Polycystic Liver Disease

Ruby Vee, BSN · Nov 27, 2016

Were the rib fractures because of the enlarged liver mechanically pressing against them? Because no, I've never seen that and wouldn't have believed it possible. Although the majority of my experience is in CCU/CTICU so I haven't seen that many liver patients.

la_chica_suerte85, BSN, RN · Nov 27, 2016

So, according to the UK's PKD Charity, rib fractures may be a rare complication of polycystic liver disease.

Polycystic liver disease

Since the pt also has kidney problems, there may be a metabolic issue with her bones causing them to be more prone to breaking. But, I guess it's just that the cysts get that big and are that fibrous that they just break the ribs. Eek. :confused:

Kooky Korky, BSN, RN · Nov 28, 2016

Ruby, yes she says they are. Shocking!

Kooky Korky, BSN, RN · Nov 28, 2016

So, according to the UK's PKD Charity, rib fractures may be a rare complication of polycystic liver disease.
Polycystic liver disease
Since the pt also has kidney problems, there may be a metabolic issue with her bones causing them to be more prone to breaking. But, I guess it's just that the cysts get that big and are that fibrous that they just break the ribs. Eek.

Eek is right! I was just shocked. I really started wondering if someone had hit her, pushed her down, wondered if she had fallen (she denied anything along those lines - violence or falls).

I think you are right about the metabolic bone issues. I need to get into her history some more.

Kooky Korky, BSN, RN · Nov 28, 2016

So, according to the UK's PKD Charity, rib fractures may be a rare complication of polycystic liver disease.
Polycystic liver disease
Since the pt also has kidney problems, there may be a metabolic issue with her bones causing them to be more prone to breaking. But, I guess it's just that the cysts get that big and are that fibrous that they just break the ribs. Eek.

Fibrous? Hmm, I was thinking they were filled with fluid. I will check out her imagining and other test results.

There is a procedure they are thinking to do - it's called de-roofing. With an endoscope, they go in and drain some of the cysts to make them smaller. I guess that won't do anything to help liver function, but it should help relieve pressure and swelling. It is really something that is very new to me.

Kooky Korky, BSN, RN · Nov 28, 2016

Thank you for the informative link, La Chica Suerte85. I was glad to see that normal liver cells are not affected, that the cysts form in other places in the liver in a way such that they do not harm liver function.

KatieMI, BSN, MSN, RN · Nov 28, 2016

Fibrous? Hmm, I was thinking they were filled with fluid. I will check out her imagining and other test results.
There is a procedure they are thinking to do - it's called de-roofing. With an endoscope, they go in and drain some of the cysts to make them smaller. I guess that won't do anything to help liver function, but it should help relieve pressure and swelling. It is really something that is very new to me.

It can help. These cysts are thin, fluid-filled cavities which press against healthy tissue and so with time destroy it, so removing pressure helps ror a while. Emptying cysts is a common palliative procedure. Otherwise, it is genetic and largely uncurable disease, usually manageable by transplant(s) when it is not too late.

Kooky Korky, BSN, RN · Nov 29, 2016

I called today to a major, world-renowned, magnet facility to ask about becoming a donor.

Well, hush my mouth. The first person (and I am controlling myself to not say "idiot' - oops) who answered the phone in the Liver Transplant section of this "fantastic" hospital could not tell me the procedure to get tested to see if I'm even close to being able to donate liver tissue. She then said the pt must be on "the List". I asked, as one might, "What List". UNOS, came the reply. I asked "What is UNOS". It's the list. Well, how do I contact the list? Where are they located? Could you point me in the right direction? "Well, who is her doctor?" came the reply, and you get the idea. Then the charmer hung up. (I didn't know the doctor right off the top of my head

because I didn't know getting tested would require me to know the pt's doctor or whether the pt was on UNOS).

