Multidisciplinary Approach: Intestinal Failure Innovations

This is to define working groups that care for kids with intestinal failure because intestinal failure is managed in many different aspects of your hospital from the NICU to the floor to outpatients to clinics with surgeons, neonatologists, and there's a lot of discussion about programmatic support, and we feel pretty, I think, passionate as a group that that's a better way to do it, but I think it's first. Nice to discuss how teams interact within the hospital to, uh, to care for these kids. So I'm gonna throw the first question to Conrad and just say, uh, to describe what, uh, what we consider as a, um, what is a team. How, how do you care for kids as a team, maybe here in Cincinnati versus others that um allow us to do things that we're gonna talk about today. I mean, the way we've looked at it in Cincinnati is that we've seen, we have sort of like a multi-pronged approach, um, where we have equal members in the team and the team starts from neonatology, um, where you have the neonatologist and the nutrition team within the NICUs. We also have the surgical team, um, that is key, having, you know, surgeons that participate within that, uh, within the program. Uh, we also have the, the GI team that. Bridges both the inpatient and outpatient aspect, um, we do have dietitians and nutritionists at work, both in the inpatient and outpatient aspect, and also the, the social, the social workers, they're, they're key members of the team, and, and basically we also consider them to be equal partners within that team. Sam, don't you agree? Oh, I agree completely, Conrad, and I think um. Uh, and, and don't forget we've recently, um, incorporated, uh, psychology. We have, uh, uh, an adherence specialist who comes to clinic, and then, and indeed we also, uh, have, uh, speech pathologists who come to clinic and, uh, work with our children who have eating disorders. Um, the, um, uh, I, I think it's been. Uh, shown, at least, um, uh, from our own experience and from the experience of, uh, uh, of others that a team approach is indeed an appropriate, uh, and important, uh, aspect of the care of these patients. Um, Dan Teitelbaum years ago, uh, looked at, uh, at survival at, uh, at University of Michigan and. And back when, each surgeon or gastroenterologist was taking care of 2 or 3 patients apiece, the, the 1 year mortality was something like 30 to 40%. Uh, and then when they developed a team approach in Michigan. Um, mortality dropped to about 5% per year. Um, and I, I think all of us have shown the same, uh, phenomenon, um, throughout our, uh, our history. Uh, here in Cincinnati, the, the, uh, we have a very mature program. It was started in 1984, actually, and back when I was in Pittsburgh, we, a group of us went, came here to Cincinnati. Uh, to model our program after Cincinnati's and, and the Pittsburgh program, and I know, um, Jeff, uh, heads that program up. Uh, the Pittsburgh program was, was, um, has been very successful and has published several papers, um, uh, really suggesting that, uh, that outcomes can be improved, uh, with a team approach. So, so 11 important thing I think is the question is, is which patients are intestinal failure patients? When, when is a patient defined as an intestinal patient, intestinal failure patient, and, and how do you, how do you interact with those patients? Monique, can you, uh, can you give it, how we define intestinal failure here and maybe we can get input from, uh, Valerie after that. So I think there's, there's two ways. So from my perspective of looking at intestinal failure, I think one is, of course, the, the length of bowel. So of course, if a, if a child has had a resection for, for whatever reason, and the diseases that can encompass why you would have, uh, resection are, are many. Um, and of course the other is, is, uh, they may have the adequate length of bowel, but their bowel doesn't work appropriately. So they're not able to absorb the nutrition and, and, and, uh, fluid in order to maintain growth or, or development in any way. So those are the kind of the two broad senses of how I Monique is a kid with medical neck in the NICU or intestinal failure patient. I think that if they're not able to, yeah, if they're not able to absorb the appropriate nutrition to maintain their growth, then there's an element of intestinal failure there. And so is there a role for a gastroenterologist to help understand better care for the premium and NICU, and I believe. That's true. I think it's really important. So Monique, but when you're in, in, in Illinois, do, do you get involved in the at-risk intestinal failure kids, or does it require there be some, um, level of failure prior to your involvement in their care in the NICU, let's say. I think it varies depending on the surgeon. Some patients, um, I'm consulted on earlier than other patients. Um, the surgeons there are also good with taking care of these patients, but I think they do recognize when the patients are unable to be advanced on their feedings, they're starting, especially when they start developing cholestasis, that perhaps I need to, um, be involved sooner rather than later in their care. Um, some of the, um, patients I'm involved in very soon, especially some of those patients who have significant loss. May have only residual 5 centimeters, 10 centimeters. I'm definitely involved very early in the conversations with the family, what life is going to be like, um, and what their options are if they choose to proceed with their care. The child is so sick that perhaps they're considering withdrawing care, what their care would be like if they were to survive, and ultimately if they would require transplantation. So I, I, I'm assuming Simon can hear me in Seattle, I think they're championed and they've presented us a lot of data to support how the um programmatic support of these patients really improves outcomes. Simon, um, how do you define your patients in Seattle and do you have a way of tracking all patients that are at risk or have intestinal failure so you can see how you're doing over time, uh, and, and know in real time the improvements that you've published and have shared with us through the literature. Um, yeah, we, we tend, our surgeons are involved pretty early, and I, I work very closely with Patrick Javid, who's uh one of our pediatric surgeons. So he knows what's going on surgically in the NICU, um, but. We tend not to get involved early unless there's some difficult conversations to be had. We've certainly influenced the management so that patients don't leave our NICU with cholestasis anymore, and then once they need to be transitioned to. Uh, home TPN and full medical management, they come to us, um, for education to be familiar with the, with the families. Certainly in the acute liver failure, uh, acute intestinal failure, that what I think of as, you know, severe neck, uh, acutely, we're not heavily involved. Uh, we're very happy to consult and be involved, but, uh, mostly that's between the neonatologists and the, and the surgeons. But I think we have, uh, we have expertise to offer. If the question comes up about end of life decisions and whether this is survivable, then we're called in very rapidly, um, and that's really where we're able to give families reassurance that that this, the traditional, um, So the response to the extreme short guarding a premature infant is not a survivable condition is now completely. You know, archaic and that with good management there are, there are great prospects for them. So yeah, we get involved early, but we, we rely very much on the surgical arm of our team to monitor them in the early stages and ensure that we get to know about them and pick them up in time. Simon, this is Todd Ponsky. Would you go ahead and turn on your camera so we can see you? I'm trying. I can't see the icons for. OK, so, so you see all of us sitting at the table right above our heads, right above our heads is a button that says start. If you just hit that button, that's your camera. No button that says start, I'm afraid. OK, well we'll figure that out, but we'll put a pretty picture of you up anyways. So, so Jeff, can you tell us about Pittsburgh? Do you, uh, do you have any involvement in developing protocols or standard ways of addressing feeding issues in at-risk kids and how you feed kids like gastroschisis or kids after neck in a standardized way, or is that dependent upon the neonatologist and the surgeon who's in the NICU at that time? I think that we have a very similar situation to what Simon described in Seattle that um whenever uh whenever there's acute intestinal failure or before um they've been on TPN for, um, generally we'd like to say the cutoff is 30 days, but whenever there is some chronicity that that develops, um, that's when we generally get involved. Um, we, we do, um, leave the initial um management to the neonatologist and often the surgical, um. Uh, staff, because a lot of the acute issues, uh, end up being, um, surgical to start off with. Now, like Seattle, whenever it's clear that a patient's going to have intestinal failure, um, and there's going to be chronicity or if there is again sort of end of life discussions or or how to proceed, then we, we tend to get involved, uh, earlier. So I think it's a very similar situation to what Simon described in Seattle. So let me challenge or ask people, we know that how TPN is administered has a huge difference in cholestasis and some of the nutritional. Complications we see. So what's the role of lipid management and TPN? Who influences that? Is it the neonatologists, the surgeons, the gastroenterologists? Are you doing it on a per patient basis, or have you kind of come up with a protocol? Anyone can step in, I think that. Of The your initial comments on the team management approach is uh is very important because it's taken time and a lot of effort to collaborate with the neonatologists and the surgeons and to come up with a a lipid management strategy that um that we do tend to employ up in the initial stages of the the lipid management strategy such as minimization of the lipids and um. Uh Reduction of some of the trace elements in the CPN and also watching things like glucose infusion rates over time through a lot of discussion with the neonatologists and the surgeons have come to sort of uh uh the same uh management strategies that we do in the intestinal rehab center here. Um, so oftentimes by by the time kids get to. Around 30 days or whenever there's criticity that's established, um, some of these some of these protocols are already in place, and then we continue them and manage them. It's kind of funny. I was thinking about this this morning coming in is that we now oftentimes have to. Pull back a little bit. There's sometimes when I think that we overdo it with regard to lipid immunization and the NICU, and by the time we get involved, I've actually begun saying maybe we need to give the child a little bit more lipid because we worry about the alternative the