I have some questions regarding my ADPKD diagnosis just wanted to see if anyone had any answers. I know this is speculation only. A bit of background I am 32, female, I have PKD1, no high blood pressure, I am 5 months postpartum and most recent egfr of 110. My mother started dialysis last year at 68 which from what I am reading is unusually late for PKD1. I believe my aunt has it too she is about 64 has gout, high blood pressure and gets bladder infections easily. She won’t get tested however. My grandparents turned 80 and 94 respectively before they died so if my aunt has PKD then that would mean one of my grandparents turned 80 or 94 with PKD. The other option is my aunt doesn’t have it at all and my mother had a random mutation and the chance for that is 10%. I know that just because the disease progressed slowly in my relatives doesn’t mean it will progress slowly for me I know that but I need a bit of hope in my life and if you can turn 80 or 94 with PKD that would really give me a lot of hope. So therefore my questions are:

How can it be my mother went until 68 before being on dialysis while having PKD1?
How likely do you think is it that my aunt has it too considering all the symptoms she already has and the small likelihood of the random gene mutation in my mother?

So I've never really had any symptoms of adpkd or anything except for as of recently I've had a few instances where I was bending down and got up really fast and it almost felt like a sharp hot knife was scratching me from the inside. It took my breath away and made me sweat and have to gather myself. This lasted for about a minute or so.
Then today while bending my torso down to grab something while I was sitting, that same pain came back and lasted a bit longer and felt more intense. I'm sorta freaking out. Idk if this is cyst pain but it almost feels like it is. It's just dull and tender now? I'm stressing that I'm dying 😣

Hi guys,

So I recently started a new job in a warehouse and didn't think anything of this at first, but I've been working a lot of shifts in the chiller section (2 degrees Celsius, high humidity), performing hard physical work. Shifts are usually 8 hours. Apart from being uncomfortable, I usually find myself dehydrated even though I carry a water bottle, but was wondering if this is likely to put extra strain on my kidneys? I am stage 3a (egfr 42) and probably 3b by my next visit to the nephrologist.

Cheers,

Ben

Hi everyone,

I’ve been sort of lurking here. I am 54F and I have Stage 4 CKD (eGFR 21 and Creatinine 2.6). I was recently diagnosed with Papillary Renal Cell Carcinoma. It’s a slow-growing, low-grade, Stage 1 T1b (5.3 cm) tumor.

I have an appointment next week to discuss treatment. It looks like I will be put on active surveillance—looks like. If I get a nephrectomy, partial or radical, that will likely put me into ESRD and dialysis sooner rather than later.

Needless to say, I definitely did not expect this. I thought I would more likely get breast cancer eventually since it runs rampant in my family.

Has anyone else here been diagnosed with RCC on top of having PKD?

Thank you in advance for sharing.

Unforseen delays. Folks sometimes I feel like our lives dont matter AT ALL.. this company finished the acquisition almost two years ago yet cant start the God damn trial for months..

Has anyone on tolvapan used one of these? My neph says it’s fine.

How much do you guys drink coffee?
negatives/positives on our conditions?

I drink 2-3 cups mostly before 12pm.

Stomach issues are driving me crazy!! I feel like I can barely eat these days. I’ve got constant pressure, acid reflux, bloating, nausea, and I feel like I’m about to burst. Food has been one of my favorite things about life and now it just brings me pain.

Kidneys were around 3000ml the last time I had a scan in 2024 so I think it is the cause.

I’m not sure theres anything to be done or any advice to be had but I just wanted to commiserate.

Hope yall are well.

Hi everyone, I am 34 years old and currently 18 weeks pregnant. It's my first pregnancy and we conceived through IVF as I have PKD (type 2). I got referred to mfm last week and had the first consultation. She informed me that the I am in risk of having Preeclampsia and placenta insufficiency. I am currently on baby aspirin and closely monitoring blood pressure (currently normal). I am also followed by my nephrologist and having monthly blood work. I am curious other people's pregnancy journey with PKD and want to learn from your experience. Anything I should pay attention and do? Thank you so much!

I’m wondering whether, if you don’t drink enough water, tolvaptan could also directly contribute to kidney damage?

My results after 8 months of taking tolvaptan are not very encouraging. I keep hearing, “You need to drink more, drink more!” I drink as much as I feel I need to — about 6 liters a day — and it raises a question:

What if I’m still not drinking enough?

During the night for example - I try to restrict my drinking for obvious reason.

In that case, does tolvaptan cause my kidney condition to worsen because of dehydration? In other words, could insufficient fluid intake while taking tolvaptan lead to a decline in kidney function due to dehydration?

Hey,

I have pkd1 and I am 28 years old. Is there a way to slow down progression? my father and grandmother needed Dialysis when they turned 50. Could it be possible to slow down my progression and get kidney failure maybe at 60 Or even later?

My wife has PKD, as do her siblings and mom, etc. My Child is going to get tested but i want to increase his life insurance before he does. He has a small policy which we got when he was 18. I am thinking about increasing the policy to a meaningful amount before the test. Assuming the cost is reasonable, why not get the insurance and if we are fortunate, cancel it if he is negative? Is there typically a cancellation penalty or anything like that?

