Polycystic liver disease patient shares experience with her disease

Polycystic liver disease (PLD) often goes hand in hand with PKD. We recently reached out to PKD community member and advocate Jenn Tompkins 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: 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 ex-husband, Matt Kirshenbaum, and I have two daughters ages 15 and 18.

Jenn’s father

PKDF: What is your relationship with PLD/PKD?
Jenn: 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?
Jenn: 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 five 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?
Jenn: Researching is a big way to face the challenges. Having a good sense of humor helps too.

Jenn Tompkins

PKDF: What is one thing about PLD you feel is lesser known that you’d like more people to know?
Jenn: 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 effect. 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?
Jenn: 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.

Check out our website to learn more about PLD.

60 Comments

  1. Vickie Brown

    I have had a kidney transplant..15 years ago..also a liver transplant..6 years ago.. due to polycystic disease..it is an incedious disease..so many different organs are affected..research seems to be difficult..my father died from it in 1967..not a lot of progress since then…praying that advancement in curing is closer…my 40 year old daughter has been undiagnosed also..I am 68….hopefully something will be close..before she has to deal with what I have been through..I feel really blessed to still be alive and healthy…prayers for all affected!!

    Reply
    • Lisa

      I’ve had a kidney transplant for almost 30 years due to PKD. Now I am affected by cysts throughput my liver from PLD and mention of getting me on a liver transplant list. You are the only person that has been through my same situation. Curious how it has all gone for you. Hope you’re well!

      Reply
      • Jennifer Tompkins

        Lisa – are you on the transplant list? I had a three day evaluation before they placed me on the liver transplant list. I was on the list for almost two years before I received the phone call.

        Reply
      • Todd

        I had a kidney transplant in 2011, and am currently doing a workup for liver transplant. My enlarged liver has caused ascities and I have to have abdominal fluid drained every 45 days or so. It’s really hard on my transplanted kidney. My liver is completely full of cysts and I guess I’m one of the few males that get it this bad as they say mostly females have this issue.

        Reply
        • Paul

          Hi Todd, my twin brother and me both have enlarged livers too due to PLD.

          Reply
        • Tong Young

          Hi, Tod, my name is Tong. I had kidney transplant over a year ago. And I have an enlarged liver full of liver cysts. Also like you, I need water tap, about once a month, about 3.2 L drainage. Glad to see your blog. By the way, I’m male also. 79 years old.

          Reply
    • Jennifer Tompkins

      I feel really blessed to be alive and healthy too! I have not looked at this blog in a long time. I have received a liver transplant since I wrote this blog. I am doing much better and am very thankful.

      Reply
  2. bambi arredondo

    Kelly Shakoor told her story about her PLD. You might be about to find the story here. My daughter has PLD & was on birth control when dx & they never told her to stop taking it. I told her to stop taking BC after reading articles on PLD.I read even soy affects cyst formation🤔 I don’t know if that is true statement or not.I just hope they fine some kind of treatment for PKD/PLD.
    If you can’t find Kelly’s story I can emsil it to you

    Reply
  3. Paula Browning

    I also have PLD along with my PKD(GFR 52) I’ve known since I was 15. It comes from my dad’s side, we currently have 13 family members with PKD. I lost my dad in 2016. In the last year I have noticed a lot of discomfort/fullness. My liver functions are all fine, my bilirubin runs around 2.1, not sure if that’s from my PLD. The largest cyst that could be measured on my liver is 12cm. I had a CT recently and my liver is very large, kidneys are completely covered with cyst of course, but not as enlarged as I was expecting. I’ve been looking for procedures that could be done just to give me some temporary relief. Have you had any procedures or surgery on your liver?

    Reply
    • Jenn

      No surgeries on my liver. The med center won’t do surgery on my liver because there isn’t a piece of liver without cysts. I’m sorry to hear about your family members.

      Reply
      • Irene

        My name is Irene, I have had 2 native kidneys removed and a kidney transplant. I have PLD and had liver deroofing/debulking 10 yrs ago, changed my life for the better. Still a massive liver with bulk and other issues but free to discuss if anyone needs as I have been very lucky so far. I am 51 yrs old.

        Reply
        • Jennifer Tompkins

          That’s wonderful! How are you doing?

          Reply
        • Kristy Swenson

          Thank you for sharing your story. I had a kidney transplant 12 years ago due to PKD, and now I am developing high blood pressure at 50 yrs old. I have PLD as well. Both native kidneys were removed. I’m wondering if my liver cysts could be the reason for my high blood pressure. I’m currently on 2 medications that aren’t really helping even though my kidney function is still very good. Have you had any experience with that?

