you you you you you you you you you you you you you you you you you you you you you you you you you you you you you you you you you you you you you we we you us. I'll start with Dr. Velesquez. She graduated from the College of the Holy Cross here in Massachusetts and then obtained her in PH at Boston University followed by medical school just one second. Followed by medical school at Albany Medical College. After a medical school, she returned to Massachusetts and completed her residency in general surgery at the Leakey Clinic. In 2008, she joined as a fellow in our surgical critical care program here at Boston Children's, after which she completed her pediatric surgery fellowship at New York Presbyterian. She returned here as a faculty member approximately 11 years ago now at Boston Children's and she has many titles which you can tell that she's worked very hard to achieve. She is the assistant professor of Harvard Medical School Associate Program Director of the Surgical Critical Care Program, co-director of our simulation program, surgical director of the ECMO program, and most recently co-director of our continual diaphragmatic pernion or CDH program. As you can tell by her roles, her career has really been focused on simulation, education, and critical care. Our second president today is Dr. Sally Batali. Special Batali completed her bachelor's in biology at Dartmouth followed by her medical degree at the University of Texas San Antonio. She then returned back to the Northeast and completed her residency of pediatric and fellowships in critical care here at Boston Children's. Following fellowships, she joined the faculty here in 2004 and is now assistant professor of HMS. She serves as the medical director of both the ECMO and CDH programs. As a surgical fellow here, I've been fortunate to have the opportunity to train under both of these incredible women and learn from them how the care for extremely sick children. I've also been fortunate to see the collaborative management techniques that they foster and the transformation in the care of the CDH children that they have created over the last couple of years. So with that time, effort, and continuity, we are honored that they are joining us today to share with us some of what they have achieved and envisioned over the future. In a talk titled Advanced Think Here for CDH at Boston Children's Hospital. Thank you. Thank you, Jamie. So I'm going to start our talk today and Sally will join on, but I also want to acknowledge the work of our other team members of Belinda Dickian Nalashmeda who have been instrumental this past year in developing our CDH program to what it is today. So today we're going to talk about our CDH program here at Children's and of what the continuum of care is here and some of the programmatic changes that we've done to try to help achieve better outcomes in advance that what we're doing here. So we're seeing your presenter view. Oh goodness, so sorry. Sorry about that. Here we go. That looks better. Perfect. So we're going to look at the continuum of care in the CDH patient and look at what's the needs assessment? Can we do better and what should we be doing to achieve those goals and then talk about some of the structural process and outcome measures that we're focusing on in the CDH program now. So in this continuum of care for CDH patients, it starts prenatally and transitions all the way out to child. And when we were thinking about this program and talk in some way together to present this talk today, we're really thinking about all the different aspects of not only this institution but beyond that are involved in the care of the CDH patient starting from the maternal fetal care center to our collaboration with the Brigham and Beth Israel for managing these babies prenatally in the delivery room. And then really the crux of our program that's centered here in our inpatient care both in the ICU and on the floor. But then we transition home and in our outpatient clinic which follows these children till young adulthood, we're really involving the care of these patients and touching on so many multidisciplinary programs and subspecialties. So in the maternal fetal care center, this is where it all begins, where these families are getting this diagnosis, something that they've never heard before and it's completely changing their world and their outlook on the pregnancy. In these visits, we're seeing both with our imaging, we're able to identify the disease and then use risk stratification based on what we're seeing based on MRI imaging with predicted lung volumes and on ultrasound, the lung to head ratio to try to to help these families risk stratify and is this a mild moderate or severe case? And then what does that mean and what does it look like for them and for their baby after birth? It also helps us coordinate our delivery plans with our OB colleagues next door and it help with how to prepare when to do it and what needs to be involved and also with our ICU and is there are we going to need ECMO on standby or is this something that we hope to manage without? Our delivery plans and our initial management in the ICU is really focused on that initial ventilator management and how do you ventilate the CDU-G lung picture in the top corner? I think helps you take a step back and realize we can't use our normal thresholds for two healthy lungs. When you're looking at what are you doing to this this tiny undeveloped lung, we're really focusing on avoiding any kind of trauma, a volume trauma, a barotroma and oxygen toxicity to these lungs to try to help get them through this transitional period. I just wanted to show you kind of the progression of a CDH patient from the initial delivery through post-repair. In that initial management