Hal Finney: Dying Outside

[R.A. Hettinga]

<http://lesswrong.com/lw/1ab/dying_outside/>

Less Wrong

Dying Outside
59
HalFinney
05 October 2009 02:45AM

A man goes in to see his doctor, and after some tests, the doctor  
says, "I'm sorry, but you have a fatal disease."

Man: "That's terrible! How long have I got?"
Doctor: "Ten."
Man: "Ten? What kind of answer is that? Ten months? Ten years? Ten  
what?"
The doctor looks at his watch. "Nine."

Recently I received some bad medical news (although not as bad as in  
the joke). Unfortunately I have been diagnosed with a fatal disease,  
Amyotrophic Lateral Sclerosis or ALS, sometimes called Lou Gehrig's  
disease. ALS causes nerve damage, progressive muscle weakness and  
paralysis, and ultimately death. Patients lose the ability to talk,  
walk, move, eventually even to breathe, which is usually the end of  
life. This process generally takes about 2 to 5 years.

There are however two bright spots in this picture. The first is that  
ALS normally does not affect higher brain functions. I will retain my  
abilities to think and reason as usual. Even as my body is dying  
outside, I will remain alive inside.

The second relates to survival. Although ALS is generally described as  
a fatal disease, this is not quite true. It is only mostly fatal. When  
breathing begins to fail, ALS patients must make a choice. They have  
the option to either go onto invasive mechanical respiration, which  
involves a tracheotomy and breathing machine, or they can die in  
comfort. I was very surprised to learn that over 90% of ALS patients  
choose to die. And even among those who choose life, for the great  
majority this is an emergency decision made in the hospital during a  
medical respiratory crisis. In a few cases the patient will have made  
his wishes known in advance, but most of the time the procedure is  
done as part of the medical management of the situation, and then the  
ALS patient either lives with it or asks to have the machine  
disconnected so he can die. Probably fewer than 1% of ALS patients  
arrange to go onto ventilation when they are still in relatively good  
health, even though this provides the best odds for a successful  
transition.

With mechanical respiration, survival with ALS can be indefinitely  
extended. And the great majority of people living on respirators say  
that their quality of life is good and they are happy with their  
decision. (There may be a selection effect here.) It seems, then, that  
calling ALS a fatal disease is an oversimplification. ALS takes away  
your body, but it does not take away your mind, and if you are  
determined and fortunate, it does not have to take away your life.

There are a number of practical and financial obstacles to  
successfully surviving on a ventilator, foremost among them the great  
load on caregivers. No doubt this contributes to the high rates of  
choosing death. But it seems that much of the objection is  
philosophical. People are not happy about being kept alive by  
machines. And they assume that their quality of life would be poor,  
without the ability to move and participate in their usual activities.  
This is despite the fact that most people on respirators describe  
their quality of life as acceptable to good. As we have seen in other  
contexts, people are surprisingly poor predictors of how they will  
react to changed circumstances. This seems to be such a case,  
contributing to the high death rates for ALS patients.

I hope that when the time comes, I will choose life. ALS kills only  
motor neurons, which carry signals to the muscles. The senses are  
intact. And most patients retain at least some vestige of control over  
a few muscles, which with modern technology can offer a surprisingly  
effective mode of communication. Stephen Hawking, the world's longest  
surviving ALS patient at over 40 years since diagnosis, is said to be  
able to type at ten words per minute by twitching a cheek muscle. I  
hope to be able to read, browse the net, and even participate in  
conversations by email and messaging. Voice synthesizers allow local  
communications, and I am making use of a free service for ALS patients  
which will create a synthetic model of my own natural voice, for  
future use. 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.