, such as the hand or a leg; eventually it will lead to problems with everyday tasks like lifting or climbing stairs.
- Paralysis
- Speech problems, including slurring words
- Voice changes, hoarseness
- Weight loss
Few drugs are currently available to treat the disease, though research is ongoing. One medication, Riluzole, has been shown to slow ALS’s progression and add months to patients’ lifespan. Physical therapy, assistive devices including braces or wheelchairs and respiratory aids, including breathing tubes, help make the disease more manageable.