fatty acid deficiency, especially in a premature baby. So it's it's a constant sort of give and take and interplay between the neonatologists, surgeons, and ourselves with regard to how to get the kid to grow, which is is often one of the biggest challenges when you're trying to do things like limit lipids, but yet try to provide, um, I guess you could say it's a hepatic sparing TPN or TPN is not going to lead to excessive liver damage. So it's always a constant interplay between, I, I think really between all three teams, and it's, it's where that communication is essential, um, but to answer your initial question, the, the protocols and the um. The strategies for especially lipid minimization are often started by the neonatologists, but it's been through years of collaboration with them that we've come to where we are today. So, I'm excited that can give us some really good insight as to some of the different ways they have to provide lipids. Can, can you kind of talk to this, uh, Gresh? Yeah, I'm adding from, uh, I'm sorry about my voice, a bit of sore throat, but, uh, uh, you know, in the UK, especially, you know, it's the same kind of strategy, whatever, uh, uh, Doctor Rudolph and Doctor Hosson has mentioned, uh, you, you know, working, uh, closely with the neonatologist, gastroenterologist, and the hepatologist, uh, and manage these complex children. The one thing I do like to stress is. That uh we should not mention the word transplant in the beginning to the families. Uh, you know, it's important to mention it, but not overplayed, because the families get too focused on transplantation. I think the focus will be on pain management and, and, and strategies, uh. Could be a management, uh, rather than transplantation, and I think that's, that's very, uh, important to uh to rememberwise all the families think that transplantation is a magical cure, get too focused on it. So I think the stress is on. And avoiding complications related to, uh, you know. Uh, so this is mainly done by the gastroenterology teams in the UK and, and the hepatology teams support them. There is a need for intestinal transplantation or a development of spaces. Right How about, how about going over to Los Angeles, Bob? Is there anything you want to add to this, this point? Um, no, I think very similar to the others, uh, certainly, multidisciplinary team effort and especially in terms of forming guidelines for many of our, our, our patients, but as we all probably appreciate. Each of these children with intestinal failure are so unique. So then there comes the individualization of care, and a lot of that in our NICU is certainly driven or led by the neonatologists, but truly with input from all other disciplines that were mentioned. So, so I, I kind of have a, a unique approach to this which I think is different than the field because I see adaptation as a normal process that every baby does at birth to accommodate the enteral nutrition needed to grow and that the gut grows in a very rapid manner and understanding that natural history in the face of loss like we do surgically or injury as we see with neck gives a natural timeline in which we can understand ways. To implement new therapies and feeding to improve this, and if you don't know how the kids that do well do well, and you don't, you won't understand where your inflection points to intervene are, and I'm a proponent that earlier intervention has a better sustainability potentially reducing and avoiding long term morbidity and mortality. And so by having a way of looking at the natural history in our institution of all. Patients that are not segregated into different units but can be seen as a whole I think offers us and we'll show you data later that gives us the ability to tell you what all patients do have gastroschisis over time as opposed to the select few that we get called about as specialists when the people that routinely care for those kids recognize they're not following the right pattern and by doing so you can implement uh not just feeding and nutritional strategies. You can identify things. I'm going to ask Conrad to talk about briefly about when we see some of these vaccinations that aren't given to kids and they get sick later, how that affected the kids he cared for here and how he was able to get our neonatologists to recognize the role of possibly vaccinating kids in the NICU, which I think was a big improvement that he gave to us. Well, I mean that's true. I think what we used to see in terms of Um, readmission rates was that almost 30% of the patients who left the hospital from the Neonatal intensive care unit without receiving rotavirus vaccinations were readmitted with that. I mean, it wasn't that they had increased stool output from intestinal failure or shotgut, but actually that they were positive for rotavirus and by just showing that data to the neonatologists we were able to convince them it's still a work in progress, but We're able to convince them at least that if we monitor these patients on our own, identify them, administer the vaccination, if they were still in the hospital, isolated them, we were able to cut that down, you know, significantly to almost zero at this point. So that at least from a cost perspective and also from an outcome perspective, that took that particular, you know, infection out of the scenario. So it made a big difference, at least, you know, with that intervention. I'd just like to amplify it a little bit. Um, uh, I think, um, one non-billable exercise that we, uh, that we employ in this institution is joint rounds weekly. Surgeons, um, gastroenterologists, dietitians, neonatologists all meet together, uh, for at least an hour and Run the list of patients in the NICU and I think that's valuable from two points of view. Number one, our, our nurses or our administrators recognize when we're deviating from protocols and so they pointed out to us or when a patient, for example, has not gotten rotavirus vaccination. Uh, and needs of rotavirus vaccination. And secondly, um, when a patient is at risk for long-term intestinal failure, um, we, uh, they, we are introduced, all of us are introduced to the family, so that helps build rapport. So I think we know all of the patients to some degree every week, and, uh, I think it's an extraordinarily valuable, uh, exercise. So, so I guess this brings me to the first maybe controversy, and I'm gonna send this to Cartland, uh, my surgeon to the right, because he has experience at Pittsburgh, a larger center similar to ours, and now he's starting a center where there's not as many people competing for patients. But the question is, who should take care of these patients because our intestinal patients usually come from the NICCU initially in most institutions, and we'll talk about referrals here in a second, but the babies that are born, is it the doctor on call, the surgeon and who should just manage that patient based on their best practices, what they believe are true, or should some of these more complicated kids directly go to team management like we are, or maybe speak to how teams like this can influence the care of patients that are cared for by the community pediatric surgeon or anyone else in your practice that may not have expertise in this. Before you answer, I just want to make sure I understand the question. Oh yes, OK. So are you, are you asking, so if someone's, I'm on call, I have, I'm the least qualified, a patient comes in, I have an intestinal failure patient. Are you saying the question is, do, let's take two situations. A kid, a kid comes in has perforated neck last night, and you did an ileocecal resection, right? Who manages that kid for the next, next 8 weeks in the NICU? So that just brought up 1000 questions. Number 1, I, ignorant enough, didn't, would never have considered that an intestinal failure patient, an ileocytectomy on a patient. Obviously that's where I need to reconnoiter my brain a little bit. That's question one. But in a patient that we actually called you, I don't know if you know, remember, I had Garrison call you because we had a kid that had gastroschisis, closed gastroschisis, had no gut. I was a primary anastomosis, so that patient clearly is short gut intestinal failure. Do I continue to take care of that patient, because I was the surgeon that took care of it? Do I say to some of my partners, you're the person that's recognized as the expert, or do I say, we should actually send this patient to another facility? So I hear. Intra and inter hospitals decisions. You know, I think that the idea of having team rounds, the idea of having algorithms that you generate within your own team that help identify the patients that are going to be at risk is really paramount to getting these kids started in the proper therapies and Even in big centers that have intestinal failure programs, it's still hard to to sort out which patients require more joint management approaches and which patients would be OK just on their own, and I think that the The struggle that happens is that these patients come in unexpected. They come in at night, they have necrotizing enterocolitis, they get an operation, they get a resection, small or short resection, or a large resection or a small resection, and those kids have immediate needs. And, and as a pediatric general surgeon, we know how to manage those immediate life threatening problems. But I think we have to, we have to be open to the idea that there are groups that can come together and manage these kids in a, in a more coherent, comprehensive fashion, and that doesn't mean that we're giving up the management of those patients. That is a, as a pediatric surgeon that that. Doesn't see this often that you don't have to give up the management and the storm enclosure and the relationship that you have with the family when you've gone through a really very life threatening situation together. You don't have to give that up. You can still be a part of that management. You can still be a part of the ongoing care of the child, and I think that in places that have referral, you know, widespread referral practices, that's really paramount for our success is to be able to maintain communication and be able to. Keep the the people that that started the care involved. So, you know, really the trouble is, is how to identify these kids and how do you, as a surgeon not taking care of these kids on a regular basis, say this child needs, needs more comprehensive care. And there are lots of definitions. There are people who say 2 months on TPN and you're and you're labeled intestinal failure. There are people who say 1 month on TPN. And there are some people who would say you have any bowel resection with necrotizing enterocolitis. I think probably all of us have patients who've had had. Maybe minimal resection with necrotizing entercolitis or gastroschisis, for instance, who It would not be predicted to have intestinal failure going forward, but as they progress, they have trouble with their motility or they have trouble with with secretory