Melanie Cole (Host): Welcome to UF Health Med Ed Cast with UF Health Shands Hospital. I'm Melanie Cole and we're discussing today the multidisciplinary means management of pediatric intestinal failure. Joining me in this panel is Dr. Janice Taylor. She's an Associate Professor of Pediatric Surgery for the University of Florida College of Medicine and Dr. Sam Cheng. He's an Associate Professor of Pediatric Gastroenterology for the University of Florida College of Medicine. Doctors, thank you so much for joining us today. Dr. Cheng, I'd like to start with you. What is short bowel syndrome? Tell us a little bit about the prevalence and intestinal failure in general in children. What is known about this now?

Sam Cheng, MD (Guest): Short bowel syndrome is when patients require parenteral nutritional support and due to loss of bowel either physically or functionally. It's not a very common condition, but it's a very penetrating condition, according to NASPGHAN, the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition.

The failure is defined as the need for parenteral nutrition for more than 60 days due to intestinal disease, dysfunction or resection. The recommended definition for short bowel syndrome is the need for parenteral nutrition for more than 60 days after intestinal resection or a bowel length less than 25% of expected. This is not so common, but it's common in our pediatric world. The most common cause of short bowel syndrome and intestinal failure is necrotizing enterocolitis. Account about like 20, 25% and coming next would be gastroschisis, it's a congenital defect, wall defect and that about maybe around 10% to 15% and then the third common reason would be intestinal atresia, named also the malrotation with volvulus. That's another cause of short bowel syndrome in children.

Host: Well, Dr. Cheng sticking with you for a minute. As we're talking about chronic medical management challenges for these kinds of situations in children, do these dominate a child's life by restricting growth, development, productivity, all of these things? Tell us a little bit about the symptoms. Red flags pediatricians should be on the lookout for, as the medical home, working with these children on a regular basis.

Dr. Cheng: Short bowel syndrome, intestinal failure as the name suggests is due to loss of bowel. The main function of the bowel is to you know absorb fluid, electrolytes and provide the nutrition for the whole body. So, when the function is compromised, the first symptoms would be the loss of fluid and so end up a lot of diarrhea and then also malnutrition, maldigestion. So, that's the first thing. This is a long process because it takes a long time for the bowel to adapt. Usually we're not talking about days or months, we talk about years. So, patients usually end up also failure to thrive, poor or retarded growth.

Host: And Dr. Taylor, help us understand some of the surgical and medical options for managing these patients.

Janice Taylor, MD, MEd (Guest): They need to be considered together. A lot of these children because they're not able to absorb nutrition normally, a lot of them will need to have some kind of a feeding tube, like a gastrostomy tube or a gastrojejunostomy tube, depending on the situation. Typically it's a gastrostomy tube. And while we encourage them to eat by mouth, or to take formula or breast milk when they're infants, a lot of times they aren't able to process and absorb feeds normally like any other baby would. And so having a feeding tube, having the access straight into the stomach allows us to say administer some of their feeds in a more slow fashion so that they can have a more gradual way of absorbing their nutrition in what little bowel they have.

So, feeding methods are one important way that we manage these children. Other methods involve various medications that we can use for nutritional absorption. As well as managing what's called bacterial overgrowth. A lot of these babies because and young children, because there are bowel isn't functioning normally, they can get a lot of bowel dilation over time as well, depending on what kind of intestinal surgery they needed that, that got them into this short gut syndrome situation to begin with. They may need to be on oral antibiotics at fairly regular intervals to help control the overall amount of bacteria in their guts to help with that absorption. Some children are also candidates for medications that involve growth factors. So, that's another way that we can manage these children and try to get their intestine to adapt out of the shortgut environment. There are other medications we use to help with fluid production, hypergastronemia in the stomach and in other things that we can do but there's no one perfect thing that works.

And sometimes what may initially start to work on a child ends up not working so great. And we have to move things around. As far as the surgical options for these babies, there are very few specific criteria that they need to fit. Not every baby, just because they have a short length of intestine can actually be a surgical candidate.

The bowel needs to be fairly dilated, typically at least four to five centimeters in diameter in order to be candidates for various types of surgeries to either lengthen the bowel. And is there a sequential or step fashion as we call it or if it's very dilated and if their length is actually not too short if it's actually an okay length, but it's just very wide, then we can do, what's called a tapering, enteroplasty to help the bowel not be so dilated to help move the food contents through.

Host: And Dr. Taylor as we're talking about these types of treatments, help us to understand the importance of multidisciplinary care because you were mentioning a few different modalities and given the complexity and with increasingly complex treatment algorithms that add new options, really to your armamentarium of available therapies, speak about this approach, the multidisciplinary approach, and who's in charge of guiding patients care.

Dr. Taylor: It really is a multi-disciplinary approach that helps these kids do better and have improved outcomes. About 60% of pediatric patients who are listed for intestinal transplant are on that list because they have short bowel syndrome. And it's been shown with some studies looking at either regional groups of intestinal rehabilitation and intestinal failure programs, or even national international groups that if children are involved with these kinds of multidisciplinary care programs, that their outcomes are better, either their outcomes without needing an intestinal transplant or liver transplant are better.

