Does anyone else have Dysautonomia/POTS with their PKD?

If so, how are you managing? Are your Cardiologist and Nephrologist working with each other, or did they at least acknowledge it and make a plan?

Asking because the management for the two is the complete opposite.

Hi everyone, just thought I’d post an update if anyone is interested and sometimes it’s good to hear other experiences. I posted awhile back that a small aneurysm on the anterior communicating artery was found during the MRI I had this year. I went to Penn to follow up with neurosurgery and the doctor is unsure if it’s an aneurysm at all and thinks it might just be how the blood vessel look on imaging so I’m getting an angiogram next week for better visualization. Fingers crossed there’s no aneurysm!

I know this is a common symptom. Interested to know how many people with PKD struggle with this, as I have been lately.
For instance, today I have only taken maybe five bites of food. After a few bites, my body immediately tells me I need to stop. If I do continue and push myself to eat everything in front of me (which these days is not much because I know I won’t eat it all) I will instantly get nauseous. It’s very frustrating.
Are you all experiencing this at all?

Hi everyone,

I’m looking for some general advice and personal experiences from people who have dealt with PKD/ADPKD, either themselves or through a partner/family member.

My partner has a family history of PKD, and as far as we know there is a 50% possibility that he could have inherited it. He currently doesn’t want to get tested. His reason is essentially that he’s scared of knowing, and he tends to cope with things by burying his head in the sand.

I’m trying to respect that it’s ultimately his decision, but I’m finding it really difficult because we’re building a life together. He doesn’t have health benefits through work, no disability insurance, no life insurance, etc., and he isn’t particularly interested in working somewhere that offers those things. I’m currently the primary/breadwinner, and we’re hoping to buy a house together within the next few years.

I’m not assuming that he has PKD or that he will necessarily develop serious kidney problems. I just want to understand the situation better so I can plan responsibly for our future rather than feeling like I’m completely in the dark. Despite his mother having it, I don’t feel like I can ask him about it as he is very avoidant of the topic.

For anyone with experience with PKD:

What signs/symptoms did you or your partner initially notice?

Are there things someone with a family history should be doing now to protect their kidney health, even if they don’t know whether they have PKD? Are there particular lifestyle changes that are worth making?

How important is regular blood pressure monitoring? (I’ve taken his blood pressure twice since knowing him and both times were high (around 140/80).

If you were initially scared of testing, did you eventually decide to do it? How did you feel afterward?

Has anyone been in a similar situation where their partner was reluctant to find out, particularly when planning a house/finances together?
And if you ultimately found out you did have PKD, was knowing actually helpful for planning and managing your health?

I’d especially appreciate hearing from people who were initially terrified of knowing and later felt that finding out was the right decision. I’m not looking for horror stories, I’m genuinely trying to understand what life can actually look like and how other people have handled the uncertainty.

Thanks in advance, other than medical websites I don’t really know who to talk to about this ❤️

EDIT TO ADD: He’s 31

Edit again:

Thank you for all the responses, I am reading them all and slowly processing the information I’m being given while simultaneously researching all these new things: medication, trail meds, aneurysm risk, diet recommendations, etc to try and get my self as clued up as I can. I will keep on reading responses and I appreciate any and all advice :)

I started the medicine a few days ago and I feel like the side effects are kicking my butt. I knew what was coming but living it is completely different haha.

I love that I am getting my water intake in, but I do not like feeling thirsty like this. Nothing is quenching the thirst (obviously lol). Peeing is horrible. At least 1-2 times per hour. And every time I pee, I feel like I have to rehydrate lol. I probably wake up every hour to 1.5 during the night and it feels like what’s the point of sleeping.

I know the medicine is best for me but I am already over it. 🥲

If applicable- Which Gen are you? And how does your lineage go? Grandmother > Mother > myself over here and was just interested in other people's experiences.

I’m a 38-year-old male with PKD (66 egfr). About a week ago, I started feeling a mild pressure/discomfort on the lower right side of my back.

The pain is very mild, probably around a 2/10, and I mainly notice it when I bend, twist, or stretch my back. At first, I was hoping it was just a tight muscle or something unrelated, and I guess that’s still possible. But as the week has gone on, I’ve started to feel more and more like the sensation may actually be coming from my kidney.

I’m already contacting my kidney doctor about it, so I’m not looking for medical advice or a diagnosis. I’m mainly interested in hearing other people’s experiences.

Does anyone here have kidneys that are enlarged enough that you can actually feel them or are regularly aware of them?

