Dying Outside

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.