Cari is living with rapidly progressing autosomal dominant polycystic kidney disease, or ADPKD, and taking JYNARQUE® (tolvaptan).
At the age of 10, diagnostic imaging confirmed Cari’s parent’s fears: she, like her father, had autosomal dominant polycystic kidney disease (ADPKD). As little could be done for a young patient with no symptoms, her parents made the difficult choice to wait to share the diagnosis with Cari until she was an adult and better able to process the information. Growing up, Cari watched her father deal with his own ADPKD symptoms, dialysis, and treatment, so the significance of an ADPKD diagnosis was not lost on her. Cari knows her parents’ hope was to protect her, but as an adult, she is now very open about ADPKD, advocates for others with the condition, and spreads awareness in hopes of helping everyone in the ADPKD community.
Please remember that none of the information discussed in this article should replace the conversations with your healthcare provider. Cari 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.
What was it like to learn not only that you had ADPKD, but that you’d been diagnosed with it for years?
Cari: It’s a lot to process, even when you have no symptoms. I didn’t know then, but my daily life wouldn’t be affected by ADPKD symptoms for at least 10 more years. At that time, not disclosing information on any chronic diseases you might have could preclude you from employment or private health or life insurance. Thankfully, there is now legislation that protects against discrimination of pre-existing conditions.
However, there’s something frightening that my generation of patients has in common: we are not strangers to ADPKD. Many of us watched our parents deal with a brutal disease daily, where the only strategy was to wait until your kidneys failed, then start dialysis or be fortunate enough to get a transplant. Receiving a diagnosis of an unknown illness is scary, but the fear that comes with a diagnosis of something that’s already impacted your family life is something else entirely.
What did living with ADPKD look like for your dad? It sounds like he went through a lot.
Cari: It’s hard to remember a time when ADPKD wasn’t a big part of my day-to-day life. When I was eight years old, my dad started receiving hemodialysis at home—something unheard of in the mid-1980s—and it allowed him to keep working full time to provide for our family. Dialysis is hard on the body, though. Even when we were young, we knew dad needed a lot of rest, and there were days where he was very sick. He was fortunate to receive a kidney that was a near-perfect match just two years after beginning dialysis. While that freed him from the hemodialysis machine, the numerous medications caused issues of their own, including those designed to prevent transplant rejection. Over the next 30 years, he had sepsis five times and three different types of cancer, and we were acutely aware of how tenuous his health was.
“I wanted to…set the best example possible for my girls by taking charge of my health, being open to research, and sharing my story with others.”
I’m sure seeing everything your parents went through affected your own approach to ADPKD.
Cari: Through all of this, my dad kept a positive attitude about the many health challenges he faced. He was respected by his doctors for how well he understood his conditions and medications. If he felt a medication wasn’t working for him, he’d speak up. He wasn’t afraid to ask questions, even if it was about a course of action they were recommending. Through his example, I learned the value of being my own best advocate and playing an active role in my care.
Was there a moment that the importance of managing your own ADPKD hit home for you?
Cari: Personally, becoming a mom brought my ADPKD into focus. I’ve had three complicated and high-risk pregnancies, but other than that, I’m fortunate that I’ve lived relatively symptom-free until the last 10 years or so. And because ADPKD can impact families through generations, I knew something of the guilt my dad experienced. I wanted to channel all of those emotions into something positive and set the best example possible for my girls by taking charge of my health, being open to research, and sharing my story with others.
“I find both relief and purpose in being open about living with ADPKD and all that goes along with it.”
When did you realize you needed to move beyond managing ADPKD on your own?
Cari: Like many ADPKD patients, the first symptom I needed to treat was my high blood pressure. It’s scary because this and other kidney diseases can move silently in your body. I was 30 before I realized that silent progression was happening to me, and I knew from my upbringing that I needed to be more proactive than reactive. I started making healthier life choices right away in conjunction with finding the right medication to control my blood pressure, and I was amazed at how quickly I started to feel better. A few years later when planning my third pregnancy, I researched alternative ways to slow the progression of ADPKD online and discovered a clinical trial for an investigational drug called tolvaptan, later approved by the FDA to treat adults with rapidly progressing ADPKD as JYNARQUE. I began taking the trial medication in January 2016.
In 2018, I transitioned from the trial to the commercially available medication. I applied for the Otsuka Patient Support Program, which helped tremendously. I also learned that the Patient Support Program can, if eligible, cover the bloodwork required by the JYNARQUE REMS program that I need to be part of to monitor my liver function. JYNARQUE can cause serious and potentially fatal liver problems, so I stick to my scheduled checkups to ensure my liver is working properly.
Being on JYNARQUE, I drink much more water than I ever did before, but over time, I’ve grown accustomed to it and found that the search for new and flavorful ways to consume water and keep track of my intake is now part of my daily routine. Of course, this is my experience, and everyone is different.
You are fairly active in the ADPKD community, correct?
Cari: Through my research on potential treatments and my quest to make some sense out of having a chronic illness, I’ve been blessed to find initiatives ranging from grassroots political advocacy to patient advisory boards focused on bringing the patient community together via technology. In addition to feeling like I’m using my disease as a positive platform, I’ve met some amazing people who truly understand me because they face the same health challenges.
Why is raising awareness of ADPKD so important to you?
Cari: Part of being open about my health journey is honoring my dad’s memory and legacy. In one generation, we’ve moved from guarding information about our health out of fear of discrimination to openly sharing our diagnosis in hope that a fellow patient will identify with us and find encouragement. I find both relief and purpose in being open about living with ADPKD and all that goes along with it. The people in my life who know about my condition understand and support me! My hope is that through my commitment to awareness of chronic kidney diseases, others will take active roles in their health journeys through early detection of ADPKD, healthy living, and becoming a strong voice for themselves and others.
Patients were compensated for their time.