I was diagnosed with PCKD about 35 years ago. My father had it, and eager to prove the 50% inheritance rate to be true, my brother and I were both tested. And I won, in that perverse way that doctors say "tested positive" for something that's actually kind of negative.
For most people, involvement with the high-octane end of the medical system comes after something relatively sudden. They up 'n have a heart attack, for example, or catch a bit of cancer. Semi-regular visits to GPs are suddenly replaced by specialists, sophisticated tests, and machines that go "ping".
My journey began a lot less suddenly, starting way back when I was diagnosed in my late teens. Polycystic Kidney Disease is a degenerative condition where relatively harmless cysts begin growing and multiplying in the kidneys, like plums in a pudding, eventually muscling out the healthy nephron cells innocently trying to do their job.
But in the decades it takes to get really bad, it's as if nothing at all is wrong. In the early years it was merely a fun fact about me, something to drop into conversation, or tick on a form. No symptoms, no adjustment to lifestyle required.
When I worked in Japan in the 90s, I let my new employers in on the secret so they could renew one of my prescriptions. It hadn't even dawned on me that I should tell them about it before they moved me over there at great expense. Being Japanese, of course, they immediately ordered up a battery of tests and scans, only to inform me that I was just fine right now. I could tell that made them feel better.
Living with an impending disease is an odd state of mind. On a day-to-day basis it's kind of like having your doctor tell you the results of your tests are in … and it turns out you're Hungarian. "Don't worry, no one will know, it makes no difference, you're free to go skiing." You really do put it out of your mind.
Oddly, the only people to ever really treat me any differently have been insurance underwriters. Each and every one of them has listened patiently to my doctors' assurances that it was unlikely to affect my longevity or quality of life, and responded with a cheerful "Yeah OK … but no thanks." It's always kind of made me wonder if they got a memo my doctors didn't.
And then somewhat suddenly, after years of impressing party guests with "Hey, cool thing about me – someday I'm going to need a kidney transplant but I've known forever and I'm cool with it …" that someday becomes … someday soon!
There is an unmistakable moment where you realize it's gone from being years away to not years away. The car crash you were told about is happening.
And it turns out that in 35 years they did not find a cure, they did not learn how to make pig kidneys a better genetic match, and despite all the cool articles on the internet, they have yet to 3D print them. It's time to actually have that conversation with potential donors who always casually said "Hey, you can have one of mine!" Some of them quietly forget they ever said anything. Some get tested for suitability and flunk out. If you're lucky someone embraces the challenge, the rigmarole and the uncertainty, and follows through on the promise. I am, for the record, lucky that way. And unimaginably grateful.
In the early years it was merely a fun fact about me, something to drop into conversation, or tick on a form. No symptoms, no adjustment to lifestyle required.
More and more of the specialist doctors and nurses I meet are like fighter pilots and jet engine mechanics – totally focused (and sharp and current) on the precise thing they do, because they do it every day. I show up to discuss my potential kidney transplant for the very first time, and they've already knocked out two of them before lunch.
There's an odd dynamic in each meeting with a new health care professional where each of us suss-out how much the other one actually knows. My GP has a tendency to Google things while I'm in her office – which is both reassuring and disheartening. Good to know she'd rather look it up than fake it, but it dispels once and for all that illusion that every physician has the encyclopedic knowledge of Dr. House and snappy delivery of an Aaron Sorkin character.
You can tell that doctors are always trying to benchmark the level at which we can carry on our discussions. As soon as they realize that I actually know what my eGFR is, for example, the tone of the conversation changes. Dropping in a few correctly-used technical terms elevates the discourse from "all the bumpy-lumps on your squishy bits" to something that feels a bit more honest and informative. The same works in aviation, by the way. Call up air traffic control with a crisp Chuck Yeager jargon-laced delivery and they'll squeeze your landing in between airliners. Sound the slightest bit confused and they'll send you around the long way.
Ultrasound technicians are my personal favourite. About 30 seconds in, as they struggle to conceal the "Whoa! What the hell are all those?" expression that has invariably crept onto their faces, I'll usually point out that I know full well why I'm here, and name drop some medical terms so they feel better talking to me about what they're seeing.
