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My PKD liver (or PKD-associated PLD)

This entry was posted on Monday, March 12th, 2018 by Frances Ryan.
Tags: tolvaptan, sewing, pkd, health, family, ego, confession

March is PKD Awareness month, so I thought I would take the time to talk about how PKD affects my liver. (Oh, yes, polycystic kidney disease impacts more than just kidneys!) To start, a few facts about PKD:

  • PKD stands for polycystic kidney disease, a genetic disorder characterised by the growth of numerous cysts in the kidneys.
  • PKD is a progressive disease, meaning it worsens over time.
  • Over time, the cysts grow and multiply, replacing normal healthy tissue and causing the kidneys to lose their function. Kidneys can grow to 3 to 4 times their normal size.
  • Dialysis and/or kidney transplantation are treatments for kidney failure, but not a cure for PKD.
  • There is no cure for PKD. But there are some very clever people working to find one.

You can learn more about PKD from the USA’s PKD Foundation or the UK’s PKD Charity.

You can read more about my personal experiences with PKD by reading posts tagged "pkd".

Disclaimer: I am not a medical professional nor do I have any professional health or fitness qualifications. This post is about my own experiences and layman’s understandings of the day. The details and links shared below are meant as an informational starting point—not as medical advice. Please consult your medical team if you have any questions or concerns about your own health.

And now, onto the story...

Regular readers will know that I have polycystic kidney disease (PKD). What they might not know is that I also have PKD-associated PLD (that’s polycystic liver disease). In fact, 75-90% of people with PKD also have PKD-associated PLD.

However, that’s not really to say that I have “liver disease” in addition to my kidney disease as PKD-associated PLD is not quite the same as the “stand-alone” form of PLD. Instead, the cysts are a secondary effect of the PKD. That also means that liver cysts develop later than the kidney cysts. These cysts are often “incidental findings and clinically insignificant” and are more likely to appear with age. Liver cysts are most often found for the first time when someone is in their 40s.

Whilst both sexes are affected by these liver cysts, women are affected at a higher rate. It is believed that exposure to oestrogen during pregnancy, the use of hormone-based contraceptive pills, or oestrogen replacement therapies speed up the growth of cysts.

Although with all of that said, PKD-associated PLD does not generally impact liver function. There are, however, other issues and complications that individuals have to deal with because of the increased size of the liver (and the cysts). And that’s what the rest of this post is about: The other issues and complications.

[Note: References with links to full articles can be found at the end of this post.]

As for me, I don’t really know when cysts first appeared on my liver. But I do know that I was not really aware of the size of my liver until 2-3 years ago (41-42 years of age). I also know that since that initial “awareness” my liver seems to have grown. It is now about twice the “normal” size and I feel as if it has pushed my ribs out a bit, adding to my issues with “PKD belly”. I have never had children and my use of hormone-based contraception is almost nil. And as a widowed, 44-year-old woman, I don’t expect either of those statements to change. However, those two things (or, rather, a lack of those two things) might explain why I’ve only recently started to have any issues with my liver.

But what does all of this mean for my day-to-day life?

For the most part, not much.

From an outwardly physical standpoint, you would probably never notice. Even if I wear fitted clothing, no one would look at me and think I’m fat. And no one would think I look pregnant (a problem that some people really do have). At the worst, someone might think I am not fully toned and muscled. (And they’d probably be right as I’ve been a bit lazy lately. I must be more dedicated to my running routine!)

The problem, however, is that because of my liver I can no longer wear some of my favourite dresses. And I can no longer purchase dresses of a certain cut or style (think 1950s-inspired cocktail frocks) because I can’t close the zipper around my ribcage—or if I can zip-up, I can’t also breathe. And I can’t go up a dress size because then I would have a dress that zipped at the ribs but that was too big everywhere else.

