If you’re watching the hit Fox show, “Empire,” starring Taraji P. Henson and Terrence Howard, you know that the key storyline is centered on Howard’s character "Lucious Lyon's" diagnosis with ALS.
Yes, that ALS. It’s the same ALS that caught the attention of the nation in a viral video campaign that had everyone from your old college roommate to your mom to celebrities like Kevin Hart, LeBron James, and Oprah Winfrey, dumping ice water on their heads in the Ice Bucket Challenge to raise money for research.
But what is ALS?
Amyotrophic lateral sclerosis (ALS), which is also known as "Lou Gehrig's Disease," is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. It is fairly rare, in that 5,600 people on average are diagnosed each year. To dig deeper into the medical side of the disease, we talked to Jeffery McClean, M.D., Assistant Professor of Neurology, Uniformed Services University of the Health Sciences, San Antonio Military Medical Center.
Part of the storyline in Empire is that Lucious’ disease is fatal. Dr. McClean says, “ALS is almost uniformly fatal.” He says, “this is because the progressive weakness eventually involves the muscles required for breathing, leading to respiratory failure and death.”
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According to Dr. McClean, “the average time from the beginning of the disease until death is about 3 to 5 years.“ However, he says that there can be many variables from person to person. “Up to about 10 percent of people with ALS may live over 10 years, from the beginning of the disease,” he adds.
Dr. McClean says that Riluzole (brand name Rilutek) is the only FDA-approved medication for the treatment of ALS, and it has been available for almost 20 years.“ It slows progression of the disease somewhat, leading to prolonged survival of about 2-3 months on average, he says. “There are currently no drugs or other treatments that can stop or reverse the progression of ALS. “
According to Dr. McClean, it is important to realize that many of the symptoms of ALS can be treated, leading to a much higher quality of life. “There is a great deal of promising research ongoing that may lead to new treatments for ALS, but we are not quite there yet,” he says.
How long after initial diagnosis can a person stay fairly independent?
This is difficult to answer because ALS begins and progresses very differently for each individual. Also there is some variability about how far the disease has progressed before the diagnosis is made. For some, it might be 18 months or even less. For others, the progression is slower, and they may remain independent, with a good quality of life, for several years.
The progressive degeneration of the motor neurons in ALS eventually leads to their death. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, patients in the later stages of the disease may become totally paralyzed.
Generally, ALS involves the nerve cells (neurons) that control our voluntary muscles. However, between one-third to one-half of people with ALS will develop some problems with thinking or behavior. For most this is relatively mild, but a smaller percentage, perhaps about 10 percent, have problems severe enough to be classified as dementia.
This generally looks different than Alzheimer’s. Individuals with dementia due to ALS tend to have more difficulty with communication, planning and decision-making, and personality changes compared to the primary issue of memory loss with Alzheimer’s.
In ‘Empire,’ Lucious Lyon has three adult sons. Dr. McClean says that in the vast majority of cases, ALS is not hereditary. “Unless there is already a known family history, the family members and children of an individual diagnosed with ALS are very unlikely to develop the disease. “
He does add that five to ten percent of cases are inherited and can therefore run in families.