What It's Like

Ironically, while it's the cysts in my kidneys that are going to cause me the worst problems down the road, the cysts in my liver are what cause me the most ongoing discomfort. (One of the quirks of polycystic kidney disease is that it can cause large cysts not only in the kidneys, but in the liver and pancreas as well.) It is rare in PKD for liver function to be impeded by the cysts (though it can happen); unlike the kidneys, the human liver has amazing powers of regeneration and is therefore less vulnerable to the formation of scar tissue that will eventually destroy all my kidney function.

The cysts in my kidneys give me pain occasionally. They twinge, or pinch, or throb for a few moments, or longer, and then stop. By contrast, I am always aware of the cysts in my liver. They cause a distension of my abdomen and my liver presses against my bladder and lungs, giving me indigestion and often making me short of breath. When my liver cysts pinch me, or burst, it feels like a stitch in my side after a hard run and the stitch won't go away. I have dreams of being pregnant, with vivid phantom muscle memories of the little baby heels and elbows kicking and pushing from inside my abdomen, and upon waking, it takes long moments for me to realize that what's growing inside me is not a baby.

I don't know how big my kidneys and liver are right now. My last ultrasound was over a year ago, at which point both kidneys measured at about 17 cm (normal size for me would be around 10 cm). At that time, the ultrasound tech measured several individual cysts of 6 cm and 7 cm -- each about the size of a lemon. Everything has surely grown since then.

Size isn't really the issue, though. As the cysts in my kidneys grow, they leave scar tissue in their wake, which means there is less and less healthy kidney tissue remaining to do the important work of cleansing the toxins from my blood. More toxins in the blood means more fatigue and less energy -- both physically and mentally.

As of my last blood work, my kidneys are doing about 40% of the job that they would be doing without the cysts and the scar tissue. This isn't too awful -- most people don't have to start on dialysis until they reach about 20%. The question is (has always been), how long do I have before I hit 20%? There is no way to know. Some PKD patients chug along at 40% for a decade or more and then quickly bottom out; some PKD patients follow a slow curve downward through increasing fatigue, nausea, and pain. It remains to be seen.

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Meanwhile, there is work to be done. Public funding for a cure for PKD is an embarrasment. The NIH's February 2008 report shows that annual research spending per patient is $60. That's right, about the cost of an Olive Garden dinner for four. Compare that with the $2,733 spent on research per cystic fibrosis patient or the $1,880 spent per muscular dystrophy patient and you'll see why there is huge work to be done.

The Polycystic Kidney Foundation is an amazing organization which uses over 80% of its budget to directly fund research and provide patient education regarding PKD. They are the only group in the U.S. dedicated solely to eradicating PKD, and last year they funded $4 million in research -- a full 9% of what the NIH spent on PKD in 2007.

Last year, with the help of many wonderful friends and family members, Michael, the kids and I raised $9,000 for the PKD Foundation. (You can read all about it here.) This year, I'd like to make that number an even $10,000. I hope you'll join us. My fundraising page is up and ready for action (and will remain up on the sidebar to your right), and as always, I will post our progress here on the blog. It really will take a village to eradicate this disease, and I'm determined to do so before Quinlan's kidneys start giving him any trouble.

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