And the liver transplant comes into play because these babies and children need the IV nutrition, the parenteral nutrition. That can cause problems with the liver over time. They either can do better for longer without ultimately needing IV nutrition or if they do end up going to transplant that their outcomes are better because they've been managed more appropriately with the various disciplines that are involved in these children's care.

So, surgeons like me, pediatric gastrologists, like Dr. Cheng also in our group are pharmacists. The neonatologists where a lot of these children will come from here at UF. We've got a core group of neonatologists from our NICU who are involved in the management of these patients, as well as neonatal and pediatric nutrition specialists.

Dr. Cheng: Yeah, I would add that Dr. Taylor we are doing a really good job in working together. A multi-disciplinary short bowel clinic. Most of the time we see patients together as a group and oftentimes, those patients and families not only have medical issues, but also have surgical problems, like a malfunctioning of the feeding tube, occlusion, or damage of the central lines, venous line. Or sometimes they have questions like whether I have an ongoing bowel obstruction, strictures versus stellations or not. So, in those cases, seeing patient separately it's not very helpful, I think that the team approach is really the best way to approach them with the best patient satisfaction.

Dr. Taylor: I agree, and it really allows to streamline their care and make things more efficient. So, they don't feel like they are being bounced around from clinic to clinic. They're seeing everybody in one place. And if I can also add too that we call ourselves in an intestinal rehabilitation program and it's a lot of times synonymous with intestinal failure programs. But I think that we really like to focus on the fact that we are trying to make the bowel adapt and rehabilitate it. You can almost say that it's a focus on the more positive aspects of things and what we're trying to do.

Dr. Cheng: I would also add in that diet is the foundation of all therapies. So, without the diet presently in the lumen, there will be no omtestomal adaptation to occur. I feel also our dietician play a critical role in managing this nutritionally disabling patient. They work together with us, to make sure patients start with right diet in the right time.

Host: Well thank you both. And I was going to ask you about the intestinal rehabilitation program at UF Health Shands Hospital and Dr. Cheng, if you would give us a little bit of a summary of the goals of that therapy. You just mentioned diet and certainly understanding each individual's remaining intestinal function is so important. How does that program work briefly if you would.

Dr. Cheng: Patients with short bowel, if you give sufficient time and support the bowel eventually become adapted and can wean off parenteral support. And without the need of even transplant. So, it's a time and otherwise required by the patient. So, our dietician play a big role and then food, feeding play a critical step. And so we have to initiate diet earlier, gradually as permitted based on the stool output. And then we will change according to the need and also oral feeding as early as possible because the combination of an enteral feeding together with oral feeding produce a greater adaptation and absorption than enteral feeds alone.

Host: And before wrap up, I'd like to give you each a chance for a final thought. So, Dr. Cheng first to you. Give us some strategies for successful outcomes and how the success of therapeutic interventions are working for you. Tell us a little bit about your outcomes at UF Health Shands Hospital.

Dr. Cheng: You know most of our patients right are now doing very well. And we manage more than 25 patients totally. And they only one patient, recently have a referral for transplant evaluations. Most of the patients doing well, on the enteral feeds and some already started on oral feeds, and then we are steadily weaning off the parenteral support. Our patients are in, in the process towards achieving, enteral autonomy. And so that's our goal.

Host: Dr. Taylor last word to you. Tell us a little bit about some future interventions, such as bowel lengthening procedures that could show promise. Tell us a little bit about what you would like referring providers to know about your program at UF Health Shands Hospital and when you feel it's important, they refer.

Dr. Taylor: Sure, I think when a provider has a concern about the absorption, about the weight gain, signs like diarrhea, failure to thrive based on a child's surgical history, or birth history that may have to do with a short length of intestine, or even a normal length of intestine, but acts like it is short gut, then that should be the time at which they should be referred to our program. It's been shown that, based on liver function and other nutritional parameters that the earlier these children get to these multi-disciplinary programs and the better they can do. I would also add that, along with just the assessment, we also work off of our collective knowledge and what we've done to help standardize care and make things more streamlined for these complicated patients that we've developed over the past couple of years, multiple guidelines for how we manage feeds, how we initiate feeds, when we decide to start certain medications to help with their overall outcomes. So, when we first meet a patient and if they haven't had surgery or even if they have, then we will probably do some imaging studies to see exactly what their bowel looks like to see if they would be a candidate for any kind of operative intervention.

Host: Thank you both so much for joining us today. To refer your patient, you can always visit UFhealth.org/pediatricsurgery for more information. Or to listen to more podcasts from our experts, please visit ufhealth.org/medmatters to get connected with one of our providers. That concludes today's episode of UF Health Med Ed Cast with UF Health Shands Hospital. Please remember to subscribe, rate and review this podcast and all the other UF Health Shands Hospital podcasts. I'm Melanie Cole.