Polycystic liver

[123432]

Hello I have pkd and also polycystic liver, I recently under went an attempted liver cyst fenestration which was unsuccessful as the cyst could not be reached via key hole surgery. The surgeon advised afterwards that I may have to consider liver transplant. This shocked and frightened me as I wasn’t expecting that since my liver function is ok it’s just that I have a very very large cyst. I believe it’s quite rare for PLD to lead to transplantation, does anyone know of anyone who has had to have a liver transplant due to pkld?

[JanWellcome]

Hi there is a lady on the PKD & Tolvaptan face book group who has had this I believe her name is Natasha, it would be worth you joining and posting this question on either of these groups I’m sure she would be happy to help. Jx

[123432 in reply to JanWellcome]

Thank you very much I will do that

[Orchidhouse in reply to 123432]

Oh, that's me! hello - I'm Natasha

[123432 in reply to Orchidhouse]

Hi Natasha thanks for reaching out. I’m not on FB but might set up account just to join the groups.

As you are in here are u able to give some background on what advice you received that made them go down the transplant route? I am seeing my consultants boss for a second opinion so would be great to have some understanding around this as it seems such a rare option

[Orchidhouse in reply to 123432]

I asked my renal team for a referral to a liver clinic because my tummy was getting bigger and bigger. This was March 2018. I was sent to Queen Elizabeth Birmingham and seen by a consultant who has an interest in PLD. He added me to his clinic in order to see me annually. It was only 3 months later a liver cyst ruptured and I referred myself back to see them. At this point the consultant simply said "time for a transplant then". No warning etc. My liver was huge. From my groin to the top of my ribs. My spine was being stretch (I grew 3cm), I couldn't bend, I couldn't breath very well, I couldn't sit up. I spent a lot of 2018 lying in bed. In hindsight, I was in a bad way. I was sent for a 3-day assessment where they test for all sorts and then the day later they listed me for transplant. I got the call 3 months after that. In fact, it'll be one year on Friday! I was in hospital 8 days only. Recovery has been remarkable and I am feeling so well, I can't believe it all happened. I worked in the gym right up to transplant (which helped) and I ate well, slept well and kept all levels of stress to a minimum.

[123432 in reply to Orchidhouse]

it’s great to hear you are doing so well it makes me much more hopeful. Thank you so much for your message.

I am not yet in as bad a condition, I have a large cyst 17cm when last measured it has made my stomach look 6 months pregnant but mostly I am pain free. I have lost weight however due to my stomach being squeezed by the cyst my appetite has reduced. But feel better lighter anyway so positive in a way.

My next move is to try to reduce stress I have a stressful job and High blood pressure so plan to find a less stressful job (if that’s possible).

I’ll wait to see what the next consultant says next month.

[tillymint16 in reply to 123432]

Hi Natasha

How do you find being on Tolvaptan or if your no longer requiring it how were you when taking it. Apparently I would have to attend a clinic once a month for blood tests which I’m terrified of!!!!

[twobirthdaze]

Hi there, sorry to hear about your plight. I have had a liver transplant due to PKL. My experience was similar with defenestration and to be honest in my case it simply made things worse because the scarring can cause that part of the liver to not function as well. I continued to develop many cysts until my liver function was very low.It did take around 8 years to progress to that level.I was extremely lucky to receive the gift of life.All went well and i have now had an amazing 11 years, although now the kidneys are playing up.I dont know your journey, but it does take many years for these cysts to develop and cause damage.I was diagnosed at the age of 17 and Liver cysts didnt become a real issue for me until i was in my 40s. I hope this gives you some reassurance about your own situation, it has a few ups and downs along the way.Best of luck to you.