Will is living with rapidly progressing autosomal dominant polycystic kidney disease, or ADPKD, and taking JYNARQUE® (tolvaptan).
Family means everything to Will. His parents modeled positive life lessons for him growing up: to live the American dream, he must do well in school, work extremely hard, and do the best he can—no matter what he was doing. On the flip side, his family was stoic and did not discuss difficult topics. So when it came to facing autosomal dominant polycystic kidney disease (ADPKD), they never discussed managing it—or discussed ADPKD at all—which later put Will’s health in jeopardy. To do the right thing for himself and his family, he sought out the best way to manage his ADPKD.
Please remember that none of the information discussed in this article should replace the conversations with your healthcare provider. Will 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.
How did you learn about your mom’s ADPKD diagnosis—and that you might have it as well?
Will: In 2004, my mom called all of her kids together and finally let us know that she had ADPKD. She recommended that we all get checked out. The first thing I did was discuss this bombshell with my wife, Judy, and then I saw my general practitioner, who ordered an ultrasound and declared I had “fatty kidneys.” That did not sit well with Judy or me, and we wanted a second opinion. We found a nephrologist that specialized in ADPKD patients. The second ultrasound told my new nephrologist everything she needed to know. At age 40, I was officially diagnosed with ADPKD.
Did you ever have any hint that you might have ADPKD?
Will: For several years, I went to the doctor’s office for my annual physical and usually had elevated blood pressure readings. The doctors and I always rationalized it away by saying it’s “White Coat Syndrome,” which is when nervousness elevates someone’s blood pressure during appointments.
To tell you the truth, my first emotion after hearing my diagnosis was relief at finding out what was causing my high blood pressure. It also explained why I had a contrast-enhanced kidney ultrasound done around age 11, and why my mom always had backaches and an unexplained hernia, took so many medications, and needed afternoon naps.
What were your next steps after getting your diagnosis?
Will: Even with all of this evidence in front of me, I convinced myself that I would be okay until I was 70 to 75. During this time, I found out that my uncle had started kidney dialysis at age 79, and my mom was 73 when she told us about her ADPKD. This felt like enough proof for me that I didn’t have to worry for another 30 to 35 years. I continued on with my life.
“I did have too much to live for—doing nothing wasn’t an option.”
What prompted you to take ADPKD seriously?
Will: In 2017, my son, Patrick, and I agreed that after he graduated from high school in the spring of 2022, we would fulfill my lifelong dream to bike across the country—me on the bike, him driving the support vehicle. That gave me four years to get back into biking shape.
By the end of 2017, I’d logged over 2,000 miles—a good start. But halfway through 2018, I noticed I wasn’t making any progress on my biking goals. Something was really wrong. I was getting headaches, I was always tired and easily winded, and my back was hurting every day. By then, I was only two weeks out from my annual nephrology appointment. When I got to the doctor’s office, my blood pressure was out of control. For the first time, my doctor could feel my kidneys through my stomach. We found that in less than nine months my eGFR—a test that measures kidney function—had dropped drastically. My ADPKD was rapidly progressing, and I now had stage 3b kidney failure.
That sounds terrifying. What did you think when you got those results?
Will: No! This is too soon. In my mind, this was not supposed to happen for another 20 years. I was in denial. Why me? Did my kids also have ADPKD? They had a 50 percent chance. My options were to wait until my eGFR fell more to make a treatment decision; start taking JYNARQUE, a newly approved drug my nephrologist recommended; or do what my mother did, which was nothing.
“I also openly discuss ADPKD with family and friends. These discussions have shown me how many wonderful people there are in this world.”
How did you decide what to do next?
Will: My mom passed away from end-stage renal failure due to ADPKD on September 11, 2007. She had chosen not to go on kidney dialysis. It was a heart-wrenching day for me and my family. A few days before my mother passed away, her last words to me were, “If this happens to you, please do something like going on dialysis. You have too much to live for.” Those words have stayed with me all of these years, and I thought of them again when I realized my ADPKD was not going to go the way it did for my mom and uncle. I did have too much to live for—doing nothing wasn’t an option.
So together, my nephrologist and I decided I would start JYNARQUE to try something that might help slow the decline of my kidney function. After stabilizing my blood pressure with appropriate medications and being authorized by my health insurance company, I was finally able to start JYNARQUE at the end of April 2019. Because JYNARQUE can cause serious and potentially fatal liver problems, I have regular blood tests to monitor my liver function as part of the Risk Evaluation and Mitigation Strategy, or REMS, program. My hope is to slow the progression of my ADPKD and the decline in my kidney function as much as possible.
What else do you do to help manage ADPKD?
Will: I made some life-changing adjustments like improving my diet and surrounding myself with healthcare providers that know about ADPKD. I also have to drink more than five liters of water per day with JYNARQUE, which causes me to go to the bathroom a lot. I pay much closer attention to where the restrooms are and have a portable urine bottle in each car. Knock on wood—I have not needed to use the bottles yet. As part of this, I learned my limitations during biking (I need to drink even more water so as not to dehydrate). Because I wake up several times a night to go to the bathroom, I never get a full night’s sleep, so I catnap whenever I can during the day, too. I also openly discuss ADPKD with family and friends. These discussions have shown me how many wonderful people there are in this world.
What advice do you have to others living with ADPKD?
Will: Be who you are and live true to yourself. Figure out who and what is important to you and your family, then hug and love them each and every day. You have so much to live for.
eGFR=estimated glomerular filtration rate.
Patients were compensated for their time.