diarrhea or something that doesn't allow them to progress, and I think maintaining maintaining a diligence, maintaining a communication, and An open mindedness about this patient isn't progressing, you know, we're not really here to put out fires. We're here to predict the progress. And when we don't see the progress that we expect, then that's the time to reach out for help. I, you know, I want to go back to Monique when you gave your definition. I'm still confused. So, what I'm learning so far here is that everyone gets involved early here at Cincinnati. Um, I don't do that, and I'm learning already that I wait till there's a problem, and then I say, maybe we should get GI involved. Probably it should be, I finished the case and say, OK, we need GI involved. I think that is that what I'm hearing is early involvement of the team, not just waiting till there becomes a problem and try to predict who's going to need that. And that's another question I still don't get is who is the patient that needs that. Obviously, the ones that have loss of a lot of gut, but the ones in the middle, I'm, I'm confused about. So early involvement. And um and then who gets involved. It sounds like neonatology, gastroenterology, surgery, nutrition. So just real quick, I don't know if this is a poll question, because I think what is the most important thing we can do for any of these at-risk children to provide them an ability to be internally independent at some point in time, and I don't know if anyone would argue that it's luminal nutrition, appropriately administered luminal nutrition. And that in my opinion that administering the nutrition is providing the stimulus growth that goes through normal adaptation of birth and development and allows the kids to heal and lack of the ability to feed kids, I think attenuates that adaptive process. So we can start to talk about if people feel there's something else. Initially more important than nutrition, but for me whether we feed the stomach, beyond the stomach, and how we feed are all important concepts that our daily hurdles on NICU rounds that generate a lot of stopping and starting of feeds, different types of feeds, changing of formulas that occur, and if it's not in a And if there's not a programmatic approach to that, you can really see very disparate care of similar patients next to each other in your same NICU, and I would argue that that probably doesn't give you an insight as to how we should be treating these kids better moving forward. So what's a poll question? So my poll question is, is. Help me, Sam. You're good at these. Um, I mean, what's the question? The question, I guess the question is, um, my first question is, is, is, um. How, how do you feed your kids, your at-risk kids? What's, what is the, the best way to feed um kids at risk for, um, for intestinal failure, ad-lib PO with an NG as a bolus, NG as a drip. Or as a post pyloric drip feeds, or is it better to just give them TPN and let them heal? Yes, that's a good multiple choice question and no, I agree that's it's an excellent question and then we can discuss which type of formula afterwards, but. I think uh we'll see what the what the group decides. So I'm hearing ad lib PO with NG bolus, ad lib with NG continuous, or not even ad lib, just NG continuous, and then post pyloric. Those are the choices. And then we'll get into formula breast milk. Let's see what the audience says here. OK, well what It's I think, I mean, the key thing also it's not like identifying who's at risk and monitoring those patients. I mean, there's certain diagnosis, so. Um, a post resection kid, um, whether it's neck or from a treasure or from an isolated perforation, is potentially, you know, you know, somewhat at risk. I mean, you have some isolated perforation kids who would do well, and then you have those who would definitely, you know, who we think have enough bowel, who would not do well. So that's one. The kids also with gastrocski. I mean some of the simple or complex as schizo kids would do well, but I think monitoring them from the start so that you can appropriately identify those who might not do well based on, you know, when are they going to open up? I mean, are they going to need significantly TPN over time? It doesn't always depend on the length of the bowel, and the reasia kids also depend on the type of atrisia and the location. You know the seizure occurred, you can definitely impact on the length of time. I know that traditionally we think about the amount of bowel. I mean people used to say about 50% of bowel. I mean we now know that kids with 50% will do well, and I mean even as low as probably about 20 to 10% will do significantly well, and some of them might be able to get off TPN. And there also used to be the dogma that if the IC valve was there or not, you know, made a difference. We also know that it really doesn't matter. I think what matters is whether they have a colon or not. It's not just the IC valve. So I mean, I think we're learning a lot more. So following these kids as Dr. Helmer said from the start and trying to identify this crazy. what a normal adaptive response history in the patients over time gives you a better idea, and I think that the idea of having the team involved from the start then helps you to build that portfolio in terms of your thought process and coming up with with with with how and when you decide that all the interventions are appropriate. So I just want to clarify who's on your team. It's GI surgery, NICU, and nutrition, is that correct, and psychology and social work, really? And they all come to these rounds once or twice a week. Yes, once a week. So it's important that, uh, the baby, so I, it starts actually prenatally. I, I prenatally consult all the gastroschisis kids and the kids who have known, and it's an opportunity to emphasize the, the data that we'll show you in a minute that suggests that there's a huge Huge benefit for breast milk in our population of kids that um we can't always mimic. Even donor breast milk is fine, but hard to grow and we can't mimic with other formulas, and I, and I can't tell you how easy it is to convince the mom that you've talked about that data prenatally, who's having trouble pumping to give it that extra chance because um I think most people would, when we get to the discussion that you have to feed these kids and. That if they're throwing up and their stomachs aren't emptying, which is a huge problem in our field, the stomach can limit the nutrition these kids can get, and it can result in us just using TPN as a form of growing and not really pushing the fact that the rest of the bowel may function in some of these kids, and you just have to look at innovative ways of post-gastrically feeding these kids to get them to get enough animal nutrition to. Do the adaption that we want and formulas like breast milk may actually um not just provide the uh immunostimulatory but motility effects of breast milk are being shown in the different oligosaccharides and the immune improvements and the the healing aspects of breast milk is telling me it's not just a nutrient, it's a therapy and it's, it's a great therapy for the patients we care for so. Here's the results to your poll, and I'm gonna turn it over to you. So, uh, almost 70%, uh, do NG, not post pyloric, NG continuous, uh, 30% do the ad lib with PO bolus. Nobody posts pyloric. So I'm curious what you think. Sorry to interrupt. Two people, I wanted to say, um, go back to 11 thing that Mike mentioned and Sam mentioned earlier, and you expressed, uh, surprise about. Having a social worker and a nutritionist involved in the rounds is, I think, really important. The nutritionist, of course, has an obvious role. The social worker, I think, does not have as obvious a role to the to the medical team, but this diagnosis and the treatment of course these families are going to embark on is going to last years, and it is a huge stress on the family, both socially, emotionally, financially. That it's, it's, I think, paramount that you have good social work support to help these families get plugged in discharging Bobby on Friday. Can you come by and see him? And by the way, they need a pump and they have TPN and they have, they have tubes and the plan. It's actually, I mean, I'm tweeting what I think are the highest, the most impressive point I've heard so far, Sam, is what you said when you said that the mortality has been shown to go down. Going back to my hospital tomorrow, I'm going to say to them, it's been shown we have to do this team approach. It decreases mortality. The numbers are on the screen. Sam can speak. I mean, Conrad. I can speak to the fact that just putting our, there's nothing that improved our out well, let him say it. I won't go for it. No, I mean, I mean, it's, it's been shown. I mean there are at least a couple of publications that clearly show that the biggest impact on, on improving outcome was the team approach. And it's not, it's not us alone, it's all centers. I mean, Boston Children's has similar data suggesting that they've reduced mortality by 2/3 in the last 5 years. In fact, the old, you know, the classic PFCON study, and maybe, maybe, uh, others who were, uh, co-investigators in that PIFCON group, it was a pediatric intestinal failure consortium. Uh, a study that was published in the Journal of Pediatrics, uh, uh, about, about 2 or 3 years ago, uh, all those data are old data, and they're really archaic data because I think in the past 5 years, outcomes have improved so dramatically that, uh, that we can't rely on, on, or we can't expect a 25% mortality or referral for transplantation. Um, I wonder if any one of our distant, uh, participants, uh, maybe Simon, uh, you were, and Jeff, you were both in the DFA group. Oh, absolutely. I mean, I think that, uh, the, what we were seeing 1012 years ago of these kids needing liver bowel transplants by 1 year or so of age, that just doesn't happen anymore. Um, the outcomes are remarkably different. Um, I think going back to that issue about how do we feed patients, everybody's looking for medicine by numbers, but I think the important thing about an expert team is that we take each patient individually and we assess what they need. Yeah, occasionally I feed post pylorically. Um, but nowadays I've got to say I'm much more inclined to give the child a chance to feed because I don't feel that pressure that I used to feel that if I didn't get them on as much enteral feed as possible immediately that they would end up with liver disease. So you really have to individualize your treatment for for these patients, and that's, that's where the experience and expertise and the and the multidisciplinary aspects of a of a. Of the team really come into play. Now we're not lucky enough to be able to round with a vast team, but, but surgeon, physician, and nutritionists get together at least once a week for our patients, and it's absolutely crucial. So the comment on the PICon data. You talked about before. I mean, I think one of the one of the true