Beating ALS’ ghastly odds a triumph, and a mystery
Stephen Hawking fascinated astrophysicists with his otherworldly theories. But he also blew the minds of medical experts by living with ALS for 55 years.
Amyotrophic lateral sclerosis (ALS) — otherwise known as Lou Gehrig’s disease — can kill someone within a few months of diagnosis, and there’s only a 10% chance that patients live more than a decade, the ALS Association says.
Some medical experts have said Hawking lived so long because he developed the disease so early in his life. The ALS Association says most people are diagnosed at age 40 to 70. Hawking was diagnosed at age 21. He died Wednesday at 76.
“We have found that the survival in younger patients is strikingly better and is measured in many years — in some cases more than 10,” King’s College clinical neurology professor Nigel Leigh said in a 2002 entry on Hawking in the British Medical Journal. “Among
Amyotrophic lateral sclerosis can kill in a few months, and there’s only a 10% chance patients live more than a decade.
people in their 50s and 60s, there is a 50% chance of surviving four years or so. It is a different beast if you start young, oddly, and no one knows why.”
Pam Shaw, a professor of neurology at England’s University of Sheffield, says she, too, wasn’t sure why some patients survive longer.
“The older you are the quicker the disease course tends to be, but we don’t really have a handle on why some people survive for longer periods than others. I wish we did,” she said.
University of Pennsylvania associate neurology professor Leo McCluskey told Scientific American that Hawking’s type of ALS may be similar to juvenile-onset disorder, which progresses “very, very, very slowly.”
“I have patients in my clinic who were diagnosed in their teens and are still alive in their 40s, 50s or 60s,” said McCluskey, who added that slow-progressing ALS occurs “less than a few percent” of the time.
McCluskey, however, told Scientific American it’s possible for patients to live on if they can avoid respiratory problems and the weakening of swallowing muscles, two main factors in life expectancy.
“What’s happened to him is just astounding,” McCluskey said. “He’s certainly an outlier.”