Wednesday, July 2, 2008

The Rest of It

First, a big wet sloppy kiss to all of you who read yesterday's post and, instead of rolling your eyes in disgust at my obvious play for your sympathy [g!], hopped on the PKD fundraising link and whipped out your credit cards. Talk about getting started with a bang! I'm all verklempt, and thank you from the bottom of my heart, as do Michael and the kids.

A number of you emailed me some very sensible questions about how I developed PKD and what my future holds, and I thought I'd answer them here. (Knowledge is power, baby.)

The form of PKD that I have is genetic and affects one in 500 people. My father was the first in our family tree to develop it (which is a bit of a mystery), and my brothers and I each had a 50% chance of having the disease as well. Luckily, I am the only one of my siblings who has tested positive.

I was diagnosed in my early 20s but did not become symptomatic until a few years ago, when my blood pressure started to climb. There is no way to know how quickly the disease will now progress. I visit my nephrologist every 8 months or so for new blood work, and I keep an eye on my blood pressure from home. Aside from controlling the blood pressure, staying generally healthy and eating a low-protein diet, there is not a lot I can do at this point to keep a rein on things.

When my kidney function decreases to around 20% (maybe 3 years? maybe 5 years?), my older brother -- who is my only family member who has a compatible blood-type -- will go through a battery of tests to determine whether he would be a good prospect to donate me a kidney. The best case scenario is that he passes the screening, I get one of his kidneys and the transplant works (many don't). I'll then spend the rest of my life on immunosuppressants and hoping that my new kidney won't fail (many do). My father's first kidney transplant failed after 20 years, which is a good outcome -- many only last 10 years (or less).

If my brother is not deemed a good match for me, or does not pass the rigorous health screenings, another option is paired donation. This is a relatively new arena, where kidney patients who have willing donors with incompatible blood types "swap" donors to get a better match. Plenty of unknowns, here, but a good potential fall-back position. This would enable my younger brother to get into the act, as well.

If scenarios 1 and 2 fail, the next step is to go on The List. The statistics of The List are pretty horrible. There are currently 76,000 people in the U.S. waiting for kidneys, and fewer than 10,000 of them receive a kidney each year. The List is growing by roughly 40,000 patients each year, and 4,000 die each year before receiving a transplant. Pretty crappy odds.

Dialysis is a life-saving procedure that cleans the blood in much the way the kidneys do. There are several types of dialysis currently available, each with particular drawbacks. None is a cure, and most people feel very sick while on dialysis. Being on dialysis is very restrictive, and is a way to borrow time, not a way to live out one's life. If my first two transplant scenarios fail, I will certainly have to go on dialysis for some period of time, which I dread.

With regard to the liver issues, as long as my liver function doesn't fail (it doesn't, in most PKD patients) and as long as it doesn't get too grossly enlarged, we will likely just leave it alone. (Alas, I fear my bikini days are numbered.) In some instances the docs will aspirate individual cysts if they are very large or infected, but even going in laprascopically creates some scar tissue and is best avoided. I do know some PKD patients who have had liver resections to remove the most grossly affected portion of the liver, but that's only done in extreme cases.

I am one of the lucky ones -- I have private health insurance that will cover the costs of dialysis and/or transplantation, when the time comes. I have group life insurance, disability insurance, and all kinds of financial contingency planning in place to help cover the mortgage when/if I get too sick to work. I have family members willing to donate me a kidney (you'd be surprised at how often that's not the case). I have a husband who loves me for better and for worse. And I have an incredibly supportive network of friends who cheer me on in all of my hairbrained schemes, and yell at me when I'm taking on too much.

It is hard to feel anything other than blessed.


Anonymous said...

Hemodialysis is hell, but peritoneal, which I was on for two months before my darling husband gave me his kidney, isn't that bad. It's like getting to actually LIVE. Doing exchanges is so much easier than being hooked up to a machine for four hours three times a week and easier on your system. I've done exchanges in a mall parking lot while Christmas shopping, in a car traveling down the highway (after getting hooked up while parked. Hubby drove!), and in various family members homes while visiting. Anyone with the same blood type can be a donor, it doesn't have to be a relative.

RuthWells said...

Nancy, thank you so much for commenting. I'd love to hear more about your experience. I thought that with peritoneal, the exchange had to be done in a sterile environment -- am I mistaken? My email is rwellsfischer at hotmail dot com.

Unknown said...

Hi Ruth! you are certainly right. Peritoneal dialysis should be conducted in a clean, sterile, dirt and dust free environment. Even pets such as dogs and cats are highly discouraged as their hairs could contaminate the dialysis system and cause serious infection.