advantages, one of the very nice things about the PISA study is it was a, it was a tight cohort that it was a period of 5 years, I believe that that a patient had to be born into that really gave you a glimpse of. What the outcomes were for that particular cohort unfortunately, well, actually fortunately I guess as Sam has mentioned, the outcomes have improved over time due to different management strategies. I think the team approach, lipid management strategies, we haven't talked about central lines at all and how to manage central lines, but all of those, all of the things that we have done to improve care have made the data from the initial PICO analysis almost obsolete with regard to looking at. Uh mortality rates and the numbers listed for transplants and things like that, um, but it's, it's an interesting study and it does give you a glimpse of of a of a huge multi-center study with a large number of patients all born within a 5 year span of each other that tells you what the status of intestinal failure was at that time. But uh like you said, I think that it's a um. You expect the mortality rate of 25% these days is Equates to uh failure um I I think that uh most programs do much better than that now. So yeah, you mentioned Lyme infections, and I think getting a standardized way of caring for these kids have improved and this is a measurable um. Result that we can have. So if you look at the PIPCO reported data in their, their study, it was well over 8 infections per 1000 catheter days. And in our institution, we broke our data down from the 3 years prior to when we generated this team we're talking about to afterwards, and our infection rate was actually higher than PIPCO. It was 12 per 1000 and as of last year it was 2. Per 1000 and even this year it's it's actually almost getting down to less than 1 per 1000 following a very standardized way of of treating Lyme so we can talk about the treatment of Lyme's which I think is important, but I think the concept that you can actually change the care that's not just in the intestinal failure of patients that are classically been TP independent for 60 days, but the kids from the very beginning that aren't getting Lyme infection. Do better than the ones that do and this is something the institution can look at and it actually validates the resources that brings us together and gives a better outcome where other people may not have thought that this is just kind of like a token that we actually have this committee. This is real real benefit for real patients that really impart the care you provide for kids. I, I think you're, I think you're right. I think it does validate the team approach and, and, and I think when you're you're talking about lot infections, I think that we all know from a hospital, um, what the state is that lot infections, and especially um. Infections within the hospital, in the NICU, the PICUs on the floors are a very, very hot topic of conversation which have led to standardized care, things like bundles, the bundle of approaches of combining best practices, having in our hospital communications. I know that our hospital participates in a multi-hospital. It looks at different bundles with regard to different safety practices, including Lyme infection. And the ability to learn from other hospitals and to identify and develop team approaches within your hospital, not only for the intestinal rehab kids or the intestinal failure kids, but for all kids in general, I think is is a very important part. With regard to especially in our case, Lyme infections, I think in Tesla rehab, you know, I think that kids with intestinal failure are a somewhat unique population with regard to Lyme infections, with regard to their propensity to get infected, as well as the type of infections that they get. And our experience with the central line management of these kids, I think has benefited hospitals and other kids and other populations with central lines in general when developing some of these protocols as well. Here's a poll question. Is it OK to feed our kids in the NICU with PPICC lines as long as they're there, or are there people that believe in placing BR reacts, and when do they do it? So, first question is, is, uh, for the kid with intestinal failure, is a PICC OK for TPN, or should they be administered through some other central access like a BRRA? Todd, Todd's typing this up, but people chime in as they want. I think Simon, I, I mean, I think all our children start with PICs, um, I have no problem with it, they need to be treated with just as much respect as any other central line, um, but they start with PICs and generally as they, uh, graduate to the, the more long term care. We go ahead and we get the permanent line placed before they go home. I think we do a combination of both, but I certainly see no problem with using a PIC when when having. I think there was a paper in JPGN, the Toronto Experience, which looked at PICC lines and the administration of PPN. We personally prefer when kids are going home. We've had very few kids ever go home with PICC lines instead of tunnel Central lines. I think it's a lot safer way to. Uh, administer the the TPN and and also implies the longevity. Um, I, I, I think that, um, using a PIICC line until it's uh stiffened and, um, and, uh, not functional, it's not, it's not the way to, to, to go, um, so I mean I think starting with PICC lines, I don't see any reason why we can't give uh TPN uh through PICC lines. But again, as pregnancy develops, the idea of putting in a central line, maybe a time of stoma takedown, maybe another time when they have to undergo anesthesia, but I think the idea of eventually putting in a tunnel central line is a good idea. I, I'm going to echo that comment, Jeff's comments. I think that In the intestinal failure population, you have to be very respectful of your vascular access. If you have a PICC line that's working well, then you should use that PICC line. It may not be a great stable form of access for long term going home, teaching the parents care, but it's there's nothing wrong with it to use in the hospital and we have had kids go home with a PICC line just for, you know, temporary measure. I think it's more important that you protect your vascular access with passion. It's really important for these kids for long term. I'm so shocked by the results, actually. I thought. I thought the majority would say PICC line. It's not the majority. Um, it's 30%. We have 30% right IJ, 25% subclavian broviac. We have someone that said femoral roviac, um. Especially since we talked about we want early intervention, we don't necessarily know who's going to have a problem, who's not. You put a PICC line in, they may not have a problem. They may not need TPN. Um, so I say save them, just like you said, save the veins, convert them to a broviac if you need to later on. I'm, I'm, I'm surprised how so many people choose the, so, so I'm not as surprised that all of our NICU kids are going to get PICC lines because that's the standard, at least here. But there's data that's starting to come out, and if you're paying attention, PICC lines are not benign, and they have different levels of complications that they can have. And so, um, having a PICC line in until it goes bad makes a whole lot of sense, as Cartland says, because you're protecting it. But you're going back to the OR on a kid to do a gastrostomy. Should you be putting a roviac in? Should you be doing that as an outpatient? Uh, the Broviacs, uh, the PICC lines tend to be. Very firm and these kids grow fast and what was the central PICC line can become embedded into different areas and we've certainly have seen some complications of PICC lines and, and I'm a proponent of PICC lines. I think that they're great in our NICU and I am just like Hartland, but I also wonder, um, at what point is it right for me to be thinking about, uh, the roback is it when the kid goes home, if the kid spends 4 or 5 months with us, should they have. The PICC line for the 1st 4 or 5 months of life, um, that's typically what we do, and I wanted to hear what other people say because, because I do think that we really became pick heavy in the past, and I suspect we're going to come back to understanding some of these kids might have some benefits to a Broviak sooner than maybe like we currently do here. It's just a point of discussion. I would say reviewing the I the IR literature suggests. And these are all comers, adults and children, that the Complication-free survival of a PICC line is only about half that of a broviac. Uh, now, this includes adults who may have more complications, but, um, and then the, uh, when I discussed PICC lines with our IR colleagues, they say, oh, PICC lines should be much more damaging to the vessel because there's far more contact with the vessel. We don't see that empirically, and I don't know whether our other, uh, centers, uh, believe the same. As I do, but I empirically, it appears that our infants do better with PPICC lines when they're in the hospital. I think one thing that relates to that is the the local practices, you know, in, in most NICUs. You almost can't be there overnight without getting a PICC line. So they, they start with that, and I wouldn't necessarily argue that a PICC line is going to be better for a preferred route of vascular access, which I think the poll probably reflects a preferred route of vascular access. I think that the, the kids get a PICC line. We should use the PICC line until it's clear that they're going to need something more long term. And one of the, one of the tweets that came through here, uh talked about using cuffed PICC lines, which I think we have not been using, but it's certainly, it's a, it's a reasonable, it's a reasonable catheter. I think that converting to a roviac or a cuff line in whatever vessel you choose is probably more local preference in some cases. I think it's clear that subclavian approach is probably inferior to a jugular approach for for risk of stenosis, but local practices in many neonatology units would would have kids getting PICC lines before they even call us. If I can jump in here just for a second, I, I, I don't want this to be overlooked at, uh, at this point. So I think previously we've, we've concurred that it sounds like or it seems like the mortality of intestinal failure has been reduced over time. And so that we're having longer and longer, long-term survival, uh, survivors with, uh, they've had intestinal failure. And I think that as disease does when you solve one problem, new problems will come up. I think that what Carlin stated about trying to preserve vascular access as much as possible is extremely important because what you can potentially foresee in the future is kids who've had vascular access sites used up stenotic central venous access. As they grow, potentially causing more complications even after they get off the TPN. This may be one of the future problems that we see as these infants and young children become adults. I think it's extremely important