Hello and welcome, Henry. More people on this site is better. Recently this website was changed drastically. It seems to me there's a lot less activity since then. I hope you can help some of us out.
I'm a first generation PKD. I'm the one with the mutated gene. I have eight siblings and nobody else has it in my family. I always worry about my children. I'm sorry to hear this has been passed on to both of your children.

Welcome Henry. I'm second Gen (at least) but the first to be diagnosed so young and have problems from it.

Hey there, welcome to the group. I don't know anyone else in my family that has it, so I'm just currently assuming it's myself alone. I have been told however that some people live their whole life never knowing they had it until much later in life if even that. I was diagnosed at 16 and I'm in my mid 20s now.

My father is one of nine children. He is the only one with PKD. His parents passed away prior to him finding out but there were never any signs that they had it. I have over 100 cousins and I am the only one that has it. I am one of three kids. My brother and sister (both tested) do not have it. But, as I said, both my sons do. It is crazy how the genes work! I found out when I was about 20. I am now 50 in Stage 3 kidney failure.

Currently I am in the Tolvaptan study. Have been in it for 3 years now. Looking at possibly another 2 years.

One of the hard things with PDK is finding doctors who really want to take the time to follow up and make the patient (me) feel important. I find that because there is nothing really they can do they have the attitude of "come back when something is wrong" I still am looking for the Dr. that has the interest to keep track and get ahead of the complications. Frustrating.

Hi Henry, welcome to the group. I am a first generation mutant, hoping my teenage sons don't have it but we haven't done testing. I'm 49, stage 4, diagnosed nearly 10 years ago. I would agree about doctors not being specialists in PKD, and not being very interested because they can't really do anything (apart from Tolvaptan now). After being hospitalised for pain management a few years ago (severe bleeding into kidney and liver cysts following snow shovelling - don't do it), I persuaded the renal team to monitor me as my GP was very uncomfortable with being responsible for me. I've gone from seeing them six monthly, to four monthly and now 2-3 monthly. The consultant even said to me, "I don't want to teach grandma to suck eggs", acknowledging that I know quite a lot already! That's OK, I try hard to keep informed and I understand that nothing can be done except monitoring the numbers from blood tests, keeping sodium intake low, drinking lots of water, avoiding aspirin and keeping blood pressure low. It's frustrating sometimes but it is reassuring to have regular contact with a doctor who knows at least something about the disease.

Yes I had a study visit this week and found out that the new blood pressure meds that the "specialist" put me on is not kidney friendly. Turns out he could of up'd the med I was already on because I was only at 1/2 the allowed dose. You should have seen the look on the docs face when I told her the "specialist" changed my meds and then said he didn't need to see me for another year with out any tests to see if new meds work.

Now I am getting lab tests done and she ordered a sonogram for measurements. Don't know if the new meds caused a growth in cysts and that is why I am larger than normal. I've put on 11 pounds over 3 months but don't seem to see(or feel) any difference anywhere else .... wondering if it is all kidney?..?..?

I know I have been VERY lucky with pain .... but this week I started with back pains. Not happy about that.

Now I just have to wait for tests to see what action we can do... where I live all the doctors work out of one office so I don't have the option to move ... I may just end up seeing the study doctor at her office ... a two hour drive... but at least I will be seeing someone who knows and cares about PKD patients.

My GP monitors my blood pressure and prescribes the medication recommended by the renal consultant. Of course, me being me, I check everything I'm prescribed! Diuretics are best avoided if you have PKD. ACE inhibitors or ARBs are best. I check my own blood pressure at home and report any changes to the GP or consultant. Seems to work well.

My liver is growing all the time - I can feel it as the liver capsule stretches. In the last couple of years my weight has not gone up but other parts of me are thinner (not good) so I guess underneath it all I've lost weight. Is your abdomen bigger? I can actually see the lumps and bumps sticking out and watch new ones develop. I don't need and imaging studies to tell me my organs are bigger. My kidneys also stick out at the back, but they might not do this if the liver were not taking up every scrap of space (and more) in my abdomen.

Sometimes the kidneys do have a growth spurt. Who knows why. Remember that caffeine is thought to be implicated in cyst formation and growth, so make sure you avoid it. Are you having renal function checked? Diuretics seem to accelerate decline in function in those with PKD.

Good luck with the tests and let us know how things go.

Tests were good. Not a lot of growth from last scans of 3 years ago. Which is positive news being I started drugs 3 years ago! However there is something with my gallbladder, it was suggested I see my Dr. I sent a message and got a reply that no intervention was needed. So I sent message back to the study doc asking what's up with the gallbladder and is it something I need to follow up on. Now waiting reply.....

Any one have gallbladder issues in relationship to PKD?

Henry, glad your kidneys are doing well. Do you know what the issue is with the gall bladder? There have been members of the group in the past who have had gall bladder problems but no association with PKD has been made. I have pain in my upper right abdomen when I eat but haven't had any imaging studies for several years so don't know what's going on in there. My liver obscures everything on ultrasounds anyway, so they're not very helpful. I think it's possible for a cyst to block the bile duct in the same way that gall stones might. Could this be your problem? Let us know how things go.

I don't have an answer yet.... waiting to hear back from study doctor as to what her concerns were for wanting me to follow up on it. Report reads dilatation of the CBD, tapering distally, no stone seen. I am still trying to get what that means. It was sad to read that I have cysts that are almost 7 x 6 cm large.

This old article suggests that dilatation of the common bile duct (CBD) may be quite common in PKD:

https://www.ncbi.nlm.nih.gov/pubmed/8604699

So it could be a cyst. I've never been given measurements of my cysts. I just know that I have big cysts and small ones, filling my entire liver and kidneys.

Thank you for the information, will discuss with doctor