Nancy is living with rapidly progressing autosomal dominant polycystic kidney disease, or ADPKD, and taking JYNARQUE® (tolvaptan).
Nancy had a plan. After her sixth and youngest child left for college, she’d return to college and become a hospital chaplain. She’d always loved her career in nursing but felt she had so much more to learn and do. Then a mysterious pain in Nancy’s back led to a shocking diagnosis of autosomal dominant polycystic kidney disease (ADPKD). She felt her hopes and plans dissolving and, instead of going back to college, she started getting her affairs in order. But with support from her children and healthcare team, Nancy figured out new plans for her life with ADPKD—an approach filled with hope and courage.
Please remember that none of the information discussed in this article should replace the conversations with your healthcare provider. Nancy 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 you first started experiencing back pain, what did you think was the cause?
Nancy: I didn’t think much of it at first. It was a dull, nagging pain that seemed to bother me most in the morning. As my mattress was more than 20 years old, I was sure that was the cause. Then one afternoon, the dull pain became a severe, throbbing pain. A week after that, I found it difficult to even get out of bed, so my nurse practitioner recommended an ultrasound to see what was going on. When the scans came back, she gently told me that the radiologist had seen multiple cysts on my kidneys and they suspected ADPKD.
You were shocked by the diagnosis. What did you know of ADPKD at the time?
Nancy: What little I knew about ADPKD came from being a nurse. It was a hereditary disease that typically causes cysts to form on each kidney and eventually leads to kidney failure, then transplant or dialysis. This was not part of my plan. I remembered that my older sister had kidney issues when she was a teenager and had died when she was just 60 years old.
How did you move forward after your ADPKD diagnosis?
Nancy: I met with a nephrologist and, still in shock, searched for words, comments, or questions. I had nothing. It didn’t help when my nephrologist said I’d probably be in kidney failure in five years—10 at the most—and the best thing I could do at this point was control my blood pressure. This was the absolute lowest part of my journey with ADPKD. In that one visit, my hopes and plans dissolved away.
After my appointment, I did some online research. The results were not promising. If there was no treatment for this condition, I wasn’t going to dwell on it. Nearly all of my kids were in college, and I was a nurse supervisor working 60-hour weeks. I didn’t have time for this. I put ADPKD on the back burner. I told myself that if there was no treatment, I had no reason to think about it. But I did have reasons—six of them.
“Now, knowing there’s a treatment for ADPKD—it’s huge. It changes your whole perspective.”
It must’ve been difficult telling your children about your ADPKD diagnosis. How did you approach that conversation?
Nancy: Though my own health concerns were upsetting, the guilt I felt about what may happen to their health was a burden. I had to remind myself that I didn’t cause this and there was nothing I could have done to prevent it. I had brought six amazing people into this world. They were launching their careers, marrying, and having their own families. With ADPKD’s hereditary impact, my diagnosis could potentially become their diagnosis. This was one of the most difficult conversations I’ve ever had. And of course, they had many questions. Their biggest concern was that I should not go through this alone. I was deeply humbled by their grace.
Where else did you turn for support?
Nancy: I prayed. I prayed a lot. And by God’s grace, a transformation began to take root. I realized that I should approach my diagnosis differently. Instead of focusing on an expiration date—paying off bills, downsizing my belongings, saving for my funeral—I started looking forward to life. Before I got out of bed in the morning, I prayed, and I was thankful for the coming day. I took time to treasure each moment. I compounded my savings into a down payment for a small home. And I decided I needed a different healthcare team—one that would share my new outlook on life. My new nurse practitioners, nephrologist, and OB/GYN specialist encouraged me to take better care of myself, engage in my future, and focus on my needs. I also joined patient advocacy walks. I learned more about how others were living with and managing their ADPKD. Through these walks, I discovered there was a clinical trial for a drug called tolvaptan, and I later learned I was eligible.
“My children also decided to take control of their futures and got screened for ADPKD.”
How did participating in this clinical trial affect your outlook on ADPKD?
Nancy: There’s good and bad in trying anything new, but this opportunity was worth it for me. I thought, This clinical trial could lead to a medication that could make a difference in my life, my children’s lives…and the lives of my grandchildren. When the trial ended and the drug was approved by the FDA, it was called JYNARQUE. My nephrologist prescribed JYNARQUE to treat my rapidly progressing ADPKD. We talked about the fact that JYNARQUE can cause serious liver problems. Because of this, I enrolled in the JYNARQUE Risk Evaluation and Mitigation Strategy, or REMS, program to get frequent blood tests to monitor for liver injury.
I do get tired a lot. It’s hard to say whether this is based on my age or the medication, but it is something my nephrologist and I are keeping an eye on. I also drink a lot of water and use the bathroom a lot. Please remember that this is my story, and your experience may be different. Now, knowing there’s a treatment for ADPKD—it’s huge. It changes your whole perspective.
What are your hopes for the future—not only for yourself, but also for your family?
Nancy: I’ve been given an opportunity to learn what is truly important in life. ADPKD is not my focus. Life is my focus. I’m thankful for the strength and courage I have been given to face my life one beautiful day at a time.
My children also decided to take control of their futures and got screened for ADPKD. As it turns out, three of them have been diagnosed. But so much has changed since my diagnosis. For them, ADPKD is in the early stages, and with continued research and development, I am hopeful for their futures, too.
Patients were compensated for their time.