My Two Gallon Habit

Move over Tolvaptan/Jynarque, there may be a new sheriff in town. On 05 June 2019 Texas-based Reata Pharmaceutical received orphan drug status from the FDA for Bardoxolone Methyl (aka Bardoxolone). So what does it mean if a drug is granted orphan status? It means that even though there are fewer than 200,000 people in the United States with the disease at any given time, the federal government has acknowledged the need for medical innovation to address the condition. Companies/drugs given this status receive certain development incentives such as tax credits for clinical testing, exemption from prescription drug user fees (used to fund new drug approval processes) and the ability to market the drug exclusively for seven years. I guess that takes the sting out of having a limited market. It may also explain why drugs such as Tolvaptan (also with orphan drug status) are so flippin' expensive! It's hard to recoup an investment when your upside is limited. So what's the...

I've been on the increased dose of Tolvaptan / Jynarque for the past week (up to 60/30 from 45/15) and I'm happy to admit... It's been a non-event. Water consumption has remained pretty much the same. No real change in thirst. One bathroom break per night. Manageable. I see my nephrologist in two weeks so depending on what she thinks, I may be moving up to the Big Leagues - 90/30 - soon.

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 ...

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.

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...

Remember that AWESOME welcome box I received from Avella with my first shipment of Tolvaptan? One time spiff. This is how shipments #2+ come packaged. I guess there wasn't enough margin in the monthly $13,000 wholesale price for 'Congrats on Making it to Month 2' packaging. ;-) Still SO grateful to have the opportunity to stave off end stage with this medication. Maybe they'll have the artificial kidney by the time I reach the dialysis stage. Here's hoping. :-D

One year ago today, Otsuka (the company that manufactures Tolvaptan) published the following  press release  (citations removed but available via the link): TOKYO, JAPAN – April 24, 2018 – Otsuka Pharmaceutical Co., Ltd.(Otsuka) announces that the U.S. Food and Drug Administration (FDA) has approved JYNARQUE™ (tolvaptan) as the first drug treatment to slow kidney function decline in adults at risk of rapidly progressing autosomal dominant polycystic kidney disease (ADPKD). ADPKD is a genetic disease with consequences that can lead to dialysis or kidney transplantation. It is a progressively debilitating and often painful disorder in which fluid-filled cysts develop in the kidneys over time. These cysts enlarge the kidneys and impair their ability to function normally, leading to kidney failure in most patients.[iii] ADPKD is diagnosed in approximately 140,000 people in the U.S and impacts families across multiple generations, since a parent with ADPKD has a 50 percent ...

Due to the risk of 'serious and potentially fatal liver injury', the dispensation of Tolvaptan is very closely monitored and the PHARMACIES THEMSELVES need to be certified under the Risk Evaluation and Mitigation Strategy ('REMS') Program. Because of this, there are currently only three specialty pharmacies in the US that are certified to fulfill Tolvaptan prescriptions: AllianceRx  (Walgreens), Avella (Optum) and PANTHERx . If you're ever in a situation where you need to go through a specialty pharmacy, check out online reviews to see how others have fared from a customer service standpoint. SERVICE MATTERS! I queried members of the Tolvaptan Community on Facebook when I had to choose. Based on their recommendations, I decided to try Avella. As I'm quickly approaching my one month anniversary of taking Tolvaptan, I was pleasantly surprised to receive a call last week from Avella. A very personable customer service rep asked me about my experience so far...

After 3 weeks on the 45/15 dose of Tolvaptan (45 mg in the am and 15 mg in the afternoon) I found myself sleeping through the night without waking up to drink water and use the bathroom. Heavenly. I was afraid to tell anyone in case I jinxed my good fortune. Then one night I was craving Culver's. The pot roast sandwich. The fries. It was delicious. And that night I once again found myself waking up every two hours with a seemingly unquenchable thirst and multiple trips to the bathroom Coincidence? Connection? It took two days of drinking water to flush that salt out of my system. And now I've returned to sleeping through the night. I knew I was supposed to be watching my salt intake before (high blood pressure) but the added incentive of allowing me to sleep through the night may be just what I need to keep my hand out of the chip bag.

Continuing with my love of cold water is my love of ice! My office has a wonderful pellet/nugget ice maker and I'll admit that I fill two water bottles with the frozen gold before I leave for the day. Maybe I should bring an insulated thermos and haul it home in bulk. Hmmm. So it's no wonder that I've tried to find a similar model to have at home. Well if you have $3,500 lying around and would like to send it to me for my ice fund, that would be great. In the meantime, I'll have to settle for something "close" to perfection. Online reviews say that the Portable Opal Nugget Ice Maker is the perfect compromise for home use. Not that it comes cheap. Amazon had it listed for $475 when I wrote this. For frozen water. You may also know this as "the good ice". Opal makes the chewable, crunchable, flavor-saving nugget ice you love. The Opal is an affordable nugget ice maker for your home. Opal only does one thing: make chewable, crave able nugget ic...

