ADPCKD newly diagnosed anyone else have this??

[Ropergirl]

Hi I am new to this site but have just been diagnosed with ADPCKD in both kidneys .. level 2 and I am scared don’t know what to expect down the road except will have to have a transplant when the time comes. I hear that a drug Tolvalpan (not FDA approved in US yet) can slow down the process but no cure except eventually a transplant. Does anyone on here have same diease that can tell me about this ??

[KelliC_NKF]

I'm sorry to hear about your new diagnosis. Although I do not have PKD I can offer some helpful information. Recent studies suggest that drinking plain water throughout the day and avoiding caffeine in beverages can slow the growth of cysts. Research is also helping us understand the genetic basis of PKD.

Studies also suggest that some treatments may slow the rate of kidney disease in PKD, but further research is needed before these treatments can be used in patients. In the meantime, many supportive treatments can be done to control symptoms, help slow the growth of cysts, and help prevent or slow down the loss of kidney function in people with PKD. These include:

careful control of blood pressure

prompt treatment with antibiotics of a bladder or kidney infection

lots of fluid when blood in the urine is first noted

medication to control pain (talk to your doctor about which over-the-counter medicines are safe to take if you have kidney disease)

a healthy lifestyle with regard to smoking cessation, exercise, weight control and reduced salt intake

drinking lots of plain water throughout the day

avoiding caffeine in all beverages

kidney.org/atoz/content/pol...

[Ropergirl in reply to KelliC_NKF]

Thank you for your response . Like o said I am scared of what’s to come and what to expect from this. Any answers I can get are greatly appreciated. I do drink lots of plain water and walk never have smoked and changed my eating habits right away to almost a vegan diet. I have lost 28 pounds the first month which I think most is water weight and clean eating now .

[LoisDuncan in reply to Ropergirl]

Yes you are on the right path a vegetarian diet can prolong kidney failure. Protein helps you heal quickly, just be aware don't stress I was diagnosed with Burgers and it took me seven years I believe that they could last longer under good circumstances.

[Ropergirl in reply to LoisDuncan]

What is Burgerd?? Thank you for responding to my question . Do you also have ADPCKD??

[LoisDuncan in reply to Ropergirl]

I was diagnosed with Burgers,it's when you're body attacks it own kidneys also called I AG necropathy. So different dignosis but has the same outcome and treatments.

[cs65 NKF Ambassador]

I, too have PKD and I remember being scared when I first learned that I had it. I think it helped me to learn everything I could about the disease. pkdcure.org is a great source of info and a wonderful group to get involved with. They have yearly walks to fundraise for finding a cure for PKD and are doing ongoing research and clinical trials with people like you who are in the beginning stages. I found out I had it at age 50 and didn't need to start dialysis until I was 65. It can have a very slow progression, especially if you take care of yourself, find a good nephrologist with a a good understanding of it, take your blood pressure pills and follow a low sodium diet. Tylenol is safe to take for pain, not aspirin or NSAIDs. For now, the only treatments are dialysis and transplant when you get to stage 5 (under 15% kidney function). If you have any questions I would be glad to answer them. Good luck and stay strong and hopeful!

[Ropergirl in reply to cs65]

Thank you for your reply so what should I expect as far as pain?? What level are you?? I would love any advise you could give me on what to expect. What about Tolvaptan ?? They use it in Canada and UK???

[cs65 NKF Ambassador]

I am lucky in that I have had very little pain from the kidney cysts, however, when they rupture you can have pain and sometimes blood in your urine. It really varies from patient to patient, so check with your doctor if you experience a lot of pain or severe pain. Some people have to have cysts drained to relieve pain if the cysts or kidneys become really enlarged. When I have pain I just go to bed until it lets up. All you can take is Tylenol. Sometimes I take gas relief tablets because it feels like you're having a painful gas attack that radiates around from your stomach to your back. I am a level 5 PKD (my kidney function is down to 5%, so I am on dialysis 3 days a week for 3 hour sessions). Like I said, you may be fine for many years until your kidney function declines to the point of needing dialysis. Also, you can get on the transplant list before you start dialysis, but you need a recommendation from your nephrologist. I believe you have to be at level 5. You might be able to take part in a clinical trial for Tolvaptan or another possible treatment to slow the progress of PKD. Check with the PKD Foundation.

[Ropergirl in reply to cs65]

Thanks again cs65 so glad you are here to talk to makes this process a little easier to deal with. I have been spending endless nights and time researching this and my mind is going crazy. I hope they find a cure for PKD soon for as many people who suffer from it. I am going to stay positive that they will and hope to stay in touch and anything else you could share with me would be great again thank you.

