Holistic Care of PUV

And, uh, you know, as we bring this session to this part of the session to an end, we're gonna open up to questions is just to, you know, just to wrap it up, some take-home messages about the importance of holistic care for patients who are born with post-lethal valves. And uh as I mentioned before, you know, one of the most important interventions that any of my team members and I can provide is the counseling. It's educating you that this is a lifelong condition. What are the important aspects of care that you need to be seeking out and advocates for your child's health. And ensuring that their kidney monitor, kidney function is being monitored, um, you know, we cannot tell how healthy a kidney is just with an ultrasound. We have to do the blood test. We have to see where their creatinine level is, and then we can calculate what their GFR, which is an actual measure of how much blood is actually being filtered and. Cleaned by the kidneys every minute. And that's the most accurate way of predicting how healthy one's kidneys are and if that child is progressing towards a uh chronic kidney disease state and also are we losing kidney function. Uh, so without those regular blood tests, uh, it's really hard for anybody to tell you how your child's kidneys are behaving and whether they're staying healthy or not. So the education, the counseling, all of the educational resources that we, uh, bring to bear to support the families in the care of their child are, I think, is probably one of the most important things that we do, uh, and I'm privileged to have this, you know, huge team that works with me to ensure that we are providing the state of the art education for the families. Another important thing is that, you know, we never give up on the bladder. Uh, it can always surprise us, uh, what sometimes will look like a really, really, um, very affected bladder with appropriate care, catheterization, um, using Ditropan or other anticholinergic medications, and sometimes Botox can sometimes rehabilitate that bladder and make it more functional. Not normal, but more functional to a point where we don't have to think about replacing the bladder with an augmentation, and we can use the child's own bladder to serve as that reservoir of urine so that they can be dry, that their kidneys are protected, and also avoid infections. It really is important to understand that pressure is one of the most important, um, enemies for a healthy kidney, and if we have too much pressure in the urinary tract. It starts to cause scarring and damaging of the kidneys that is irreversible. Unlike the bladder that might recover some function, once the kidney is damaged, it's not going to recover function. That injury to the kidney is permanent, which is why Doctor Clay and I are very, very particular about ensuring that every time we see a child that we know where they are, what's going on, if there's been a change in the kidney function, what happened? Um, we have changes that we can predict and we can mitigate, and there are unfortunately some changes that are just a part of growing up. Um, as your child grows, the body's going to require more and more kidney function, and if the kidney's not able to provide that from its reserve, then we will start to see that the kidney function starts to decline, and that's what we call, you know, uh, an unavoidable change in the kidney function because it's just part of your child growing. But too much pressure in the bladder, that's avoidable. We should be avoiding that injury, recurrent repeated kidney infections, that's avoidable. We should be doing everything we can to avoid that. Uh, certain food substances, dietary habits, uh, becoming dehydrated, these are all things that can be avoided, can negatively impact the kidney health, and so we are very vigilant about these things and anything that we can affect in a positive way, we're gonna make sure that we teach you how to do that. Sometimes, unfortunately, parents will say, my child pees fine and so everything must be OK. Unfortunately, just because your child is urinating and there seems to be appropriate amount of urine production, doesn't mean that everything is OK in the urinary tract. Uh, it can be very misleading and again, the only way to really know if a child's kidneys are working well is do that blood test. All urine is not good quality urine, and what I mean by that is you can have a child who makes 2 or 3 L of urine a day, and they're constantly thirsty. Um, the kidney function, um, as Doctor Clay mentioned, the kidney is really a master organ for us and it does so many things besides just make urine. But when we look at the two main functions, it is there to clean and filter our blood, get rid of the toxins, so those toxins can be excreted out of our body. It also just as importantly, is concentrating the urine, and what I mean by that, it is pulling back the clean water back into our body. The human body is about 70% water, and if we lost a lot of fluid, we wouldn't be able to function. And so the kidney is constantly monitoring how well we are hydrated and deciding how much fluid we want to send out in our body as urine and how much fluid to pull back, and it's doing that in a very, very, very elegant manner throughout the day, whether we're awake or asleep. When a kidney is injured, it loses that ability to concentrate the urine, and that's one of the first signs of kidney injury. And when that injury occurs and you can't concentrate the urine, the child starts to make very, very large volumes of dilute urine to a point where they may be making 2 or 3 L of urine a day. That means they have to keep up with 2 or 3 L of fluid a day, sometimes not possible. And there, Doctor Clase would then come in and say maybe we need to supplement the child with overnight fluid replacement. Either through a feeding tube or a G tube, uh, and then from a urological standpoint, we're saying, you know, all that urine's being stored in the bladder, is it damaging the bladder? So, this is where we have to be very, very careful and say that, you know, again, urine production is something that we have to monitor. It's not always a sign that everything is OK. It can actually be an early warning sign. You have to always be careful when we're taking care of a child who has a concentrating defect because when a child is sick, they don't want to drink, and if they have a fixed urinary output, they are going to make a lot of urine regardless of whether they're drinking fluid or not. And in young babies, when they get dehydrated, that can be really critical. They can get very sick very quickly. So we teach the families that if their child is not drinking, And they're having that large volume of urine coming out, they need to come to the hospital so we can start an IV and keep their child from getting dehydrated because that dehydration causes an ongoing secondary injury to the kidney. As we mentioned, neurological development may be impaired. It could be a result of the prematurity. It could also be a result of these toxins that the damaged kidneys are not clearing well. We just don't understand. And as Doctor Clay mentioned that children with kidney damage, they don't assimilate the nutrients properly either, so there's multiple facets that can explain why the neurological development in children. is not going well, and that's actually one of the metrics that my colleagues in nephrology will use is, what is the brain's brain, you know, head circumference? Is that growing properly? If it's not, it means the baby's brain's not growing properly, and that's an indication to initiate renal replacement therapy with dialysis. We can never forget that a baby with valves potentially at some point may have had some injury to their lungs, and so we always have to be mindful that during times of cough and flu seasons that we're providing that extra buffering for these children so that if there is anything going on that we get them to see the pulmonologist and get them tested with pulmonary function testing to see do they have the appropriate amount of pulmonary reserve or do they have some true pulmonary hyperplasia that can be a risk factor for them as they continue to grow. And then the last thing is, you know, we really have to address the individual as a whole, so the kidney health, their bladder health, their developmental health, their nutritional status, their psychosocial well-being, and also the psychosocial well-being of the family and also the siblings, because when you have a child with a chronic illness, you're spending a lot of time coming to the hospital to make sure that that child is OK, and the siblings sometimes feel left out. So we're really trying to create this program that takes care of all of the needs of a family who has a child with postlethal valves. So, uh, it is really our privilege to be able to provide this comprehensive care and, uh, you know, while we're chasing after your child, it's with really well-intentioned, um, desire to provide the highest level of care that we can and um we can only do this as a team led by our very, um, dedicated coordinator, Nicole, so she, she really has made a huge difference to the care of the children that we've been providing over the past year that this center has really been in existence.