in that top left picture you're seeing our admission test x-ray and one of the things although there's a repulsive in place, there's a large gastric dilation and some air distally and then over the course of hours to a day you see how that affects the air if it's not adequately decompressing, shifting the lung you have more media-stinal shift and more compression of that lung on the right side that can affect your ventilator management. So we often have to do add chunks like placing a second sump to decompress and you can see that in the third x-ray on the top really helps and helps expand that lung and give you more volume and potentially prevent ECMO. After repair you see that empty space with that high-bropastic lung and then over time and patience and our gentle ventilator management were able to get the child exhibited. All of them don't go so smoothly and there's a cohort of patients that despite our best management to require extra support. Here are children's over the course of the last two decades about 43% require ECMO. When we take a step back and look at who are the patients that have required it with the diagnosis of left side and a liver up 75% of this patient population does go on ECMO and that's followed by the right side diaphragms where the liver is up in those as well, 60% go on ECMO. Among all comers on left-sided CDH 40%. So there's a significant percentage despite how we were managing them with the ventilator due to require additional support with ECMO. Unlike some other disease processes that resolve quickly because of that lung hypoplasia, these are often long runs. Three weeks, four weeks runs of ECMO are not uncommon. Historically early on in ECMO and there are often kind of arbitrary stops of if there was no improvement or you're still on in four weeks the time time's up and you come off. Now there's no real accepted limits on the length but we do know that survival decreases significantly as the duration of ECMO increases and when you get beyond five weeks, their survival is about 15%. So we're really trying to do what we can to optimize the lungs, optimize nutrition so the babies grow and limit the complications on ECMO to try to get them off. When a baby is on ECMO, it kind of changes that management. You can see this is a right-sided CDH who went on initially right after birth and you can see from those ECMO canally and in that top picture the the heart shifted all the way over to the left and in these cases we would repair early on that first day because fluid shifts and the heart being shifted all the way to the left we can have issues with flow so that's often the impetus to go to the OR early on that first day after cannulation to repair. And then you can see that first two weeks looks kind of discouraging not seeing any tidal volumes, whiteed out chests, the media stonems still shifted but with patients, lung recruitment and in time and growth you see by day 20 the media stonem has shifted, the lungs have opened and at six weeks an excavated child and outpatient x-ray on the bottom off of all supplemental oxygen. After that initial trend delivery event management trying to figure out when's the right time to take these children to the operating room balancing their ventilation needs the degree of pulmonary hypertension we try to figure out when is safe as so we're not creating a situation that potentially requires ECMO in a patient that was otherwise stable so we want them to stabilize physically logically have that transitional fetal circulation happen and the pulmonary hypertension relax as much as possible. And a patient who's not on ECMO that'll be a minimum of really 48 hours after birth and it can be days or longer depending on how they're doing. And as I mentioned the typically once we're on ECMO we do like to repair enough in that early period before there are significant fluid shifts that can affect both flow on ECMO as well as risk of bleeding after surgery. What we've learned over time because the babies like to or we do better with spontaneous ventilation that there is some transition when we do paralyze these children to go down to the operating room. So what we have learned is to do this trial of paralysis in the ICU an hour or two before going down so we can modify the ventilator so we're not transferring down with an acidotic patient who's hypercarbic and then going to be undergoing the stress of surgery. We've also learned over time and experienced that our patients are not taking the risk of bleeding. Keeping the children on the ventilator avoiding hand ventilating down to the third floor and then hooking up to the anesthesia circuit was often both stressful and led to significant respiratory acidosis. And we've now over the last many years have left the children on our ventilator on the ICU and brought everything down so they weren't ever disconnected so we didn't have to deal with that part of that care. They take great coordination of care with the anesthesia team and our respiratory therapist to help with the ventilator in the operating room to help with our management goals during the operation. If there are issues with pulmonary hypertension and using nitric and went to use nitric and helping with that physiology. So there's a tremendous amount of coordination that happens at the time of the OR. These are pretty pictures. Most of our cases are open repairs but every once in a while we do get a child that is maybe a postnatal diagnosis or a small defect that looks like there's just balance in the chest that we are able to do this thorcuscopically. You can see the defect there with a nice diaphragm that was able to be closed primarily and then reinforced with a patch. When we're talking about these defects trying to classify them and then