Congenital ESKD - A Family Diagnosis

The second speaker is Kara Short. Also, Kara Short is a pediatric nurse practitioner with over 12 years of pediatric nursing experience. She joined the acute dialysis team of Children of Alabama in 2016 as their first inpatient nurse practitioner. She has been an integral, integral part in standardizing the care of all ICU patients. Requiring kidney support therapy and also has a special interest in the neoninatal dialysis program, uh, which performs on hundreds of patients there. She's a co-founder of the neonatal and infant Course of kidney Sport, which is done quarterly, a conference that teaches neonatal dialysis, nephrologists, neonatologists, and nurses. Yes, what a tall task to go behind these wonderful experts today. Um, and I was glad Mel was able to join. I'm gonna try and share my screen really quickly. OK, so my name is Kara Short. I'm the acute dialysis nurse practitioner, um, at the UAB Department of Pediatric Nephrology under the Pediatric and Infant Center for Acute Nephrology at Children's of Alabama. That is our free-standing pediatric hospital, but we are affiliated with UAB. Um, I do not have any disclosures this morning. I will discuss briefly some off-label uses for some machines and products, and then just a heads up, the parents of all the patients that I'll be talking about today did agree to share their information and photographs, uh, with, uh, Pecan for this talk and several other talks that we do about them. So I just wanna kind of give an overview of what our institution looks like as far as the care that we give for patients with end-stage kidney disease. This is the UAB Children's of Alabama data from 202014 to 2020, um, and this is all, uh, in our NICU, um, we see these patients. And if you look over the course of the years, um, we've had more patients with end-stage kidney disease that we are receiving into our institution and taking care of, and our percent survival rate, um, is slowly getting better. So, um, we're, we're learning a lot over the years and we're, uh, trying to do better for these patients. And just a note of um, from some of this data, um, all of our patients at Children's were started on CRRT either CVVH. Via modified Aquadex or ECMO, uh, before transition to PD or HD, um, and then our smallest patient was 1200 g and the largest patient of this set of patients with end-stage kidney disease was, um, 3.6 kg and then three of these patients listed here, uh, did require ECMO at birth and they all survived hospital discharge. So just a little window into kind of the patient population that we see. Um, here are a couple of pictures of some of the babies that we have taken care of with end-stage kidney disease. We have Landon on the left, uh, Charlie Belle in the middle, and Kennedy on the right. So I first like to discuss kind of what the goals for our patients are for this patient population. Uh, we talked a lot about this today already, um, appropriate diag diagnosis prenatally, including the wonderful teams that you have at your institutions, maternal fetal medicine. Um, urology, radiologists, um, to figure out what's going on and, um, make sure that we, um, are including all of our multidisciplinary teams and including, um, meeting that, meeting with those teams before these, uh, babies are born and having really good plans like we already discussed. Uh, survival to birth is obviously a goal and Uncomplicated delivery, appropriate respiratory supported delivery, um, and then for us, uh, we are stand-alone Children's Hospital and so for any patient who may need an escalation of care such as ECMO or dialysis if they're end stage, uh, we really push for immediate transfer to Children's of Alabama and we have the beauty of being connected by a, um, walkway, uh, to UAB. So sometimes there's really, uh, an easy way to get these patients over. And then the really big thing for us with these babies, um, and we harp on this, uh, is prompt initiation and nutrition. We're not doing these babies any favor if we cannot adequately give them nutrition. And so if that means initiating dialysis, um, then that's what we do, uh, because we gotta grow these babies, um, for best chance of survival. Next up, we're talking today about, uh, parents, um, this, uh, this last talk. And so what are the goals for our parents or our caregivers, um, Initiating frequent communication by phone, um, in the beginning is super important. These moms have just had these babies, so they may still be in the hospital. They still may be on medications, um, you know, it's a very stressful time for these patients, uh, for these parents who know that their baby is gonna have issues when they're born, and so it's super, super important that we touch base with them really quickly after we get the baby and give updates, um, frequently. Uh, giving appropriately timed education of the disease process and current status. Um, these are complicated patients with comorbidities, um, that take several members of, um, the healthcare team to take care of them, and so giving all of that information to parents