[KidneyCoach NKF Ambassador in reply to cs65]

Once kidney function reaches 20% one can be referred for transplant evaluation. Many are able to get transplant prior to needing dialysis. Blessings

[KidneyCoach NKF Ambassador]

I understand the panic I went through when diagnosed. I was shocked yet expecting the diagnosis at some time as it is very prevalent in my family. Some people live their entire lives in Stage 2, 3 without ever losing anymore kidney function. Some people die never knowing they had PKD. It progresses differently for each individual and there are currently lots of research and development towards better treatments, cures etc. Have hope. I was diagnosed at age 21 and didn't need dialysis until age 44. I probably could have prolonged my function but unable to control blood pressure, kidney stones and infections for about 10 years (had no health insurance to provide medications). That being said, I have been on dialysis nearly 16 years, so one *can* lead a happy, fulfilling and enjoyable life even when on dialysis. I would definitely go to kidneyschool.org and pkdcure.org to read more about what you can do. Drink plenty of water, avoid dark sodas (or sodas altogether), avoid caffeinated beverages, avoid licorice, don't smoke, maintain a healthy weight, exercise and stay active, control blood pressure, quickly treat any UTI's, do things that make you happy (hobbies etc). Diagnosis is certainly not a death sentence nor the end of life. Only a few simple changes can have a very positive impact in prolonging your kidney function and your life. Have hope. You are certainly not alone. Many Blessings

[Ropergirl in reply to KidneyCoach]

Thank you for your response and info . I really appreciate this and it makes things a little easier to handle. I do research a lot and it scares me reading some of the stuff to where I stop reading but reading stuff from actual people makes it easier.

[KidneyCoach NKF Ambassador in reply to Ropergirl]

well, it can be scary, but remember in your research that most studies involve large groups of people and not individuals. Studies can be wide and general but may leave out certain criteria that may not pertain to you. Many studies will or will not include people over age 70 or under age 35 or those who are overweight or underweight, employed or unemployed et al, so don't let studies impact you negatively. We are all individuals and must receive individual treatment. Hang in there. Blessings

[Ropergirl in reply to KidneyCoach]

That’s true didn’t think of that I guess when your dealt scary diagnosis you don’t think straight or about that stuff. Heck I cried for a week before even reading or talking to anyone so chatting with you is such a reliever and thank you so much . Hope we can stay in touch blessing to you my friend.

[Ropergirl]

I also have two cyst on the outside of each kidney that the doc wants to drain has anyone else had this happen if so did you have them drained??

[Barlander]

My husband was diagnosed with IgA Nephropathy also known as Bergers (a similar kidney disease) 20 years ago. It was only four years ago that his kidney function got really low but with careful attention to his diet he managed to depend on his own kidneys right up to a year ago. It was at this time that the kidney function dropped so rapidly he had to start dialysis. He goes three times a week now but we are still living a good life and have even booked a holiday abroad. So don’t be scared. You could go years before your kidneys need dialysis.

[Ropergirl in reply to Barlander]

I just heard you could only live 3 years on dialysis and that scares me and you have to wait forever for a kidney and most people die before it happens so that just scares me so much because I have two little grand daughters I take care of. So is it true you only survive 3 years then if no transplant you die?? Ugh

[cmaxwell78]

Hello, Ropergirl . I have ADPKD as well, tho I was diagnosed 30 years ago at age 9. I am currently on the Tolvaptan clinical trial in the hopes that it is stopping, slowing, or shrinking growth of my cysts. I see you've already gotten some great advice on this thread. One I would add is to stay positive! Everyone's progression is different. I try not to assume the worst in my own situation and it helps me take things one day at a time. Best of luck!

[Ropergirl in reply to cmaxwell78]

I read about Tolvaptan wish they would approve it here my doc wanted me on it yesterday as the saying goes but waiting to see if FDA approves.. how long you been on Tolvaptan?? What level are you at?? You live in the US and do you know if any more trails for tolvaptan ??

[cmaxwell78 in reply to Ropergirl]

I'm stage 3b, I've been on Tolvaptan for 2 years now. I have it on good authority that the pharmaceutical company that produces Tolvaptan will have a date before the FDA this Spring to review the results of this study. Yep, I'm in the US.

[Roxxtar99]

MAGNESIUM ... don't let these AMERICAN money GRUBBERS shorten your life span ...

[Ropergirl in reply to Roxxtar99]

What do you mean MAGNESIUM Roxxtar99???

