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It's a family affair

Both of my brothers have ADPKD as well.

The odds of a PKD parent having a child with PKD are one in two. The odds of having two with PKD are one in four, or 25%. Three? a 12.5% chance. We "beat the odds" (i.e. the PKD is strong in this line).

Thankfully - for me - my PKD is less aggressive (although still not good). While I have a bit of a "PKD belly", it's not excessive. And instead of true pain, I only suffer from discomfort.

Not so for my little brother.

Despite being nine years younger, his kidneys are MUCH larger. You can actually see the outline of them on his belly.

And instead of discomfort, he experiences true pain.

So much so that he recently went in for surgery to have the size of some of his larger cysts reduced.

The surgery is called Percutaneous (through the skin) Sclerotherapy and is an outpatient procedure.

Larger cysts are identified through a CT scan or MRI, during which the doctor inserts a needle to aspirate (drain) and then sclerose (destroy) the inner lining (often by injecting alcohol) to prevent the cyst from filling with fluid once again.

I'm happy to report that, while sore, my brother says that the pain is down and that the procedure
was a good decision.

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Artificial kidney

I have a somewhat fanciful thought of what an artificial kidney would look like. I see a device that is shaped like a kidney (of course) but is completely clear so you could see all the blood circulating through it. Granted, once it's implanted no one's going to see it but - hey - it's my vision. The closest picture I can find of what's in my mind's eye is this image. Granted, that's a picture of a kidney-shaped paper weight and it's a little cloudy, but I know you can picture it too. My, oh my. My imagination is not even close to reality. According to a post from the American Journal of Kidney Diseases , there are three new approaches being explored right now: the automated wearable artificial kidney ('AWAK'), the wearable artificial kidney ('WAK') and the implantable artificial kidney ('IAK'). My money's on the IAK and you'll understand why once I show you the concept pictures of the other two. The AWAK is said to ...

One more for the books

I had the quarterly appointment with my nephrologist the other day - one of the "bonuses" of being on Jynarque is that they monitor, monitor, monitor the heck out of you. And I'm happy to say that all my levels are stable - eGFR (we've decided to call me early Stage 4 since I've been teetering between 3b and 4 for the past 5-7 months), liver enzymes, calcium, phosphates, glucose, potassium, sodium, Vitamin D... Oops! Then there's that nasty Intact Parathyroid test. TOTALLY different story... Not only is it higher than last year - and increase of 75% - but now I'm outside the normal range. Sound the alarms! But wait. What exactly is the parathyroid? What does it do? And is this normal for a Stage 4 PKD patient? The parathyroid glands (four of them) are about the size of a grain of rice - or a pea, depends on your source - and they are located on your thyroid. They release a hormone (PTH) that regulates the amount of calcium in your blood ...

Live in the moment

I'm a thinker, a what if-er. I analyze things to within an inch of their life. Live in the moment? What's that? Nothing is straight forward with PKD. The unpredictable nature makes it impossible to create solid plans. And the decisions you think you can make are often conflicting. Case in point, Tolvaptan. Taking it should slow down kidney decline, delaying my need for dialysis and/or transplant. Yet the older I am before I need a transplant, the higher my Expected Post Transplant Survival score is (a lower score means greater survival rate and, if 20 or less, a high quality kidney). But I'm really hoping to stave off dialysis/transplant until there's a viable implantable artificial kidney. See what I mean? I guess the bottom line is that I have to have faith and trust that whatever happens was meant to be. What are the words from the Serenity Prayer? God grant me the SERENITY to accept the thing I cannot change, the COURAGE to change the things I can and ...

And so it begins

At my last appointment, the nephrologist actually offered to give me a referral for transplant. And then a week later I got THIS in the mail from my insurance company. Crap! It's getting real now. If something like that doesn't take the wind out of your sails, I don't know what will. Granted I had let my doctor know a couple of months ago that I wanted to be screened and ready to go as soon as my eGFR hit 20. Did I think it would be this year? No. My estimates were three years from now. Yet the combination of the four point eGFR drop + the transplant referral leads me to believe that my DOCTOR believes I should hit 20 within the year. I don't think I'm going to be able to last until the artificial kidney comes out. :-(