When I first heard about Tolvaptan, I truly wondered if I'd be able to choke down the amount of water needed to keep my kidneys hydrated and happy. I like water, but TWO GALLONS?!? Yikes. About nine months before starting on the meds, I purchased the Primo Water Cooler Dispenser + Pet Station . I live in a rural area and have well water so wanted to make sure that I wasn't ingesting minerals that could harm my kidneys. Also, I have softened water and wasn't sure if it was increasing my sodium levels - better safe, right? Plus - given that I have three dogs - I loved the idea of tying it together with a water dish for the pups. READ THE DESCRIPTION BEFORE YOU BUY! So somehow I thought it was a self-filling water dish. It is... but only if you self-fill it by pushing the button to fill it. DOH! Oh well. Over the summer, I enjoyed the crisp, clear water but I didn't truly appreciate it. Enter Tolvaptan. Now I CRAVE water. The colder the better. The icier th...

Oh how I hate the morning commute to work. Oh how I hate the traffic. Oh how I hate the wasted time. And now... Oh how I hate the fear of peeing myself because I'm stuck in my car in a traffic jam and am unable to get to a bathroom in time. The other day I was sitting in my car, feeling stranded on the freeway as I waited for the other cars to start moving so I could make my way to work and, more importantly, A BATHROOM! Because there's not much else to think about as you're staring at the bumper in front of you, I started playing the what if game. What if I can't hold it? What if I pee myself? I can't go home and work because my laptop is at the office. How could I get my laptop without embarassing myself too much? Oh wait. I have a cardigan sweater on. Could i possibly wrap it around, make my way to my desk and then flee before anyone's had a chance to witness the shame? Okay. I think I can make it. Cue the traffic. It moves. I get to work on ti...

I'm two weeks into my Tolvaptan adventure and I'm happy to report that liver tested with no change to the pre-medication levels. A shout out to my liver. Way to go! One of the potential side effects of the medication is liver damage (which has been proven to be reversible as long as it's caught early). Discontinuation of the drug causes the reversal. Fortunately, the reported incidence of liver damage is relatively low at only 4-5%. More recently, in large registration trials of long term therapy in patients with ADPKD, serum aminotransferase elevations occurred in 4% to 5% of patients on tolvaptan, compared to only 1% of controls.  Furthermore, clinically apparent liver injury occurred in approximately 0.1% of treated patients.  The time to onset of illness ranged from 3 to 7 months (Case 1), but occasionally arose during long term therapy (Case 2: after 3 years of intermittent use). National Institute of Health Crossing my fingers that we'll be able to incr...

As much as I hate to admit it, my life now revolves around my meds. Don't get me wrong, I've been on other maintenance drugs for a LOONG time. But somehow the timing of when I took them never seemed to be top of mind to me. No more. Not now that I'm on Tolvaptan. Late doses mean that my sleep suffers. Lack of water means my kidneys suffer. So I started using an app - Medisafe - to help me keep on track. It allows you to interface with your pharmacy (CVS, Walgreens, Rite Aid) or clinic and download your current prescriptions and dosages. All you need to do is schedule each one, select an alarm sound and you're good to go. When the time comes, an alarm sounds and a screen like the one above shows up on your phone. Take your dose, click on the Take button (showing as 'Un-Take' sine I actually took my dose) and go on with your day. The app stores a record of when you took everything so you can talk to your doctor about schedule compliance....

Just took the last dose for the week and I have to say that it went pretty well. The biggest change has simply been planning ahead and being aware. Bringing water with me when I'm out and about. Scouting for bathrooms before I need them. Keeping a full water bottle beside me when I work. Using the bathroom before meetings, before leaving home, before leaving work. Prepping water bottles to have bedside before i go to bed. Manageable. Totally manageable.

Fri Apr 5 2019 - Isn't it strange how quickly your new crazy becomes your new normal? Last night I went through less than two litres (64 oz) of water and was actually able to sleep for 2 1/2 to 3 hours at a shot. Now I'm wondering if something is wrong. Isn't that just too funny? Whatever it is, I'll definitely take it. I actually woke up feeling almost refreshed.

Wed Apr 3 2019 - And THIS is how we prep for bed. Bring it Tolvaptan. Bring it.