is really hard, especially those parents. Who may not understand as well as others. Um, and so it's really important to gauge the education level, um, and the comprehension level of these families and giving education piece by piece, um, in, in a manner that they can tolerate and they can handle. Uh, you know, there are a lot of emotions going on when these babies are born, um, it's very stressful, and so, uh, being able to gauge how much those families can take in, you know, hour by hour or day by day is really important. Um, it's important for us to give support for these families to make informed decisions regarding their child's medical care. Mel just talked a lot about how their institution, um, integrates so many different teams to be able to teach these families what these diagnoses mean and what their options are. Um, one or two people in the healthcare team for these babies cannot really give them the information that they need to make these decisions. And it's important that we all get together, that we engage with the other team members to give them the information that they need. Um, engaging with these teams and keeping the family frequently involved. Sometimes you have families who deliver a baby and it's their 3rd baby or their 4th baby and They live, um, we're in Alabama, so they live rurally, maybe 3 or 4 hours away, um, they don't have childcare for their other children and so they may not be able to be at the bedside and it's really important to remember that although they're not right there, that it's still important to keep them involved and giving them the support they need. Um, working to establish rapport is super important from a nephrology perspective. If these babies survive, we're gonna take care of them until they're 18. years old, so we've gotta start laying the foundation for a really good relationship, um, and tapping into our resources, so our social workers, our case managers, um, the nurses that they may see after the NICU, involving them, um, at the appropriate time to give the support, um, that these families need to take care of these complex patients and then educate on the NICU and dialysis jargon, and I think that this is so important because it empowers families. To know what's going on. So if you teach them vital signs and what is an appropriate vital sign for that baby at that age, and if you teach them the jargon that we use to talk about dialysis or the ventilator, um, you know, they feel more confident when it's time for them to step up and represent their, their, their child's needs, um, and so it's really important and that we encourage the bedside nurses to do this as well, to teach them the words that we use and And to help them understand, um, the medical jargon that they're gonna hear a day to day basis and then it's important for us to be able to bring that down to their level when we're teaching them as well and kind of, you know, relate that and help them understand where we're going forward. And then finally, most important, it, it's so important to allow them to be parents to their baby. I mean, you know, they did not envision this when they found out they were having a baby that they would be in a NICU, maybe on ECMO. Um, ventilated, you know, on dialysis, and so the idea of them holding their baby after birth is, is not there and that's not a reality and so it's really important for us as healthcare providers to remember that that's such a normal and special, um, thought and a bond and we need to do all that we can to allow them to still be parents. So I'm gonna talk a little bit about a patient and then you're gonna meet his mom and uh I wanted her to be here live and I actually think she's about to stop answering my calls because I'm always calling her to um help us with our support group or do things so I do have her on video today and so you will meet her at the end of this little. View of, of Landon, um, and she will talk a little bit about her perspective throughout her NICU course. So this is Mr. Landon Puyam. He was born in August of 2018. His birth weight was 2.9 kg, and he was 37 weeks gestation. Um, maternal history was positive for oligohydramnius, which then became anhydramnio. Um, prenatally diagnosed with bilateral hydronephrosis. Um, megacystis, probable bladder outlet obstruction, and so, of course, kidney dysplasia on a prenatal ultrasound. So he was delivered at UAB right next door to us. He was intubated at delivery. He did receive two doses of surfactant. He was placed on the oscillator and he was shipped over to us, um, on day one of life. Looking through his hospital course, um, on day two, we decided that it would be best for him to be placed on ECMO. That was a team decision between our neonatologists, our surgeons, our nephrologists, and the family. Um, he was requiring presser support at this time, um, but we were able once we got him on ECMO to start some nutrition. Uh, he hung out for a while, uh, but on day 5 of life, we decided to start some dialysis via our ECMO circuit, um, and that is when he went on dialysis for the first time. A couple