Stephen Hawking, who died Wednesday at the age of 76, developed ALS at the age of 21 – meaning he lived for more than five decades with the disease.
The renowned scientist was very much the exception in this, as most people die within two to five years of diagnosis.
“We do have a few people who are living in Canada with the disease for around 25 years, but even that is very, very rare,” said Tammy Moore, CEO of ALS Canada.
ALS, or amyotrophic lateral sclerosis, is a progressive disease for which there is no cure. There are about 3,000 people in Canada living with ALS, with another 1,000 or so diagnosed every year. Unfortunately, about the same number die annually.
In its most common form, it often first shows up as muscle weakness, where people might start dropping things or tripping over their own feet.
WATCH: Stephen Hawking exceeded the expectations of doctors and lived to 76-years-old with ALS.
Moore’s cousin, who was diagnosed when he was 45, found that he was dropping his hockey stick. “As it moved its way in, he lost his ability to pick up a drink, to feed himself and then to be able to walk, and eventually affecting his ability to speak and to breathe.”
It’s a “progressive paralysis,” Moore said, and there are only very limited treatments available at the moment, though there are drugs being developed that give her hope. “But it’s likely to be a cocktail that’s going to be required because of the complexity of the disease, and will be very expensive.”
“We do see people with longevity,” Moore said. “But we also have to keep in mind that often those people also have a lot of assistive devices that enable them to have longevity or help them with that.”
Although his disease progressed very slowly, Hawking also benefited from quality care and assistive technology.
He had a tracheostomy in the 1980s after a bout of pneumonia, which left him breathing through a tube. After he and his first wife divorced, his nursing care was covered by a grant from the United States. He was in a motorized wheelchair most of his life.
In the last few years of Hawking’s life, the only thing connecting the brilliant physicist to the outside world was a couple of inches of frayed nerve in his cheek.
As slowly as a word per minute, Stephen Hawking used the twitching of the muscle under his right eye to grind out his thoughts on a custom-built computer, which could also speak for him.
WATCH: In this interview from 2017, Stephen Hawking said he was looking forward to a space trip with Richard Branson
Access to such communication devices makes a big difference to people living with ALS, Moore said. “You can appreciate that without eye-gaze technology that Stephen Hawking was using, he wouldn’t have been able to communicate to the outside world whatsoever.”
“So if someone’s in need of a medical intervention, for instance, I could be sitting beside you and choking to death – I couldn’t tell you unless I had my eye-gaze technology set up.”
In over half of ALS cases, like Hawking’s, individuals have no cognitive impairment as a result of the disease. “So someone is fully cognizant, fully able to feel but unable to move their body,” Moore said.
The types of assistance provided vary widely across Canada, she said. She also believes that there needs to be more research into the disease to understand why it progresses so slowly in some people, like Hawking, and so quickly in others.
To people living with ALS, Hawking was a source of inspiration.
“I was diagnosed at the age of 22, Professor Hawking at the age of 21,” said Eddy Lefrançois, who was diagnosed with ALS 25 years ago.
“At the time of my diagnosis, he had already been living with ALS for 25 years. He inspired me with his continued achievements, even though he was living with ALS. ALS did affect his body, but not his ability to think.”
“I thought he was a brilliant man with a love for life. He seemed to have lived his life the way he wanted on both a personal, and professional level.”
WATCH: The University of Cambridge paid tribute to Stephen Hawking in a video released after his death on Wednesday, March 14, describing him as one of the world’s most brilliant minds.
Carol Skinner, who has been living with ALS for nearly five years, notes that the tributes to Hawking didn’t mention ALS before his other achievements. She also remembers Hawking’s humour. “His sense of humour made him relatable and endeared him to popular culture. Though trapped in his own body due to paralysis, a terrible symptom of ALS, Stephen never lost himself,” she said.
“Like Stephen, I was given 2-3 years left to live by my neurologist. This September it will be my 5th year living with ALS. Stephen focused on living, not dying, and I struggle to emulate this part of him every day.”
Although Hawking wrote that he was deeply depressed when he was first diagnosed, he eventually felt like he could be “quietly satisfied” with his life.
“I accept that there are some things I can’t do,” he told The Associated Press in 1997. “But they are mostly things I don’t particularly want to do anyway.”
Then, grinning widely, he added, “I seem to manage to do anything that I really want.”
–With files from the Associated Press and Reuters