ultimately seeing how this classification grouping, how we can look at their outcomes overall because a defect like I just showed you a primary repair CDH defect A and their outcome is going to be much different than someone who has essentially an age-genesis of the diaphragm and how do we want to try to compare like things. So this the CDH study group came up with this classification to try to help with that. In the defect A's are generally the primary repairs that have a nice diaphragm. B has a significant amount of diaphragm still present and then when you get to a D that's essentially no diaphragm and you're sewing a patch around the ribs both anteriorly and posteriorly. Once we're out of this initial period of ICU care and ventilator management it's been a long course in the in the hospital on the floor. Typically the children are on the floor for about as long as they're in the ICU. Our median hospital like the stay is 45 days but often can be several months and during that time our management priorities and our focus is on their growth and nutrition to help with their long growth and with that you know comes along with their management of pulmonary hypertension. Often the sedation mean that has been happening from their ICU course and management of their pulmonary high glauctasia over time with growth we have we're able to wean our oxygen and diuretics and try to simplify the care for when they ultimately go home. Once they go home you know there can be many issues not only relating to their lungs but the other systems that are involved in the multidisciplinary clinic here at Children's is actually the longest standing clinic started in 1991. Dr. Wilson and Dr. Buckmiller had been at the the heart of this clinic over the last decades and following these patients until they're young adults until they can turn into an adult care. We've seen over nearly 500 patients in this clinic and it we rely on the expertise from our pulmonologists our dieticians developmental pediatricians cardiologists neurologists and all looking at these patients and some of the needs that they have as they grow and develop. Excuse me. So in looking at this spectrum of care of patients with congenital diaphragmatic hernia where the the opportunities to improve our outcomes and and where should we focus? When you look at this 25 year review from this CDH study group of when you're looking at just mortality over that 25 year period there's been some improvement in mortality but we're still hovering close to 30 percent so how do we get to that next next level. We look here at our historic experience you know mortality or survival rather of 45 percent in the 80s and you know Dr. Wilson and others at that time we're trying to figure out how can we get these babies how can we keep them alive and that transition from immediate repair to delayed repair and then the focus on permissive hypercapnia really led to significant improvement in survival at that time. Our outcomes here have improved but we need to get to the to the next level. Our overall survival is close to 80 percent in the patients who are not on ECMO a 95 percent survival but the ones that are on ECMO are 57 percent so we have some work to do in figuring out where where we can really make an impact in these outcomes and when we look at our data here from our registry it's really that C and D defects that we need to focus on and how are we managing them from the prenatal from the delivery room and through their hospital course that we can improve the survival in this patient population. Is there anything in the literature that can help us with this? There have been consensus guidelines from both ELSO, the ECMO consortium as well as some other CDH consortiums both here and abroad to look at what has been reported in the literature to come up with some consensus. What we can say for optimal ventilator strategy is gentle ventilation with permissive hypercapnia is can lead a help with better outcomes. In terms of management of pulmonary hypertension there's no real benefit in the studies that have been done with nitric oxide. steroids is that beneficial? Well there's no real evidence to support that either both either prenatally or postnatally. Is there a way that we do ECMO that's better for these patients? The mode although we do VA ECMO for all of our diaphragms, some centers do VB and there's really no mode of ECMO that has been shown to be better in the duration also hasn't proven out in any study. Is there anything with what we're doing in the operating room that can help? Open repair does have significantly fewer recurrences but when you're talking about mortality it doesn't change that and looking at the type of patch is the PTFE that we use is the most durable. Does it matter earlier late repair? The overall timing hasn't been shown to impact outcomes but except for on ECMO it may have some benefits. So overall there's no real high level evidence to help us guide our management to show outcomes. Our mortality is close to just over 20 percent. How does that stand up to other institutions? When we look at the FIS database this is from the period of 2012 to 21. Children's is in here in red and you have mortality where just over 20s and there are some centers below that. We have one of the highest numbers of CDH programs in the country as well but we still when we're looking at mortality there's this outlier here and what are they doing to have such significantly lower mortality? Close you know under 10 percent here what's different and if there are way we can achieve that improvement in outcomes. This is just another slide showing the case mix. So our patients you know are sick. The length of stay is longer again with one other outlier there. So we know there's other