of days later, he was able to come off ECMO. They placed a temporary 7 French catheter in his ECMO cannulation space, and we started CVVH via the modified Aquaex. He came off dopamine almost. At 2 weeks of age, um, at 3 weeks of age, we decided he needed a more permanent catheter and so we placed a barred 6 French power line, uh, and tunneled that to the right IJ, and that was his first, um, cuff catheter that we used for him in his hospital course. At 5 weeks old, we decided he's finally stable enough for VCUG which confirmed what we thought, which was that he was a PUV. Um, he did have a cystoscopy and transurethral resection of that at 5 weeks old, and we placed a G tube. At 6 weeks old, he was able to be extubated to a nasal prong vent, had a setback at 8 weeks old, um, he decompensated, culture negative sepsis, he did require pressers and was really, uh, sick during that time. Here are some pictures of him during his hospital course, you can see on the left, um, uh, much, uh, worse off than, than he was as he kind of improved on the right. Um, he did have pulmonary hypertension at birth, which eventually resolved in the month 2 and 3. He came off nitric oxide and sildenafil. Um, his catheter was displaced due to growth, so we replaced that around month 2 to 3. He had a UTI, um, that was E. coli, so that was another setback. At 16 weeks old, we were able to place a peritoneal dialysis catheter, um, and then we replaced his vas cath again at that time. He was going to the OR, um, because it was not working well. He was weaned to nasal cannula and later to room air, and then 3 weeks after we placed the PD, we started using it, uh, and then found out, whoops, he had bilateral inguinal hernias. So we had to repair those and hold off on PD again. So he went back on CBVH via the modified Aquadex. He did get a treatment for club Celis sepsis for about 2 weeks, and then we were able to restart his peritoneal dialysis successfully at 5.5 months old, about the same time, he finally reached full feeds. Um, he had been supported with, uh, enteral nutrition and TPN during that time prior, um, but finally reached full feeds and full nutrition. At 6 months and 1 day, we really thought we were about to send him home, got another UTI. Um, so urology, our urology friends came and did some work for that. We treated him for his UTI. We got him a circumcision, and then we were finally able to discharge him home at 6 months and 3 weeks. And here he is, happy to be at home and away from our hospital and away from all of us, um, taking care of him and home with his family. Um, around month 9, mom noticed he was making a lot more urine. Um, we trialed him off PD and he did great. Uh, by month 14, he was taking all of his feeds by mouth. We did still keep his G tube from feeds and meds, and this is him here on his first birthday with a picture of him, um, in the hospital, uh, on day 1 or 2 of life, uh, to show the comparison of where he's, where he's come. So most recently, uh, he remains stable at CKD5. He has a GFR between 10 and 15. All of his feeds-in meds are by mouth. He is on some medications for renal support. His weight gain is appropriate, and oh man, his development is, is on target. He is a very feisty. Toddler, toddler. Um, in January 2021, they had their first transplant evaluation and his vessels were not yet large enough for a transplant, so they are sitting on him off of dialysis, uh, waiting on him to get a, a bit bigger for the surgeon to be happy. Here Landon is with his family, his mom and his dad, and these are just the final stats on, on Landon. So he was born and went on ECMO, and he needed ECMO for a week. We put him on Aquadex and that's how it was his, uh, um, dialysis modality for a large amount of time, 151 days. He was 45 days on the vent. He ended up with 4 vascular catheters during his hospitalization. He was discharged at 6 months and 25 days, and today he is almost 3 years old, 2 years and 9 months. So here are just some updated photographs of some of the patients you saw earlier. We have Kennedy on the left and then Miss Charlie Belle, Miss Nova and Landon here, um, and it's always a nice reminder of why we do what we do, um, when we get these babies on day one of life and, uh, they're very, very sick. So the next thing I wanna do is play for you guys the video of Shia, that's Landon's mom, um, and some questions she answered for me, and so let me pull this up. And we will get started reviewing that. So the first thing I'm gonna ask her is what are some things your healthcare team did well? Hm, well, I mean, looking at Landon, I would have to say you did everything well, but to be more specific, um, you guys listened to me, you were kind, you were patient, um, You, you know, you, you gave me the opportunity to give my input, um, and it was just, I don't have anything negative to say in terms of, um, things that you did or didn't do well. What do you think we could have done better? Um, I don't think you could have done anything better because I was always asked um what I thought about anything that you guys were going to do