A man goes in to see his doctor, and after some tests, the doctor says, “I’m sorry, but you have a fatal disease.”
“That’s terrible,” the man says. “How long have I got?”
“Ten,” says the doctor.
“Ten?” the man asks. “Ten what? Years? Months?”
The doctor looks at his watch. “Nine.”
This is not quite an accurate depiction of how I got the news. I didn’t learn I had a fatal disease in ten seconds. But I did learn I had one.
I have ALS, Amyotrophic Lateral Sclerosis, also known as Lou Gehrig’s disease. ALS destroys the motor neurons that allow control of voluntary muscle movement. The prognosis is for progressive weakness and paralysis, and death within 2 to 5 years, typically from respiratory failure.
There are, however, two hopeful aspects of this disease.
First, ALS does not usually affect the higher brain functions. I will retain my ability to think and reason as usual. Even as my body is dying outside, I will remain alive inside.
Second, like most diseases described as fatal, ALS actually kills through a specific mechanism: progressive weakening of the muscles responsible for breathing. When breathing fails, there are two options: die, or go on a ventilator. Over 90% of ALS patients choose to die at that point. And many of the ones who go on a ventilator do so involuntarily, when they go to the hospital in crisis and end up on a vent before being able to express their wishes.
But for those who do choose life, most people on ventilators report an acceptable quality of life. People are actually much worse at predicting what their quality of life in a hypothetical situation would be than they think. There is a well known psychological effect, the “hedonic set point,” in which people tend to adapt to changed life circumstances and eventually return to a set point of happiness. People overestimate how unhappy adverse changes will make them, and underestimate their ability to cope.
My intent is to go on a ventilator when the time comes. I may even still be able to write code, and my dream is to contribute to open source software projects even from within an immobile body. That will be a life very much worth living.
Stephen Hawking is probably the most famous person with ALS. He has survived for over 40 years with the disease. He can only communicate by twitching his cheek muscles to select letters with a computerized system, slowly typing out his messages at about 10 words per minute. I am optimistic that by the time I need the same kind of assistive technology, it will have further improved.
[Hal Finney was diagnosed with ALS in August 2009, the same year he became the first person besides Satoshi to run Bitcoin. Despite his disease, he continued programming, developing a Bitcoin wallet using eye-tracking software at roughly 1/50th of his previous speed. He passed away on August 28, 2014, at age 58. His body was cryopreserved at the Alcor Life Extension Foundation.]