Deidre is living with rapidly progressing autosomal dominant polycystic kidney disease, or ADPKD, and taking JYNARQUE® (tolvaptan).
After an abdominal ultrasound in college, Deidre suddenly learned that not only did she have autosomal dominant polycystic kidney disease (ADPKD)—she had a family history of the condition, too. Without much information on ADPKD, Deidre did not take it seriously until her endocrinologist and then nephrologist stepped in and suggested she start treatment. Afterward, Deidre had the chance to meet others with ADPKD and learned what facing the condition could mean. Now she takes charge of her own health and wants others with ADPKD to know there is a supportive community for them out there.
Please remember that none of the information discussed in this article should replace the conversations with your healthcare provider. Deidre 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.
It sounds like your ADPKD diagnosis came as something of a surprise. What was your moment of diagnosis like?
Deidre: It was! When I was in college, I struggled with several inexplicable recurrent health issues. The healthcare office at my college sent me for an abdominal ultrasound and that’s when they found them: my big, cyst-covered kidneys. I was told in a phone call that I had something called ADPKD and I would need to see a nephrologist. I didn’t know what that meant. I told my dad and his response was, “Your mother has that.”
My parents divorced when I was young, and I was never close to my mother. Her side of the family didn’t really exist in my world, so I didn’t know about the several relatives that had ADPKD. In my world, it was just me.
What was your initial understanding of ADPKD?
Deidre: When I met with a nephrologist, they explained what ADPKD was and sent me home with a small book. It was not in-depth. My takeaway was that all they could do was monitor the disease and eventually I would go on dialysis.
When I was switched to a different nephrologist in the practice, it only further reinforced that unconcerned feeling. That doctor was so nonchalant that it made it even easier for me not to worry. Basically, “keep an eye on your blood work and we’ll deal with this when things get bad” was the message I received.
Without much guidance on ADPKD, how did you face it?
Deidre: For a long time, I didn’t. I was eventually dropped from the nephrology practice for too many rescheduled appointments. I even thought I didn’t necessarily need a nephrologist, so I just saw my primary care provider to check my levels. I didn’t really talk about my ADPKD unless I was filling out medical history forms. If there wasn’t anything I could do about it, why even think about it? It was a low priority.
“I knew I needed to do something. Together with my doctor, I decided to start on JYNARQUE because it was the first approved treatment for adults at risk of rapidly progressing ADPKD.”
Were there any steps you eventually took to manage your ADPKD?
Deidre: Basically, my endocrinologist got me to a nephrologist because she knew I wasn’t doing anything about my ADPKD. That nephrologist introduced me to JYNARQUE at my very first appointment after reviewing my medical history and previous lab work. He felt that I would be a good candidate, as he explained that at that point, I had rapidly progressing ADPKD. Even though the treatment was new to the market, he was enthusiastic about it, as he had a patient who had success as part of the clinical trials.
Was there anything about JYNARQUE that appealed to you?
Deidre: At first, I was hesitant. Sure, JYNARQUE had been shown to slow kidney function decline, but with the possible side effects, I might have to urinate all the time. My doctor had also 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. But still I wondered, Did I really need this? Were the side effects worth it?
As I was researching JYNARQUE, I also wondered more about ADPKD. I researched it online and almost fell over. I knew I needed to do something. Together with my doctor, I decided to start on JYNARQUE because it was the first approved treatment for adults at risk of rapidly progressing ADPKD. It was definitely a change for me. The drive to drink water was incredible and I was using the bathroom a lot, but soon I felt that my body adjusted. It’s shocking how much urine my bladder can hold now! I do have to plan a little more and make sure I have a water bottle at all times. I always know where the fill stations and bathrooms are. I even have a device to help me urinate outside easily because there are not always bathrooms available! Of course, this is my experience, and the experiences of others may be different.
The biggest thing for me is having a medication—something I can take for my rapidly progressing ADPKD now, rather than just keeping an eye on it.
“The biggest thing for me is having a medication—something I can take for my rapidly progressing ADPKD now, rather than just keeping an eye on it.”
Did anything else happen that changed your outlook on ADPKD?
Deidre: Several months after starting JYNARQUE, I had the opportunity to attend meetings where I met other people with ADPKD, which I had never done before. I heard the stories of some of their family members who had complications from dialysis, failed transplants, or died in their 40s or 50s. It was terrifying, but I also realized those stories were of the past. I was lucky. For the first time, I found strength and courage. I was taking the necessary steps to manage my condition. I was actually doing something.
How are you proactive about your ADPKD now?
Deidre: I make sure to drink lots of water and follow a plant-based diet. I subscribe to ADPKD newsletters and joined a couple of social media groups for the disease. The support groups are like a lifeline. It makes a world of difference to have support from someone who knows what you’re going through. I find I’ve also become more active in the groups and I’m excited to support others. I hope that I can open that door for someone else and let the ADPKD community in for them as well.
Patients were compensated for their time.