What Stephen Hawking Can Teach Us About ALS

Stephen Hawking was as renowned for his battle with ALS as for his theory on black holes. His success and visibility made the wheelchair-bound physicist, who died March 14 at age 76, a symbol for living productively with a debilitating disease.

By Julie Vargo, Editorial Contributor

Stephen Hawking was as renowned for his lifetime battle with ALS (or amyotrophic lateral sclerosis) as for his cosmic genius and theory on black holes. Because of his success and visibility, the brilliant, wheelchair-bound physicist, who died March 14 at age 76, became an unwitting symbol for living productively with a debilitating disease.

Also known as Lou Gehrig Disease, ALS affects about two in every 100,000 people. “Stephen Hawking was completely outstanding in many ways,” says Dr. Tawfiq Lahham, a neurologist with Orlando Health Physicians Neurology Group who specializes in neuromuscular diseases.

The Cambridge University scientist was diagnosed with ALS at age 21. “We do not typically see this manifest in people that young,” says Dr. Lahham. “The average age of onset is approximately 60 years. As in other neurodegenerative conditions, the cause is unknown, and only 10 percent to 15 percent of all cases have a hereditary link. When you don’t know the cause, it is hard to stop the disease from progressing.”

Doctors gave Hawking only a few years to live — a normal prognosis. “Average life expectancy is usually three to five years from onset of symptoms,” says Dr. Lahham, who notes every case is different. “There have been dozens of clinical trials, but none of them have identified a therapy to stop or reverse the disease.”

While there is no cure, medical care can prolong a patient’s life. “There are some FDA-approved treatments that slow down progression and increase lifespan,” says Dr. Lahham. “The sooner treatment begins, the more effective we are. Patients, who attend ALS specialty clinics, maintain their weight and use support for breathing symptoms generally live longer.”

Diagnosed early and supported by family, Hawking defied odds, surviving five decades longer than expected. He married twice, fathered three children, became a bestselling author and researched the origins of the universe. He made guest appearances on shows such as “Star Trek,” “The Simpsons,” “Futurama” and “Big Bang Theory. “All the while, ALS progressively weakened and paralyzed his limbs as well as core muscles and the diaphragm, which affected his ability to breathe, speak and swallow. He used a voice computer to speak.

“This is another sad part of a motor neuron disease such as ALS,” says Dr. Lahham. “Most ALS patients have normal cognitive ability, but are physically trapped inside a body that is wasting away.”

The early signs of ALS are subtle. Patients notice painless muscle twitches as well as weakness and wasting. Less commonly, the initial symptoms may be impaired speech, swallowing or breathing.  Recurring and progressive signs such as difficulty opening a jar or frequent tripping are red flags that need to be evaluated by a neurologist.

Stephen Hawking, The Ice Bucket Challenge and Community ALS Walks have helped raise awareness about the disease. “This is important for many reasons,” says Dr. Lahham. “Especially for those who are suffering symptoms, but have never heard of motor neuron disease. Having access to appropriate medical care can prolong and improve patients’ quality of life.”