          Reply
    • Phyllis Giardina

      Yes I have had liver fenestration surgery 11 years ago in which 6 out of 11 cysts were removed and cauterized. The largest was 14cm. Just recently I began having symptoms again and recent scan shows 8 cysts with the largest measuring 7.5cm I’m going in for consultation but would prefer to not have liver surgery again.

      Reply
    • Christina Curry Poore

      i have had several large liver cyst drained or deroofed. and it does offer some relief, i was on the tolvaptan study and that was a Godsend for 5 years until the conclusion of the study and at over 1000 dollars a mg, i could never afford the treatment.

      Reply
  4. Diane M. Peters

    I have PKD /PLD . I am 63 this June will be 10 yrs. since my kidney transplant.. have large liver cyst. So far I’m able to handle the pain from liver and two big old kidneys. With my Faith and Trust in Jesus Christ.
    I lost my mom at 52 and my brother at 53. My niece has already had transplant at 41. Her brother is now on dialysis at 42 . My daughter has been diagnosed she is 41 and her son 24 , was diagnosed this past Christmas.
    Hate this disease . PKD HAS BROKE OUR HEARTS SO MUCH. PLD IS ONLY BOTHERING ME. SO FAR.

    Reply
  5. Tammy Richmond

    My Name is Ray RIchmond, I would like for you to get into contact
    With my wife who has Polycystic Kidney and Liver Disease, Her name is Tammy Richmond, Her Life journey with this disease will leave you in awe of all the stories you have Published.

    Diagnosed this Disease Christmas Day 2005.
    Tammy Richmond
    773-992-8756

    Reply
    • Janet Purdie

      Hi my friend Barbara has it can you email me your story please

      Reply
      • Kenneth Perry

        A pkd transplant patient 2014 after 8yrs on dialysis both native kidneys removed 2010 due to size, my liver now so large with pld it spans both side of my rib cage & below now experiencing severe abdominal pain. Located UK

        Reply
        • Barb

          I have the same issue. I have an appointment with the liver dr on Friday to see what they can do. I’ve gained 25 lbs in my belly. I’d be interested to hear your treatment plan

          Reply
          • Michele Heffernan

            I had kidney transplant 9 years ago, still have both old kidneys that are still enlarged. I now have PLD, My liver is so enlarged covered in cysts giving me so many symptoms, shortness of breath, itchy burning skin and eyes, fatigue, lightheaded, constant pain throughout chest and abdomen, colitis, duodenitis, gastritis, reflux, bowel and bladder incontinence, always full etc,etc,. This disease is depilitating and quality of life is nearly as bad as having PKD, I have gone up 3 dress sizes around my abdomen and put on weight, which I was told you are meant to lose weight which I don’t understand. As my function is ok the doctors refuse to put me on the transplant list, there are days that I just don’t want to be here. I put in a happy face and apart from the ever growing alien growing inside of me, nobody would no the pain and suffering I am experiencing. Has anyone else experienced these symptoms, I just feel like if I mention all these symptoms, people will just think I am a hypochondriac.

  6. Anne Edwards

    I have PKD and PLD, diagnosed age 29. I’m now 56. I recently have a liver cyst ablation procedure done to aspirate a very large cyst filled with 16 ounces of fluid. The ablation done with alcohol causes the walls of the cyst to basically break down so that the cyst won’t fill up again. I feel so much better since they drained that huge liver cyst. Yes, I’m often asked if pregnant too. Oh well.
    Anne Edwards,
    Brandon, MS

    Reply
    • Nancy

      Anne, Can you tell me, did you have to go to a special hospital to do this, and was it done by what type of doctor? I am desperately needing relief from large liver cysts!

      Thank you!

      Reply
      • Peggy

        Hi my name is Peggy .I am 59 and I was diagnosed with Severe polycystic liver disease 11 years ago. My only option for pain relief is ablation and drainage of liver cysts. The best care received is by a Heptoligist, (liver doctor)at a University based hospital .Dr Vincente Torres at Mayo Clinic in Rochester is an expert on PLD.
        Dr Richard Green at Northwestern Memorial Hospital is excellent too.
        Good luck .