with land and I was always given the opportunity to um agree or disagree, so I was never rushed or bombarded or overwhelmed in making decisions. You guys basically, you know, you gave me your um Your expert advice, but the decisions were always ultimately up to me. The next question I asked her was how did we do managing Landon's other needs? And for example, um, mom and dad's need to hold him and play with him, physical therapy, occupational therapy, sleep. Schedule, regular baby schedules, things like that. Yeah, I held Landon, um, when it was, when it was time and when I could hold him, um, my first time holding him was 3.5 weeks and it was obviously because he was on EC mode and he had so much going on, he, he couldn't really be touched, um, but once I was able to hold him, I didn't have any issues. Um, I would hold him for 3 or 4 hours at a time. You know, I would just sit there and hold him. They allowed me to hold him as long as I, as I wanted to, you know, unless he needed something that um required him to be in bed, but I was able to hold him as much as I wanted to. What would you tell other parents if they were about to have a baby like Landon? Maintaining a positive attitude is like going to be immense. Um, the situation is what it is. You know, if, if you have a negative attitude or, um, a sad or depressed attitude, as hard as it may be not to, it, it's not gonna work out well for you because, you know, you're gonna see everything. You're gonna see, for me, Landis's lungs collapse before surgery, he coded right before my eyes, so many different things, um. But that's the advice that I was given from my aunt. She was like, you have to maintain positive, and that's what kept me going. I was always positive. I was always upbeat, um, and that's the type of child that Landon is. I think because of that, I was never sad or depressed around him. So always, always, always, no matter what, maintain a positive attitude. Sure there are going to be days, you know, you feel like giving up and you feel like it's too much, but overall just maintain a positive attitude. And the final question I have for Shia is how do you feel about the setbacks during Landon's hospitalizations? For example, his need for several lines and the hernia repair after we began using his PD catheter. As a parent, of course, there's frustration because like you said, sometimes they had to be changed just because he was growing. But um, It was great that he was growing, so it was kind of like a catch-22. You can't be upset that he's growing. I mean, no, I don't want him to have constant surgeries, but yes, I want him to grow because growing was a part of him getting to peritoneal dialysis, which was also a part of us going home. Um, it was not expected, but, um, I just dealt with it. I just rolled with the punches. So that is Shika Poyam. She's a wonderful mother and a fantastic, um, person to have had the privilege of working with, and I'm still working with her kind of to figure out how we can go forward, um, and use that patient perspective, uh, to change the way we care for these families and, and patients, and it's more of kind of an organic. Discussion, um, and not really something that we've tried to kind of nail down and write down yet, um, but, uh, she's been an, a, a great advocate for her son and almost even just kind of teaching us how to encourage our families to be advocates for their own, own child, own children. So she's a fantastic mom and I'm glad that I was able to share her with you guys today. Thank you, Kara. That was amazing because I agree with you, patient perspective is always important in this journey. It's a long journey. One of the questions that has been addressed by, I think Dr. Lim, he sent a question about what is your approach, Kara, for patients with bilateral renal agenesis. So, um, the patient I was talking about, the smallest patient that we have taken care of, um, in our NICU that was, uh, 1200 g was a patient with bilateral renal agenesis. And for us from the nephrology team at Children's, we have the idea that if maternal fetal medicine can support this family prenatally and if the neonatologists feel like they can support this, um, baby once they're born, we are ready to do whatever it takes to, um, provide dialysis for this patient. And so for us in our institution that looks like having the baby come over as quickly as possible, having the neonatologist stabilize them, get them on ECM if they need to, um, having surgery consulted and urology consulted, anybody that we may need on our team, um, and then us starting dialysis as soon as needed, and for those bilateral renal agenesis patients, we know that they're gonna need dialysis and we know that they're. need nutrition. So we will start dialysis day 123 of life, um, as soon as we can, and we feel like for them, um, you know, avoiding fluid overload like that was spoken about earlier, um, is, uh, is helpful for us in managing this patient. So we treat them like any other patient. If we've got a team in team in the NICU who is willing to do the things for them, then we're