Not being too happy about the lack of information and flabbergasted that this world-class hospital was really just a schlock joint where they don't seem to really want donors, I called back and got the RN Director of the program. She was much more informative and I know now the next steps I have to take. But I am also shocked to learn that this particular hospital only works on children with hepatopathology. Also, she told me that there are enough cadaver donors these days, so that people like me, who want to help a specific person, are not all that sought after. She said liver was not like bone marrow or even kidney transplantation, in that

She suggested I call other facilities on either East or West coast or see if UNOS can offer any direction.

I thought people were just crying for organs, but I guess I am wrong. Or maybe there's not enough money the hospital can make. I believe donors really just are no longer needed except for kids. Maybe I am just hurt and irked but I will think long and hard before feeling sorry enough for someone else again to offer this kind of self-sacrifice.

Live and Learn

KatieMI, BSN, MSN, RN · Nov 29, 2016

Oh, Kooky...

people ARE crying for organs, but transplant medicine is an area full of, to say that as mild as possible, totally crazy people more than anything else excluding acute mental health crisis centers. The war stories I'd heard while working there put practically everything else to shame.

Being very short, in case of polycystic kidneys one of the main probelm is that these cysts grow up everywhere in internal organs. So far, we cannot set up a human bodyshop and transplant firts a kidney, then liver and then pancreas, then cut off spleen, etc. Unfortunately, some of these people do not qualify for renal transplants for this reason alone, plus there come usual disqualifying factors of h/o non-compliance, substance abuse, not being willing to live within 30 min direct drive from the center, etc.

Now, as a random person, you have approximately 1:100.000 chance to be another random person "perfect HLA match" for transplant (important for kidneys) and add to that blood antigens. In the list of US bone marrow donors, approximately 1 out of 430 persons eventually becomes a donor; other 429 never have a patient for whom they would be a good match. Transplant centers do test random strangers but mostly for research and also for avoiding family feuds and litigations, honestly.

The List(s) are ranking lsts of the patients avaiting transplants. They are run according to extremely sophisticated algorythms and not available not only for public but for 90% of people directly involved in the process to avoid cheating. A few years ago a family managed to bump their dying daughter to the very top of the adult list so that the child could get adult organ; they had to go almost to Congress for that.

The best thing you can do if you would like to donate is to register your driver license as "donor". I do understand your noble move and passion to help this unfortunate patient, but by simple and clear math your chances to be a "match" are not that great, leaving alone all other process' sophistications and technicalities.

Kooky Korky, BSN, RN · Nov 30, 2016

Oh, Kooky...
people ARE crying for organs, but transplant medicine is an area full of, to say that as mild as possible, totally crazy people more than anything else excluding acute mental health crisis centers. The war stories I'd heard while working there put practically everything else to shame.
Being very short, in case of polycystic kidneys one of the main probelm is that these cysts grow up everywhere in internal organs. So far, we cannot set up a human bodyshop and transplant firts a kidney, then liver and then pancreas, then cut off spleen, etc. Unfortunately, some of these people do not qualify for renal transplants for this reason alone, plus there come usual disqualifying factors of h/o non-compliance, substance abuse, not being willing to live within 30 min direct drive from the center, etc.
Now, as a random person, you have approximately 1:100.000 chance to be another random person "perfect HLA match" for transplant (important for kidneys) and add to that blood antigens. In the list of US bone marrow donors, approximately 1 out of 430 persons eventually becomes a donor; other 429 never have a patient for whom they would be a good match. Transplant centers do test random strangers but mostly for research and also for avoiding family feuds and litigations, honestly.
The List(s) are ranking lists of the patients avaiting transplants. They are run according to extremely sophisticated algorythms and not available not only for public but for 90% of people directly involved in the process to avoid cheating. A few years ago a family managed to bump their dying daughter to the very top of the adult list so that the child could get adult organ; they had to go almost to Congress for that.
The best thing you can do if you would like to donate is to register your driver license as "donor". I do understand your noble move and passion to help this unfortunate patient, but by simple and clear math your chances to be a "match" are not that great, leaving alone all other process' sophistications and technicalities.

Hi, Katie, and thanks for this information and for caring. I am going to look up UNOS and learn as much as I can because I really was startled about what I learned on that phone call and by what you have posted, which is most informative. Thank you again and God bless.