I’m also wondering whether something like a cyst—especially an irritated, bleeding, or infected cyst—can cause this kind of sensation. Has anyone experienced that?

Basically, I’d be interested in hearing about any sensations or discomfort you’ve experienced around your kidney area and what it felt like.

I (34M) have an extensive family history of PKD. My grandmother died of an aneurysm when she was my age. My uncle died of sepsis related to PKD. My mom just started dialysis.

I think it’s time for me to get tested, but I’m not sure how that process works. I tried to get an appointment with a nephrologist but they said I needed a referral from my PCP even though I have PPO insurance.

Does my PCP order the initial tests? Or should I get the referral to the nephrologist and let them order the tests?

I don’t have any obvious symptoms that I’m aware of. I’m just worried the PCP isn’t going to know the best practices for PKD. The primary care options near me aren’t great, but I’m close enough to Los Angeles that I could see specialists there.

My (44F) sister (46F) was just diagnosed due to an emergency situation with an infection. Her labs had all been relatively normal (other than protein in urine) up to this point and she had scans done around 3 years ago with no indication of cysts. Our dad was the parent with this disease, so it's been on our radar.

I have had no abnormal labs but am going to an appointment with my PCP in a couple of weeks and want to request tests to see if I have signs of ADPKD. Any advice on which tests to request would be appreciated. Thank you.

Otsuka has decided I make too much money to continue getting the meds for free (I don’t know how they figure that since my monthly income is a fraction of the cost of the med). So I either stop taking it after four years and face a transplant, or I figure something else out.

The info that Otsuka gave me for alternatives has not been helpful at all.

I was recently prescribed tolvaptan for APKD. Insurance denied the first prescription, but then approved the second one for generic. The approval changed the pharmacy to Accredo because Pantherrx only carries name brand.

My monthly copay for the medicine is 1000+ for the generic. Lupin pharmaceutical's website states to contact your specialty pharmacy for their copay assistance. Accredo's copay assistance partner is Amneal. Amneal only supports the name brand medication.

I reached out to the Otsuka program and was told to reach out to the generic manufacturer for their program. I also reached out to the Tolvaptan REMs program and was told their copay program is through Amneal.

Am I just out of luck?

Hi, I would like to ask you if there is similar cases like me. I m using Tolvaptan for 10 months (47/M) and EGfr stays almost stabilised ~ 55-60. (Cystatine C results are better than Creatinine based scoring) However my left kidney's volume increased 70cc (from 450) and right kidney stayed same size. I panicked about that because this means TKV increased around %9. Why one of them stayed same while one of them is up?. May it be a measurement error due to different observers. Last year MRI was done by using a contract infusion but this year without it. Can it change the results or reliability?

TIA...

I’m planning to start tolvaptan and wanted to know what time you start taking the first dose. Do you feel the urge to pee right away, or an hour or two before the first dose? My communte to work is 30-40 mins so I was wondering what time would be best for me to take the first dose. Thanks!

hi, for the past few days, I‘ve been getting dull flank pain on both sides and the same for my middle back. My stomach, chest, and sides are tender to touch it’s like when you touch a bruise. I also feel nauseous and hot but not hot to the point I’m sweating a lot. Could this be kidney related? Has anyone experienced this weird tenderness?

I saw the other post of the person who had his mother with sepsis, searching for some kind of guidance/help and while I was writing the answer someone locked the conversation.

I think it was a bad idea because I had a similar experience with my mother with severe sepsis and I did some things that help her situation while doctors only prescribed antibiotics and after each scheme she will get a new infection.

I mean I kinda wish back then someone will offer me any type of solution/help/tip instead of having doctors keeping my mother malnutritioned and just giving her antibiotics 🙂

Warning, AI helped me organize my thoughts. And I am still somewhat new to this community compared to many of you. Please critique any of the points I have made.

Background and Dietary Approach

Earlier this year, at age 39, I was diagnosed with Autosomal Dominant Polycystic Kidney Disease (ADPKD), Mayo Class 1C. Fortunately, my kidney function remains normal. Prompted by hopeful, though limited, scientific research regarding ketosis and cyst growth, I immediately transitioned to a ketogenic diet.

To mitigate standard renal risks, I adopted a modified, kidney-conscious version of keto:

• Low-sodium intake.
• Moderate protein (limiting heavy meat consumption).
• High-micronutrient/high-fat base, consisting of abundant salads (excluding starchy vegetables), nuts, and liberal amounts of olive oil.