there ready to go. So, talking about that, um, If One of the team members not willing to proceed, how strongly, you know, would someone like you be stepping in and advocate for the family and say, no, we should be aggressive. You know, that's a great question because I feel like in the time that I've been at the institution I am now, things have evolved, um. Widely because when I first came, there were discussions about these patients and, and the, the question of is ECMO a good idea or are we just prolonging the inevitable and Landon actually was a patient who, um, our nephrologist felt like, you know, given his diagnosis prenatally that he was not compatible with life and it was the neonatologist who really pushed and said, let's do this, let's try him on ECMO. And um they said, OK, that's fine, and look at him today, he's not even on dialysis right now, you know, he ended up getting renal re like some enough function to hang out almost 3 years old off dialysis. And so that patient really kind of shed light on us of, of, you know, at day 0 of life, it's maybe unfair to say what these patients are capable of later. And so we're kind of a full-court press institution. We have awesome resources. Uh, we've done a ton of baby dialysis and we've gotten good at it and so we do everything that we can for any of these patients who are born and, you know, we still have families who will withdraw care. We still have patients who don't survive, but, um, he was actually a patient who kind of, I think opened everyone's eyes to, oh wait, maybe this, maybe we don't know what we're talking about a day, days um of life one. So just to Kara's point, it is incredibly common for these kids to be just um very, very sick in the first few days of life, mostly from pulmonary and, and hypotensive type issues. But the reality is, is if you can get them over that, um, the, those, those issues sort of disappear. The pulmonary hypertension does not tend to persist like we oftentimes see in diaphragmatic hernia patients for whatever reason. Um, we, we can get beyond that. So, um, you know, the, the trick is trying to figure out within the first couple of days who's the true pulmonary survivor. And if you have tiny lungs by X-ray, you've got bilateral chest tubes in, you're not ventilating, you're not oxygenating, you're hypotensive. Those kids declare themselves, but when kids are um responding to your therapies, I think it's very reasonable to, to be aggressive and to go forward if that's what the family wants. Mhm Kara, what are some of the challenges, you know, these patients, despite we do significant prenatal counseling, still, when the baby is born, they mention those, you know, this is overwhelming even more than we mentioned. What are the challenges, daily challenges that you face in such in such a patient population? Um, I had a mom tell me the other day, she said, you and the NICU were so harsh in the beginning about the realities of what my son's life would look like, and it was very, very hard for us in the beginning, and she's like, and now on the other end of it, he was getting ready to go home after, you know, 3 or 4 months in the NICU. She said, I understand why you're like that, and I don't think that we are perfect in how we deliver information. Um, I'm an optimist, but you have to really be a realist when these babies are born, and it is shocking the number of babies we've had in the last couple of years who were not diagnosed prenatally, mainly because these families were not getting prenatal care, and so then you kind of have that barrier of, OK, where are we gonna go from here if you weren't even getting prenatal care, um, but just kind of trying to, to really, really identify the, the learning curve of these families and where their education level is and where their. Apprehension level is, um, but really being truly honest and, and harsh sometimes like the mom said, um, to get your point through, uh, I think it's, it's, it's easy for us to look at a baby on ECM and say, oh gosh, you know, this is really bad, but for someone who has never seen anything medical and they walk down a NICU and there's 4 of these babies lined up, you know, that to them may not seem as scary as it is to us. They just think, oh look, they do this all the time, it's gonna be fine, so. I agree. Thank you. Um, I think there is no, if there is any question from the panelist, Doctor Lim, go ahead. Alan and um Kara, I really enjoyed, you know, both of your talks as well as, you know, we, we learned so much from other speakers that um have presented today. And uh I, I really hope that people do see both from the medical surgical side as well as from the patient perspective and nurses's perspective, um, of how challenging this could be. Now, that's say, you know, we, we have presented Mainly, I will say the good side of the stories, um, had Any family, uh, come to you, Ellen and Kara, and say, we wish we haven't gone down this road. Um, And um They, or any patients that came to you and say, this is totally something that we don't expect. We thought that we would be in a