First, some key bits of information about PKD:
- PKD stands for polycystic kidney disease, a genetic disorder characterised by the growth of numerous cysts in the kidneys.
- PKD is a progressive disease, meaning it worsens over time.
- Over time, the cysts grow and multiply, replacing normal healthy tissue and causing the kidneys to lose their function. Kidneys can enlarge to 3 to 4 times their normal size.
- Dialysis and/or kidney transplantation are treatments for kidney failure, but not a cure for PKD.
- There is no cure for PKD. But there are some very clever people working to find one.
But this story isn’t about the medical and scientific side of PKD, it’s about the human side. It’s about living with PKD and finding a balance between “being sick” and living a full and happy life. So, here goes!
My PKD story
I was diagnosed with polycystic kidney disease when I was five years old. At the time, I was the youngest person in American diagnosed with the disease. Because I was so young, and because (like many PKDers) several other family members had the disease*, it didn’t feel like a life-changing diagnosis. It was more of a confirmation that I, too, had it.
Growing up, I was very aware of PKD and I knew that, one day, I would likely be faced with kidney failure leading to dialysis and/or a transplant. But I wasn’t worried about it. It was just, well, life with PKD. I was also very aware that having PKD didn’t mean I wasn’t able to live a full and active life. I was an active child (and I have the scarred-up knees to prove it!) and I was a varsity cross-country runner. And I knew that there was very little preventing me from continuing on the active lifestyle. (However, my ITP diagnosis in the mid-1990s has had an impact on my activity levels.)
As an adult, I have had very few complications from my kidneys. I get occasional infections and ruptured cysts (once whilst running; ouch) but I don’t have the constant pain that some people have. And whilst my kidneys are enlarged, and I sometimes feel like my abdomen looks large because of them, I still have a fairly flat stomach and can wear form-fitting clothing without my kidneys showing.
Importantly, I have been able to maintain my blood pressure extremely well and my kidney function and other related tests are well within “normal person” range. In fact, I was told at my last appointment that my tests are better than many healthy people my age without kidney disease.
However, I am not a normal, healthy person. No, between my PKD and a second, unrelated chronic disease (idiopathic thrombocytopenia purpua, or ITP) I am actually one of those people classified as “infirm”.
And that’s where the “living with PKD” bit comes in.
You see, to maintain my health as best as I can—whilst slowing the progression of the disease and staving off as many infections as possible—I have made conscious efforts to alter my behaviours. I limit my caffeine intake to one cup of coffee (most days) and am mindful of my protein sources and intake. I also work to keep to a lower sodium diet and I limit my alcohol consumption. And, of course, I run and try to keep as active as possible—all in an effort to keep my blood pressure down and my overall physical health high.
My doctors have said that I don’t have to restrict myself as much as I do, and have said I can have more coffee and booze if I’d like. But I would rather limit them now when I don’t have to, then to have to cut back drastically when (if?) my kidneys start to give out.
All of that said, however, I am very lucky in that I haven’t had the troubles with my kidneys (they’re called Bob and Dave, by the way) that many people have had. Yes, compared to my sisters—one of whom has already had a transplant; another suffers from debilitating pain—I haven’t had any trouble at all. However, I know that PKD is a progressive disease and I acknowledge that my luck could run out at any time.
It’s that “luck running out” bit that scares me now. It never seemed as scary when I was married, and I had a supportive husband, but he died nearly six years ago so I now have to face my illness alone. And I do admit that I worry I will become very ill at some point and I won’t have anyone to care for me; I worry that I will end up as a burden on someone else because I don’t have a husband or children to look after me.
But those fears aside, I don’t really find PKD to be a scary thing. After all, I am full of hope that there will be great strides made towards a cure. And maybe that cure won’t help me, but maybe it will help the next generation of PKDers.
* My family’s history began with my maternal grandmother, who developed the disease through a genetic mutation. It was later learned that my Mum had inherited the disease, by which time she had six children—four of whom would later be diagnosed (only two are PKD-free). At least four nieces and nephews have PKD, as do some of my Mum’s siblings and their offspring. There have also been four transplants in the family: My mum, an uncle, an aunt, and my second-eldest sister.