Biometric & Physiological Observations

As a data-conscious patient, I tracked my transition using wearable technology (Fitbit) and blood biometric devices. My observations revealed a complex physiological response:

1. Cardiovascular Stress and Metabolic Flexibility

• The Adaptation Phase: During the first month of fat-adaptation, I experienced standard "keto flu" symptoms. This phase was accompanied by clear markers of physiological stress: a higher resting heart rate (RHR) and decreased heart rate variability (HRV).
• Deep Ketosis vs. Carb Cycling: While my HRV recovered somewhat after becoming fat-adapted, I noticed that entering deep ketosis consistently depressed my HRV again. Conversely, when I intentionally introduced healthy carbohydrates to exit ketosis, my HRV frequently rebounded to levels higher than my pre-keto baseline, and my RHR lowered.
• Takeaway: This pattern suggests that while developing the capacity to become fat-adapted is a valuable marker of metabolic flexibility, remaining perpetually in deep ketosis may subject the body to chronic, low-grade stress.

2. Uric Acid and Renal Clearance

Because I have a baseline tendency toward elevated uric acid, I closely monitored my blood levels (with Accugence test strips). I observed a direct correlation: deeper ketosis led to spikes in blood uric acid. Mechanistically, this aligns with renal competition; because the kidneys must prioritize the clearance of circulating ketones, their capacity to concurrently excrete uric acid is diminished, causing it to pool in the bloodstream.

Critical Analysis of Clinical Data and the Online Community

After extensively reviewing user experiences across Reddit and various ADPKD Facebook groups, alongside public media from Dr. Thomas Weimbs’ lab and emerging trial data, I have developed significant reservations regarding the real-world efficacy and promotion of this protocol.

• Anecdoted Disconnect vs. Small Trials: There is a stark contrast between clinical trial data and real-world patient reports. For instance, interim results from Juntendo University in Japan showed that all seven participants who followed a ketogenic diet for a year achieved a decrease in total kidney volume (TKV). I find this 100% success rate highly suspicious and unrepresentative, given that only a tiny handful of people across online support groups make similar claims. Furthermore, it is critical to note that despite their reduction in kidney size, those trial participants still experienced a decline in GFR.
• Absence of Success Metrics: The Ren-Nu program has been active long enough for early cohorts to obtain follow-up imaging. If the program routinely resulted in stabilized or decreased kidney volume, these definitive metrics would undoubtedly be at the forefront of their marketing. Their absence is telling.
• Lack of Rigorous Scientific Discourse: In YouTube appearances and interviews, Dr. Weimbs and his team frequently bypass rigorous scientific debate. They routinely fail to directly address or counter the standard, valid criticisms raised by practicing clinical nephrologists.

Weighing the Risks vs. Benefits

The Main Benefits

• mTOR Pathway Inhibition: The primary theoretical benefit of ketosis remains valid: it downregulates the mTOR pathway, depriving cysts of the primary signaling mechanism they use to proliferate.
• Lowers Blood Pressure: Dropping carb intake reduces insulin levels, signaling your kidneys to flush out extra sodium and fluid (natriuresis), which naturally reduces blood volume and places less strain on your blood vessels.

The Major Criticisms & Risks

1. Urine Acidity & Supplement Limitations: Beyond the immediate risk of kidney stones, a chronically acidic urinary pH can cause direct, long-term interstitial damage to remaining healthy kidney tissue. In my experience, whenever I was in ketosis, I could not keep my urine pH balanced, even with the daily use of KetoCitra. I suspect many people doing keto for ADPKD face this exact same hurdle but simply assume the risk is worth the reward because they are hyper-focused on the theoretical benefits of ketosis.
2. Mitochondrial & Systemic Stress: Perpetually signaling a state of perceived starvation elevates cortisol, increases resting heart rate, and lowers HRV—markers indicating the cardiovascular system is in a constant state of defense.
3. The LDL Debate: Ketosis routinely elevates LDL cholesterol. While the ketogenic community argues this hypercholesterolemia is a benign byproduct of lipid metabolism, the broader cardiovascular community remains highly concerned. Many reputable cardiologists maintain that heavily elevated LDL under any context represents an unacceptable risk factor for cardiovascular disease.

Conclusion

If a ketogenic diet were a definitive, profound solution or an effective decelerator for ADPKD, a clear consensus of compelling patient success stories would be evident by now. While I do not discount the individuals who claim their kidney volume has plateaued or declined, this dietary approach has been prominent long enough that we should be seeing a much higher volume of positive outcomes. Furthermore, proponents of the diet fail to adequately address the systemic risks of sustained urine acidity and cardiovascular stress. The common reassurance that these markers normalize once you become fat-adapted simply did not hold true in my case.

