[Thorzdad]

Stephen Hawking has passed away.

[The Final Dakar]

Man had no business living as long as he did. He was incredible.

[reader50]

I'll miss him. We'll all miss him - probably no antigrav or FTL in our lifetimes now.

[subego]

Originally Posted by The Final Dakar 

Man had no business living as long as he did. He was incredible.

Not to take away anything from him, but I get the impression it was decided he couldn’t have had “standard” ALS, and instead had a rare, related condition.

[ghporter]

We have lost a massive mind with an immense imagination. And a pretty much razor sharp wit, too. He was frequently quite flip - even caustic - in interviews. Clearly he did not take his grave illness with the same gravity as people around him did.

And his longevity is a very positive thing quite aside from his accomplishments in science. For one thing, he’s a hopeful icon for people who face a diagnosis of ALS. For another, he’s a figurehead for people facing almost any obstacle: he made enormous strides in human understanding of...well everything, and he did so AFTER being diagnosed with ALS.

So how did a guy who was diagnosed at 21 manage to live some 18 times longer than his original prognosis? It’s complicated, but that’s true about almost everything related to ALS. What specific muscles are affected, how quickly those muscles are affected and how quickly those affects become problems, and a plethora of other factors have a hand in how the disease progresses.

ALS is characterized as a group of related neurological diseases that are typically thought of as “lower motor neuron” diseases. There are two classes of motor neurons: upper motor neurons that emerge from the brain and connect farther down in the spinal cord, and lower motor neurons that connect between the upper neurons and the individual muscles.

While the most obvious impacts of these diseases are muscular in nature, many forms of motor neuron disease affect upper motor neurons and sometimes parts of the brain that are unrelated to movement as well. Some victims can develop a particular form of dementia that is directly related to the disease.

The most common causes of death in all forms of ALS are respiratory failure and pneumonia, but malnutrition is also an important factor. When the diaphragm becomes paralyzed, breathing is no longer going to happen on its own. Impaired inhalation AND exhalation (which is not 100% passive) put a person at a high risk for developing pneumonia.

Malnutrition comes from failure of the very critical swallowing muscles. Swallowing is a highly choreographed operation, coordinating breathing and movement of food and liquids into the esophagus. A few muscles becoming paralyzed can cause ALL oral consumption (food and drink) to become a potentially fatal risk.

One thing that Dr. Hawking had going for him was his drive. He had stuff to do, and wasn’t going to put up with obstacles. Another was that he had an enormous amount of support. That translated into his communication system, his powered mobility, and eventually 24/7 respirator support (and presumably some alternate form of nutrition, but I haven’t found much about that).

Finally, while most people who are diagnosed with ALS have a fairly short lifespan after diagnosis, the rate at which the disease progresses is always unique to the individual patient. It’s quite probable that Dr. Hawking had a combination of otherwise robust health and some sort of genetic makeup that helped slow the progress of the disease.

And dammit, I had questions for the man. Now I’ll never get to ask him about his ride in the Vomit Comet, playing a hologram of himself in Star Trek, or any of the other important parts about living with a debilitating, chronic disease. As an occupational therapist, that’s a huge loss all by itself.

[subego]

Originally Posted by ghporter 

For one thing, he’s a hopeful icon for people who face a diagnosis of ALS.

I get the impression, from the go-to person in the family for terminal illness, there is no hope once you’re diagnosed. It’s the only one he says he’s had to deal with where there’s a prevailing sense of defeat from all the literature and all the doctors.

He mentioned the thing about Hawking in passing awhile back. It’s certainly possible he’s incorrect.

[Waragainstsleep]

I remember 20 years ago I spotted an ad in New Scientist to apply to work for him. It was a 1 year internship of sorts where the successful applicant was responsible for maintaining and improving his voice synthesiser. It included travelling around the world wherever he went. I bet it was quite an experience for those who got to do it.