Locus Online
23 March 2006

Dear Locus,

I have been diagnosed with amyloidosis. That is a rare blood disease which affects only 8 people out of a million each year, and those 8 per million are divided among 22 distinct forms of amyloidosis. They are distinct enough that while some have no treatment at all, for the others, the treatment that works on one will have no effect whatsoever on any of the rest. An amyloid is a misshapen or misfolded protein that can be produced by various parts of the body and which may deposit in other parts of the body (nerves or organs) with varying effects. (As a small oddity, amyloids are associated with a wide list of diseases ranging from carpal tunnel syndrome to Alzheimer's. There's no current evidence of cause and effect, and none of these is considered any form of amyloidosis, but the amyloids are always there. So it is entirely possible that research on amyloids may one day lead to cures for Alzheimer's and the Lord knows what else. I've offered to be a literary poster boy for the Mayo Amyloidosis Program, and the May PR Department, at least, seems very interested. Plus, I've discovered a number of fans in various positions at the clinic, so maybe they'll help out.)

Now in my case, what I have is primary amyloidosis with cardiomyapathy. That means that some (only about 5% at present) of my bone marrow is producing amyloids which are depositing in the wall of my heart, causing it to thicken and stiffen. Untreated, it would eventually make my heart unable to function any longer and I would have a median life expectancy of one year from diagnosis. Fortunately, I am set up for treatment, which expands my median life expectancy to four years. This does NOT mean I have four years to live. For those who've forgotten their freshman or pre-freshman (high school or junior high) math, a median means half the numbers fall above that value and half fall below. It is NOT an average.

In any case, I intend to live considerably longer than that. Everybody knows or has heard of someone who was told they had five years to live, only that was twenty years ago and here they guy is, still around and kicking. I mean to beat him. I sat down and figured out how long it would take me to write all of the books I currently have in mind, without adding anything new and without trying rush anything. The figure I came up with was thirty years. Now, I'm fifty-seven, so anyone my age hoping for another thirty years is asking for a fair bit, but I don't care. That is my minimum goal. I am going to finish those books, all of them, and that is that.

My treatment starts in about 2 weeks at the Mayo Clinic in Rochester, Minnesota, where they have seen and treated more cases like mine than anywhere else in the US. Basically, it boils down to this. They will harvest a good quantity of my bone marrow stem cells from my blood. These aren't the stem cells that have Bush and Cheney in a swivet; they can only grow into bone marrow, and only into my bone marrow at that. Then will follow two days of intense chemotherapy to kill off all of my bone marrow, since there is no way at present to target just the misbehaving 5%. Once this is done, they will re-implant my bmsc to begin rebuilding my bone marrow and immune system, which will of course go south with the bone marrow. Depending on how long it takes me to recuperate sufficiently, 6 to 8 weeks after checking in, I can come home. I will have a fifty-fifty chance of some good result (25% chance of remission; 25% chance of some reduction in amyloid production), a 35-40% chance of no result, and a 10-15% chance of fatality. Believe me, that's a Hell of a lot better than staring down the barrel of a one-year median. If I get less than full remission, my doctor already, she says, has several therapies in mind, though I suspect we will heading into experimental territory. If that is where this takes me, however, so be it. I have thirty more years worth of books to write even if I can keep from thinking of any more, and I don't intend to let this thing get in my way.

Jim Rigney/Robert Jordan

Update 3 April: Information about donations for Amyloidosis research is posted here.

© 2006 by Locus Publications. All rights reserved.