Karen is living with rapidly progressing autosomal dominant polycystic kidney disease, or ADPKD, and taking JYNARQUE® (tolvaptan).
Before Karen was diagnosed with autosomal dominant polycystic kidney disease (ADPKD) at the age of 42, she knew she possibly could have inherited kidney disease from her mother. Since the time Karen was a child, she watched her mother progress from weakness and fatigue to dialysis to the need for a new kidney. When Karen discovered she couldn’t donate her own kidney because of the family history of kidney disease, well, that was when she started to understand how this information could impact her future.
Please remember that none of the information discussed in this article should replace the conversations with your healthcare provider. Karen is sharing her 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.
When did you first notice that you may have inherited your family’s kidney disease, let alone ADPKD?
Karen: I didn’t think I would get ADPKD. Three out of four of my cousins were diagnosed with it, but none of my siblings had it. I was active, healthy, and never had any of the symptoms that I watched my mother go through. Even on the day I felt a bulge in my abdomen, it never occurred to me that I might have anything wrong with my kidneys. I suspected I could have polycystic liver disease, or PLD (something else my mom had). I was hoping it was just my gallbladder. Who needs a gallbladder?
But my body did have a glitch. I went in for an ultrasound and discovered that not only did I have PLD, but also ADPKD. I knew I needed to learn as much as I could about both diseases as soon as possible.
What made you think about getting ahead of your ADPKD progression?
Karen: The thought of going through what my mother did was one I did not want to bear. At first, I relied a lot on my local doctors. I saw a local nephrologist, who looked at my ultrasounds and blood work and cheerfully said that I had nothing to worry about. He said that, aside from the cysts on my kidneys, I was asymptomatic. Based on the doctor’s reaction, I think he saw ADPKD as something that was diagnosed and treated after my kidneys no longer worked. I felt like there was nothing to do but wait—wait for my mother’s fate. I wasn’t okay with that. I wanted to learn more about both ADPKD and PLD.
“The thought of going through what my mother did was one I did not want to bear.”
Watching your mother’s progression with ADPKD must be incredibly motivating when it comes to making your own medical decisions.
Karen: In a way, it was. My mom’s story is very different than my own so far. I lost her due to complications of ADPKD when I was 33, nine years before I was diagnosed with the disease. My mom didn’t have the same level of care I have had—or an available treatment option. She went on dialysis at age 53. She received a transplant two years later, but her health continued to decline. After seven years, she had to go back on dialysis. She remained hopeful that she would receive another kidney.
One evening, the night before Thanksgiving, she went into the hospital for what was supposed to be a routine procedure. Because of the damage to her kidneys from ADPKD, she couldn’t process the pain medication that the hospital gave her and spent the last week of her life in an unresponsive state. That is an image I try to block from my mind as much as possible and a fate I wish to save myself from.
Learning from your mother’s experience, how did you go about taking control of your own ADPKD progression?
Karen: In the process of researching as much as I could on my diseases, I came across an online PLD forum that posted information about ADPKD. I learned about a Phase II clinical study for a treatment that showed evidence of slowing kidney function decline in adults with rapidly progressing ADPKD. I signed up for the study, feeling I didn’t have anything to lose and I could contribute to the research.
My doctor had told me that because JYNARQUE can cause serious and potentially fatal liver problems, I would need regular blood work to monitor my liver function. He enrolled me in the JYNARQUE Risk Evaluation and Mitigation Strategy, or REMS, program to help me keep on track with my blood work.
Since taking JYNARQUE, I have experienced an increase in thirst. I have water with me at all times to keep myself hydrated. Drinking water has become my new normal, and of course, the more water I drink, the more I have to go to the bathroom, so I’ve also become a master and connoisseur of bathroom locations.
“Having ADPKD has strengthened my determination and willingness to take care of my health, to believe anything is possible, and to remember that hope can empower us.”
Having ADPKD can leave some people weary. How has ADPKD changed your outlook of the future?
Karen: I grew up to be a teacher and a performer. Teaching and performing magic are about engaging people and helping them feel that anything is possible. Maybe that is a reflection of who I really am. I want to feel important and believe that anything is possible. Like everyone, I want to have a purpose and hope that things can get better in the future.
My ADPKD experience has humbled me by helping me not take my life for granted, especially as I get older. I never had a bad outlook on life since being diagnosed, but I knew that I never wanted to have a hopeless fate. Having ADPKD has strengthened my determination and willingness to take care of my health, to believe anything is possible, and to remember that hope can empower us. Magic gives us hope that anything is possible, but science and our willingness to continually learn make that hope a reality.
Patients were compensated for their time.