        Reply
    • Evy Young

      Hi Anne,
      That’s so hugely helpful! Thank you so much.
      I also have PKD and PLD and had a kidney transplant 8 years ago.
      Currently having a huge lot of discomfort from a very sore liver and ribs being pushed out on the right. In fact they are broken and can’t heal due to the huge kidney and liver on that side.
      My nephrologist (I live in the UK) just said they are reluctant to take the kidneys out and also said that they can’t drain the liver cysts as they re-fill within 5 years. Knowing that it can be done with alcohol to destroy the cyst walls in the liver is an absolute revelation! Thank you so much Anne! Wishing you and all other PKD /PLD sufferers all the very best and good health!
      Evy
      Scotland

      Reply
      • Maritza

        My mom has polycystic liver disease. Over a decade ago, she had surgery to remove a massive cyst in her liver. The first Doctor drained the cyst and, in a week, grew back. On the second surgery, the new surgeon decided to do an experimental surgery, and it worked. The cyst was open and reduced to the size of a golf ball. The doctor filled the inside with my Mom’s fat tissue, and the cyst did not grow back. I believe they drained half a gallon of liquid. The cyst had sacs inside, and the cell was alive, so they regenerate even if the cyst was drained or cut. I have one cyst in my kidney and a stone so, I was trying to get information about the polycystic disease in the kidney. My Mom is 70 and has never gotten any cysts in the kidneys, but she has many liver cysts. What kind of Doctor diagnoses PLKD? My doctor referred me to a urologist. It didn’t seem like the right choice.

        Reply
        • Jennifer Tompkins

          Have you tried a hepatologist?

          Reply
  7. Donna Willingham

    hi, I’m Donna. 66 years old. PKD and PLD. My kidney transplant is 5 years. My liver has 1 cyst the size of a grapefruit. and multiple cysts as well. they are complex cysts filled with blood and proteinaceous content. I am tired a lot. and happy. happy to be alive. I weigh 135 and am 5 6. yes. my belly is distended. I was recently diagnosed with arterial malformation of the colon, portal hypertension gastropathy and gastric varices. I feel blessed. and I appreciate your input and your experiences. I’m also an innovo patient. Thank you all for sharing and may you receive many blessings. Love, Donna TX

    Reply
  8. Anna

    Thanks for sharing your experience.

    Reply
  9. Robert

    Hi ,
    We loved your blog on “Polycystic liver disease patient shares experience with her disease”. It was very informative.
    If you are interested in learning more about Gastroenterology procedures and services check out our website http://www.midstategastroct.com/ for further information.
    Thank You,

    Reply
  10. Debra McGinty-Poteet

    I am 63 and had a kidney transplant 19 months ago and had my pkd kidneys removed laproscopically at Mayo AZ 5 months ago. Now I am dealing with my PLD liver which is large and has heavily dispersed cysts. They started talking about liver transplant with me today and I would like to avoid that if possible and try one of the somastatin therapies. Where do I go to get into a clinical trial for that? Mayo Rochester with Dr. Hogan, UCSF? I am in San Diego.

    Reply
  11. Margaret Nelson

    Hi,
    I am 64 years old and recently had an elevated ALT level, so my doctor scheduled and US of my liver and right kidney. A group of small cysts were found in my liver, but none in my kidney. My doctor referred me to see a gastroenterologist and my appointment is at the end of this month. I don’t know of any personal family history of PKD or PLD, so I’m hoping my liver cysts are nothing to worry about. Is any one aware of liver cysts being anything else? Are there people who have PLD that don’t experience any problems from it?

    Reply
  12. Norah Jhonson

    We should keep a routine check for Polycystic liver disease and its signs, and contact an able liver surgeon at the earliest. Thanks for sharing this post. Stay safe and stay happy.

    Reply
  13. Karen

    I am 50 with PKD and PLD. I had a left nephrectomy a couple of years ago. I have been having severe pain on liver. Had a U/S yesterday and received a call today that I have a large cyst on my liver. I’m waiting now for a call to go get it taken care. My abdomen is distended, my reflux has gotten worse and I just feel run down.

    Reply
  14. Aysha Davies

    Hi.
    Would be interested to know if there is anyone from the Uk or Wales here with PKF/PLD.

    Reply
    • Janice McFadyen

      Hi, I’m from Scotland and have PKD &PLD. I have varices (portal hypertension) satiety and a very distended abdomen. I get tired and breathless very easily. I am waiting for CT scan to see if there is much change since January. I would love to talk further to someone in same boat as me.

