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New to Dent's

IsaacsMom Message
14 Feb 2014, 05:05 PM

Hello, My 5 year old son Isaac is in the midst of a Dent's diagnosis. It all started with his Kindergarten screening where doctors noticed a high level of protein in his urine. From there it's been a lot of back and forth and testing and re-testing and right now we are at the final stage of confirmation, genetics. Our lab work will be done tomorrow and with results in the next couple months. During this anxious waiting period I'll continue to look for as much information as I can. I think a lot of my focus will be change in diet, as that's something we can control now. Everything is still registering...the rarity of this all baffles me, yet also intrigues me. No matter the genetic findings, my son has some form of Kidney disease and the one thing I can say I'm looking forward to is being part of a group of people that all want to do their best to keep their kidneys healthy. If anyone has some unique information or is in the same spot I am right now, please don't hesitate to share. Thanks!
IsaacsMom Message
15 Feb 2014, 09:32 PM

Thanks Vicky, When was your son diagnosed? Did you guys already go through genetic testing?
Vicky_p Message
15 Feb 2014, 11:02 PM

He was diagnosed in December 2012 we had the genetic tests done and was confirmed. He's 9 now but we've been having appointments with the nephrologist since he was 3. We live in the UK, so we needed special permission to get the tests done.
IsaacsMom Message
16 Feb 2014, 12:33 AM

Vicky, This is a rather personal question, but is your son a bed wetter? My son had a severe issue with bed wetting this past year and though it's gotten a bit better, he still practically overfills a diaper every night. My doctors said its just his age and the fact he's a boy but I wonder if it might be in relation to his kidneys. I can picture him needing nighttime coverage for years to come-hopefully not though. Is your nephrologist in touch with any of the Dent's specialists here in the US? Do you think you will be traveling to the Dent's conference in June?
Vicky_p Message
16 Feb 2014, 07:36 AM

He isn't a bed wetter but we do have to push fluids with him as he is polyuric (makes too much urine) which could explain why your son is wetting the bed. However some children do struggle to stay dry at night. My son's team doesn't have contact with anyone in the US. They have a network of Nephrologists here that they can discuss cases with. However I don't think they use it enough as we have spoken to doctors who have very different opinions on how my son is cared for. I wish we could come over to the Dent's conference, we have 3 boys (only 1 has been diagnosed so far) so we couldn't afford the flights etc.
minu Message
16 Feb 2014, 06:01 PM

Hi Isaacs mom. Welcome to the forum, and we will see you in Chicago if you are attending. I also want to hopefully reassure you and mention my son also wore a night diaper until the age of 5. But i know our neighbour's son at the time of same age did too, and he had no Dent. Their sisters had no bedwetting issues as toddlers, so from my perspective I have to agree with your doctor that it is because he is a boy and it should stop hopefully very soon as it did with our son who he is now an adult. But I know every case is different.
IsaacsMom Message
16 Feb 2014, 06:18 PM

Thanks for your thoughts minu. We plan to be in Chicago and are looking forward to it. Did you go to the first conference? May I ask how old your son is? And how is he conscious of this disease as an adult?
minu Message
17 Feb 2014, 12:17 AM

Hi IsaacsMom. Yes, my son and I did attend the first conference and will be going to Chicago. He will be 22 in March. I don't want to write too much for him (we can talk in Chicago) as he also participates in this forum and could answer himself! He is in a unique situation that he is very conscious about Dent disease for the simple fact that he is studying biology, interested in the medical field and genetics, and has been working in nephrology labs for the past two years. He even did research regarding Dent at Mayo the last two summers! But in a personal level, he leads a normal happy life and, I believe, does not dwell on his condition, even though his GFR is down to 22%. He has tried to change his diet lately, following Jay's excellent example.
IsaacsMom Message
17 Feb 2014, 03:48 PM

Wow Minu, That's great that your son's interest in his own health has lead him to such a role. I look forward to reading any of his posts or hearing more about him eventually. I have an appointment with a nutritionist coming up and plan on looking into finding one that works more specifically with kidney patients. Who is Jay? Is he or she a nutritionist?
minu Message
17 Feb 2014, 05:10 PM

I am guessing the nutritionist, among other things, will tell you to reduce salt intake and not overdo protein. My son was tested, diagnosed and started taking meds when he was 3 years old. Jay is another young man who is a Dent patient who has seen a change in his numbers after changing his diet. Just check his thread on Diet in this same forum!
IsaacsMom Message
17 Feb 2014, 05:14 PM

