Post 2 of 5
An understandably common question I get is,
"Any progress in getting a transplant?"
For the last year I've had to give the same disappointing answer: "No, first I have to get some genetic testing done to see how aggressive my disease is, because I have an autoimmune condition; they won't want to take live donors if the kidney isn't likely to last long."
The testing was originally supposed to have been done by New Year, but come March, I still hadn't even gotten approved to have the samples taken, never mind getting the results. Finally, in April, I got to do that, and the report came on July 6. All I could tell from the technical, sciencey language was that I did have several abnormalities, but not the ones they might have expected to find. But on July 21 we had my annual visit to the transplant clinic, and the doctors there evaluated the report.
You have to understand that my condition was fairly recently identified as a distinct condition: it was originally called Membranoproliferative Glomerulonephritis, Type II, and that's the name my first nephrologist gave to me when I was diagnosed. But as they learned more about the disease, they decided that membranoproliferative was an inaccurate term, so the modern trend has been to call it Dense Deposit Disease. Now they tell me that further research has led them to classify some DDD patients into an even more specific condition called C3 Glomerulonephritis (the C is for Complement, which is an element of the immune system). I'm not sure if it's more a sister or a daughter to DDD.
I'm not exactly sure how it works, but essentially, the "Alternative Complement Pathway" of the immune system is activated by some perceived threat (yeast, bacteria... kidneys?), and a kind of chain reaction happens that leads to the creation of something called TCC/MAC (Terminal Complement Complex or, more descriptively, Membrane Attack Complex) that targets and destroys the threatening tissue -- in my case, the wrong tissue. I think it essentially pokes a hole in the cell membrane and blows it up.
Not much research has been done on it. In addition to being new, it's super-rare. When it was all DDD, they said that 2 or 3 people per million get it, worldwide. If you split that group into DDD and C3GN, you could say that I am literally one in a million! If I had to have a chronic, life-threatening illness, at least I got an interesting one.
Places with a population of around one million:
Oklahoma City, Oklahoma
El Paso, Texas
New Orleans, Louisiana
Salt Lake City Metro Area, Utah
There's your daily trivia.
But, applying 2.5/1,000,000 evenly across the world population, it means that in the world's top countries in medical innovation there would be approximately:
750 cases (people) in the U.S.
160 in the U.K.
200 in Germany
300 in Japan
160 in France
85 in Canada
35 in the Netherlands
150 in Italy
and so on.
That's a lot of people in a room, but not in a nation. It's hard to get a lot of research funded for a disease that affects such an infinitesimal part of the population; sponsors want to invest in fields with the biggest potential impact. Plus, studies themselves are limited by the number of potential subjects. Limited funding + limited sample size = not much research and even fewer conclusions. Thus, practically any treatment is experimental.
They have a new drug that basically interrupts the Alternative Complement Pathway before it creates the MAC. They've had some success with it in holding off the progress of the disease. The doctor I spoke to suggested that I may need to take it after transplant for the rest of my life. It's very expensive, and there is that experimental element, so the insurance will fuss.
Summing up, the DNA tests seem to indicate that I have C3GN, and it's practically certain to attack the new kidney, and they don't have a sure way of stopping or curing it. So, the verdict was:
No live donors.
I'm at peace about it. Of course, it is a bit of a disappointment, but it's also sort of a relief. It would be awful if, say, my brother gave me a kidney, and it only lasted a couple years or, heaven forbid, my body rejected it. So, I just have to wait on the list for a cadaveric kidney. I'm so grateful to all the family members, friends, and even some friends of friends who offered to be evaluated as donors for me: it's a touching demonstration of love, and even though it won't come to pass, I count the intention for the deed.
It is also a relief to finally have a definite path before me after having been in limbo for a year. I have to get an echocardiogram and dental clearance, and I also have to get my blood tested to see if the transfusion introduced any new antigens (I think) to my system, because that would narrow the field of potential donor matches. That should, I hope, all be done within 6 weeks, and then I'll be put on "active" status on the waiting list, with credit for having been waiting since May 2014.
The doctor said that, based on my age/life expectancy and blood type, he'd expect a 2 1/2 to 3 year wait (total). Giving that estimate some cushion on either side, it would be some time between Summer 2016 and Autumn 2017. Ish. There's a man living near us who had his third kidney transplant last year, and if I remember correctly, they got a phone call basically saying, "We've got a kidney for you if you can get to Salt Lake within 3 hours." So they just dropped everything and went. I'm thinking I'll make a "transplant bag" with the essential stuff already packed as much as possible and a list of what else to throw in, so when the call comes, I'll be able to leave immediately.
When I was in Driver's Ed watching the "Be a Donor" presentation, I would never have imagined myself in this position -- certainly not within 15 years. It's a strange kind of anticipation, waiting for a match: on the one hand, it's a relief to think that I'm not putting someone else’s life at risk by taking their kidney, with no certainty of its lasting long enough to justify the sacrifice; on the other hand, I am instead going to benefit by some person's sudden death.
I don't hope for someone to die to give me a kidney; I can only hope and pray that those people who must die will have chosen to be organ donors.