      Reply
      • Lisa

        Hello Janice, how are you doing?? I’m in same situation as yourself, two years after your comment. I also live in Scotland

        Reply
    • Rachel Hope

      Hi Aysha
      I live in London and have both liver and kidney cysts. Keyhole Surgery on my liver cysts in January proved useless as they re-filled within 2 weeks and now they are worse than ever.

      Reply
      • Maritza

        The cyst is made of live cells and divided inside like a honeycomb. Each space produces the liquid, so if the fluid is drained, the cyst gets filled again. My Mom was treated over a decade ago in UCSD in San Diego, CA. The surgeon cut the cyst to a small size, clean everything inside the cyst, leaving it to hollo then filled it with fat tissue. Her surgeon was the chief of the liver transplant unit at the time.

        Reply
      • Maritza

        My Mom was treated over a decade ago in UCSD in San Diego, CA. The surgeon cut the cyst to a small size, clean everything inside the cyst, leaving it to hollow then filled it with fat tissue. Her surgeon was the chief of the liver transplant unit at the time. The cyst has not grow back it is still the size of a golf ball.

        Reply
        • Jennifer Tompkins

          That’s amazing! I’m so glad.

          Reply
    • Dannette

      Hi, am 50 and just returned for the doctor. My liver has some small cysts one larger at 4 cm and one cyst on my kidney. My Dad Uncle and brother have PKD. How fast do the cysts spread and grow once they appear. And what is considered a large cyst? My Dad is 70 and only has one kidney enlarged and really painful. His kidneys are still doing a pretty good job though. I can’t find anything on how fast it grows and spreads.

      Reply
      • Jennifer Tompkins

        I believe my largest liver cyst was 13 cm. I had several 5 to 7 cm cysts and innumerable smaller ones. I have since received a liver transplant. When I left the hospital, I was 18 pounds lighter with my donated liver. My cysts grew fast within two years.

        Reply
        • Christina Curry Poore

          my largest liver cyst was 27 cm, they removed 5/8 of a gallon of fluids so i lost 5 pounds in mere minutes, best i had felt in a long time

          Reply
  15. Todd

    I am 57 and had both polycystic kidneys removed with transplant in 2011. Currently I’m dealing with hypotension and mild ascities based off my PLD which has large cysts throughout it. My doctor says I need a liver transplant and could do partial from living donor. My liver is huge and though I don’t have much pain I feel zapped of energy especially on hot days. So far I have had my stomach cavity fluid drained twice and second time was 7 litres. Apparently it is very difficult to get approval for transplant when your liver is still functional. Anyone else have same issues.

    Reply
    • Jennifer Tompkins

      Yes, the med center wrote exception points for my MELD score because with PLD, your liver function is not affected. They write about your other symptoms that a huge liver is causing.

      Reply
  16. Angela welch

    Dad is 78 PKD living related transplant 1995, liver enormous thousands of cysts, can’t eat much pressing on other organs etc. too old for transplant. Too many to aspirate. Otherwise Very healthy. Being told surgery too risky needs something says quality of life maybe a 4. Wants surgery. Who are best Drs for surgery please? Need to find drs familiar with this and anyone have reduction surgery? Thank you

    Reply
  17. Rachel Hope

    Thank you for your story Jenn.
    I was diagnosed with PKD and PLD when I was 36 after a couple of years of doctors treating it as gastric reflux due to the swelling under my ribs. I had enough and managed to get a scan where I was told I have liver cysts but then told I also have kidney cysts too. None of my family have it and they also checked my mothers records (she passed away from cancer 27 years ago) so luckily for me it’s spontaneous.
    It has gotten worse since I had my son 2 years ago and now often get asked if I am pregnant, I would say I look about 6 months. I am a small build and it makes me embarrassed and constantly aware of what I look like. I too have had a cyst burst which resulted in an ambulance trip, I have never experienced pain like it even after childbirth. I had keyhole surgery which drained 2 litres of fluid from cysts on my liver but they returned within 2 weeks and are now much worse. I am considering another procedure as I am only 39 and I want to be able to enjoy playing with my son.
    This condition can really affect your mental wellbeing as there is nothing you can do to prevent it. I just hope now that I haven’t passed it on to my son.

    Reply
    • Jennifer Tompkins

      I’m so sorry, Rachel. I hear you.

      Reply
    • Jennifer Yee

      Hi Rachel- I am in the same boat as you. I am 40 and my PLD became noticeable after my second child. It is a daily mental battle. Can you tell me what procedure you are thinking of having/had? I have talked to my doctor a few times about resection, as my liver has not grown in 2 years. I really think the growth was due to hormones I was put on during pregnancy.