institutions that have some better outcomes. We don't really have great evidence in the literature to guide us so how do we improve? And this has been the focus and our goal over the past year when we've been charged with building, revising the CDH program, how are we going to improve those outcomes to reduce our mortality and our morbidity? And some of the structural changes that have gone along is to really cohort this population so we can develop a team and a system that can take care of these patients and we can learn from each patient if we're doing things the same way we can learn what's working and what's not. We've tried to streamline our approach to the care from the prenatal consultation all the way through their hospitalization and in doing so that's required more support for decision-making, frontline and being there to offer the expertise throughout the day and night. This surgical core that has been established over the past year has included Dr. Sticky Weldon and Kim. Dr. Demarie is finishing his critical care fellowship and will work excited to have him join just in another month. And the people who have been involved in really trying to look at all aspects of the care and coordinate with the multidisciplinary teams are the core for us that have been meeting weekly and really trying to advance the program. And then adding the expertise from our respiratory therapists as well as nurse in the ICU to help coordinate and collaborate with this with this care. Some of the specific changes that we have someone that's on call all the time to be able to help manage these patients. One of the things that we wanted to make sure is that the care is the same whether it's 10 p.m. or 10 a.m. and that the people had someone to go to day or night. We actually started a nightly rounds with the on-call ICU team at 9 o'clock via Zoom and that includes the surgeon and many of our core come on those rounds as well as the fellows, the ICU fellows who are on the team and most importantly the bedside nurse, the respiratory therapist and the ECMO specialist. So we can make sure that everyone is on the same page. We have a plan and that we can continue to advance the care day and night. Other structural changes to the program that have also helped, you know, we are, we recognize that this is a collaborative program that involves many sub-specialties and we wanted to make sure that we're including everyone and the perspectives in managing these patients. We started a weekly rounds on all the impatience both in the ICU and on the floor. This initially started with just a few of us and when other members heard that we were doing this. They wanted to be involved to be able to both hear about the trajectory of the patients and then provide the expertise. So now it's a true multidisciplinary rounds with our surgical teams, ICU, for teams of pulmonology, cardiology or dietician feeding team are all participating in this care. We update with all the ICU attendings that round attend on team three and manage these patients. So we're learning from each other and and trying to improve the care from our experiences. In our clinic, this has really grown over the past year. We've been fortunate enough to have an outstanding nurse practitioner in Lindsay Lemire who has really taken the clinic to the next level. She has a wonderful rapport with the families that she's meeting in the hospital and helping with family education and some of the outreach that we're only going to expand in the future. We collaborate with our cardiologists and the pulmonary hypertension team and Dr. Mullen to coordinate our care on an outpatient basis. The clinic has evolved it used to meet twice a month and we've realized that in order to provide this comprehensive care, we needed to expand this or expanding too weekly. And we also noted although it's a small cohort, there's a complex group of patients that we share with our KTEM are chronically ventilated patients and we've created combined clinic with the KTEM as well that meets quarterly so we can truly provide the comprehensive care to these very complicated patients. And what changes are we making to the management of these patients? I'm going to now transition to Sally who is going to go through these. Thanks Jill. I'm going to share my screen with you. We have the same slides that we wanted to be able to advance on our own. In slideshow mode there, hope everybody can see that. So, Jill talked a lot about the structural changes that we as a group thought were important to improving the care of the CDH patients. But what specifically are we going to do different in terms of how we take care of them? And I think the important thing we realized from the beginning was that in all of these different areas, there was potential for improvement. But there were also experts who had been working on their own for many, many years to develop their expertise and to improve their management of CDH. And you know, this is really a world class program that we started with. And how do you go about improving something that's already functioning at a very high level, but to try to really move the needle on the outcomes we needed to go back and look at how everybody had evolved their practice. And I thank the idea of streamlining and providing guidelines and trying to manage like patients in a similar fashion every time was the sort of a spirit that we went into this process with. And we've had great partners in each area. I'm not going to talk about each one of them, but I do want to show you some examples of partnerships and places where we feel like we really have, we sort of started out. We've really a beginning of the process. And this is areas where we have had some, what we feel like are early wins. One of them is