[susys]

Hello-lots of good advice here, but some misleading advice too. First I would recommend a PKD group on Facebook so you can talk directly to people with this disease, rather than a general kidney group. Next, Bergers is nothing like PKD (although us PKDers get that often) except they are both kidney diseases that lead to kidney failure. While those of us with CKD have some experiences that are the same, particularly in diet and side effects of progression, PKD has additional symptoms due to the effects of the cysts on our bodies, like crowding, pain, cyst ruptures, crowding of the diaphragm and intestines, back pain, etc, in addition to possible liver cysts, and brain aneurysm (ask your nephro for a MRA-one should be ordered every 10 yrs if results are normal). Next, since this news is shocking to you, I assume no one in your family has this disease. That is a big clue to me. PKD is genetic. There are 2 adult forms-PKD1 and PKD2 (ADPKD, not ADPCKD-Autosomal Dominant Polycystic Kidney Disease, which means if one parent carries a dominant allele for the disease that means that parent also has the disease, and then there is a 50% of the disease being passed to each offspring). The first form, PKD1, which is a missing code for a protein on chromosome 16, causes kidney failure around mid life, but it could be sooner or later. Scientists DO NOT know the triggers for when cyst growth speeds up, so if anyone tells you their miracle diet or supplement will slow down cyst growth, they are lying-everyone's progression is different and MANY things have been tested and nothing yet has been found to determine these differences (more on Tolvaptan at the end). One person's story of being vegan is not proof that being vegan changes a genetic disease. In this case, you would be aware of PKD in your family assuming they didn't pass away at a young age from something else. PKD2 is very different in progression. This mutation is on chromosome 2 and is not a deletion of a code but an incorrect code, so the patient makes some, but not enough of the protein (cysteine), which controls cyst growth. That means that PKD2 people rarely go into kidney failure, and unless it was discovered by a random scan of the pelvis, they never even know they have it because they almost never go into kidney failure. The only exception here is that some people can mutate not through the passing of genes, but through a spontaneous mutation during fertilization. So, it is important for us to know, in order to help you, how do you know you which one you have? How were you diagnosed? Who else has kidney disease in your family? How can you be sure you have type 1, which is more common only because more people know they have it and we can document it, compared to type 2? You would only know if you had a genetics test (through a doctor) or a family history presented to a nephrologist. People with type 2 have very little to worry about, and I feel this may be the case for you, so please don't panic until you know for sure. There are nephrologists who don't even know the difference. Can you find a PKD specialist in your area? This would be best. NEXT, there will be no cure for you. I don't mean to be awful about that but you need to understand genetic diseases. The error in your DNA code (and mine!) is in every cell in your body. Until we can change every cell in your body, your cells are going to pass that error to every new cell they make. Now, there is a significant advance in gene editing with a machine called CRISPR, which you may have heard of. For our disease, this would help people who don't have cysts yet-mostly very young people, but the machine can't even do that yet. The main hope in helping genetic diseases is that it would be used to edit out the mutation in the sperm or egg, then allow fertilization outside the body and then in vitro. Setting all that aside, as someone who is mid-life, post transplant with a living donor, who never went on dialysis, with both sisters who are positive for PKD, and who's mom had her transplant for 24 years before passing away, I completely understand why it is scary and you worry about what is to come. I can tell you the only thing that does is ruin your present day. You will mostly feel yucky in stage 5 which can be 5-10-15 years from now. There are 110,000 people waiting for a new kidney who are going through the yuckys right now. Don't freak out-you will be ok. Do your bloodwork, learn how to read your bloodwork (by the way, in recent years, the low-caffeine for PKD has been disproven-just moderate. Same for protein. Same for sodium-don't overdo but kidney patients are notorious for hospitalization for sodium that is too low-and that makes you VERY sick). Go to your scheduled appointments. Make it your business to know everything the doctor says and more. Avoid NSAIDS-they break down through the kidneys, unlike most meds which the liver breaks down. General doctors don't understand this, so if you see a GP for an injury, remind him/her that you are avoiding NSAIDS due to kidney pain so they can recommend something else. WATCH YOUR SUPPLEMENTS! As you progress, your kidneys will have a harder time filtering micronutrients and taking vitamins and minerals can cause a build up in your system. This is a big reason to know how to read your blood work. To the person who gave you magnesium advice-bad. Only take if your nephro tells you to. Contrary to fake health news, most people are not low in Mg and don't need it orally. This is one that can build up and literally poison you. A good nephro will have you add Vitamin D around stage 3, as your parathyroid (this is NOT the thyroid gland and it does not have the same job-it is only named that because it is below the thyroid glands in your neck) will struggle. This gland helps regulate your calcium levels (which can also build up in your system when the kidneys struggle-they are partners with the parathyroid gland). If your parathyroid goes kaput, don't worry. You can take synthetic hormone supplements (harmless! Do not compare them to reproductive hormones). Make sure you are prepared for long term illness with a solid job and health insurance. I can not stress this enough. If you think being sick is stressful, imagine worrying about paying the bills and being sick. If you want to go back to school or change careers, do it now while you feel good. I did exactly that. Do not think the government will take care of you. That is for minimum care. If you are otherwise able-bodied, make it your priority to find a career that will give you sick days, medical leave for surgery, and decent health insurance. Maybe things will change with our system's health care, but maybe things won't. As someone with a chronic health condition, you can not sit and wait to see what will happen. Your health-your responsibility. Lastly, Tolvaptan-I was in the study for 7 years. It is suggested my progression was not slowed due to its use. I should mention I am also a science teacher, so reading scientific studies is easier for me than most people, and between my PKD specialist who is the regional clinical trial supervisor, and all the reading I have done (btw, one person posted that the FDA would be having the results released-yearly results have been released all along-that's just part of the approval process-it's not a big deal). This drug is the first of it's kind and not that great. While it has been approved in other countries, most of those socialized-medicine countries are not covering the cost. The reason? And the same reason the US hasn't approved it yet? The results are not that overwhelming. There is a minor change in progression. The expectation was NEVER that it would be able to stop cyst growth. And since this means patients will STILL need a transplant in the future, possibly as someone now older, and in overall more poor health, it's not reasonable to put people on it for decades, especially due to the inconvenience of it making your insanely thirsty and having to pee all the time (compromises your life when you have to pee almost every hour). Yes, this gets a little better over time, but is it worth the overall benefit of delaying transplantation by a few years? Probably not, which is why it still hasn't been approved. Even if it does, it is highly unlikely insurance companies will pay for it-not because they are evil, but because the results don't justify the cost (which according to folks in other countries is in the $1000 per month-US dollar comparison). It's easy to pin hope on it when we have no other reason to hope, but that doesn't mean this is something that will be life changing. There are other clinical trials now-you can find them on PKDCure.org, and being in early progression, you may be interested. You will also get free care and bloodwork from experts who know the latest about our disease. Again, look for a PKD specific support group on FB (I like PKD Cysterhood) and see your doctor regularly.

