I woke up Wednesday morning to the terrible news that Stephen Hawking had died.
While I never met the man, and didn’t really understand all he was saying about black holes, I learned a lot from him about what is possible for people with Amyotrophic Lateral Sclerosis (PALS).
At his diagnosis, Hawking was reportedly told that he would only live a few years and that there was nothing that could be done about it.
He beat his prognosis by many decades. While he appeared to decline a lot in the beginning of his illness, his progression seemed to slow, or even stop in later years. His course opened my eyes to the idea that there can be marked variability between PALS, and even within a given patient at different times.
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I have since validated this single observation in the 2000 PALS I have seen at the Duke ALS Clinic (www.dukealsclinic.org) and in a large database called PRO-ACT (http://n.neurology.org/content/86/9/808.long). Much to my surprise, I have even found a small group of PALS who regain significant amounts of lost function (www.alsreversals.org). Our community now has a lot of interesting research underway to try and understand this variability. If we can figure out why some PALS’ progression is very slow, stops, or reverses, we might be able to make this happen more often.
And it wasn’t just the length of his life that was remarkable, it was all that he packed into it. He wrote papers and books that expanded our knowledge of the universe, he traveled all over the world giving scientific lectures and making hilarious appearances on popular television programs like “The Simpsons” and “Big Bang Theory,} and he had a large family that appeared to love him very much. While ALS weakened his muscles, it clearly did not touch his spirit. The equipment he had, including his power wheelchair and speech-generating device, undoubtedly helped make these remarkable achievements possible as well.
I will continue to point to Stephen Hawking whenever I make a new diagnosis of ALS in my patients, pointing out that it is possible for PALS to live long and absolutely amazing lives with this disease, and that there are things our clinics can offer to help.
Richard Bedlack, M.D., Ph.D., a neurologist, leads the Duke ALS Clinic.