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My PKD story

This entry was posted on Wednesday, March 11th, 2015 by Frances Ryan.
Tags: pkd, health, fear, faith, confession

As part of World Kidney Day and National Kidney Month, the PKD Foundation has asked people to share their PKD story. This is mine.

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.

You can learn more about PKD from the USA’s PKD Foundation or the UK’s PKD Charity.

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.

Comments

Email me if you want to know anything about PKD, I’ve lived my life and it’s not as bad as it seems.  Been on PD, refuse to go on Hemo Dialysis, had 2 Kidney transplants , first one live donor rejected (Brother in law) second one extended Kidney cadavar.  Anyhow if need any advise I’m willing to share.

by Shari at 8:10pm (GMT) on August 23rd, 2017

hi there. i was in tears reading your story as it is very close to home for me.my daughter was digonsed with a very rare HPV virus at the age of 3 . has had 46 operations up to the age of 10 . now was dioganised with PKD at the age of 19 , with kidneys and liver full of it . it hurts so bad to watch your beautiful daughter go through so much and not being able to fix her in anyway.Emily is almost due to have her first miracle baby boy and i cant wait . i look every day to find a cure for her , but im still to find nothing . Emily will most likely go onto diaolis at the age of 30 plus . i blame myself everyday for all the sickness that she has had to go through , although i havnt been told that i have it as im to scared to find out. i hope every thing goes well for you . please email anything back

by donna hale at 2:12am (GMT) on February 25th, 2018

Hi, Donna. Thank you for your note.

I am so sorry to hear about your daughter and her medical struggles. I am sure it must be hard for you to watch her go through this, but I hope you find a way to be gentle with yourself.

PKD is a life-long disease, but it doesn’t have to be a life-changing one! Most people manage to live a full and healthy life despite it (if you can consider having kidney disease healthy!). Even those who’ve gone through dialysis and transplant can live fairly normal lives. (I have many family members who’ve had transplants and they just keep going and going and going… including my 70+-year-old mum!)

There is no cure for PKD at this time and the likelihood is that there won’t be a cure for a very long time. However, there are some potential treatments that can slow the progression of the disease. For example, the drug tolvaptan. It is being used quite widely in the UK now and is starting to be used in other countries, too. It has not yet been approved in America, but it is hoped that it will be soon. It might be worth your daughter asking about that and other potential treatments when she visits her doctor next.

In the meantime, keep reading up on the disease as much as you can. And don’t beat yourself up about it. You don’t need that kind of stress, and you can’t support your daughter if you are overly stressed!

All my best to you,
Frances

by Just Frances at 1:08pm (GMT) on February 27th, 2018

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