much better place with your team, but we, we, we are now not in that position. Earth population since I've been there, I've not had a single parent say we wish we hadn't done it. And I was speaking to one of our nephrologists recently who's been there almost 20 years, and she said the same thing. Not a single family has said, I wish we hadn't gone through this, um, and it's a hard road. I mean, you know, even if you get to transplant, that's not a perfect fix for these patients. I mean, transplant, you know, you've got medications you gotta take, appointments. So, um, that makes me feel better when, you know, you're, you're presented with one of these babies that you look at and you say, this is gonna be a really long, hard road for the healthcare team and the family. Um, and so I like to kind of keep that in mind. I have the same experience. Um, most of the families, and, uh, do feel like they've been well educated. They, um, do know what to expect. We are, you know, talking to these families daily. Um, what I'm, what I've heard more is on the families that we haven't been successful, I've heard gratitude. I've heard thank you for giving us the Opportunity for giving our child a chance and for giving us the opportunity to spend time with our child and even when we, uh, you know, stop therapies on these children, um, they don't necessarily die quickly, um, and we've even had, uh, babies where we've stopped, um, interventions, stopped their dialysis and transitioned them to home and let them die in a home environment. Um, we've also had times where they've stayed in the hospital for 456 days. Until, until they succumb to their, uh, their, their renal failure. But overwhelmingly what, what I've heard from families is thank you. Um, I'm sure there are families out there that, that haven't felt that way. Um, I have not heard from them, uh, and I'm sure that, uh, they may feel differently in 1020 years, but, um, For the most part, by the time the families get to the NICU, uh, they've already decided in their mind that this is what they want to do for their child, and I think that is the one thing that really, um, uh, helps them accept when things don't go well is that they feel that it was their decision. And, um, I don't know about you, Alan, um, and also the audience still uh with us at this hour, um, after hearing what, um, Kara had spoken about and as well as, uh, what, uh, Doctor Ekenazi, uh, previously had spoken about in session two of this Reno series, you know, uh, are you ready to offer ECMO, uh, and go as far as offering ECMO? Uh, to this patient population. So if you can type, um, your response in the chat, uh, as audience and also let Alan, you know, comment on that, uh, quickly before, uh, we turn to our moderator to conclude the session. That'll be wonderful. So our experience at our center is that if we can ventilate these kids, typically they, they tend to go on to be pulmonary survivors and if we're failing at an oxygenation level, then we'll go ahead and, and, and put them on ECMO with full disclosure to the families, uh, that this is why we're doing it. Uh, as I alluded to in my talk, the beauty of that is that I have access for dialysis and hemo uh filtration, uh, which makes it just, uh, all the easier. So, um, it is something we don't offer lightly and we don't talk to families about it prenatally at our institution, mostly because our families are so overwhelmed and the chance of them needing to go to ECMO is still relatively small, so we tend to not burden the family with that at, at, at the prenatal consultation, um, visits. Um, but it's definitely something that we talk about as a team and offer to families when we think that it's, it has the potential for a good outcome. I have a neonatologist who always says to the family, your baby will tell us where they need to go, and I, I think that's a good message to remember because um when it gets really tough in those 1st 1 or 2 or 3 days, you know, it's really kind of goes back to we have all these interventions and you know, and Institution like mine, we have people with, we have buy-in from everyone at this point, um, but we can only do so much and so it's always a nice reminder that, you know, we can only do so much as we've got coming back and especially talking about, you know, the pulmonary hyperplasia, you can only do so much with teeny tiny, tiny, tiny lungs. I think my personal experience is that one of the things that makes the long-term issues is if your child has, has significant enough lung disease that they're gonna end up with a tracheostomy and mechanical ventilation. When you look forward to the eventual transplant and immunosuppression that that is, that that entails, having a trach is a massive obstacle, and so that's why it's so important early on to try and make a determination of, you know, can this child be technology-free from a respiratory standpoint, um, because that tells us a lot about their long-term risks of a good outcome. Thank you all so much for your insights, um, and your expertise that you provided, uh, to, to the session today. Um.