      Reply
      • Avigail D

        Jennifer your comments resonate with me. I was in clomid for fertility treatment to get pregnant – I did not know I had PKD and PLD until after pregnant and found my and my liver cysts grew rapidly. I want to educate other women to be careful in fertility treatment. Also- I am looking for a good liver doctor who specializes in PLD – any referrals you all have trust in working with? Thanks and grateful to have this community.

        Reply
        • Mary Ruth Webb

          Abigail,
          I don’t know where you live, of course. I’m in North Carolina and the Nephrologists and Hepatologists at the Transplant Clinic at UNC in Chapel Hill are top notch. I would suggest you reach out to your nephrologist (if you don’t have one, get one, along with a hepatologist). Find your nearest Transplant center.
          Online, there is PKDiet.com, which is a group of people with PKD and PLD who share what they have learned with each other.
          Best to you.

          Reply
  18. Tracy Smith

    Hi I am 53 and just found out I have cyst liver disease plus I have cysts on my kidneys and a lesion on my spleen. Is this the same thing as PLD? It explains why I always feel like I have a fist stuck up under my rib cage. I also have IBS-D which seems to run my life. I am waiting to hear back from my doc. What is considered a large cyst and what is considered small. I also have hemangioma some both liver and kidney.
    I was just diagnosed with 1st stage renal failure as well with a score of 61.
    I am not sure where to even begin. Does diet have impact on all this?

    Thanks

    Reply
  19. Kathy Illingworth

    I am 69 years old and have PKD ( waiting on a transplant). I also have a very large liver which has grown a lot in size over the last year. The largest cyst has gotten bigger at an alarming rate. I look pregnant and It affects the amount of food I can eat at one time and and overall quality of life. My doctor is going to talk to some of his colleagues and see about adding me to the liver transplant list also. Has anyone had a liver and kidney transplant at the same time? I have heard that it is better for them to come from the same donor.

    Reply
  20. Mary Ruth Webb

    I’m 71 and female. I have PKD and PLD. My very large liver has given me much more discomfort and pain over the years than my kidneys ever did My kidney function was down to a eGFR of 6 when I went on dialysis. I never felt that bad except for fatigue from anemia. I was extremely blessed to get a transplant on December 1st, 2020, after only 3 months on dialysis. For some reason, after the transplant, my liver discomfort is less. My liver is very big on both sides of my abdomen but is working well.
    A note: Don’t be afraid of a Hepatitis C positive donor. I accepted one and because of being 70 years old, I didn’t think I would ever get a donor. I immediately contracted Hep C, and it was cured after a two month course of Mavyret. For me, it was an easy medication to take with no side effects at all. After a year, I am still Hep C negative and feeling good. I do still avoid certain things /foods that I know are not good for my PLD liver like soy, caffeine, heavy fats, and chemicals.
    Wishing all of you the best.

    Reply
  21. Gloria Clark

    I am a 55 year old female. I have PKD and PLD. I have been wanting to hear stories of people that have PKD and PLD. I accidentally stumbled up on this blog page and is so very thankful. I am truly encouraged by all of your stories. The PKD has taken out many of my family members. My mother had PKD and was in dialysis, believe it or not, almost 30 years before she passed away in 2019. I have a 59 year old brother and male cousin that are on dialysis. I’ve had aunts, uncles, and cousins that battled PKD. I’ve seen them all suffer through this dreadful genetic disease. My GFR is 17. I too look like I am 4 or 5 months pregnant, even when I wear a waist trainer. I get asked all the time. It’s so embarrassing! Both my kidneys and liver are fully covered/filled with the cysts. I have had all of the evaluation tests for a kidney and liver transplant and recently received approval for a kidney/liver transplant. I don’t know how things will turn out, but trusting God with the process. My question to all of you is how did you afford all the out of pocket expenses? I wish you all the best.

    Reply
  22. K Wilson

    Good to hear from others about their experience! I was diagnosed last year and apparently have at least three 10-14 cm cysts, but the doctor does not want to put me on a transplant list because I am only 45. Anyone have referrals for surgeons in the mid-Atlantic?

    Reply

Submit a Comment

Your email address will not be published.

Advocacy

Awareness

Education

Research

Subscribe

Enter your email address to subscribe to this blog and receive notifications of new posts by email

English Spanish