really the work of predominantly of Jill and the land of it begins back at the the MFCC, but a partnership with the MFM groups at the VI and the program. We recognize early on that the period of time around delivery and transport and admission to the MSI CU is a very high risk time, high intensity time. A lot of resources are required. And coordinating to streamline care during that time is really valuable to everyone. Everyone appreciates it. I think we all know a good delivery and transport and admission when we see it, but how can we set up some guidelines and structure that makes it so that we try to have, you know, sort of an optimal experience every time. So one of those has to do. We recognize with being able to have as many patients as possible delivered during the daytime hours and early in the day so that all the resources are available not only at the delivering institution, but all the way through until the ICU. And it's absolutely true that we're going to have patients deliver in the middle of the night. And we have to be prepared for that. But I think particularly with high risk deliveries, the optimal time is during the day and Jill and Belinda have been partnering with the MFMs, including going to even some meetings delivery planning meetings with the with the MFM group at the program that happened weekly to try to strategize for each of these moms, particularly the high risk ones. And then on going collaboration and coordination with our newborn medicine colleagues at the VI in the Brigham to help them understand why it's so important to have similar care for babies in the delivery room every time to create an efficiency and get patients through the delivery room process transported and, you know, to seven south in an expedient fashion. We also of course wanted to look at the admission process. And I think we all know all the things that need to be done for a CDH patient on admission, but quite frankly they, you know, happen sort of in the past that happened, then in whatever order seemed appropriate for that patient at that time. And I think it's definitely was a place where we could have tried to have the standardized process, both for how we prepare for admissions and assigned roles and how we have people ready to do what they're going to do when the baby arrives. And then in terms of exactly what we do in the order of things, things like getting a weight on admission, when babies are really sick, it's easy to bypass that because you want to get them, you know, all hooked up to everything and the weight seems like that might not be something that we want to spend time doing. And reality, the weight takes, you know, 10 to 15 seconds and it's absolutely vital to being able to provide the right amount of medications, the right ECMO flows, etc. So we've, you know, prioritized that, prioritized transitioning directly to the ventilator, which really means avoiding a lot of hand ventilation, which is kind of an uncontrolled title of volume at times. Regardless for therapists, they do a fantastic job, but it is clear that, you know, being able to pay attention to every single breath you give is a challenge that the ventilator can do that well, getting our chest x-rays and then trying to get a vascular access in a similar way when it's possible. Not every baby is a minimal, you can, the UAC and UBC, but it's a rapid way of getting access. It gets us labs early. It's a painless process for the baby that doesn't acquire tremendous amount of sedation. And so we've been trying to optimize getting that done as soon as possible after, after admission. The nurses and the respiratory therapists have partnered with us on these admission guidelines and, and I think they've made a real impact on how we, contribute to that stability during this high-risk admission time. And then probably the biggest amount of work that we have done so far in terms of creating guidelines and partnering with experts is an arm mechanical ventilation strategy. We've already said that we want to enjoy, avoid injurious ventilation. What does that mean? We have tried to have a ceiling, sort of, for our pecan-spin-fury pressures to not go higher than a 23-year-old max, 25 centimeters a potter, avoiding tidal volumes that are higher than 5cc per kilo. Jill showed you the picture of that tiny little it's a lateral lung. And, you know, that really, you know, those patients don't have tidal volumes that are like you and me. Their tidal volumes are lower. And so using a lower tidal volume strategy with lower tips, but then using rate, higher rate if that's needed to be able to improve ventilation. That's something we've always used high rates in the past, but other institutions have had success with going up to 80 and 100, and our respiratory therapists have really partnered with us to try to take on that strategy. And it has worked really well for a number of babies, particularly in the 12 hours after admission and 12 hours after CDH repair when it's, they just need to sort of bridge a gap of instability and being able to use very high rates during that time has helped keep a number of these babies off the backbone. And then, sedation management is vital and important. This is really a partnership with our nursing colleagues, and they have worked hard to reach out to our, the NICU to understand all of the developmental care that allows sort of non-pharmacologic means of keeping these babies comfortable and well-citated. And so, that's definitely part of the mechanical ventilation plan because not only does sedation affect how long a baby needs to be out of ventilator because they need to be doing the spontaneous breathing, but the spontaneous breathing is something that we like is good for their overall outcome. And a less sedation means