early on to try to preserve as much faster access as possible, not only for the sake of The fact that it's it's one of the indications for intestinal transplant, but also for the for the long term health and survival of these kids as they get older. I wonder if I could ask Rob and how often they're doing intestinal transplants for lack of vascular access though. Yeah, I mean, I think, uh, you know, from the UK, uh, uh, in our center, the pediatric gastroenterology and the vascular access team is very well established. So actually, our transplant anesthetists are the part of the vascular access team, which makes that they have uh some responsibility to preserve venous access for as long as possible. So I think whatever Jeff said and some others have said, we have to treat these veins and these lines with respect, and they have to be done by a dedicated team, which is used to doing the vascular access all the time, which means that we can maintain vascular patency for a long period of time. What's happened now, as Simon has said, is that we're no longer seeing those young children with liver problems, uh, coming for intestinal transplantation, but we are seeing some children coming with impaired vascular access now for intestinal transplantation, you know, particularly in the short bowel syndrome patients because they stay in the hospital for a long period of time. And, and, and that's why we are propagating that these lines need to be done by dedicated vascular access teams or pediatric surgeons or anesthetists who are experienced in placing them via the percutaneous way and with an ultrasound guided technique. Yes, and I agree with what Geresh said. I would also add to answer Sam's question, just as the emphasis and the appreciation on lipid sparing effects of, of uh. With regards to liver disease for these patients is now really well understood in our field. I think there's also been this emphasis on the importance of maintaining vascular access, and within the last 3 years, I can't, we have not transplanted a child because of limitations of vascular access. So I think the outcomes in this area, as has been shown at Cincinnati with reducing infection rates and preservation of vascular access is coming a long way in the field. So, so one thing I think that this brings up is a really protocolized way of managing these, these lines for, uh, like ethanol locks and or how we handle fevers. So I'm gonna ask Maria because she was just recently finishing her fellowship. She got all the calls. Tell us about what is our management of when to use alcohol locks, how we manage lines, and when a child has a fever, what's the protocol here. So as you mentioned, it's a it's a nice standardized approach that we've we've developed over time, seeing significant improvement in in maintenance of lines in general. So yes, the standard phone call from the parents in the middle of the night, my child has a central line and and has a fever. Unfortunately that warrants a visit to the emergency room. But what we've done is we've established a a nice means of communication obviously with the families as they call us, but we've also implemented a way of communicating directly with the emergency room staff, um, with a phone call as well after speaking with the family, um, about the importance of implementing therapy sooner rather than later. So, uh, of course we call the emergency room, uh, folks after we speak with the family, and we have a, uh, um, uh, a list of things that we list off, of course, weight and, and the appropriate, uh, medications based on the child's history and Lyme infection. Um, uh, pattern of drug resistance in particular, uh, which again as they enter the door we're able to implement care very quickly, which in fact makes a difference when it comes to outcomes if you're at home at night, kid has a fever of 101, how do you know about that? That's the beauty, that's the beauty of uh. Electronic medical records, of course, um, we have uh a lot of this information is listed in a certain tab under the child's problem list so we're able to see in fact after discussion with the with the uh appropriate folks what is the is the best antibiotic therapy for them, um, again it's all kind of listed in a nice fashion in in our electronic medical records after reviewing the history in the past so we know what to implement when they when they come into the door, which is a huge help. By the team of these adults, these gas pediatric gastroenterologists into the NICU implementing that so that information the NICU becomes available to the kid that's been discharged home and has been home for 1 month is 5 month old, and they're calling Moe, um, knows all that stuff that happened in the NICU. Talk, talk about alcohol locks. When do we use them? Those are key, I think. Um, I think there's, there's several different ways that we implement alcohol locks. Um, one is, uh, the type of line, I think is, is a, uh, a thing that we think about. The other is, is dependent on how many Lyme infections a kid may have had in a year, particularly we, we, our cutoff is generally 2 or more. Um, but there's also room to say that there. Attending preference and whether to whether to start ethanol locks, um, and that's been hugely beneficial as well, um, you know, the duration of the dwell is anywhere from 2 to 6 hours, um, again, depending on, on the, the type of line, the size of the line, um, and that again has been pretty impactful in terms of reducing our, our bloodstream infections in general in the hospital. So what makes you say you can't use an ethanol lock? Are there certain organisms? What's the, what's your criteria? So I, I think that the only thing that I've encountered in my experience has been the type of line per se. There's been some, uh, some resistance to turning on this line can't accept this type of ethno lock. But actually, if I, if I may kind of bounce back a question, um, and I don't know if this is something for the group or, or for the folks, uh, across the, uh, uh, the country. There's been some kids that we've seen come in that have actually been on antibiotic locks, which I think is an interesting, um, thing to discuss because we typically don't embrace that that same policy, um, so I'm wondering what the group's experience has been with antibiotic locks in particular in in their own institutions. So, um, go ahead, I heard someone wanna speak virtually. So, um, we use ethanol ox, but, but occasionally we get an unusual organism and someone, other people have mentioned to me that in those situations they've actually tried. Antibiotics that are specific to that organism. Um, I'm very aggressive with ethanol locks, um, and also we have, I would, I'm curious after other people give input about antibiotics, what, you know, the lines have been cut. So how do you know the volume that you're supposed to give? Do you have a way of calculating that by looking at an X-ray, blah, blah, blah, blah blah. So, um. Has anyone here used antibiotic locks? Yes, so this is Simon. Before ethanol was regularly used, we used to use antibiotic locks, but it's far less resistance to ethanol. But the other question that you had about, you know, how do you know the volume? We have a standard across the hospital. We've got rid of all polyurethane. Um, indwelling catheters, they're all elastic so that we know that we can use ethanol. We have the surgeons measure the volume directly when they're placed and it's recorded in the notes. And then we also work with our ER. So there's a standard protocol that they follow when a patient comes in with a suspected Lyme infection. And I certainly don't tell the parents or the or the ER that they have to have a fever. If the, if the parents say the child looks funny, they think they've got a line, they smell funny, they're behaving funny, then they've got a Lyme infection until proved otherwise, and we're very aggressive in that manner. Who takes care of Lyme infections and not to underscore that these kids get sick very fast, and I think all of us can give you examples of a child that probably could have done very well and came in with an overwhelming Lyme infection in the ICU and you just never got them back to where they were before. So don't underestimate. Um, how much damage can be done, certainly over time to the liver and others, but Lyme infections are a big deal, and I think they, we'll show you data here in a minute that the kids that have had Lyme infections don't do as well as the ones that did not. Implementing protocols about how the emergency room deals with patients that have a central line and a fever has really changed our experience while I was in Pittsburgh and Jeff Rudolph might be able to expand on that a little bit, but it's very easy to follow these kids as something as simple as their growth curve, and You can, you can plot every time they had a line infection. They're growing, growing, growing, they drop down and it takes them a little while to recover and get back. It's, it's a real detriment to their progress to have recurrently infections, and I think it's really important to Be aggressive with getting those kids started on antibiotics immediately when they arrive to the emergency room. I mean, I think that that um both the, the, the chronic effects of uh multiple line infections are important, and I think a great reason to to think about um uh prophylactic strategies such as uh ethanol locks, um, and, and, and, you know, we, I, I don't think ethanol locks should be a a a crutch. It goes without saying that, uh, that proper line care and good education. I think one of the things that I learned in Cincinnati. The most was the education of parents and in the hospital, the education of staff with regard to proper care of the line and sterile technique, which can't be underscored, but I agree with the idea of protocols within the emergency room. We have set up a protocol. Of the children's emergency room where kids are flagged if they have intestinal failure in central line and if they are on TPN and have a fever, they are immediately brought back to one of the trauma rooms or the acute care rooms, and they're supposed to be seen within. A certain period of time and antibiotic doses, the timing of blood cultures, initial labs, and the initial antibiotic doses, we all have goal times for that, and I think that what we don't always what we strive to do to meet those goals all the time. I think they do a fairly good job in the emergency room at doing that, but it's taken collaboration just like within the NICU with the emergency room doctors and the emergency room physicians to understand that it's very important to start care and the treatment of these antibiotics, these infections rapidly. So can someone comment, maybe Sam or Simon, about the damaging that effects of the ethanol lock to the polyurethane catheters that have been reported and, and what is the lowest weight kit and, and that you would use an ethanol lock for because those I think are common questions. Yeah, well, I think the polyurethane issue