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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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It's getting real...

I looked at my phone messages on Monday night and was excited to find a call from the Mayo Clinic. Would they tell me that I had to wait for further deterioration before I could be evaluated for transplant? Or would they tell me to make the drive to Rochester to go through a battery of tests and meetings? It turned out to be the latter. Yay! They're going to evaluate me for transplant in April! Wait a minute. They're going to evaluate me for transplant... Crap. As they say in the movies (?), shit's getting real. I'm trying to stay positive, telling myself that this way I'll be able to get my name on the transplant list as soon as my eGFR hits 20. I'll be able to maximize my time on the list. But every so often, the positivity slips and the fear and anxiety appear. And the questions. All the questions... Will I be able to keep working? Will I be able to afford this? How will this affect my retirement? Will I be able to retire early? How will I pay...
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Teach us to fish

I've recently started trying to educate myself on current research and insights around PKD and all the diseases that seem to show up along with it.  While it's exciting to learn that we're on the verge of significant innovation in how CKD and PKD are diagnosed and treated, I find myself a bit disappointed. Instead of shouting the news from the roof tops... instead of spreading the excitement, the medical community hides the punchline. They share their findings in dry journal articles, steeped in language that is hard to understand. I realize the audience for the articles is not Joe or Jane Public. I realize that they're scholarly discussions of research methodology, statistics, inference and conclusion. I also realize that this is potentially a single data point that may, or may not, contradict current beliefs. Regardless, wouldn't it be nice if these studies included a simply worded summary of the work? Something easily consumable by the general pu...
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Truth

The need is real! I don't know if it's because prior to Tolvaptan I routinely used mind over matter to delay my trips to the restroom, but I think I'm doing okay these days. Is it possible that I built up bladder muscles over the years? Don't get me wrong, the need to pee is omnipresent. But it's one of those things where I can make note of it, finish what I'm doing (or finish a couple more things) and then make my way to the facilities without the fear of letting things fly. But I'm not stupid. You'd better believe I'm making the restroom my last stop before starting my 45 to 60 minute commute to/from work. A soggy crotch is NOT becoming.
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Expensive but affordable

There's no denying it. Tolvaptan - brand name Jynarque - is CRAZY expensive. The wholesale price is just north of $13,000... A MONTH! Dear God. I've searched and searched to find out how much pharmacies are beings charged for the drug, as well as my insurance company, but I've yet to find a source. Surely there has to be some kind of 'negotiated price' like you see with healthcare?!? My doctor suggested increasing my dosage - after I'd already received the next month's supply - and I about hyperventilated. There's no returning drugs and the thought of wasting $13,000 of pills left me queasy. So instead, we decided that I'd stay on the lower dose for another month and then increase the dose the next time. Deep breaths... Regardless, Otsuka (manufacturer of the drug) is working to make sure that the patients' out of pocket for the drug is manageable. Through their MyPASS program , patients with commercial insurance can acquire the pr...
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Round 2 deja vu

My doctor decided to keep me at the initial 45/15 mg dose for another months so I'm anticipating a similar water consumption. Here are the first four of seven (?) or eight (?) five gallon bottles of water. My local grocery store has a water bottle fill station for an extremely reasonable 39 cents a gallon. The frugal side of me thinks I should invest in reusable water bottles and take advantage of the savings. The lazy side of me thinks that it'd be a total hassle to wash the bottles, keep bugs and dust from falling into them before I'm able to refill them, remember to take them with me to the store (I can't even remember those reusable shopping bags) and then stockpile a source of bottle caps. It's just so much easier to buy these recyclable five gallon bottles. Lazy beats frugal.
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