(A summary Randy composed for people who are just catching up with the blog. He put it together sitting at Holly's bedside after the November 11 nephrectomy.)
May 24 -- changed life arc
I had entirely expected that my PKD would proceed much as my father’s had. That is not show symptoms until my fifties, but it was not to be. I'm in my early forties so have a long career of immunosuppression ahead of me.
I'm so glad so much has changed in transplantation over the last 27 years. But who knows? The next 5-10 may bring us the DNA-matched kidney or the marker-washed pig kidney! no more immunosuppression!
August 2009 -- an intermittent struggle but going forward
It was an uphill battle to get the U surgeons to agree to take my native kidneys out. Much push back and such specious logic as "You don't get lower back pain with enlarged kidneys." (Oh really?)
I happened to be having an awful day with pain (it comes and goes for a few days at a time, then a week or 2 off) when I was in for an appointment and a doctor walked in to see me with my leg and knee flexed up on the exam table while I cried in pain. . . that finally made an impression.
We still a second opinion from an orthopedist to make sure I didn't have classic back pain. Bless him, he pulled the MRI up and saw it at the same time I did. In a cross-section at my navel, you could see my spine, and then nothing but kidneys right out to the borders of the frame. "I'm not a kidney doctor, but even I can tell your pain is probably coming from these (he paused to try to phrase it more elegantly, then gave up the fight) gigantic kidneys."
People are interested in what my experience might has been like at various point as the disease progressed -- well, at 40 percent, I was pretty much as active as ever, though perhaps not as energetic as I'd been in my 20s. What really took the toll was the dread of what would eventually, assuredly happen to me. (I fully expected to go downhill on dialysis until a cadaver kidney became available.) At under 30 percent function, I had a huge wakeup call when my blood pressure meds stopped controlling the pressure and it went right through the roof. I couldn't think clearly or concentrate on anything. That's when I found out just how bad off I was -- a day before my 40th birthday.
The plan is to have an "open" nephrectomy in which they open me up from the breastbone down past my navel -- like a big old-fashioned C-section. [Ironic since the size of the Kidneys gives people the impression Holly and other PKD women are pregnant. rss]
The plan also includes taking out a bunch of "while we're in theres" such as my gallbladder, my appendix and perhaps my spleen if the left kidney is too far involved with it. I rather dread the whole thing, but the amount of pain I've had even since the transplant tells me there's no question I need it. [There was no need to remove the spleen. rss]
May 26
I don't think of wanting this nephrectomy as vanity. [Women with PKD often are mistaken for being pregnant and individuals who make this mistake create embarrassment for themselves and annoy the woman they are talking to. rss] I think these two things that are each 12 inches long by 6-7 inches in diameter must weigh QUITE a bit. Having all that on my back, knees, and feet for the rest of my life is more than I'm up for.
Isn't it strange how PKD takes different families and individuals? A family close to us has it but it most often affects when they're elderly. The sister of the family member we know best has it, though, and has far more problems with liver cysts than kidney cysts.
It's funny how -- well, ignorant -- even health professionals are about PKD. A doctor of my acquaintance had a nurse for years who'd worked for a doctor whose daughter had given him a kidney. We'd go over my records every time I came in and establish that I had PKD and I inherited it from my father. And then she'd ask, "Did you ever consider donating a kidney to your father?" I always just said "no" and let her think I was some kind of slacker instead of getting into "Well, my kidney wouldn't have lasted him as long as the one he's got, and losing one would cut my pre-transplant lifetime in half."
I was very relieved when she retired and we didn't have to go through this routine anymore.
July 23
Just came across another new twist:
I was curious about GFR (the measure of the flow rate/function of kidneys) after I got Cheryl's. Judging that my own kidneys were down under 12 percent or so, adding Cheryl's only increased GFR to 48 percent. I wondered at the time if her kidney, though doing a great job, was a little shocked by the experience.
I'm now up to about 54 percent function overall, and so asked my coordinator about this.
She said that a lone kidney left in a healthy person (a living donor) senses it's on its own and simply performs even more to handle the extra. Such a kidney might increase its GFR by as much as 20 percent at a year following the transplant of the other.
In my case, the new kidney will also adapt to handle more work, but likely will not hit the 20 percent extra due to being inhibited by immunosuppressants. (The usual immunosuppressant conundrum.)
So, at some future date, Cheryl and I could be getting 130 percent out of those kidneys!
August 10
You know, I haven't blogged for perhaps 2 months. It seems like I have so much to do -- and most the "exciting" stuff is over -- but people do ask. [It’s not exciting to you but we all think it’s pretty swell. rss]
Oct 12
Yeah-- I HAVE to get into that blog again. As one of my outstate friends noted, "You went back to work and poof! nothing!" Work is my life -- was here 16 hours yesterday -- and I have been concentrating on that and projects at home with all the energy I've got. Amazing what you can get done when you're not sleeping 14 hours a day. . .
Nov 8
Mine may not be the face that launched a thousand ships, but I have an immune system that's launched a research study!
My surgeon -- when she got back into town from that ski trip -- called to say that yes, we were a go on the transplant, and besides that, this episode had inspired the team to look into low-level DSA measurements and exactly how much they do count in long-term transplant survival.
"We got to talking about your situation, and it seems like time to look at it further," she said. "We agreed that someone should look at people with mild DSA reactions like yours and see how they did over the long term.
"And that means I get more work!" she said cheerfully. (As much as she loves doing surgery, and she has said just that in so many words, she's also apparently ready to slice into long-term tracking studies to ferret out this information.) [Holly has a post about this event here. rss]