is, uh, maybe, may indeed be a myth, um, we, um. Uh, the, it, it's supposed to dry the polyurethane catheter, and, uh, and it is supposed to make it more prone to breakage. Um, but, you know, Dennis Mackey, who was really the guru of, of ethanol locks, uh, Mackey from, uh, from Madison, Wisconsin, an adult ID guy. Um, had, um, really demonstrated in vitro that polyurethane catheters have not, do not change their, their elastic characteristics when exposed to ethanol, just as long as the silastic catheters. So, um, if, if we're desperate, we'll occasionally, uh, Put ethanol and still ethanol in a polyurethane. Interestingly, I just pulled the audience and almost half of them have never used ethanol locks. Can, can we give a 32nd summary and maybe I'll try to find a reference of how we do an ethanol lock? Yeah, there are several strategies available. I think, um, uh, most people use 70%, but I I know that in Pittsburgh now, they're, they're cutting it down to 35% ethanol, and uh we use uh ethanol locks as a dwell when the patient has a uh uh a TPN window. So, certainly, by definition, the patient needs to have be on cycle TPN. Um, and so there has to be a period of time off TPN so the, the ethanol is utilized to, to, uh, dwell into the catheter and again there are charts as to appropriate volumes according to lines, um, we seem. To have much better luck when we use a 6.6 catheter than when we use a smaller catheter. Uh, PICC lines, it can be used in PICC lines, uh, but some of the smaller PPICC lines tend to occlude when you use ethanol. Um, the, um, the second aspect of it is, um, and again, it's, you know, we're. Theoretically, you can, since you pull the ethanol back, you can use it with any child, but in practice, most of the younger infants who are NPO can't tolerate a TPN window, so you really can't utilize an ethanol lock in that group of patients. Um, but the, um. Um, the, uh, uh, timing, uh, it should be during the window, but, uh, uh, uh, some centers have said you can get by with 3 times weekly, um, ethanol locks, and they, they've shown very good results, um, utilizing it, uh, every Monday, Wednesday, and Friday. So, it's, it's the, the specifics have not been well worked out. It would be very nice, would be a multi-center study to actually ascertain. Um, what protocol works best. Um Val, uh, you, you have a broad geographic region that you cover. Um, do these practices work in Chicago where you may have a patient in, uh, Uh, Eureka, Illinois or wherever or Antioch, Illinois or or South Wisconsin somewhere. I think that we're able to educate the families on how to use the ethanol locks. Um, I think that it is, it has clearly been demonstrated we can do that. Um, I think one of the other issues you mentioned before, we talked about transplanting patients for lack of vascular. Access. Um, and definitely I think, you know, because of the referral error I get, I may get patients who've had 2 or 3 PPICC lines or Broviacs. So I think also underscoring the need that perhaps a patient who's going through all of this access is a patient who needs to be sent to a rehab center sooner rather than later because that's the patient who may end up needing to be transplanted for vascular access that maybe if we've got them a little sooner, may not need to be. Mm. Sir. Any other comments out there on central lines? We could talk about this all day. I'm curious, what is your experience in terms of using ethanol locks versus toroidin locks? I mean, I know you guys sometimes use that. So Hi Gary, what's your experience over there with uh ethanol ox? No, we, we usually don't use ethanol locks. In the UK there is a predominant use of toraloidine locks, which are other kind of, uh, locks which are available to prevent line infections. And there is a huge, uh, there is a multi-center study which is being planned by the pediatric intestinal failure centers in the UK to prove the efficacy of Torolodine. It has already been, uh, published, uh, in a few occasions. That this is equally effective in, uh, preventing, uh, Lyme infections. But again, I would like to stress that what my pedia what our pediatric gastroenterology team says is that Lyme care is the crucial thing, and, and providing Lyme care to the families is crucial. And if you have a child who's getting a recurrent Lyme infections, then revisiting that line care issue again is critically important before uh we proceed to any kind of locks. And so what is your indication for locks then? Uh, uh, you know, usually, if, uh, there are recurrently infections, and despite, uh, uh, uh, you know, making sure that the families are taking good care of the lines, then you would institute torollary locks in these children. So, just to finish up on central lines, if a kid has a bacterial infection, let's say it's strep versus uh Pseudomonas versus Canada, uh, is everyone pulling the Canada line? Are they treating through the gram-negatives and hoping the gram-positives can be managed and having a threshold to take them out? What, what's the, what's the thought in that UCLA on um the type of infections and how that influences their management? Well, I think, uh, in general, uh yes, uh, knowing our experience with fungal infections there and, and how sick some of the, the young ones can get, they're, they're pulled uh uh more promptly, but having said that, um, as we were talking about some of our uh chronic intestinal failure patients who. Have somewhat limited, you know, limited vascular access. We, we have, uh, uh, certainly in the last few years treated through, uh, uh, even fungal infections, although I think our, our recurrence rates once you come off of antibiotics are certainly higher and then in some of those instances also implementing not just uh systemic antibiotic therapy but uh also simultaneous uh lock therapy, uh, can be helpful. Rob, do you use, do you use, uh, TPA intermittently on those kids? Improving lines as well because we have a dedicated vascular access team which can put in lines if our line infections are, are not appropriately controlled with antibiotics, the child continues to have a high temperature, rather than waiting for the child's condition to progress, we have a low threshold for removing lines because, uh, we know that our dedicated vascular access team can, uh, insert lines and preserve vascular access in the long term. Does anyone use TPA routinely? Yeah, for infections for for recurring infections, uh, treatment of a uh a fiberman sheath. Uh, if we get an acute obstruction, then, then we'll try it, but, uh, we don't use it for infection. Likewise. Mhm. Well, let's, let's take this time to transition a little bit and we were talking about line, so let's talk about TPN because it's a natural next step. That's what the line there is for. And, and I, I'd like to have people's thoughts on, on the role of the lipids versus, uh, the lipid restriction versus the different types of lipids. So, um, Uh, we certainly can reduce cholestasis. Does that matter? And, and, and what's, how are people approaching this? I'm going to have Sam talk about what we do here first and then interest in everyone else's comments. Yes, uh, the, um, uh, there are many potentially toxic aspects of TPN. Um, anything from, uh, excessive glucose to excessive manganese to, uh, uh, excessive, uh, quantities of, uh, sulfated amino acids to excessive, uh, uh, lipid. Uh, but, uh, from the work that Clayton did in the UK. What, 25 years ago now, um, it really appears that there's a very, the strongest association with, with irreversible liver disease is with, uh, the administration of high doses of, uh. Uh, soy-based lipid preps, um, and those soy-based lipids are, uh, laced with phytosterols, which are deleterious. The phytosterols, uh, not only are cleared very poorly, they share the, uh, the cholesterol heterodimer, uh, uh, transporter, uh. Um, uh, in the hepatocyte and the, uh, and, um, that is, by the way, down regulated by endotoxemia, and, um, then, um, the, once the phytosterols build in the hepatocyte, it reduces the, uh, the quantity of the nuclear receptor, the, the expression of the nuclear receptor, the. FX receptor and, and that's really instrumental in, in maintaining bile acid homeostasis. So the hepatocyte is damaged when you give high doses of phytosterols. So what dose is, is optimal? Well, none is optimal, but, um, but we see that our patients who are at risk for long-term TPN. Uh, seem to have a much lower risk if we limit the, uh, the overall, uh, lipid intake to 1 g per kilo per day. Um, and if they're developing cholestasis on that dose, we may sometimes go down to 1 g per kilo per day every other day. Um, and, um, recognizing that they may become essential fatty acid deficient. Um, but utilizing that approach, we've reduced our, uh, cholestasis rate to less than 5% of the patients. Um, it's extraordinarily rare to see a patient now who's, uh, cholestatic for more than a week or two. and, um, uh, ultimately, uh, we do have available, uh, fish oil-based, um, lipid, uh, the, uh, made by the same company as, uh, uh, as, uh, intralipid. Uh, it's called Omegavent, and, um, Frasiniuscabi makes both of these products. And, um, And, uh, omegaben may have the theoretical advantage of being an anti-inflammatory, uh, because it's, of the fish oil being an anti-inflammatory, um, type of, uh, of lipid emulsion. Um. We, it's been our overall impression that some patients who don't respond to lipid restriction can achieve some benefit from, uh, fish oil-based lipid. So we have both options available, but we generally go to lipid restriction first. Um, and, uh, with a great deal of success, uh, I'm sure Conrad can speak to that point. Um, our data are really pretty impressive. You just explained what you do, the lipid restriction. Is this controversial or is this unrecognized? People just don't know to do this. What, if you would go to other places, what would you find with the mistake being made? Mhm. I, I would say that oftentimes, uh, when neonatologists drive the, the bus. Um, the, uh, lipid restriction is far less rigorous, and that is based in part because of the concern that if you lipid restrict, you're going to deprive infants of linoleic acid. And adversely affect brain development. I see. OK, so it's the liver versus the brain. Yes, I see. Yes, exactly. So oftentimes we'll see patients who have been managed by neonatologists who are still getting 2 to 3 g per kilo per day. OK. I'm trying to put this as a polls high lipids because they, uh, they find it easier to manage glucose control instead of giving them adequate energy as glucose and using their lipid and just as, um, essential fatty acid replacement. They, um, they think that they can run their glucose low and not have to resort to insulin so often, but, uh, in our experience, we managed to convince our neonatologists that lipid restriction is a good thing and And I've got to say we just don't see cholestasis come out of our NICU. Um, we do have the IND for the, the fish oil based, um, but I've got to say in recent years