Thanks! I'll check out that thread. Our whole family has started a low salt diet to support Isaac's need for diet change. I know there is more to learn, more to add and more to omit though.
kjsjd Message
12 Mar 2014, 08:09 PM

Hi IsaacsMom Glad you found this site - it has been invaluable to us - welcome!!. Just picking up on one of your questions, my son (now 12) is a bed wetter too, so you're not alone...! The whole diagnosis process is a difficult journey and there are lots of questions at present unanswered, but Lada et al are fantastic and one day we will have it cracked! Keep strong and good luck.
Kiwi Message
23 Mar 2014, 06:27 AM

Hi All. I have just spent the last hour reading all the posts on this site and very keen to be part of it. We live in New Zealand. My son aged 11 has just been diagnosed with Dents and it looks like 3 of his male cousins and 2 of my cousins kids have it too, although the rest are yet to have a genetic test to confirm. The Doctors we are working with haven't seen Dents before. We would be keen to have any material from the conference in Chicago emailed or posted to us. My son is currently on lisinopril. He tried chlorothiazide but it interfered too much with life producing too much urine so had to stop it for now. We don't seem to have a citrate supplement here and would be keen to know the names of supplements some of you have mentioned you take to see if we can get them somehow.
IsaacsMom Message
27 Mar 2014, 07:42 PM

Hello Again & to kjsjd thanks for the welcome :) Quick update. I put myself in a bit of a bubble while awaiting a official diagnosis from genetics. I heard from the lab at the Mayo Clinic the other day and they may have the results to us on Friday! With Isaac recently in the hospital related to dehydration from a stomach bug in combination with his diuretic, diuril, I have tossed myself back into the online hunt to learn more. I had to give my brain a break for a few weeks...this is a lot! Aside from the diuril, his doctors also have him on a potassium supliment and we are full force into a low salt, no processed foods diet. We're tracking his sodium intake and some days are as low as 700mg and as high as 1200mg. Tracking it helps us keep a good balance. Question, what type of vitamin if any have you given your child with Dent's. There are so many that I'm hoping to get an answer from our nephrologist when we see her next week. I am weary of one with too much calcium/vitamin D.
minu Message
28 Mar 2014, 01:53 AM

Hi IsaacsMom. My almost 22 year old son, diagnosed when he was 3 years old, has never taken any vitamin supplements. I think we were actually told he should not take Vitamin D tablets. He gets his nutrients simply by eating, hopefully, nutritious healthy food. And Kiwi, the citrate supplements my son has taken are called Urocit-K and K-Citra. (K for Potassium). They are both the same. He has also taken chlorothiazide for most of his life. He started by taking half a pill in the morning and the other half in the evening, but sometime in his teens the doctor changed the timing so that he now takes the whole pill (25 mg.) in the morning. Don't know if this (taking it early in the day only) would help your son with bedwetting issues if that is the problem.
melly1624 Message
30 Mar 2014, 12:47 PM

Hi all, I am Jay's mom. Sorry to have been absent from the conversation and am just catching up. Obviously, Jay has also been busy with work, life, etc. I will let him know that he needs to re-enter the conversation. Briefly, I your conversation brings back many memories…Jay will be 31 this June. We first knew there was a problem when he was two, when he passed his first, and only stone (but no diagnosis at that time. At age four, he was mis-diagnosed with medullary sponge kidney disease--a diagnosis that stuck until he was about twelve. As he began to enter puberty, he suddenly had a severe drop in kidney function, which would not have been consistent with the diagnosis, and we were sent for genetic counseling, which had just become available at the time. It revealed that Jay had Dent, it skipped his younger brother, and of course, I was the carrier. Being adopted, I did not know of any family history. I will have Jay weigh in to let you know of his regime of meds over the years, and his more recent and quite experiment diet protocol (basically protein free). So here we are…almost 20 years later after being told that he would need a transplant within one year as he entered puberty. We aren't fooling ourselves. The nephrologist has been very clear. It is not a matter of "if," but "when" we go to transplant. But Jay feels well and our hope is that for him, it makes sense to hold off as long as possible for that eventuality. He is on "the list," and we may have a potential living donor who is not a blood match but a tissue match, so we are hopeful that there will be options when that time comes. In the meantime…one day at a time. I'll contact Jay to rejoin the conversation. And if you are able to come to the Chicago meeting in June, our family will be there!
jgustafson Message
30 Mar 2014, 02:16 PM