more ability to do that. And then, a more recent addition to our strategy, both on ECMO and the mechanical ventilation has been using prone positioning. This is a prone positioning life we use for ARDS or COVID, but really more of a way to another adjunct to try to get the lungs open. We've always positioned these babies way up on one side or way up on the other, but using actual prone positioning has helped us to open some lungs when other strategies weren't working. The mechanical ventilation pathway that we have been working on is in 20 versions over a year, but finally published on the Boston Children's Clinical Pathways site. This was a revision of the CDH scamp that was the baby and brainchild of Jay Wilson and John Arnold and Patrice Benjamin and others. That scamp was really meant to look and see whether a high-frequency oscillatory ventilation was a way that babies could be rescued and not have to go on ECMO when they were having troubles with oxygenation and ventilation after delivery. The scamp really was essential in kind of proving that there were eight patients during the scamp time that were on high-frequency ventilation and seven of them ended up on ECMO. That strategy didn't really work as a rest you at least in our hands at Boston Children's, but we used this scamp in a sort of framework and have evolved a mechanical ventilation pathway that includes all of our current care. We're going to be rising this pathway frequently because we're learning, as I said, as you said from every baby, but there is at least our first version published and we are trying to use this to help everyone in the ICU be able to manage these patients in a similar fashion when that can happen. As far as ECMO is concerned, what kind of changes are we making there? Really, over the past couple of years, we have moved to a strategy, for example, of weaning ECMO flows as opposed to the old way that we basically would wait till the lungs were open and we thought that they had babies had a chance of coming off and then doing cycling and clamp trials from full flow ECMO to see how the babies did with really no ECMO. This is a strategy where we wean the ECMO flows over days to weeks and as we go down, we get them full mechanical ventilation and we see how the lungs are working and how they're contributing and whether the baby needs ECMO over a longer period of time. A number of advantages to this and other institutions, many other institutions, have used this strategy. It's working well for us so far and during that time of weaning ECMO flows, this is the time when we're using things like prone positioning to try to get the lungs as open as possible to expand those tidal volumes and allow separation from ECMO. Sometimes now that separation isn't happening like Jill said, till four and five and six weeks on ECMO and in years past, we would have thought that after about four weeks there really wasn't a chance for getting better. A lot of this patience on ECMO is tied in with how aggressively we are with dioresis because that's also a strategy that we use to try to get these patients off that mo and over dioreasing and creating kidney injury has really heard of us in the past and I think part of this strategy of being patient is to allow for gentle dioresis and slightly negative fluid balance every day as opposed to trying to take large amounts of fluid off every day. And then I think the other exciting thing in our future for ECMO is a transition in our anti-acquisition. As you all know, we've used Heparin for anti-acquisition for these babies and everyone on ECMO for many years. It's a terrible drug to try to titrate to create anti-acquisition the way we can monitor it doesn't really work. We've all contributed to that literature. But by Valorudin is a strategy that other institutions have used and specifically for our CDH. Dr. Puders probably in the audience and his laboratory has definitely shown that Heparin can have some effects that go against lung growth that are not helpful for lung growth. And so we're interested in switching over to by Valorudin which is a direct dromine inhibitor that will require us using centrifugal pumps for these babies and we have new centrifugal pumps in the institution, a spectrum medical at the device that has been that the week purchased to replace some of our old devices and prepare for the HAL expansion. So this is all coming together and hopefully in the next couple of months we'll begin to trial this transition which hopefully can make a big impact for our ECMO management. And then we partnered with the nurses as we've said. This is just a to show that there's an creation of a bedside manual where not only do we you know try to provide this optimal environmental care and reduce sedation for babies but we try to make sure that every baby's getting that you know kind of all the time. And so that's you know I want to shout out to all of the nurses that are part of the development of these educational materials and have really partnered with us in the ICU to try to streamline care. So how are we going to know when we're making all these changes whether they're having any impact. We have to thank an effort from the critical care center for outcomes and the PC core part of the PC core the Care Operative and Critical Care Center for Outcomes Research and Evaluation who developed for us specifically Alon Gave and Ali Reza Akhendi and Nalashmada developed a CDH dashboard that is updated automatically when patients are discharged at polls data from the EMR and also from the CDH registry in surveys for each of these patients that Terry Buckmiller and Lindsey Lamar painstakingly gather data for and all of it goes into the dashboard including data on mechanical ventilation and fluid balance and