Joseph is living with rapidly progressing autosomal dominant polycystic kidney disease, or ADPKD, and taking JYNARQUE® (tolvaptan).
Autosomal dominant polycystic kidney disease (ADPKD) was always a force in Joseph’s family. It can be traced back to his paternal great-grandmother, affecting Joseph and many of his relatives. In Joseph’s family, the consequences of ADPKD included early death related to health complications, removal of kidneys, dialysis, and lots of transplants. Joseph saw his relatives respond to ADPKD in a variety of different ways, from ignoring it to an intense focus on diet and exercise. He chose to face ADPKD proactively and pursue treatment options. Joseph wants others with ADPKD to do what they can to take charge of their condition.
Please remember that none of the information discussed in this article should replace the conversations with your healthcare provider. Joseph 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.
ADPKD plays a big part in your family history. How did you understand it as a kid?
Joseph: At some point growing up, my parents told my brother, sister, and me about this disease that my dad had that could also affect us. My younger brother found out that he had ADPKD in high school.
The reality in our family is that ADPKD means that somewhere around age 55, you may be put on a transplant list and receive a new kidney. If that isn’t possible, then you may face dialysis three times a week for the rest of your life.
What was it like to watch multiple family members get transplants or go on dialysis?
Joseph: Many of my family members, when it was their time, successfully received transplanted kidneys. They recovered quickly and went back to work and life feeling better. That was not what I saw in my dad’s life.
That sounds scary. What did your dad have to face with ADPKD?
Joseph: My dad had his first transplant in his mid-50s. Things went well at first and there was a lot of optimism. However, he had many complications and went on to have two more transplants in the next 10 years. Between the high points of the transplants, there was a lot of dialysis and several hospitalizations due to infections from the immunosuppression medications. In fact, my mom estimates that my dad has been hospitalized over 20 times (and still counting) in a little over a decade for complications directly related to his ADPKD and the transplants.
For several years now, his third donated kidney has been damaged and functioning at a reduced level, but it has been adequate to keep him going and active. He does know that if this one fails, his body won’t be able to handle another transplant.
When my dad had his first transplant, ADPKD became real for me in a way it never had before. I remember thinking, Oh man…that’s going to be me one day.
“When my dad had his first transplant, ADPKD became real for me in a way it never had before. I remember thinking, Oh man…that’s going to be me one day.”
What was it like when you officially got your ADPKD diagnosis?
Joseph: I was around age 30 when my family doctor ordered a kidney ultrasound to see if I had ADPKD too. I remember where I was standing in my bedroom when he called to tell me that it showed cysts on my kidneys. It was real. I knew I would have to deal with the consequences of ADPKD like many others in my family.
What did you decide to do to help manage your ADPKD?
Joseph: A few years after my diagnosis, my cousin, who also has ADPKD, connected me with some people at a major research clinic who were doing some drug studies related to ADPKD. I went through some short-term studies with them and then began my first long-term study with tolvaptan, which was later approved by the FDA to slow kidney function decline in adults at risk of rapidly progressing ADPKD. It felt good to be a part of research that could benefit other people with ADPKD…and that I myself might benefit from the drug, too.
My nephrologist at the clinic and the other study coordinators were quick to share the importance of a healthy lifestyle. Through my many, many trips to the clinic, I learned the importance of exercising, watching my protein consumption, controlling my blood pressure, and staying at a healthy weight. I was pleased that all of these things plus tolvaptan would create an arsenal to help me in my fight to slow down kidney function decline.
“My goal for my children is for them to see we’ve done everything we can about ADPKD, and it doesn’t have to stop them from living their lives or dreams.”
What made you decide to stick with tolvaptan as a treatment option?
Joseph: I began to wonder if tolvaptan—plus living a healthy lifestyle—could slow the decline in my kidney function, and I tried to imagine what kind of an impact that would have. After watching all of the ups and downs that my mom and dad have gone through with ADPKD, these thoughts seemed very attractive.
After the FDA approved tolvaptan as JYNARQUE, I continued with the medication and kept my nephrologist from the clinic. 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.
I have had to make adjustments while on JYNARQUE. I drink a lot of water every day. Wherever I go, I need to bring a full water bottle and keep my eye out for a bathroom. The swing between thirst and the pressure of a full bladder is constant. My kids have gotten used to that fact whenever we travel. They understand that Dad needs to stop to use the bathroom a lot, and they roll with it. Keep in mind, this is just my experience and others' could be different.
You saw various ways of handling ADPKD growing up. How do you try to model life with this condition for your own family?
Joseph: My goal for my children is for them to see we’ve done everything we can about ADPKD, and it doesn’t have to stop them from living their lives or dreams. They know that I have some kind of disease, that I’m also doing everything I can to fight it, and I’m trying to help other people with ADPKD.
My family still travels, pursues the vocations we are passionate about, and lives life not because I have ADPKD, but in spite of it. I rest in the fact that I am doing everything I can to slow the decline of my kidney function. We don’t ignore ADPKD—we deal with it and move on with life. Hopefully, there will be lots more options for the generation to come, so that they don’t have to let ADPKD define who they are and what they can do.
Patients were compensated for their time.