However, I am attempting to alter some of my dresses—specifically the ones that have a band of fabric around the ribcage where the bodice and skirt portions meet. The band of non-stretch fabric is very unforgiving, but the remedy is fairly simple: Elastic. I’ve been trying to insert short lengths of elastic in the band at the seams where the bodice and skirt meet. This way, the fabric expands around my ribcage. I really only need a small amount of give right below my bust, but if I give myself an extra little bit of give, I can eat a bit more, too!

From an internal standpoint, my liver hasn’t made much of a difference at all. It has not impacted my abilities to exercise or breathe and I can run and jump and dance around without any physical awareness of my internal organs. (Unless I am at the end of a 10-mile run, in which case I am very aware that my lungs hate me!) Of course, my liver does have a little bit of extra space, as it can move into the vacancy left by my splenectomy! [Read more about running with PKD here.]

But whilst my activity levels haven’t been impacted by my liver, I have started to wonder about the connection between my liver and my appetite. I am generally fine with the amount of food I eat at mealtime, it’s the snacking that seems to be changing. It used to be that I would snack in the evenings, even though I wasn’t hungry. But now I feel that I am less enthusiastic about snacking. I might think about a snack, and I might even go to the kitchen and grab a snack. But I won’t always eat it all. For me, it is not a feeling of being too full to eat, it is more of a lack of greed. I don’t need to eat the snack, and I am happy to forgo it.

I know that a lot of people with PKD and associated PLD talk about a lack of appetite or a feeling of “fullness” despite eating very little. But I don’t really feel that this lack of snacking is because of my kidneys or my liver. And I certainly don’t feel “full”. I just don’t feel the need to snack. I have, however, wondered if my advancing age might have something to do with the change of habits. I have also wondered if it is because I am taking tolvaptan, which means I am drinking a lot of extra water, which might mean that I am feeling satiated without the need to snack. (There is that whole thirsty vs hungry thing to consider.)

Of course, my experiences are not the same as others I know. In fact, my experiences with the whole of my PKD has been quite different than those around me. So, to get a different view, I asked my sister if she would talk about her experiences.

My sister’s experience
My eldest sister, Veronica, has had a different experience than me. (For both PKD and her associated PLD.) Some of this could be down to the fact that PKD affects people differently, even in the same family. But some of the liver difference might also be that Veronica had two children and used hormone-based contraceptives for about 20 years. This means that she has had a lot more oestrogen in her lifetime than I have (even if you ignore our 4-year age difference).

Now in her late-40s, Veronica began getting comments about the size of her abdomen (and questions about being pregnant) from her mid-30s. With two very large kidneys and a liver that measures 3-4 times the “normal” size, it is not surprising that she has people rubbing her “baby bump” on occasion—especially as she can look at least 7-months pregnant at times. However, she does note with great pride that her size has helped her win those baby shower games where you guess the expectant mother’s size.

Like me, Veronica has a lot of cysts on her liver. Unlike me, however, she is very aware of her cysts. She has a large cyst (the size of a grapefruit) that sits quite near her ribcage. When it gets irritated, she spends the day in bed with a heating pad while taking shallow breaths because it hurts to breathe. Between her kidneys and her liver, her lungs feel compressed, making physical activity a struggle and a single flight of stairs can be a struggle. Compare that to me, a (non-elite) marathon runner, and you can really see how both PKD and PLD affect us differently!

Because of her size and shape, clothes can also be a struggle for Veronica. She has recently found a new style of bra that doesn’t have underwire (it hurts) and she has adopted leggings and other stretchy fabrics for her go-to trousers (comfort is key!). She has also found a line of tops that help to camouflage her belly and is thinking about wearing long, flowy dresses as another option. (Although, personally, I think she should wear a fitted dress that highlights her belly, then boldly sip a cocktail or glass of wine at a bar—just for the reactions! That is what I will do when/if my liver starts to make me look pregnant.)