other clinical parameters. I didn't want to risk trying to go on the website of his lives but this is a screen shot and shows you just at the very beginning some parameters that are available and that can get updated with every discharge from the hospital. We will recognize some of these numbers as the ones we have presented to you earlier in the talk but we also from this can and you can see the tabs across the top the top can create some grass that really begin to help us understand our outcomes in different areas. This is just an example and first of all I'll get the caveat that the 2021 data is really the first 13 patients of 2021. We've had a large full list here at the end of the year, some of whom are I would say thicker than the ones at the beginning of the year that we had some sick ones back there at the beginning and those patients are not included in this dashboard as of yet. So the 2021 data is not complete but in terms of things like this reducing the max begins for Tory pressure. I think we're very happy to see that pulling all of the data from all of the patients and all of their begins foray pressures in 2021 we haven't gone over 23 and that was something that we set out to do to reduce ventilator and do sling injuries so it's great to see that kind of improvement in hope that it continues but we'll also continue to monitor it and it will help us know when we need to do something differently to improve best strategy. And then I told you we were using higher rates sort of instead of going up on PIP and title volume to ventilate babies more and you can see here that there's definitely evidence from all of these babies charts that that is happening that we are using higher rates no matter how you look at it whether it's spontaneous or total rates or the maximum ventilator rate we've been higher in in the past year. Fluid balance has been something interesting to look at we have been making efforts to try to improve fluid balance prior to the surgical repair and throughout the ICU stay but it's great to see from this that we are making headway with that you can see really you know the fluid balance being you know positive 25 CCs per kilowatt per day in 2021 before the repair and that just makes things go so much better in the operating room and in the perioperative period when the babies aren't incredibly fluid overloaded and this is just showing that that continues throughout the ICU stay that in the past couple of years we have reduced the intake the overall intake volume during the ICU stay as well as the overall total fluid balance and then what about some balancing metrics we haven't really targeted a length of stay for patients in terms of trying to improve it but this is just to show you that for patients without ECMO the I average ICU length of stay is around 10 to 12 days and that certainly hasn't changed hasn't gotten longer with our interventions and for ECMO patients right around you know 40 to 50 days again there was an outlier and soundliers in 2019 but otherwise we say pretty much a similar to where we had been before and then mortality is obviously the needle that we know are overall interested in improving for as far as our you know inpatient outcomes again this is only the first 13 patients only after the very last patient from 2021 is discharge will we have the complete data but we like the trend this is a sure pop plot for mortality just at our institution averaging around 20% and hopefully we will be able to have this trend continue obviously one year does not make statistical significance in the numbers but you know we're going to continue watching this and watching where the trend goes and then as far as outcomes outside once patients get out into the world our CDH program has contributed for years to the literature about long-term outcomes in terms of pulmonary function and VQ matching these are just example papers over the past couple of years from the outpatient world and from another contribution from the outpatient world just to highlight a presentation coming up at SCCM in in April Jill O'Hara one of our chief critical care fellows along with her mentor Ben Albert and other folks from the sort of outcomes and the CDH program and the out and the outpatient clinic looked at functional status scale in the areas around hospital discharge the 06 month 6 to 12 and greater than 12 months follow up and showed improvement in functional status over that time these are scores that are pulled from the clinic notes and the discharge summary and we're really excited that you know not only was Jill able to show this but also that we can continue to do functional status scores and include those as part of our dashboard of outcomes going forward we learned how to do it that it's easy to do and feasible and thank you to Jill and Ben and everyone else for contributing this to the to the literature and to our experience so i'm going to pass it back over to Jill for the end here but i just want to say that she and i both and and the entire team have a vision for where we're going for this with this program that goes beyond what we've talked about today that really tries to evolve and you know enhance our center for excellence i'm going to let Jill give a little bit more about this and then wind it up with some acknowledgments. and Sally i just you know i think we are very fortunate um we were tasked with um trying to advance the CDH program but it started as a world class program you know the the efforts of uh Jay Wilson and John Arnold and and Terry Buckmiller over the years to to build this program um you know we're excited to take um what they have done and then use um kind of the tools both from the ICU and the outcome center and and using data um to really help enhance this this