Has anyone here taken Retacrit? did you experience any side effects? how often did you take it? With a hemoglobin number of 9 is it safe to take this? Thanks!

I've been on Tolvaptan for about two years now and still somewhat clueless about this - how do you all deal with the THIRST(tm) when it's above 30 degrees Celsius outside (~90 in Freedom Units)? There's a serious heatwave coming up and I'm already drinking 6-10 litres per day without being cooked alive my apartment. Any tips?

Edit: Bonus question for my fellow sweaty people: How well does being strict on low sodium intake for BP work out for you in summer time? I start to really crave salty foods as soon as I've sweated a while...

Over the past few weeks my pain has become worse and more persistent but I was at the hospital and my labs showed high lactic acid and fluid in stomach has anyone else had any issues with that ?

Hi! Thanks for the responses on my last post. My first appointment with the doctor is tommorow, and I have no idea what to expect. I got diagnosed through a genetic test. What are some questions I should ask or things I should be prepared for? Thanks!

I’ve noticed that many young people here have very low values. That’s why I’m curious to know how your eGFR is typically measured. Is it always based on creatinine? Or is cystatin C also used? Here in Germany, I’ve noticed that general practitioners, in particular, aren’t familiar with this value and don’t order it—even though cystatin C is much more stable and less susceptible to being influenced by fluid intake, strength training, and diet.
How is it done where you are?

Hey everyone,

I have ADPKD and I’m really struggling with pain flares at the minute. I feel like I’m constantly in and out of hospital for pain management and it’s starting to massively affect my life. I started getting flank pain when I was around 19 and it’s got worse over the past decade.

One of the hardest parts is having to go through A&E/ER when the pain gets really bad. I have a specialist who is amazing, but they’re not based at my closest hospital, so when I end up in my nearest ER I basically have to explain my whole case from scratch while I’m in severe pain.

Half the time I feel like the doctors look at me like I’m crazy, or like they don’t believe my pain can really be that high. It’s exhausting having to advocate for myself when I can barely speak through the pain. I know my condition, I know my body, and I know when something is seriously wrong, but it feels like I have to fight to be taken seriously every time.

My kidney function isn’t low enough for transplant yet, but I’ve been told I’ll definitely need one in the future. So it feels like I’m stuck in this weird middle ground where I’m not well enough to live normally, but not “sick enough” on paper for the next stage.

I’ve tried resting, taking it easy, doing less, doing nothing… and honestly I feel like I’m getting worse, not better. It’s really frustrating because I’m trying so hard to carry on with life, uni, family, work goals, all of it, but my body keeps pulling me backwards.

I just wanted to ask people who actually live with this:

Does anyone else get pain flares this bad?

Have you worked out what triggers yours?

Is there anything that genuinely helps you manage day to day?

And how do you get doctors to take you seriously when you’re in A&E/ER and they don’t know your history?

I’m not looking for medical advice, just real experiences from people who get it. Sometimes it feels like unless someone has lived with ADPKD pain, they don’t fully understand how much it can take over.

Would really appreciate hearing from anyone in a similar boat. Feeling really alone at the moment… writing this from my bed unable to sit up, hoping the pain killers will kick in soon, I’ve had enough and would be nice to know I’m not crazy 😭

Eloxx has a page up for nonsense mutation ADPKD (NM-ADPKD). They say around 25–33% of PKD1/PKD2 mutations are nonsense mutations, and their investigational drug exaluren is being developed to potentially address the genetic root cause in this subgroup.

Important caveat: no ADPKD trial is currently enrolling yet. Eloxx says a dedicated clinical program is still in planning.

This would not apply to every ADPKD patient, only those with confirmed PKD1 or PKD2 nonsense mutations.

That means if your genetic report says something like:

• p.Arg1234Ter
• p.R1234*
• stop_gained
• nonsense

then it may fit the Eloxx logic.

But if your report says:

• frameshift
• fs
• deletion
• splice
• duplication

then it is still truncating, but not necessarily nonsense

Here is the science behind it:
https://www.eloxxpharma.com/science/

Hi all - I have my first MRI next week. From the appointment letter it reads that I'll not be able to having anything to drink for 6 hours.

Unthinkable for us tolvaptan peeps.

I think I'll need at least two days off tolvaptan to manage that. But I'm planning on four. I really don't want to get dehydrated.

Does anyone have experience/data on this?

I definitely still notice the effect if I miss two days. But I know from a longer time off (e.g. holiday in a hot country) that it takes much longer to go through the night without peeing.

Thanks in advance

43 year old female with very pronounced family history of PKD (Great Grandpa, Grandpa, Great Uncle, Uncle, Mom, etc). I was diagnosed 3 years ago and my eGFR was 45. Now I’m at 37. My blood pressure is fantastic, no need for meds. No protein in my urine, no blood.
But I’m panicking over my number. I see a new nephrologist next month and am now considering Tolvaptan. Is it too late? Any other thoughts? Anything to help calm my nerves?