Percentage of Children with OEIS that Develops into ESRD

So another question that uh is coming to us is what percentage of children with the omphalocele atrophy, imperforate anal spinal cord anomalies will have um end stage renal disease requiring either dialysis or a transplant. It's not a straightforward answer to that. I think it all depends on where that child is starting off life with in terms of do they have both kidneys? Are both kidneys working well? Um, and if the answer is yes, that they have both their kidneys and both their kidneys are working well, then the answer in today's day and age that that child should never end up in a situation of end stage renal disease requiring a transplant as a result of their malformation, because we should be with appropriate neurological care, be able to protect the kidneys and ensure that as we progress through the different care plan that's personalized for that particular child, um, whatever we're doing is ensuring that the kidney is healthy. Now it's important to understand that there are other reasons why you, you might get kidney damage later in life, so cardiac disease, high blood pressure, diabetes, obesity, all these things impact your kidney health. Some of those conditions actually now are starting, we're seeing them in children. We used to think of those conditions as adult onset diseases, but we now know that cardiovascular disease, high blood pressure can start early on. So it is important that as your child is being cared for by your pediatrician, that they are checking blood pressures on your children on a regular basis, um, checking your child's urine for any evidence of protein, which is one of the first signs of kidney injury, and if they do pick up that your child's blood pressure is not reading normally. Uh, maybe getting that checked out by a nephrologist, and one of the things that the nephrologist will do is something called an ambulatory blood pressure monitoring where they send your child home with a special cuff that they wear all day long and is measuring the blood pressure continuously. Sometimes we will have a phenomenon called white coat hypertension, which is the, for a child who is. Got a chronic medical condition, going to the hospital for many different doctors and tests and blood tests and catheterizations, there is an anxiety that might develop in your child. Oh my gosh, I'm going to the hospital, and that anxiety can itself fuel high blood pressure that's transient, and so that's white coat hypertension, and that needs to be distinguished from true high blood pressure because that true high blood pressure, that sustained high blood pressure that is going to cause the damage to the kidneys. So, um, if your child is born with normal kidney function, then what we've learned is that with growth there is going to be some changes, but we call that stage progression. So in our experience here, and we've been tracking this data now for the past 8 years, is that in our care we can. Reliably ensure that there's only a one stage progression going from CKD stage 1 to maybe CKD 2, or if your child start off with CKD2 going to stage 3, but we've not seen a lot of our patients go from stage 1 to 3 or 2 to 4, and very few of our patients have actually gone on to require transplant when they start off with healthy kidneys. The children that progress to kidney disease are the ones that come to us later in their care, where maybe the kidneys have already suffered irreversible damage, and unfortunately, once the kidney is damaged, we can't change that outcome and a damaged kidney, so once you're at CKD 3, it's much easier for that kidney to get hurt again and keep losing more function. So it really is something that we focus on is protecting your child's kidney health. And you will hear that from us over and over again. Um, we pay very intense attention to small changes in, um, kidney function, to trends, um, even if your child's creatinine, for instance, which is a, a measure of how well the kidneys could clear a byproduct of muscle metabolism, um, and is often used to keep an eye on kidney function. So even if your child's creatinine remains within the normal range, and there's always a range in lab values, but it's trending up even within that normal range, we wanna pay attention to that because we wanna say what's causing that? Has your child grown significantly? Well, that might be normal. Um, has your child's growth stayed about stable? What else might be going on? What might we be missing with the bladder that could be impacting the kidney function? Because by the time a creatinine doubles, you can consider that you may have lost half of the kidney function, and we don't want that to happen. Absolutely. And one of the things that, you know, you as parents have absolute control over are your child's nutritional habits, you know, their dietary habits. So having a well balanced diet with, uh, you know, if you look at the USDA, they've got the plate. If you go to their website, they've got what is a well balanced diet, they've got the plate with all the different fractions of making sure that we have the grains, the proteins. The vegetables, the dairy products, and ensuring that your child gets all those is going to ensure that they grow as healthy as they can, that they're protecting their kidney function, and that their brain development is going to be normal. So it's very critical, the nutrition that a child gets and just as importantly, what they're drinking. We really want to make sure that they're getting an appropriate amount of water. Dehydration can actually stress out kidneys a lot and that can cause ongoing damage to the kidney. And so how do you tell if your child's dehydrated? Um, one simple way is, you know, look in their mouth. If they don't have a little pool of saliva underneath their tongue, they're probably a little bit dehydrated. And also teach your children to look at the toilet bowl before they flush after they've gone to the bathroom. The only time their urine should be yellow is when they wake up in the morning and go to the bathroom because they've fasted all night long and not had anything to drink all night long. So the early morning urine might be yellow, but after that you want to ensure that your child is drinking enough fluids that their urine is at best clear or if anything, maybe a light yellow color, and that's going to protect their kidney function. It's also just as importantly going to reduce the risk of them having kidney stones. Um, one thing is that some medications do color the urine, so vitamin B complex pills, if your child's taking that, that might make their urine yellow anyway. Uh, but just a good rule of thumb to keep in mind.