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And the call finally came

Fri Mar 29 2019 - It finally happened. I received a call from the specialty pharmacy. They received my prescription. They received all necessary paperwork. They received insurance approval. They were ready to send. Was I ready to receive?

YES!!!

A little background. I have Polycystic Kidney Disease - Autosomal Dominant ('ADPKD') to be precise. 'What the heck is that?!?' you may ask. A most excellent question...
ADPKD is the most common hereditary kidney disease affecting over 12 million people worldwide. It's estimated that between 1 in every 400 to 2,500 people has the disease. It can affect women and men, across all ethnic groups. (pkdinternational.org)
An equal opportunity, inherited disease. Gee. Aren't we lucky?
Healthy kidneys are about 10–12 cm long and are found either side of the mid back. They’re filled with about one million special tubes for filtering the blood. These are called nephrons; they filter out waste products and excess water (making urine).

In people with ADPKD, the lining of individual nephrons can balloon out, causing fluid filled cysts to form. Over time, more and more cysts appear and increase in size, leaving fewer healthy nephrons and space to filter the blood. People with severe ADPKD can have kidneys as large as a football. (pkdinternational.org)
Everyone with the ADPKD gene has a 50% chance of passing this mutation along to each of their children. My family hit the lottery. My grandmother died from PKD (at the time, doctors didn't really know what was wrong with her). The ADPKD gene was passed along to both my mother and uncle (two of two children). Both have had transplants (very blessed). And of my mother's three children, we're three for three. We're still waiting to see how our cousins fared in the lottery.

There is no cure for PKD. None. Nada. As for prevention, all you can do is eat right, exercise, control your blood pressure and wait for things to progress to End Stage Renal Disease ('ESRD'). At that point your choices are dialysis, possible transplant and/or letting nature run its course. Pretty dismal-sounding but don't give up hope!

A year ago, the FDA approved a drug that has been proven to slow the growth of the cysts which will delay the progression to ESRD. Now we finally have hope! And THIS is what my Friday call was all about.

On Monday, a Fed Ex package will arrive at my home with a drug called Tolvaptan aka Jynarque aka Samsca (I swear the people who name these drugs must be altered at the time).

Let the games begin!

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Round 2 deja vu

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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. :-(
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Words to live by

I never knew - couldn't even fathom - how tough it is to live with a chronic disease. Sure I've been hypothyroid for YEARS but it never really felt serious. I take a little pill once a day. There are no side effects. I get my energy back. Good and good. Polycystic Kidney Disease is nothing like that. There is no cure - save transplant. But even then you take anti-rejection drugs for life. If you forget your meds, you risk transplant rejection. How's that for adding a little anxiety to your anxiety? There is a single drug that SLOWS DOWN the disease's progression, but that promise of dialysis and/or transplant is always right over the horizon. The side effects of that drug are constant thirst and the knowledge of every public rest room within a hundred miles. And while you wait for the disease to progress, you get to experience soul draining fatigue, anxiety, physical discomfort as your kidneys expand to fill your abdominal cavity, the look of a pregnant belly (...
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It's getting real...

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What a night!

Tues Apr 2 2019 - Oh dear God! What a night! That plant? Yeah. Could have been me. Not taking my second dose until 530pm? Never going to happen again. Wow. Just wow. I woke up every 90 minutes. I went to the bathroom every 90 minutes. I GUZZLED water every 90 minutes. I felt like I was traversing a desert and hadn't had a drink in days. I drank three liters (about 3/4 of a gallon) of water DURING THE NIGHT! Who does that?!? So here are my learnings from my first day & night on Tolvaptan: Don't take the meds late - make sure I get that first dose in at 6am and that second dose in at 2pm, Start building up that bladder durability during the day. Act like it's strength training. Feel the urge and delay going as long as is feasible (accidents are not an option). Hopefully this will allow me to sleep longer than 90 minutes. If I'm going to drink that much water, I'm prepping it before going to bed and having it bed side. Last night I was running up...
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