we've used it extremely sparingly and essentially only on patients who are referred in with cholestasis because we just don't see it in-house. That's our experience. There's a question from the audience, is there a benefit to giving a higher dose of lipids, for example, 3 g per kilogram per day, once a week, as opposed to 1 g per kilogram per day? My answer is I don't know. I, I would think if you, if you have adequate lipoprotein lipase, it might work, but I, I always worry that you might, you might not, the infants may not be able to clear that high a dose of, uh, as satisfactorily as they should, but I don't think anyone has the answer in terms of, of being hepatopprotective. So, so is is essential fatty acid deficiency a real concern? Are people having that as a problem? And how are people screening for that? Anyone out there, any of the other people? I know what we do here. How about in, in, in Seattle, do you guys get deficient in Seattle? We, we screen, I think that the standard, uh, metrics for fat essential fatty acid deficiency are based on malnourished children, not on TPN, and so the high meat acid, the alter tetrienne tetrine ratios, I don't know that they necessarily apply in the same way to patients who have been supplemented with. You know, abnormal mixes of essential fatty acids. I do worry when we see very, very low omega 6s on our essential fatty acid profiles when they're just on fish oil, and we feel much more comfortable with, with gradually working towards a mixture, um, but we don't ever see symptomatic essential fatty acid deficiency. There's a question from the audience. In order to compensate glucose compensation, how high are you pushing glucose to compensate for the low lipids? Jeff, what's your experience? Yeah, so, um, we push our glucose infusion rates, but I, I think that it's important to remember that the higher the glucose infusion rate that you have, the less efficient or less optimal glucose is used as a caloric source, and the more that it's pushed towards, um, uh, towards fatty acid, uh, and fat deposition. Um, so I, I, we have uh an age-based, um, uh, limit generally. It's generally around 15 to 16 in the premature and the or the infants, and it gradually goes down from there as kids get older and progress to teenage years and adulthood. We, um, I think one of the things that we have accepted is the fact that the days of achieving. You know, I don't, I don't think the goal of an intestinal failure patient on chronic TPN is maintaining the 50th percentile for growth or maintaining what would be considered a normal growth pattern. I don't know what the growth curve of a kid with intestinal failure should look like. I don't know if. They should be judged upon the same scale that all infants and children are judged on. And you know, I think the most important thing is that we maintain or that we that they continue to advance along a growth curve. I think that by restricting lipids. Um, and not having other, uh, and, and being limited with the amount of glucose that you can, uh, can give, uh, it can sometimes affect growth, but it's, it's, it's, um, for the most part, we are able to, to, uh, get the kids to, um, to grow, but I will tell you that they are a smaller population than what, um, uh, what would be considered the standard, um, uh, what standard growth charts would, uh, would portray. I don't know, I'd be interested in hearing what everybody else, I think one of the problems with the growth chart is. Um, or, or using growth charts to really assess growth in this population is, is the fact that um we're artificially giving these kids nutrients. And so we can make the growth charts look the way we want them to. And the question is, is it really the best thing for them um to maintain what we consider average growth um in in the general population and risk things like liver disease and and fat deposition. Yeah, I'd be interested to hear what other people in the audience, uh, or other people on the panel have to say as well. Rob, what's, what's your experience out there? I mean, especially also in terms of monitoring essential fatty acid deficiency. Well, uh, as I said by Simon, we do follow whether it's patients on lipid minimization protocols or on our omega 3, we do follow monthly essential fatty acid profiles and looking at trying to tetrane ratios. Uh, I think clinically we aren't seeing evidence of essential fatty acid deficiency. And I agree with what Jeff said that you have to look at the whole picture growth, development, and also liver function when we're taking into account how much lipid or for that matter calories that we're providing for these children. So is anyone worried about neurocognitive effects? Uh, I know I am, but I'm not sure it's justified. Anyone have a comment? I was more worried about them when they had liver disease. Yeah Is anyone screening these kids at 2 years of age to see uh what the effects of uh limiting lipids are? We've done some neurodevelopmental outcome studies, uh, which Patrick Javid has led, um, but we have not looked specifically at Separating that, I mean, all our patients are on a lipid restriction now, so we don't have a comparison group. Can I just add that in the compare, uh, it's very difficult to, uh, associate the neurodevelopmental outcome, uh, just with the, the lipid minimization strategies or with the use of alternative lipids. There are several other factors such as prolonged hospitalization, recurrent hospitalization, or the insults that they would have had in the neonatal period, which would have an impact on the neurodevelopmental outcome of these children. In the UK we usually have access to small lipids, so we don't usually see any symptomatic essential fatty acid deficiency. Although saying that the measuring of the essential fatty acid profile is much more difficult in the UK than it is in the US because of the NHS restrictions. So, so, to transition again, I, I, I put up a slide that I think was very helpful to us when we started our programming and looking at data in prospective form. It's, uh, it's data presented to us by our colleagues in Toronto, Streis and all, who gave a normogram that allows us to know what the estimated length of the intestine of our babies are. And, and for common discussion among patients to know what the percent of their remaining bowel is to an estimated number, um, I think has given us some data we'll show you in a second that helps to look at the natural history. I don't know if, if people are familiar with this, the references in the, in the, uh, in the guidelines and we base. Ours are the length of the baby, which is not the easiest number to reproducibly get in the NICU, um, but what it does is at the time of surgery when we record the amount of bowel resected and remaining, it gives us an estimate, to compare a 28 weeker to a 32 weeker, so that uh similar is similar. I don't know if anyone wants to discuss this other than the point. I just wanted to show it, uh, so that the, the people involved in the tele know that I think this is a valuable resource to start to understand how your patients are doing when compared to others, uh, in the literature and in other centers. Anyone have anything to add to this? So, So based on this, One of the things that we've done here in Cincinnati is that we've mandated that we always, all the surgeons will record the resected bowel length at the time. I think it's important it's resect at the time of resection and not out because the bowel does significantly lengthen um when it's been removed and that any ex vivo measurement in time is going. To be falsely too long and remaining bowel as well and based on having done that um over this group of, I think this is like 99 patients we're showing we've looked at different effects of the extent of resection on the time it takes to wean from parental nutrition. If you look at the red bars, um, we break it down to, to children that have had like spontaneous intestinal perforation where essentially they didn't have any uh functional bowel removed, versus ones that just received a resection that was less than 10%, up to 50% of their estimated bowel length, and then greater than 50%. Um. And I think this goes to old data that says that as Conrad alluded to, once you lose about half of your bowel, there's an inflection point of, of increasing TPM, but I think it also suggests that the differences between just a hole in the bowel in a resection of 10 centimeters or 30 centimeters, most of these kids are still on parental nutrition. Nutrition at our institution at least for 2 to 3 months, um, and, and I think that's an important concept to discuss with the NICU and the families and the team there where they may feel like they've just done an operation on a baby and it was a simple little thing and therefore you're only gonna maybe need a week or two of parental nutrition so um. I think these are data that are largely not uh easy to publish because they're not hypothesis driven and they're and they're in a population of kids that in truth are not in many people's estimation are intestinal failure patients because they've been on parental nutrition, they go home and they're on in and they grow well, but I think they teach us a lot. Um, are there anyones that have any comments on that? Out there. Jeff, I see you thinking. I was just totally my, I was just uh getting my camera adjusted. Uh, one thing at a time, I guess, um, you know, I, I think that the um. Um Uh, you know, the day that you, you have, um, uh, before you, I, I think the idea of the, the estimate, uh, uh, trying to, to measure small bowel length, uh, remaining in the estimated small bowel length is, is a, is a very important, um, concept as well. Some of the other things that you, you, uh, uh. Uh, point out, I think that the, uh, the graph that you're showing up now is, is, is really, is a very nice one because it says what we all sort of think and and and preach and now you have some data that's associated with it. So, um. You know, I think that, um, uh, both estimated small bowel length as well as uh some of these other factors that you're, you're talking about, you haven't, um, with regard to the, you know, oscopy versus no oscopy, breast milk versus uh uh formula, and then we had already talked about the infection, so. It's um Nice data. It's a, it's uh, I agree, it's a lovely data, but what, uh, is quite important is what the function of that length of the bowel is, rather than the absolute length of the bowel. So, what, what is your best measure of function? Uh, feed absorption is the best measure of function. The amount of, uh, feed that the child will tolerate, and the ability to be able to progress that feed would be the best measure of function that we can use in any child with, uh, uh, you know, short bowel syndrome. Also, you know, the ability. Uh, uh, uh, to try and, uh, manage the fluid balance appropriately so that you are in a position to wean the child from PM or give the child nights of PM or with advancing entry fees, or could be used as