Zack is living with rapidly progressing autosomal dominant polycystic kidney disease, or ADPKD, and taking JYNARQUE® (tolvaptan).
Zack was diagnosed with autosomal dominant polycystic kidney disease (ADPKD) 25 years ago. For a long time, even with a family history of the condition, he never thought he would be impacted by ADPKD. A former officer in the U.S. Coast Guard and fairly accomplished triathlete, Zack saw himself as linked to strength, stamina, and perseverance. Being affected by ADPKD made him suddenly feel like a 1965 model pickup truck, as opposed to a high-performance machine. But after living with ADPKD for several years, Zack has learned the key to managing it—knowing your limits, assembling your support team, and investing in your future.
Please remember that none of the information discussed in this article should replace the conversations with your healthcare provider. Zack is sharing his own thoughts and experiences, so please keep that in mind as your thoughts and experiences may be different.
Talk to your doctor to see if JYNARQUE is right for you.
You have a family history of ADPKD. Before you were diagnosed, what did you understand about the condition?
Zack: Growing up, I knew my mom had some kind of medical condition. I saw her trying several medications to combat her symptoms. By the time I was in college, I knew that it was a hereditary polycystic kidney disease, so there was a chance I could have it as well.
How did your mom’s experience with ADPKD prepare you for managing it?
Zack: I took my cues for how to live with ADPKD from my mom. She focused on preserving and protecting her kidney function. Together, my parents adapted to the disease, but they never let ADPKD dramatically impact their active lifestyle. My mom is [82], with no kidney transplant, and isn’t on dialysis. I hope my future is similar. My aunt tried to ignore her ADPKD, and it cost her her life way too early.
Even though my intent was to follow my mom’s example, there were some ways I had to adapt to manage my own ADPKD.
Do you have an example of how you adapted?
Zack: Sure. The first lesson I learned was: know your limits.
As an Emergency Manager in the U.S. Coast Guard, I responded to the worst oil spill to ever occur in the Gulf of Mexico, the Deepwater Horizon oil spill off the coast of Louisiana. My job was to coordinate the planning efforts to stop the flow of oil and clean up what had already discharged. It was exhausting, stressful, and physically and emotionally draining work. I worked 16-hour days for four weeks straight. Finally, I was released just before the Fourth of July weekend. My wife and I were going to spend the weekend in San Antonio to celebrate and relax. However, on the drive over, a cyst in my kidney burst.
“Assembling your support team is key to managing ADPKD.”
Oh my goodness! Had you ever experienced anything like that before?
Zack: I hadn’t, and having a kidney cyst burst is excruciatingly painful. My mom describes it as equivalent to “giving birth,” which makes me even more appreciative of her having me!
The pain caused me to go unconscious. Luckily, I was not driving. My wife took me to the hospital. There, I realized I was not invincible, I was not Captain America. I wasn’t courageous; I was careless. I ignored my ADPKD. It didn’t ignore me. It was a vicious, painful reminder that I had not been following my mom’s principles of preserving and protecting my kidneys. I had to reexamine my work–life balance and set new limits.
What helps you maintain those limits and keep managing ADPKD every day?
Zack: My wife really deserves all the credit for getting me to where I am today. I met her a little later in life, so ADPKD has been a part of our marriage from the get-go and, like my parents, we’ve managed it as a team. She researched nephrologists, insurance, pharmacies, formularies, treatment availability, copays, drug interactions, etc. She also puts up with me shamelessly drinking both our waters at restaurants—and then having the audacity to ask our waiter for refills!—and then at home having to pause movies so I can run and pee all the time! She is so patient, encouraging, supportive, and understanding.
My extended family is also a great resource and support network. Assembling your support team is key to managing ADPKD.
“When I talked with my current nephrologist about taking JYNARQUE, it was because I felt it could make a difference in my life years down the road.”
What do you do in terms of slowing down the progression of your ADPKD?
Zack: The silver lining of being diagnosed in my late 20s is that I was able to take early steps to manage my kidney function through lifestyle changes, diet, exercise, and medication. Those early steps also led to me finding out that I had rapidly progressing ADPKD years later because my nephrologist regularly monitored my eGFR, my kidney volume, and the size of my cysts through sonograms and MRIs. I invest in my future with ADPKD even now.
When I talked with my current nephrologist about taking JYNARQUE® (tolvaptan), it was because I felt it could make a difference in my life years down the road. By taking JYNARQUE now, I’m investing in my kidneys’ future because JYNARQUE has been shown to slow kidney function decline in adults with rapidly progressing ADPKD.
Before I started JYNARQUE, my doctor and I discussed that it can cause serious and potentially fatal liver problems. That’s why I have regular blood tests to monitor my liver function as part of the JYNARQUE Risk Evaluation and Mitigation Strategy, or REMS program, due to the risk of serious liver injury. My doctor also warned me that I would have to drink large quantities of water when taking JYNARQUE. I didn’t see that as a negative consequence. Now, having to use a bathroom because I’m drinking so much water sure is! But personally, I can handle it. I just have to be more deliberate and methodical in my plans and activities. Of course, that is my experience with JYNARQUE.
Someone should talk to their own healthcare provider to see if JYNARQUE is right for them.
Overall, how do you view your journey with ADPKD?
Zack: Living with ADPKD is challenging. Sometimes it’s a struggle. But you can manage. My life has evolved. Not dramatically, and some may say even for the better.
eGFR=estimated glomerular filtration rate; MRI=magnetic resonance imaging.
Patients were compensated for their time.