So wait... there's a cure?

Hello internet, it's 2018! I can't believe I remembered my password.

Here I am, some 7 years later. I'm 29 and living in New Orleans and am a Montessori teacher. I teach Montessori, ages 6-9, grades 1st through 3rd. I also still have PKD- and technically I have Polycystic Liver Disease as well, on account of having so many cysts on my liver.

So it's 2018 and, this June, they released a treatment for PKD called JYNARQUE (or tolvaptan). It's FDA approved which is amazing, and it's coming to me at a doctor's appointment near me on Tuesday. I have to get an Ultrasound on Monday and do some blood work and then I guess it's the big day the next day. This is all coming at the beginning of the school year, as my kids come back on Wednesday.

It's so crazy to me that you can google PKD treatment and there is an actual website just talking about it: https://www.jynarquehcp.com/clinical-trial-efficacy?utm_source=Google&utm_medium=cpc&utm_campaign=ADPKD_Treatment_E&utm_term=treatment_for_pkd

It's crazy enough to me that I decided I would start up the old blog again. So as I was saying earlier, here I am some 7 years later.

My dad is doing GREAT! He's staying on his medicine and just celebrated his 11 year kidney transplant anniversary with Lori. Last year, the family (minus me- I was in Houston doing Montessori training) went down to Kansas and saw Lori and celebrated the 10 year anniversary. I made her a really corny button bracelet (inspired by me teaching grades 1st-3rd) that was teal for PKD and had New Orleans red beans on it (aka kidney beans). We also got her another New Orleans red bean piece that was more classy.

Lori continues to be a badass and my dad does as well. He's still working full time, plus some at Notre Dame, as he is doing free-lance work with various dance companies. He is also writing a book on lighting design for dance as if he needs more things on his plate. Look at this amazing human that's still going strong on his donated new chance at life.

My little sister, Wilhe got diagnosed with PKD as well and she is a fighter and a winner and I know she'll be fine. She is a junior BFA theatre major at Ball State and due to her course load she is waiting on trying to treatment due to the side effects.

So that leads me to where I am tonight. Since I have this appointment scheduled, it is all becoming quite real to me. Even though the concept of real in terms of accepting my disease and my projected fate is confusing concept. I was doing some googling and I found a blog that someone wrote about their journey starting on Tolvaptan. I remember the drug- when I was at the PKD Convention whatever year that was, I remember them talking about this study. I think at that time I hadn't started my testing programs. (Which by the way, I did one in Denver and then another one in Denver, just not at the children's hospital. I'll have to reread this blog to figure out where I left off... I just saw 2011). I remember them talking about how it could be dangerous due to salt levels but that it was getting closer, but not ready, for any trials in the US. But hey- it made it here, not only through trials but as an actual FDA approved drug.

I feel like I'm rambling.

My brain is going a mile a minute. I'm checking back on my "myOschner" website, looking at appointment information. Looking at my doctors listed, seeing that I have one listed as my "transplant". That's another thing- I started going to Oschner hospital in New Orleans and I have the most AMAZING nephrologist. His name is Juan Carlos Velez and he has so many answers to my questions and such great knowledge about PKD which is incredible. I was able to give them all of the information that I had from the two testing programs I was involved in and they went over it with a team of fellows and then Dr. Velez and I had a very informative conversation about the future and everything.

There was a period of time where I was really experiencing a lot of pain related to my kidneys and I was dragged down and frustrated, because I was trying to teach and just be "normal" and was rejecting my non-normal diagnosis. I know it's normal to the PKD community, but you get what I mean.

Thankfully that period of time is over. If I do have kidney pain it's not as intense and it doesn't last as long.

So anyway- this blog. The link to it is here: http://justfrances.com/tolvaptan-tales-a-four-month-reflection

Her blog is way more up to date (obviously) than mine and very informative and wonderful. Do people even use blogger anymore? Maybe I'll do some updating to the design after this.

I'll be honest, I found it when I was googling the side-effects of Tolvaptan and alcohol because, well, I'm 29 and I live in New Orleans and your girl likes her wine. I had scoured the treatment website but nothing was said in the description about alcohol consumption. I was concerned because I had a friend who was coming to visit and I obviously wanted to take him out and down to Frenchman street which inevitably involves cocktails- we are the city of the drive through daiquiris after all. I wasn't sure about them coming this weekend because I don't want my blood work to be off because I had a weekend of fun and had probably more than 3 drinks per night.

So he wanted going to come the next weekend, but then I got to thinking. What if on this new medicine I just can't drink at all anymore? I'm not even 30 yet! I'm not ready to stop having fun and drinking, and plus I'm a teacher and we live on wine. So then that spun me out and I just started freaking out. Is this going to change my whole life if I start this medicine? I already know I'm going to have to pee a lot, but I already wake up about 3 times a night to go. The bathroom is right across the hall from my classroom at my school, so theoretically I could handle it.

Am I ready for that? What commitment can I make to my health? How much do I care? I deleted that last question at first because I do care a lot, but I have also been very firm in deciding that I will live my life WITH PKD, not BY PKD. I don't know y'all, it's a lot. I think it's worth it. I hope it's worth it.

But is it? This is the question. This is a treatment, not a cure. How many friends are going to reschedule or cancel plans or not call me as much because I can't party like I used to? Wine is "my thing".

I think I came back to this blog because I'm now at another crossroad of my disease. The floor is up there... am I going to run up the stairs, walk, or just sit down and have a rest, and live in the moment.

Writing this blog and doing those testing programs were hands down the best choice and smartest choices I made as a teenager. I believe it got me through so much and put me in such a good headspace. I'm sure at some point tonight, I'll pour another glass of wine and read through my old posts and revisit a lot of emotions.

I am accepting that tonight PKD has won. However, I'm gonna call it a win for Lydia as well. I have some exciting possibilities that will open to me next week and that's pretty cool.

Nice talking to you, internet. Until next time...

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