a marker of function. Yeah, I, I would, I would agree with Girish, but just turn it around slightly and that you can pour in lots of feed and some children tolerate it, but it's your ability to wean down the TPN. That's the most important while maintaining good growth. Going back to Jeff's point, I used to, I used to think that we should put up with poor growth in, uh, in. Intestinal failure patients for the desire to get them off of PN, but nowadays we're seeing kids growing with linear growth above the 50th percentile consistently for those patients that have the genetic potential, and I think we should aim to do that. What we don't see is these excessively fat kids with, with poor linear growth, and that's important. It's not to push too hard, but there's no doubt that liver disease and recurrent infection was the Um, was crucial to their, their poor growth, and now we're seeing much, much better growth, but it's really your ability to wean down TPN while maintaining growth. That's, that's how I judge function. So Simon, in Seattle, a kid has a junal atresia who was primarily repaired, went up to like, let's say 20 per kilo one. 25 per kilowatt enteral feeds and it starts having emesis nonbilious with a fairly large amount. The kid's being fed through an NG tube. What's your strategy? This kid's now 4 weeks post-op and his by x-ray and exam, no obvious obstruction. Yeah, you know what, this is a common thing. No obvious obstruction. That doesn't mean that they're not obstructed. Um, and I think that we keep interrogating that bow. Um, we make sure that the contrast studies are done in our center, um, that we talk to the radiologists and they follow the contrast carefully through the areas we're interested in. We scope, um, And we, uh, we will attempt to, you know, get the scope down as far as we can. Let's go back to the contrast. What's the sensitivity and specificity of an upper GI in your institution for these kids? Having a, a, a, a mechanical problem you feel. In, in the majority of cases, it will pick them up, but the absence of it does not mean that there's, there's not an issue, um, particularly with um low down anastomotic strictures, we'll see dilation and then the, the. The team will say, well, the barium enema looks fine, it flows in. But actually you do a delayed film and it's flowed in, but it doesn't flow out terribly well, or you put a scope in and um you can, you never, you can get both sides of the anastomosis with the scope, but you can't ever see the anastomosis itself, and I think there are times when the anastomosis is not necessarily strictured, but it's kinked or it's not quite, it's not quite grown in alignment and slightly twisted. And we've redone, you know, I've convinced my surgical colleagues that we work with closely, that in these circumstances there is a functional anastomosis, uh, anastomotic problem, and that it resolved with, with revision. So you're saying the upper GI can't rule out a problem. Yeah, definitely. So, so the sensitivity is reasonably low. It can give you an answer, but it can't exclude the problem. That's right. So Conrad can speak to some of our data here on upper GIs and, and the need to have better modalities to understand a functional obstruction from inflammation and the healing process versus a mechanical one that's going to ongo and give children problems. Yeah, I mean, I, I mean, I think the issue that Simon and you have brought up is that you can't rely on just one test. I think that the, if the problem persists, then you need to. Try to figure out what the next steps are going to be. I think we struggle about whether doing all the radiological interventions or probably convincing the surgeons to go back in, and that's, you know, 5 to 10% of kids that we just can't figure out what's happening. I think that's, that's key. I don't know what Sam's comments or thoughts are. Oh, I, I agree completely. I think we've seen a number of patients who had a Relatively normal upper GI or an upper GI that shows dilation, where the radiologist says, uh, oh, this is probably a motility issue. And in fact, uh, fortunately, Michael is, um, is uh much easier to convince than some of our colleagues uh to go in, and Michael has actually found a mechanical obstruction. Uh, and I can't overemphasize enough the value. Of endoscopy, actually visual visualizing an anastomosis to ascertain what the diameter of the anastomosis is, you know, that brings up the issue of, of step procedure and whether sometimes steps might be more meddlesome than than helpful. Um, uh, uh, Carla, do you, do you have any thoughts about that? I wanted to bring up a couple of points about that, um. If if upper GI is not finding the problem and you believe that there is a problem, do you use in your institution CT or MR enterography is one question. And then what would be the role of motility studies in those children before you make a decision to go back for an operation? I think scoping them first would be, I mean, we, we've struggled about doing MRI's and CTs. We've not felt like that's not our next step. I think going in to take a look is usually the next step. Yeah, I, I definitely agree that um moving on to address your question about step and, and reoperation, I think that in those kids that you are convinced you have only evidence that there is an anastomotic problem, then. While it may be, it may be with some, with some anxiety that you talk to the families about going back to the operating room for a problem that you believe is there but haven't been able to prove it. That's that's something that deserves some attention, but I think the decision to operate on those children is, is, I think, rarely met with frustration. I think you do the operation and those children have some noticeable improvement. I think the, the application of SEP to all children that have dilated bowel is now something that needs to be. Looked upon with a little skepticism, I think that it's probably more important to find out why the bowel is dilating in the first place. And talking about stuff is a great, we're gonna have a break in 5 minutes, and that's a conversation of itself, but I think just to complete the idea of the kid that's unable to be advanced in feed. So to go back and just to make sure I have the algorithm straight, uh, BE first or an upper GI first? I think it depends on how short the patient is. If it's an extremely short patient, our surgeons will first do a barium enema. Um, but we will also, after that, if that's not, um, suggestive or does not give a hint of what the problem is, we will proceed with the upper GI. Mentioning the MREs or the MRIs, especially for a younger kid, at least in my institution, are very difficult to do. Um, they can't, they need to be sedated for it. You need to put the contrast in their stomach, however, um, so they tend to be very difficult to do in a younger child. And, and the big bugaboo for us is that these kids that have dilated. of bowel, particularly when they're full with air, um, the ability to get an MRI image on these dilated segments of bowel, um, is disappointing. I think our, I think we are looking forward to our colleagues in radiology learning how to better give us functional studies with the MRI on the intestine, but in the ones that we've used where they've given us the answer that there's a stricture, I think I already knew that, um, where I really wanted to know is a dilated bowel. That also is full of air. Is that a functional problem or a mechanical problem, and that air is really the death of a good image from an MRI standpoint, and I think those things need to be worked out. I'll tell you we have a very mature motility program, but I personally find motility sometimes more confusing than helpful. I, I think the, the motility abnormalities seen in short bowel syndrome are not well. Uh, developed, they, they're not well described in the literature or in practice. So, uh, we may see all sorts of motility problems in these children, and I, I, I less and less ask for, for manometric assessment in this group of children with dilated bowel. I want to clarify some of the terminology, so. Uh, the term, you know, these kids that, that the specific case you gave is the jujunal atresia, um, and they do an upper GI and there's no stricture seen on upper GI. The term anastomotic problem is probably a better term because there may not be a stricture, and there may, and is this an overrated thing that there may be dismotility instead of global dysmotility, focal dysmotility at that big dilated segment. So it is a motility problem, but it's a surgical problem because it's at the anastomosis. It may be, and I, I think one of the learning points I've had over the last few years is that the best functional study of the ability to feed a child sometimes is a post-gastric tube that's dripping in because the rate limiting step in some of these kids is just for gut dys motility, and we've had really great success in our some of our kids by decompressing the stomach. At the time of distal feeding and not worrying about gastric residuals and the gastric volumes and have demonstrated that we can still feed the distal bowel and as we feed the distal bowel there's probably a feedback mechanism that improves for gut emptying over time that has been beneficial and the and the biggest dismodal problem is a reversible b is proximal and is limiting the ability to distally feed and so being able to distally. Assess that before decisions to operate have, I think, avoided some surgeries in our patients that I think have allowed us to wean them off of TPN and, and to really move forward and it's hard to convey how that that's what our team is good at is suggesting those in the individual patients where, um, I agree with Simon that we might wanna go back to surgery, but I often like a trial of of distal feeding before just to prove to me that that's what I'm gonna find. Let's um. I want to give a quick, maybe, uh, yeah, a summary of where we're at and then because we're going to go to break and and what are we going to talk about when we get back. So I, I, I was, we were able to talk about the multidisciplinary approaches to intestinal failure, and I think it was important to highlight that maybe not the sexiest things, but the routine things that we do every day and these kids make really big outcome. Differences and having an approach to these kids that include more than just isolated segments of your hospital, I think are going to give you better outcomes and should be part of, I think, your goals and that as you implement some of the things that we've done with line care and stuff into your programs, I think you'll see the same benefits we did. I saw one. One person put in that they want our protocols, so we have these things available and I'll try to provide them to the Globalcast so that when it's posted you'll be able to see at least here in Cincinnati what our protocol is for ethanol locks and for managing fevers and whatnot so you guys know what we know. Look forward to coming back and.