[Ropergirl in reply to susys]

Thank you for your response it’s very helpful .. I don’t know if a PKD specialist I. My area I wish I did . The nepho just came in and said I have ADPKD no test so maybe when I see him Thursday I will ask how I can be tested to see if I am PKD 1 or PKD 2 hopefully 2 if I have to have it. I am on a PKD site on FB but will look up the one you suggested. I hope we can continue to chat because you are very informed on this and it makes it easier for me to have some one like you to chat with. Again thank you

[cs65 NKF Ambassador]

Hi Ropergirl,

I would check the pkdcure.org site to see about PKD 1 and PKD 2. I believe those are different genes or areas on the DNA. If you have ADPKD that means you have a usually adult onset form of the disease and there is a 50/50 chance you will get it from a parent because it is a dominant gene. Also, you have a 50/50 chance of passing it on to your own child. There is another form of PKD called ARPKD which happens to infants - this is a recessive gene, so both parents must carry it to pass it on to their child. To complicate things, a person can also have a genetic mutation which causes PKD, in which neither parent had the gene. In any case, if you are interested in doing a clinical study (which you may qualify for since you are in an early stage of the disease) I would check out pkdcure.org. You most probably will have several years before your disease becomes a critical problem, so take good care of yourself and remain hopeful! There are even people who never have to go on dialysis! As far as life expectancy on dialysis, I personally know someone who has been on for 9 years and is 90 years old and is doing well! I just read about some one who has been on for 40 years and is in her 70's! I believe the record is 43 years. You do have to take care of yourself (diet, exercise, taking your meds, watching your fluids intake, get your flu and pneumonia shots) and hopefully avoid complications. So don't let the thought of dialysis scare you - you can also try to get on the transplant list through your nephrologist when the time comes. Hang in there!

[Ropergirl]

Thank you this does make me feel better

[Ropergirl in reply to Ropergirl]

I couldn’t find the clinical testing site could you help me with it?? The doc did no test just told me it was ADPCKD and I heard you needed to be tested to see what exactly was the PKD a person has is this true and what test is done ??

[cs65 NKF Ambassador]

Have you tried signing up for the PKD Foundation blog on pkdcure.org. There should be a link to research related topics. That's where I would look. They occasionally send me notices of clinical testing, but my disease is too far advanced to participate. Or you could call or email the PKD Foundation from the website.