Veronica has also had a lot more appetite issues than I do. She has two modes: eating a lot and eating very little—but she doesn’t always know which mode she will be in until she starts eating. And it doesn’t help that she experiences near-constant heartburn. She explained that she feels like she is in a perpetual state of third-trimester pregnancy—with all of the discomforts that involves. (Although I’ve never experienced that, it doesn’t sound pleasant!)

A similarity that I share with Veronica is our need to pee. Of course, our reasons for peeing are quite different. For Veronica, it is because her (massively!) enlarged kidneys and liver take up so much space in her abdomen that her bladder doesn’t have the space to store urine for long. For me, it is because I am taking tolvaptan, which (basically) tells my body to create more urine (hence creating a cycle of fluid intake/output).

So, I suppose that when I complain about my own liver issues (darn dresses!) I should be happy that I do not have the same level of problems that others have. That said, I am still frustrated with the fact that my liver means my ribcage is one dress size larger than the rest of my body. Still, I have a sewing machine and a creative streak, so I will be fine in the end. And if I do get to the point where I look pregnant, well, that is just an excuse to wear a fitted frock to a cocktail lounge!

As always, I am happy to hear from others in the comments below or by private message

Also: A great big thank you to my sister, Veronica, for taking the time to share her story. It is much appreciated!

Disclaimer: I am not a medical professional nor do I have any professional health or fitness qualifications. This post is about my own experiences and layman’s understandings of the day. The details and links shared below are meant as an informational starting point—not as medical advice. Please consult your medical team if you have any questions or concerns about your own health.

Chauveau, D., Fakhouri, F., & Grünfeld, J.P. (2000). Liver involvement in autosomal-dominant polycystic kidney disease: Therapeutic dilemma. Journal of the American Society of Nephrology. 11: 1767–1775. [[Available from: http://jasn.asnjournals.org/content/11/9/1767.full]]

de Miranda Henriques, M.S., de Morais Villar, E.J. (November 2015). The Liver and Polycystic Kidney Disease. In: Li X, editor. Polycystic Kidney Disease [Internet]. Brisbane (AU): Codon Publications; Chapter 17. [[Available from: https://www.ncbi.nlm.nih.gov/books/NBK373392]]


Hi, PKD runs in my family on my dads side. My sister has always had high blood pressure and she had pre eclampsia when she was pregnant with my niece 20 years ago but was only diagnosed with PKD 4 years ago. We recently found out by accident that my 19 year old son also has it, he was having a scan on his abdomen as he’s had a few digestive issues when the person doing the scan said the words ‘does anyone in your family have kidney issues?.....’ I went with him to see the renal consultant and he suggested I got tested. I was absolutely convinced that I would not have it as I have an underactive thyroid which runs in my mums side of the family so assumed I took after her if you know what I mean! Anyway I went for a scan and was told I have multiple cysts on my liver and cysts on my kidneys and I’m now waiting for an appointment with the consultant! I still feel quite shocked but I suppose I should be grateful that so far I’ve never suffered any symptoms (apart from starting Yoga a few weeks ago and feeling sick and weird afterwards which I’m thinking might have been due to pressure on the cysts?) Anyway I found your website as I’m also runner and now I’m also a runner with PKD

by Karen Stephens at 7:19pm (GMT) on March 12th, 2018

Hi, Karen. Thank you for your comment. I am sorry to hear that you and your son have PKD, but I hope that it doesn’t cause you too much trouble.

Personally, I would take the fact that you’ve not had any issues as a good sign. If you’re managing to keep your BP under control and you’ve not had any issues up to this point, it is quite possible that you can go quite some time without any complications. (Not everyone with PKD has problems, and some people make it into the 60s+ before any issues arise.)

I am not a Yoga person so I don’t really know how/if that would impact you, but I suppose that if you had large cysts, or cysts that sat “just right/wrong” you might feel them a bit. I would certainly add it to a list of things to ask your consultant about.

I’m sending loads of positive thoughts to you for your first consultant visit!

by Just Frances at 11:20am (GMT) on March 14th, 2018

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