To beat my own analogy nearly to death, this phase of my story is more like a holding pattern. Numbers that were previously "getting worse but still fine" are now no longer fine. Creatinine and potassium are high, iron is low and diet is suddenly a concern. I'm still normal on the outside but now the thing that has never had any effect on my day-to-day life actually does.
A low-potassium diet is, for the record, remarkably frustrating, inasmuch as potassium is distributed randomly across every food group in a way that makes remembering what to eat and what to avoid nearly impossible. It's high in oranges, but not limes. It's high in potatoes, but not rice. It's high in Guinness, but not Scotch. Oh, but root beer is fine.
I have literally taken to carrying around a detailed chart of things that are high and low in potassium because it's impossible to memorize. Whole grains, for example, are in the "No" column, while rice and corn are OK. That means – I kid you not – I have a printed sheet from a Registered Dietician telling me to AVOID Raisin Bran, Shredded Wheat and granola and choose instead Froot Loops, Lucky Charms and Trix. There is no better feeling than looking across the breakfast table as the kids plough dutifully through their muesli, pouring myself a man-size bowl of Cap'n Crunch, and saying "Sorry kids, Doctor's orders!"
Cooking for vegetarians, when you think about it, is quite easy. Anything but meat. Even gluten-free people (I don't mean people with actual celiac disease) are easy to cook for by comparison. Just leave out the wheat. (Or frankly, you can just bluff, and as long as you say "Don't worry, it's gluten-free!" the Placebo Effect will continue to delude them into feeling fantastic!) But when friends invite their low-potassium pal to dinner and ask "What can you eat?" the answer involves a tedious discussion about every single ingredient they were considering – a process that reduces both potassium intake and future dinner invitations.
All of which leaves me in the curious position of wishing I was just a tiny bit sicker so we could get on with this already. Until then it all seems rather manageable in that "First World problem" kind of way.
By comparison, I can tell from the questionnaires and forms I fill out that most people in my condition are also quite sick in a more tangible sense. People assume I have mobility issues. They expect that I'm diabetic or have a heart condition. They ask if I can get myself to my appointments on my own or use the washroom without assistance. But even now, I'm not actually that sick, I just have kidney disease.
Basically I wake up each day feeling fine, my kidneys having slowly caught up on their duties overnight. I then proceed to poison myself slowly over the course of the day, but not so much that we really have to do anything about it.
That leaves me with little else to do but wait for my symptoms to get worse, and wonder about my transplant potential.
Which brings me to my soapbox. The waiting list for a cadaver kidney in Ontario is around 10 years, which is appalling. It's appalling partly because organ donation is one of the few remaining aspects of human health care that we're still nervous or self-conscious about discussing. In the last few decades we've grown comfortable – even quite cheeky – encouraging women to check their breasts or telling men to check their prostates and their balls. But we still can't get behind making organ donation an opt-out rather than an opt-in. We send perfectly good organs to the crematorium every day because, by and large, no one feels it's their place to advocate for donation with the family of a recently deceased loved one. No one wants to sprint up to Mom and Dad after young Jason impaled himself making an awesome YouTube FAIL video and say "Hey, would you mind if we got to work on all the good bits he left intact?"
Policy needs to change too. While 85% of people are said to be in favour of donation, only a fraction have filled out their donor cards, and even a completed card can be over-ridden by distressed next of kin, meaning even that percentage isn't guaranteed. Because Canada is virtually the only developed nation without a coordinated organ donation strategy, wait times are vastly different depending on your postal code, Ontario's are the worst.
Unfortunately, no politician wants to be known as the guy who campaigned in favour of divvying up the recently deceased, so reform shows little sign of gaining much traction.
That's too bad because transplant people are awesome now, medications are improving every day, and the quality of life for most transplant recipients is much like mine has been for the 35 years leading up to it – pretty much normal, but with an asterisk.
For the time being then, I'll just keep circling the airport.
But hey…If you're Type O, I currently have an opening for a new best friend!