On March 14, 2018, the world mourned the loss of Cambridge professor Stephen W. Hawking, PhD. Perhaps the most remarkable part of his passing was the fact that he was 76 years old—he wasn’t supposed to live past 23. Surviving the next 55 years took constant care, determination, and—though it seems strange to say it—incredible luck.
In 1963, Dr. Hawking was a promising doctoral student at the University of Cambridge when he developed unusual clumsiness. He fell down a flight of stairs, according to author Kitty Ferguson’s biography, Stephen Hawking: An Unfettered Mind, and his speech began to slur. His doctors diagnosed him with amyotrophic lateral sclerosis—ALS, which is also known as Lou Gehrig’s disease. The prognosis: He had two years to live.
How did Dr. Hawking escape this death sentence? This is where chance creeps in: Although within a couple of years his speech was nearly unintelligible and he couldn’t walk without assistance, Dr. Hawking nonetheless had an extremely slow-growing form of the disease. “Two to five years—that is the common prognosis for ALS,” Lucie Bruijn, PhD, MBA, chief scientist for the ALS Association told Reader’s Digest. Only 10 percent of patients survive for more than ten years. “I was just at a big meeting with colleagues, and no one had ever seen such a case,” says Dr. Bruijn, “Only one clinician there had a patient that survived 20 years.” Leo McCluskey, MD, associate professor of Neurology at the University of Pennsylvania, told Scientific American that slow-growing forms of ALS occur in “less than a few percent” of patients.
One of the mysteries of ALS is how it will progress, explains Dr. McCluskey. The condition attacks your muscle control centers—your motor neurons. There are two sets—higher motor neurons in the frontal lobe of your brain and lower motor neurons in the spine. The disease can knock out the higher group, the lower, or both. Dr. Bruijn points out that ALS may also damage other parts of the brain, leading to dementia.
ALS kills by disabling the muscles that help you breathe and swallow, which is why good care is so crucial to a patient’s survival, points out Dr. Bruijn—and it played a key role in Dr. Hawking’s story. On a 1985 trip to the CERN laboratory in Switzerland, Dr. Hawking contracted severe pneumonia (a common risk for ALS patients). He became so ill that doctors recommended stopping life support; his wife refused. Instead, the staff doubled down on his care: After a tracheotomy and around-the-clock nursing, Dr. Hawking recovered.
Dr. Bruijn points to research in Europe demonstrating that patients live longer and fare better when they get medical help tailored to ALS. “The care he received and the technology he was able to take advantage of—the motorized wheelchair, the communication system—helped Dr. Hawking continue his research and engagement in life.” She speculates that his active life may have, in turn, contributed to his longevity.
“Dr. Hawking was a global icon for his intellect, and also because he continued to live a vibrant life with ALS,” she says. Learn more about ALS—and how to help in the fight against it—here.