program and and and contribute to reducing mortality and morbidity and also from an outpatient center to really start looking at what we can do for families um collaborate with um with um making protocols for for the family outreach education materials um and truly providing comprehensive care from the prenatal period to young adulthood and thinking also about that transition to adult care that's the next step that has not been tapped into and what do these young adults with progressive pulmonary hypertension and chronic lung disease where do they go and who's managing them so there's a tremendous amount of opportunity um and we are uh looking forward to uh continuing to enhance this program i think as you can see um that it takes a village there's just countless number of people um that we have worked with um just in this past year um as a as a leadership program and continue to build uh relationships with and are looking forward um to working within the future we wanted to specifically thank doctors uh fishermen and burns for for charging us with this to challenge us to to improve our outcomes and say you know there's some places that have better outcomes and what are we doing here we started off um you know at a great place but how do we make it better um and that was from the efforts of uh from doctors Wilson and Arnold and all that they've done uh through the years to get us there and we're very excited to take this um to the next level I think on behalf of every um the four of us uh Sally Belinda and Elish and I we um want to thank uh you for your support and we really look forward to the collaboration um with everyone and I think um we especially want to thank our uh CH children and their families as we go through this and uh this is a girl um every year I get a picture of her she's actually someone who Dr Wilson uh counseled prenatally and I uh took care of uh right after birth and um you know it makes it worth it when you see this little girl on eckmo uh she spent a month on eckmo and had several recurrences and and lots of hard um conversations and and all that and every year at an anniversary to get that smiling face it makes it worth it and makes us want to push ourselves to do better uh so I thank you thank you everyone for your time and today well I'm gonna just jump in we have very very little time uh and I want to um um uh thank Sally and Jill but the entire team put this together I am old enough to remember um my uh early years my fellowship and thereafter um at the bedside in the old p5 i c u and it really was John Arville and Jay Wilson who were at the bedside you would be there in the in the blue chair all night long and one of them would always be there or on the phone with you um um as we grew as an institution as a faculty and uh both a career and surgery um we got a little bit diffuse uh in our ability to focus um and everything we do with Boston Children's National World Class we do with focused excellence with people who really devote themselves to it and Jeff and I a Bob Shammergore for me uh really have committed to bringing us back to be um the best in the world uh and um um Jill was a little polite and saying that we've we've passed these guys just we have we have we've committed institutionally they were going to do this uh Jeff and I are our um standing back and and applauding but providing the resources uh and and and driving the commitment at the moment um we've made enormous progress um obviously Sally was being a circumspect about that that survival graph um we do hope that trend will we're not going to jinx ourselves we do hope that will continue um but it is obvious uh if you look at these children that they are making a difference this team you are making a difference and there are many people who are disciplined who didn't get speak today or weren't even mentioned uh it is making a difference we will continue to evolve uh one thing that we will likely see is an increasing volume of CDH patients why will that happen that will happen because of what you're doing um like everything else that's rare and complex uh in medicine a social media controls the flow of patients we see that in many of our other programs the social media was a little bit negative uh uh in the last years uh towards in fact we had you know with the honest we have families in Boston who chose to go elsewhere to deliver their baby uh for the um expectation of a better outcome that is reversing the social media will um recognize and reward what you have done uh and we are also uh as many of you know making the huge institutional commitment to um to enhancing our fetal program to include even fetal intervention uh and that is likely to attract even more patients with Dr. Madprenie. I want to congratulate you guys we have to continue to evolve for those of us at the bedside uh uh you know who pass through at any moment uh you'll always see Jill and or Belinda at the bedside we cannot continue um to drop excellence on the backs of two people um just in our meeting with the leadership group shortly to talk about how we can do that um we thank you all for your incredible commitment um and um um um um hooray and thank you. Well said um thank you both for the work you've done. Thank you. You'll and um Sally thanks from uh all of us. I guess I would only ask um the interop time period is a really dynamic period here and I I don't think there was very much in here about what we do with management and there may be an opportunity there as well so uh I look forward from the anesthesia perspective to look at how we might optimize that aspect of the care as well but thank you both I know this is a huge lift and you're doing a great job thanks. Thank you, Chair.
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