Good Morning. This is Jay. I apologize for the delayed in response as I've been pretty busy with work lately. As my mom stated, my basic approach has been to greatly restrict the amount of protein I consume throughout the day, specifically "animal proteins" (Meat, Fish, Dairy, Eggs). My strategy has been tocompletely eliminate Meat and Fish consumption and have greatly reduced dairy and egg related product consumption. My diet relies heavily on rice and pasta based dishes, along with salads, sandwiches, and pizza......all just without meat. I moved to this diet in the fall of 2010 and have yet to experience any negative side affects from the change. In fact, since I made this switch my GFR improved from 18 to 24, I've lost about 10 pounds of needed weight, and feel better overall now than I did 3.5 years ago. As for the meds I take I am currently on: Uricid K, Potassium Khloride, Allopurinal, Eperelone, hydrochlorithizide, and Vitamin-D. I will be providing some more regular communication moving forward through the Dent's Disease Blog, where I hope to inform the Dent's community on things I am doing with my diet and to take control of my disease. For much of my life, I just accepted the fact that I had a kidney condition, but until recently never really even asked "what I could do to help". While I know that at some point I will likely need a transplant, I've had a newfound mindset the last few years that I believe has delayed the need of a transplant by at least a few years (per my doctor I was about 6 - 12 months away from needing before I switched). I would encourage any Dent's patients looking for ways to try and take control of their condition to adjust their diet. You don't need to go as extreme as I did and could try and do 1 meatless meal a day, but in all reality this change was pretty simple to do once I saw the results. (After 2 months on diet Creatinine went from 4.3 to 3.5). Hope this helps. If anyone has any questions they want to ask me personally, feel free to email me at Thanks
Jack1 Message
23 Apr 2014, 03:11 PM

Hello, I am new to the Rareshare community and sort of new to Dent's. I have been aware of a Kidney problem since I was 11 (I'm now 27) when it was noticed in a standard medical test for a Visa that I had very high protein in my urine. Following this I had a series of further tests, and a biopsy and was finally told that I had "probable Dent's Disease" in my early teens. The expectation was that Genetic Testing would be performed but the University (in the UK where I am from) didn't have enough samples / money to go through with it. I have had a succession of Nephrologists since finishing with my Paediatrician, all of whom have given me different advice, pills, no pills, low oxylate low protein diets, no diets. Excluding a 1-1.5 year period where I took allopurinol, Amiloride, Bendroflumethiazide, and Ramipril I have taken no meds. The only proactive advice I received prior to the medications was to drink plenty of fluids and eat a diet that is not high in salt. I had always drunk lots and so this wasn't difficult. As such as a teenager / adult I have maintained a pretty constant intake of appx. 5-7litres per day. My latest Nephrologist recommended that I have the genetic testing done, as then it would confirm the appropriate direction for the Treatment. This came back positive and I have Dents Disease 1. This new outlook has spurred me to take a more proactive approach myself, so I have joined the Contact Registry etc (Lada please let me know as I think I m supposed to be joining more than one registry having read some of the posts on here). One of the main interesting things that I have noticed in reading the forums is that lots of people seem to be relatively asymptomatic (as am I) and I would be interested to know if this reveals anything in particular about the severity of the disease in each individual. Look forward to hearing from you all, J
minu Message
23 Apr 2014, 04:58 PM

Hi Jack. Welcome to the forum. Lada would be in a better position to answer your questions as I am no doctor. But as a mom of a 22 year old (asymptomatic like you) Dent patient that has known since he was 3 years old, I would say, yes, the severity and speed of loss of kidney function varies from one individual to another. And, unfortunately, like other kidney diseases, your kidney function in most cases keeps decreasing gradually throughout the years and you do not feel any symptoms until your kidney function is extremely low (in need of dialysis or transplant). My son has been taking a variety of meds since he was 3 (allopurinol, thiazide, fosinopril, potassium citrate and now crestor), but I do not necessarily think they have really helped in slowing down the disease. We will never know. I agree with your doctors and Jay, that probably the best thing is to eat healthy (low protein, low salt) and drink lots of water.
LadaBL Message
21 Jan 2015, 09:58 PM

Dear Jack, Sorry for the late reponse. We were terribly busy submitting all the required paperwork that would allow us further funding for Dent disease, which we have finally achieved. I am also personally very busy on my FGF Dent study, which is giving me some exciting preliminary results I will be able to share. Now we have 5 more years of funding and we plan to use it in the best way possible. There is a difference between the Contact registry and the Dent Disease Registry. Contact Registry only has the contact information, whereas the disease registry has the particular information about your disease - blood tests, urine tests, which are stored de-identified. Meaning by number and not your name.This information can later be used to draw some conclusions on disease progression, prognosis etc. Proudly, we started with 10 and now have 130 pts in our Dent Disease Registry. Did you get in contact with our coordinator Barbara Seide? Lada