My Two Gallon Habit

About four weeks ago I had an appointment with my nephrologist. Love her. She's been a great advocate for me as my PKD progresses, going out of her way to do things like prescribe Tolvaptan / Jynarque (I'm her first patient on the medication) and doggedly pursue a radiologist to get a total kidney volume (TKV) calculated from a recent CT scan. One of the things we discussed briefly was my report of fatigue. She suggested a TSH (thyroid) test to which I shared that I had recently been tested and my levels were within the acceptable range. Somehow that's where the conversation stopped and we continued onto another topic. A couple of weeks later as my fatigue continued, I reached out to her and asked if we could add iron and Vitamin D tests to my next lab appointment (I go in every four weeks to monitor liver function while I'm on Tolvaptan). Yay! She said yes. Fast forward to today. I got my labs done - and got the results back. My interpretation of the resul...

The other day I stumbled upon a webinar that Risa Simons put together for the PKD Foundation back in 2013. She spoke about taking an active role in managing the disease and not waiting around for things to get so bad that the only action was reaction. I can relate. I'm on a journey to learn as much as I can about this disease that has seemingly taken over my family - maternal grandmother, maternal uncle, mother, me and (I believe) both of my brothers.  Yeah my mom stacked the odds with all three of kids getting PKD (a 1.25% conditional probability for all my stats-minded friends out there). We're still waiting to see how our cousins fared in the genetic pool. Fingers crossed that our deck was stacked and that they'll emerge unscathed. But as much as I'm focusing on the education end of things, I'm not taking much action on the physical wellness end of things. Don't get me wrong, I'm active. But I'm also carrying around extra weight. And every time ...

While I'm sure that Miromatrix would shudder at my description, it looks like they're developing the equivalent of a test tube kidney which could be used for transplant. While the science and bioengineering behind it is extremely complex, this is how I understand it. Miromatrix uses decellularization and recellularization technology to create a transplantable kidney. Huh? They take an dead organ - it doesn't even need to be human - and using detergents (I'm guessing they're stronger than Dawn ;-) ) they strip away all the cells. What remains is the kidney's extracellular matrix (collagen, enzymes and glycoproteins) and all its chemical cues. Then the "generic" organ is repopulated with the transplant candidate's cells, eliminating the risk it will be rejected upon implantation. Early studies have shown that it takes from four to eight days to culture the transplantable kidney and, even better, the new organ produces urine when tested. B...

And it's time. Time to take the next step. I'm graduating from training wheels to the big girl bike with the banana seat and the sparkly streamers on the handlebard (Yes - I'm dating myself as a child of the 70s). My dose of Jynarque/Tolvaptan is going up from 45/15mg to 60/30mg. I have a couple of days to prepare. Although I'm not quite sure what there is to prepare FOR. Some people say that thirst will increase EXPONENTIALLY at the higher dosage. Others say that they see no difference. Given my relatively easy experience with the medication, I'm hoping to be in the latter camp. Regardless, I start the new meds on Monday which is Memorial Day. And I have the entire week off as vacation. So bring on the increased thirst! Bring on the extra bathroom breaks. I have time.

For all the medical issues I have, for all the uncertainty that exists in my future, for all the modifications I need to make to my lifestyle... I feel honestly and truly blessed. Yes. I have an incurable disease that can possibly lead me down a path towards  dialysis, organ transplant, lifelong immunosuppressive drugs and early death. But... I don't have diabetes. I don't have a history of aneurysms in my family. My blood pressure is well-managed. My risk for cardiovascular events is low. I work for a company with good healthcare. My job allows me the flexibility to go to doctor appointments and labs.  I have friends and family who love and support me. Could be much, much worse.

Oh what a glorious weekend it was. I had a plan. Things I wanted to get done. Strenuous outdoor work. And I did it! I cleared the burn pile and turned it into an attractive rustic fire pit. I leveled uneven ground. Cleared brush. Cleaned up flower beds. Walked my dogs. Cooked. Cleaned. And then hit a wall. A wall called Monday. Ugh! Even though I paced myself, I know now that I pushed myself too much. Instead of winding down, I kept working on projects and housework until 11pm. I should have put myself to bed at 8pm after a hot shower. So even though I thought I understood the 'slow down and listen to your body' mantra, I proved to myself that I still have much to learn. About myself. About my limitations. About this disease. About my new normal. And now - at 8pm - I do what I should have done last night. I go to bed.

One of the worst things about living with a chronic illness - ADPKD in my case - is that you wake up one morning and find that suddenly you have limitations. What? I'm used to powering through things. My dogged determination is my signature! Sadly, not any more. I've noticed that I'm tired more often. And when I'm working on things - like gardening - that I start out strong but quickly run out of steam. Take, for instance, my fire pit. Last year I thought it would be a great idea if I created a landscaped fire pit at my house. I had the perfect place for it and I thought that it would add a great focal point to my backyard. I started digging up the area (yes, by hand. I never said I was particularly smart ;-) ) and barely made a dent. Then winter came and Wisconsin was buried in snow. Now in May the snow has finally melted (but check back with me next week - it may be back) and I started thinking about that fire pit again. Hand digging didn't seem to be ve...

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...

Last weekend I attempted to hold on to my old normal. I have three Rhodesian Ridgebacks and one of the things that I enjoy is watching them run (and compete) in a dog sport called lure coursing. The event was about three and a half hours away in Iowa. So I packed up the camper, aka the most expensive dog kennel you'll ever purchase, and we made our way south. Uneventfully so. I set up camp, got the pups walked, fed, settled and then allowed myself to collapse. What a lot of work. But I slept well and woke up refreshed and ready for the day. I tried to be prepared. I brought four gallons of drinking water, along with a water dispenser pump. I had a 10 pound bag of ice to keep my drinking water COLD. I had every intention to stay well hydrated. I thought I was. But when you're outside all day in the sun and wind sometimes it's difficult to tell. Epic fail. By the end of the next day I was so tired, drained and dehydrated that I wanted to cry (which was crazy sin...

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 (...

Energy. What's that? During my last nephology appointment, I mentioned that my stamina was declining and that I'm, in a word, TIRED! My doctor's first thought was to check TSH (thyroid-stimulating hormone) levels. Excellent idea. Unfortunately, my levels are medicated, controlled, recently tested and within the 'normal' range. Oops. To be fair, my energy levels are reminiscent of the months prior to being diagnosed with hypothyroidism - not quite as severe, but still not good. On days I work from home - I'm a data analyst/programmer - I frequently have to either take a nap over my lunch break or crash once I'm done for the day. On days I go into the office, I often myself in bed by 8pm (and sometimes earlier). Sigh. This is no way to live life. I've asked about this in the  Polycystic Cysterhood Facebook group  and others agree that they've seen their stamina and energy flag as kidney function has declined. At least I'm not alone. U...

Now how's THAT for a t-shirt?!? I want one. I'm not familiar with the company that supplies it but I believe it's one of those 'if you design it, we will create it and they will buy it' places. The Spreadshirt site offers the shirt for about $20 plus shipping and allows you to select size, shirt color and (I believe) the kidney color. The designer, Emblem Design, has other kidney designs as well. A purchase may be in my near future.

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 ...

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...

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.