How Stephen Hawking is still alive, defying ALS and the worst expectations

Hawking's amazing survival has left some doctors unable to believe that he suffers from ALS

On April 20, 2009, a moment arrived that doctors had foretold for decades. Stephen Hawking, a scientist who overcame debilitating disease to become the world’s most renowned living physicist, was on the cusp of death. The University of Cambridge released grim prognoses. Hawking, diagnosed with amyotrophic lateral sclerosis (ALS) at the age of 21, was described as “very ill” and “undergoing tests” at the hospital. Newspapers ran obituary-esque articles. It seemed time was up for the man who so eloquently explained it.

But, as is his custom, Hawking survived.

Hawking shouldn’t be able to do the things he now does. The 73-year-old shouldn’t be able to deliver meditations on the existence of God. He shouldn’t be able to fret over artificial intelligence or humanity’s capacity for self-destruction. And he most definitely shouldn’t be able to attend the BAFTAs — Britain’s academy awards — settled inside the wheelchair that has carried him for decades, expressing admiration for a recent biopic that paid homage to his struggle. But yet, he is. And he does.

“He is exceptional,” Nigel Leigh, a professor of clinical neurology at King’s College London, told the British Medical Journal in 2002. “I am not aware of anyone else who has survived with [ALS] as long. What is unusual is not only the length of time, but that the disease seems to have burnt out. He appears to be relatively stable. … This kind of stabilization is extremely rare.”

This description is not in any way unusual. More than a decade later, when Hawking turned 70 in 2012, more researchers were baffled and amazed. Anmar al-Chalabi of King’s College London told the Associated Press Hawking was “extraordinary. … I don’t know of anyone who’s survived this long.”

So what makes Hawking different from the rest? Just luck? Or has the transcendent nature of his intellect somehow stalled what seemed an imminent fate? No one’s quite sure. Even Hawking himself, who can expound at length on the mechanics that govern the universe, is circumspect when it comes to an accomplishment that rivals his academic triumphs. “Maybe my variety [of ALS] is due to bad absorption of vitamins,” he said.

Hawking’s battle with ALS was different from the beginning. And those differences, scientists say, partly explain his miraculous longevity. The onset of ALS normally occurs later in life — the average age of diagnosis is 55 — but Hawking’s symptoms materialized when he was very young. It began with a stumble.

“In my third year at Oxford, I noticed that I seemed to be getting more clumsy, and I fell over once or twice for no apparent reason,” Hawking once wrote. “But it was not until I was at Cambridge that my father noticed, and took me to the family doctor. He referred me to a specialist, and shortly after my 21st birthday, I went into hospitals for tests. … It was a great shock to me to discover that I had motor neuron disease,” the name for the group of progressive neurological disorders that includes ALS.

Stephen Hawking, aged 12 (Rex Features)

Though the early diagnosis resigned him to a life of sickness, it also granted him a chance at surviving the disease longer than those who are diagnosed much later. “We have found that the survival in younger patients is strikingly better and is measured in many years — in some cases more than 10,” Leigh told the British Medical Journal. “… It’s a different beast if you start young, oddly, and no one knows why.”

Leo McCluskey of the University of Pennsylvania told Scientific American that ALS primarily kills in two different ways. One affects the breathing muscles. “So the common way people die is of respiratory failure,” he said. The other is the failure of swallowing muscles, which can result in dehydration and malnutrition. “If you don’t have these two things, you could potentially live for a long time,” he said.

But as long as Hawking has lived? For his part, Hawking says his work, focused through his disability, granted him years that wouldn’t have been available to others. Someone in a more physical field — like, say, Lou Gehrig, the New York Yankee who contracted ALS in his 30s — couldn’t have functioned at so high a level. “It has certainly helped that I have a job and that I have been looked after so well,” Hawking told the New York Times in 2011. “I am lucky to be working in theoretical physics, one of the few areas in which disability is not a serious handicap.”

If anything, Hawking illustrates the very different ways ALS can afflict its victims — “just an incredible, incredible example,” McCluskey said.

It has also given rise to one of the most striking contrasts of pop science. There is Stephen Hawking’s atrophied frame, slack-jawed expression and slumped shoulders. And there is Hawking’s unmatched mind, inhabiting the stars.

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