Actor Sam Shepard has died after a private battle with amyotrophic lateral sclerosis—the disease made famous first by baseball player Lou Gehrig, and more recently by the Ice Bucket Challenge.
In recent years, most news coverage of ALS has focused on the Ice Bucket Challenge—the viral social-media phenomenon that was started in 2014 to raise money and awareness about the disease. The profits from the Ice Bucket Challenge really did go on to fund a new discovery that may help future ALS patients, but scientists still have a long way to go toward understanding the disease, developing treatments, and hopefully one day finding a cure.
An average of 15 people are diagnosed with ALS every day, and as many as 30,000 Americans are currently affected by the disease. But besides the buckets of freezing water and the occasional celebrity diagnosis, many people know very little about this fatal neurological condition. To learn more, Health spoke with Eva Feldman, MD, a neurologist and ALS researcher with the University of Michigan Health System. Dr. Feldman did not treat Shepard, but she's counseled many other patients and their families about the realities of ALS.
It can affect the body and the mind
ALS attacks the nerve cells that control voluntary movement, making moving the arms, legs, and face increasingly difficult over time. And while the disease usually doesn’t affect a person’s intelligence, some people with ALS may have depression or impairments in decision-making and memory, according to the National Institute of Neurological Disorders and Stroke.
“We used to think that patients with ALS did not have trouble thinking, but we now know that probably 20% of patients will have some problems with cognition,” says Dr. Feldman. That’s one reason ALS treatment plans should take both physical and mental health into account, she adds. “Our patients see doctors, nurses, nutritionists, physical therapists, speech therapists, and social workers—and we know that patients have better longevity if they’re treated in these multidisciplinary settings.”
It’s not necessarily an inherited disease
About 5 to 10% of all cases occur within families, but 90% of patients with adult-onset ALS have no family history of the disease. More research is needed on possible risk factors, but research has shown that military veterans—especially those who were deployed during the Gulf War—are twice as likely to develop ALS.
Other environmental elements have been associated with an increased risk for ALS, as well. Dr. Feldman and her colleagues published an article last year, for example, linking pesticide exposure with development of the disease; another recent study found that workplace exposure to electromagnetic fields may raise people’s risk, as well.
It’s more common in men and white people
About 5,600 new cases of ALS are diagnosed annually. It's about 20% more common in men than in women, and 93% of the patients registered in the ALS CARE database are Causasian. One recent study also suggested that white-collar workers are more likely to die form ALS, compared to people who are less affluent and less educated.
The highest concentration of ALS cases are diagnosed between ages 60 and 69, but younger people can also develop the disease. In fact, Pete Frates, the creator of the Ice Bucket Challenge, was diagnosed with ALS in 2012 at age 27.
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Symptoms aren’t sudden
Someone with ALS doesn’t just wake up one day with an inability to move their legs or arms. Symptoms may be so subtle at first that they often go unnoticed. Early signs can include cramps, tight and stiff muscles, slight twitches, or difficulty chewing or swallowing.
“From first symptom to diagnosis is usually about a year,” says Dr. Feldman. “Frequently, a patient will wait until the symptoms become more worrisome to see a doctor.” Even then, doctors may suspect arthritis in the neck or spine, before a patient’s true diagnosis becomes clear.
Eventually, patients lose vital functions like breathing and swallowing. Most people live for two to five years after symptoms develop, and most eventually die from respiratory failure.
People with the disease may be living longer
Even though there is no cure yet for ALS, clinical trials have shown that a drug called Riluzole may prolong life by seven months for people with the disease. And just this year, the Food and Drug Administration approved another medication called Radicava—the first new drug approved for ALS in more than 20 years.
These drugs do not reverse the damage already done to motor neurons, or the related symptoms patients are already experiencing. But starting treatment early—both medication and respiratory therapy—can help slow the disease’s progression. And while patients today still face an arduous and uphill battle, there’s also reason to be optimistic, says Dr. Feldman.
“Since the Ice Bucket Challenge, there’s been a substantial resurgence in research by the scientific community, and there are many excellent scientists working to understand the pathogenesis of ALS,” she says. “I’ve been doing this for over 25 years, and we’ve made more progress in the last five years than we did in the previous 20.”