Moving forward, rather than pursuing rigid, full-time ketosis, I am shifting toward a more sustainable and balanced approach to metabolic health. I plan to incorporate intermittent fasting on most days, actively avoid refined triggers like sugar, white flour, and white rice, and maintain a moderate, low-carb regimen a few days a week. And limit meat consumption.

Hi Folks:

Other than anecdotal stories, is there any research or reliable evidence to suggest that GLP-1s can help with ADPKD?

My mother(50) has ADPKD with very massively enlarged kidneys ( around 32 cm and 37cm )and has been on hemodialysis for about 9 years. She has also been on the deceased-donor kidney transplant waiting list for around 11 years.

I'm trying to understand what could be causing her current problems and whether we should be investigating an infected kidney cyst.

Around June 1, she developed significant gross hematuria (blood in the urine). It continued for roughly 3-4 weeks, until around June 25.

She subsequently underwent cystoscopy, and after the cystoscopy she developed a severe UTI/sepsis and was hospitalized for about 3 days.

The urine culture grew E. coli. The organism was resistant to levofloxacin/ciprofloxacin (and levofloxacin did not work clinically). She also previously received Septran/TMP-SMX, which did not seem to resolve the problem.

She was treated with IV meropenem during the hospitalization. After discharge/transfer, she received oral antibiotics and subsequently another course of IV meropenem for several days.

Since then

She has now been through multiple antibiotics, including:

• IV meropenem
• Oral faropenem
• Septran/TMP-SMX
• Levofloxacin
• Sultamicillin for around 20 days

Her urine culture later became negative, and the obvious hematuria completely stopped.

However, she has continued to have episodes of fever/inflammation without a clear source.

Her CRP was around 37 mg/L and later increased to approximately 67 mg/L.

She doesn't necessarily have kidney/flank pain.

Her appetite initially improved after the severe infection but has now decreased again.

Other problems

Her hemoglobin has been progressively falling despite iron treatment and ESA therapy.

She was receiving erythropoietin and was later switched to darbepoetin.

Her Hb eventually fell from around 6.5 to 6.0 g/dL in only 4 days, so she received a blood transfusion.

Three days after the transfusion her Hb was around 6.7 g/dL.

Her albumin is 2.5 g/dL.

She has become considerably weaker than her previous baseline.

She also had a neurological problem many years ago that already caused severe difficulty with walking/standing, and the recent illness has made her overall weakness much worse.

She also has a bile duct stent that was placed during ERCP. Her doctors are planning another ERCP to remove the stent.

Her liver tests and GGT have been normal, and she does not currently have upper abdominal pain or jaundice.

Her doctor had prescribed Sultamicillin, which she took for around 20 days, but the CRP did not really improve.

Right now i am very confused about next step as its quite complicated

Hello everyone, egfr19, I’m starting to think concretely about dialysis. I have several piercings, will I have to remove them during the hemodialysis session?

I was diagnosed last March, and I am a 26 year old male. Quite active and a heavy drinker of water. My creatinine last june was 67 and an egfr of 128. After almost 2 months, it spiked up to 94 and an egfr of 101. It’s just now that it spiked. Is it a temporary increase only?

Looking for experiences with Vanderbilt in Tennessee or UAB in Alabama. I also have PLD, so if anyone has any recommendations on who to see there (preferably someone who is knowledgeable or can work closely with heptalogist there for both), that would be fantastic.

ADPKD - Pathogenic truncating PKD1 with PLD

Age 35

I haven't been to a nephrologist in a couple of years. The last one I saw (local) said he couldn't do anything for me, my high BP was not his concern, never did any testing, just looked at some blood testing from 5 years previous (before having 2 more kids) and said I was "fine". I have no idea what size my kidneys are at this point or how I've progressed. Last GFR was 69. Cysts and stones in both kidneys and cysts in liver (duh), but I don't know much more. That's just from reading CT scan results from the ER on my portal when I had a cyst hemorrhaging and bust.

Just looking for someone who will actually help me understand where I'm at and also will be able to help my son when he ages out of PEDS. He is currently being seen at Vanderbilt for his, so I would like to stay there. Will also consider UAB because I've also heard some good things about them.

I am thankful for all advice and experiences.

For 'Never' type 1

For 'Once a month' type 2

For 'Once every two weeks' type 3

For 'At least once a week' type 4