Polycystic liver disease (PLD) often goes hand in hand with PKD. We recently reached out to PKD community member and advocate Jenn Tompkins-Kirshenbaum to share her experience as a PLD patient, how she has connected with others, and little-known facts about the disease:
PKD Foundation: Tell us a little bit about yourself.
Jenn Tompkins-Kirshenbaum: I was born and raised in Omaha, Nebraska. My father is from Nova Scotia and Hamilton, Ontario, Canada. My mother is from Silver Creek, Nebraska. My husband, Matt Kirshenbaum, and I have two daughters ages 15 and 18.
PKDF: What is your relationship with PLD/PKD?
JTK: PKD runs in my family on my dad’s side—his mother had PKD and large liver cysts. He was diagnosed with PKD at age 58. He began to have high blood pressure and they couldn’t figure out why. A CT scan showed the kidney cysts. I researched everything I could about PKD. I found the PKD Foundation website and watched several of the education videos. I was pregnant at the time and asked the doctor to look at my kidneys for cysts at the next baby ultrasound. The doctor didn’t see any cysts, so I thought I didn’t have PKD. Twelve years later, though, I started to have high blood pressure. We did an ultrasound and I had kidney cysts and innumerable liver cysts with one of the cysts being very large. I went back to the Foundation website and researched everything about PKD again and PLD. Dad is 74 years old now and his kidney function is 50%. He has not had a transplant. He has liver cysts, but they are not huge.
PKDF: What has been your experience with PKD and PLD? What have been the biggest challenges?
JTK: A big challenge is that PLD is rare. Some doctors don’t know about it. The general public doesn’t know about it. It took a long time for me to find someone else who had PLD. Kim Beger, Nebraska’s Volunteer Chapter Coordinator, said she had met a woman from St. Louis at one of the PKD Foundation’s conferences who had large liver cysts. Eventually, we were connected. We spent an hour on the phone and she told me about her surgeries—that meant a lot to me. Without the PKD Foundation, I would have never been able to make a connection like that.
There are other challenges, too. You tend to feel full all of the time. If you overeat, you throw up. You start to eat less and then you lose muscle mass. Twenty-six times I have either been asked if I’m pregnant or have been congratulated on my pregnancy. I think I look three to four months pregnant, but close friends say it’s more like 5 months pregnant. I wear a lot of maternity tops and shirts. The cysts can be very painful. One time they hemorrhaged and I ended up in the ER. Sometimes it’s hard to breathe—the cysts push out my rib cage.
PKDF: How have you faced those challenges? How do you feel you’ve grown from them?
JTK: Researching is a big way to face the challenges. Having a good sense of humor helps too.
PKDF: What is one thing about PLD you feel is lesser known that you’d like more people to know?
JTK: When you are on the liver transplant list with PLD, your Model for End-Stage Liver Disease (MELD) score is determined by exception points. The transplant team writes a letter for you every three months asking for two more exception points. When a person with regular liver disease is on the transplant list, their liver function dictates their MELD score.
Unlike PKD, the cysts in the liver do not affect the liver function. Many people have asked me questions about my liver disease: Are there certain foods I can and can’t eat? Can I drink alcohol? The diet doesn’t have any affect. It’s the DNA mutation that has caused cysts to grow, not food nor drink. Researchers have advised not to take estrogen.
PKDF: How do you raise awareness for PLD?
JTK: The PKD Foundation has been very helpful in raising awareness for PLD. Dr. Marie Hogan from the Mayo Clinic has great videos about PLD on the PKD website.