When do you think adult children of PKD should be tested?

[okarol]

As you may know, my dad had PKD, died at 28 years old, so obviously had a very aggressive case. My 2 sisters and 1 brother have inherited the disease. I wondered about this question because my sister has PKD, but her 2 adult children (son is 34, daughter 32) do not know if they have PKD. My other sister with PKD has a 25 year old daughter who also has not been checked. Is there any reason to get diagnosed if you have no BP problems?
I have actually never been checked, but have always had low BP, and Jenna's neph now says at 54 years old it's unlikely that I have it. (I avoided any diagnosis so as not to put my insurance eligibilty at risk, which was advice given by a doctor friend.) A side note, in case someone is wondering, Jenna's CKD is from a bad bladder, not PKD. So after all that, here's my question: How do have the discussion with your offspring about PKD, and at what point should they be evaluated?

[willowtreewren]

This is a great question!

We advised our daughter NOT to get tested if she did not show any symptoms, but when Carl got listed, she went ahead and got tested in order to give him a kidney, if possible.

My niece and nephews whose mom has PKD have NOT been tested. She is not a transplant candidate and the oldest is too scared to find out.

Getting insurance for us was REALLY an issue after my husband innocently got tested when his father was diagnosed. He was basically "un-insurable" for a while. We established a group plan at work and after a year he was able to join it.

If for no other reason than the insurance issue, I would advise against being tested.

Maybe others have some compelling reasons on the flip side. I'd like to hear.

Aleta

[charee]

Both my sons were tested just after i found out i had pkd my dr wanted them tested they were only 12 and 14 at the time and they both have it they have yearly blood tests and keep a check on there blood pressure. so far so good.

[idahospud]

  I had ruby my doter teased when she was 15 . She has polups thy did not say moor than that because of inc. But now I know that theres a good chains she will have it we woch it and remind the Dr. win its time for her fiscally She's 23 now and theres bin no Chang so fare Carol

[Wattle]

It is a personal choice on screening for PKD.
I find it a very difficult topic. There is a huge amount of guilt and responsibility I feel towards my childrens health.
The insurance is an important factor. Although you maybe holding them back from medical advances in retarding the cyst growth. There is a clinical trial going at the moment which is looking into the reduction of the cyst growth. I would prefer they had the chance to manage the problem before symptoms arise.

There is only so long you can hide your head in the sand.

[okarol]

I am sorry Wattle. You have to know that you're not responsible, but you're not the first person I have heard of that feels guilty about PKD.
My brother had his son tested before he turned 8 years old. He has PKD, he has not tested the 2 younger daughters.
The clinic trials are not for children, but hopefully will help young adults, if they work.

[Wattle]

The clinic trials are not for children, but hopefully will help young adults, if they work.

Your right Okarol, the trials aren't for children but are looking promising in the results so far. Your sisters kids could benefit from the trial. I was showing symptoms way before my thirties and would have jumped at any chance to reduce the cyst growth. I am sure you are well aware of the drugs they are using in the trial, so I will not go into it.

Your nieces and nephews are not children anymore and I am sure they have thought the process through. When they are ready they will get themselves tested. Maybe point them in the direction of the PKD Foundation wed site and the trials available.

I hope they are all free of PKD. 

[okarol]

That's the reason I brought up the question, because of my family. My sister's have to handle their family situations as they see fit. But I am going to talk with them about it.

[paul.karen]

I say if it runs in your family get tested.  it is only a blood test and if you find out you have it you can be proactive with what you do with your body.  As far as eating better and excersize.

My older brother and two younger brothers (both adults) refuse to be tested.  They say they dont wana know if they have it.  I also use to say this unitl i almost had a stroke. 

A blood test is such an easy test.  Just Do IT.

[willowtreewren]

There are insurance ramifications, though. After my husband was tested and found positive, our insurance rates went sky high. And he was "un-insurable" so we couldn't change companies. For a while there almost all his payroll was going to insurance. All because of the PKD diagnosis.

Now our daughter is going through some of the same stuff since she was tested to see if she could be a donor (and was found positive). We just educated her all along about how to be proactive. The drill is good for you even is you don't have PKD.

Aleta

[Meinuk]

I am second generation PKD.  I was diagnosed with enlarged kidneys as an infant (my medical records have my diagnosis as being consistent with Bright's Disease - an old fashioned name for CKD).  My urine protein and creatinine were tested every six months.  When I was six, I started having kidney infections, and then the testing began, first with x-rays then IVP etc etc etc.  I was put on prophylaxis Septra (bactrim) for infections at age 7, and was genetically counseled about my disease.  My mother died with PKD -  ESRD when I was 13.

I have had medical insurance most of my life.  First government employee insurance (through my father's family plan) and then in college (through the university health service) then always through work. When I was living in Europe, I was self pay - I am lucky that I was never sick.

I chose not to have children.  But my advice to those of you who have young kids - keep them healthy and keep them informed.  They see you and what you are going through - that is only part of the story.  They did not know you when you were healthy (like they are now).  encourage them to live their lives, but also encourage them to go to the doctor, have routine blood and urine tests.  If the disease should manifest early (like mine did) then you will have no choice in having it charted that they have PKD.

Most people live a very long life before PKD manifests.  If you are careful, a healthy lifestyle can stem the disease.  I was 40 when I first started dialysis - even though I was walking around with 15% GFR for almost 5 years, and I have been <25% for most of my adult life.

Genetic testing will just confirm the obvious.  I am against it.  A low protein diet, cutting caffeine and watching BP and weight can work wonders.  (I always wonder if I had cut down on the coffee during school and not gained 60 lbs when I came back to the US - could I have bought even more time?)

Find and keep a great nephrologist (even if this means going to another city twice a year).  The HALT study is good, and showing progress - and only for adults, but no matter what, I would let a kid be a kid - they have the rest of their lives to be in studies, and if the PKD is aggressive enough - you'll have to treat it anyway.

And finally, if you are a parent with PKD suffering with guilt, STOP IT NOW.  Your kids see a lot more than you know.  Stop blaming yourself.  I, for one, am happy that I was born.  Yes, it sucks that I have a debilitating genetic disease, but everyday, I am thankful to the woman who had me.  I not only got her disease, but I got her hands, her laughter and her capacity for life - it was worth the trade off. 

I just reread the original question - sorry if I got off track - the same goes for adult children.  Keep them healthy - they can decide if they need to be in a study - after all, they are adults, they can make an informed choice.

[okarol]

Thanks Meinuk - your post is very good. It reminded me that even I feel guilty for Jenna's renal failure, and we don't have PKD. But I often wonder "Did I miss something that could have made a difference?" or "Did something in the pregnancy cause this?" even though I know I was very health minded when I carried her, and a very attentive mom - I still have that angst because she has had so much to deal with so young. But you're right - stop it!

[twirl]

my nep doctor told me not to go looking for it -
Missy's were seen at birth -- she is the one with the diaphmatic hernia
Stasie's were seen later in her teen years as she had female problems
Bubba - we do not know yet -- he is 19

[paris]

Meinuk, that was a lovely post filled with wisdom.   It brought me to tears.   

[twirl]

my twin sister and my older sister did not get PKD
my mother and her father were the only ones in that generation to have PKD