January 8, 2012 in Nation/World

Physicist celebrated on 70th

Few survive as long with Lou Gehrig’s disease
Maria Cheng Associated Press
Associated Press photo

Professor Stephen Hawking sits in his office at the University of Cambridge in England in December 2011.
(Full-size photo)

CAMBRIDGE, England – British scientist Stephen Hawking has decoded some of the most puzzling mysteries of the universe but he has left one mystery unsolved: how he has managed to survive so long with such a crippling disease.

The physicist and cosmologist was diagnosed with Lou Gehrig’s disease when he was a 21-year-old student at Cambridge University. Most people die within a few years of the diagnosis, called motor neurone disease in the U.K. Today, Hawking will turn 70.

“I don’t know of anyone who’s survived this long,” said Ammar Al-Chalabi, director of the Motor Neurone Disease Care and Research Centre at King’s College London. He does not treat Hawking and described his longevity as “extraordinary.”

“It is unusual for (motor neurone disease) patients to survive for decades, but not unheard of,” said Dr. Rup Tandan, a neurology professor at the University of Vermont College. Still, Tandan said many longtime survivors had ventilators to breathe for them – which Hawking does not.

To mark his birthday today, Cambridge University is holding a public symposium on “The State of the Universe,” featuring talks from 27 leading scientists, including Hawking. For 30 years, he held a mathematics post at the university previously held by Sir Isaac Newton. Hawking retired from that position in 2009 and is now director of research at the university’s Centre for Theoretical Cosmology.

Hawking achieved all that despite being nearly entirely paralyzed and in a wheelchair since 1970. He now communicates only by twitching his right cheek. Since catching pneumonia in 1985, Hawking has needed around-the-clock care and relies on a computer and voice synthesizer to speak.

Lou Gehrig’s disease, also called amyotrophic lateral sclerosis, or ALS, attacks motor neurons, cells that control the muscles. Patients typically suffer muscle weakness and wasting, become paralyzed and have problems talking, swallowing and breathing. Only about 10 percent of patients live longer than a decade.

People who are stricken at a young age, as Hawking was, generally have a better chance of surviving longer. Most people are diagnosed between 50 and 70. Life expectancy generally ranges from two to five years after symptoms set in.

For some reason, the disease has progressed more slowly in Hawking than in most. Al-Chalabi and colleagues are analyzing a DNA sample from Hawking, along with those of other patients, to see if there is something rare about his disease or any genetic mutations that could explain his long survival.

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