Rob Steele, MD (Host): Welcome to Pediatrics in Practice, a CME podcast. I'm your host, Dr. Rob Steele, Executive Vice President and Chief Strategy and Innovation Officer at Children's Mercy, Kansas City. Before we introduce our guests, I want to remind you to claim your CME credits after listening to today's episode. You can do so by visiting cmkc.link/cmepodcast, and there's a little button there to hit claim CME, push that.

Today, we are joined by Dr. Iris Lim-Beutel to talk about chronic constipation and an overview of inflammatory bowel disease. Dr. Lim-Beutel is a surgeon and Associate Director of the Comprehensive Colorectal Center at Children's Mercy, Kansas City. She completed fellowships in both pediatric general surgery and pediatric colorectal surgery at Cincinnati Children's Hospital Medical Center, an old stomping ground of mine, although I believe Dr. Lim-Beutel came well after me, looking at how young she looks.

Dr. Lim-Beutel is particularly interested in long-term functional outcomes for patients with Hirschsprung's disease, anorectal malformation and chronic idiopathic constipation. Additionally, she brings expertise in surgical management of inflammatory bowel disease. She hails from the Philippines and grew up in New York and has a keen interest in bringing quality pediatric colorectal surgical care globally, which I should mention also that I think you relatively recently, came back, I guess it was maybe last year, from a medical mission in Tanzania with Mending Kids. You know, I might start there, and maybe give a shout out about Mending Kids. I know that they provide free life-saving surgical care to sick children worldwide. You've been involved with Mending Kids for a while?

Iris Lim-Beutel, MD: So, last year was my first trip with them, but my mentor, Dr. Jason Frischer at Cincinnati was with Mending Kids, has been working with them for the past decade, and was recently honored at their gala last week for nine trips that he's taken to Tanzania through Mending Kids. And through him, I learned about the organization. It's a really great one where all of their funding is directed towards surgical care of children throughout the world. They are branching out, not just from Tanzania and Kenya, but also to the Philippines, where I come from. So, I'm hoping to connect with them in the next few years to try to build a relationship with an institution in the Philippines and kind of do a similar thing that my mentor has done with Cincinnati and Tanzania.

Host: Well, on behalf of the kids of Kansas City and the children of Tanzania, we thank you for everything that you do. Well, welcome Dr. Lim-Beutel. Let's jump into constipation, something everyone-- you know, it's lunchtime here. We'll go ahead and jump into that conversation either way.

the term constipation gets thrown around quite a bit with just the lay public. Maybe you could give us a sense of what's the difference between chronic constipation, the types of patients you take care of, and just constipation in general.

Iris Lim-Beutel, MD: Yeah, I think that's a really great place to start because part of my job that makes it a bit of a challenge is that people already have their preconceived notions as to what constipation is. Some patients will say, "Well, I'm not constipated. I poop all the time." But then, there are others that say, "I'm constipated, because I don't poop every day," but they don't have accidents throughout the day. So, I just want to lay down kind of the idea you don't have to poop every day to not be constipated. There are some people that they poop every other day and that's fine for them. That's just how their body is. We don't have to make ourselves "regular" and go to the bathroom every day after your first cup of coffee.

I think where constipation becomes chronic and therefore problematic is that you don't have a pattern with regards to your stooling. There are times where you poop all the time to the point where you don't know that you're pooping. You're actually having what we call overflow incontinence, where you have hard rocks of stool stuck in your rectum, and your body just releases liquid stool around those rocks because they need to release it somehow, but you don't have control of that. That's an example of severe chronic constipation.

There are some parents that come in and they say, "Why does my kid have constipation as a diagnosis? They clog the toilet all the time." Well, that's also a problem too. They don't poop regularly, but when they do, it's this giant explosion, for lack of a better term, and they're clogging the toilet, that's also a problem.

Host: Yeah. And I imagine, culturally, particularly with different generations, I mean, as a general pediatrician, the ideas of what constipation are often were different between the grandparents and the parents, and then maybe even friends that come with the parents. So, I appreciate the definitions.

Let's move to more pathologic, more potentially concerning pathologic processes like Hirschsprung's disease. What are the typical signs of Hirschsprung's? And maybe even focusing outside of the neonatal period, because I know a lot of times it'll present in neonates, and that workup becomes readily apparent. But I imagine you probably see patients with Hirschsprung's that are older than that.

Iris Lim-Beutel, MD: Yeah. Like you said, Hirschsprung's is typically diagnosed in the neonatal period. But in places like Tanzania and the Philippines where you don't have regular NICU care, or the last couple months, I have diagnosed Hirschsprung's in children over the age of 15 months. It does happen. I think big critical signs to look at is your child is having difficulty stooling regularly. It's never an association to the type of food. If a child has a viral illness, sure, we can give them a pass and say they probably have a hard time pooping because they haven't fed very much or that sort of thing. But if they are consistently having issues pooping, that should be a red flag. But I think most importantly, I think most specific to Hirschsprung's, as you can get without getting a contrast study, is explosive stools, explosive gas, and the need to have some sort of rectal stimulation, either via an enema or a suppository to stool.

In fact, one of the recent patients I diagnosed with Hirschsprung's who's much older, about two years old, the mother told me that the only way she could get her son poop was if she did a suppository. And lo and behold, you know, the first step when we evaluate these patients is to get a contrast enema where contrast is placed up the anus. And so, we get to see what the rectum and the sigmoid looks like. And for that child, they had an abnormal, what we call rectosigmoid ratio. So in a normal person, the rectum should be bigger than the sigmoid colon, because the rectum acts as the reservoir for poop before it comes out. But in Hirschsprung's disease, often the rectum is much smaller than the sigmoid colon. And so, that will then trigger us to do a rectal biopsy.

Host: That observational finding, I think that's probably the first time I've heard that. That's a really good one. It makes total sense. I'm used to just hearing about the skip lesions that you'd otherwise, see in a contrast study. So, that's a good pearl of wisdom there.

Just pulling a little bit further on chronic constipation, and once that's really diagnosed, what is the typical plan of care? And when, if ever, do more invasive type of procedures, when are they required in chronic constipation?

Iris Lim-Beutel, MD: For chronic constipation, the first thing that we do at our center is make sure that we've ruled out all other possibilities, any anatomical issues that cause constipation or motility like Hirschsprung's. So if you have ruled out everything, and the child is still constipated, then they carry the diagnosis of chronic idiopathic constipation. And I think this is where our bowel management boot camp program really shines. I run that with one of our nurse practitioners. It's a week-long program where at the beginning of the week, so we usually start this on the first Friday of the month, we meet the family, we meet the child, try to get a sense of what has been going wrong with pooping and what are our goals at the end of the week.

And starting the following Monday through Thursday, we are in direct communication with them throughout the week, changing either their enema program or their laxative program, and getting daily x-rays. So, we actually can corroborate their stooling report. They have a poop log that they have to fill out on REDCap. And we match that with the x-ray they're getting that day. And then, at the end of the day, our nurses communicate with the family and say, "Dr. Lim and our nurse practitioner wants to change your regimen to XYZ. We'll talk to you tomorrow and we'll see what the x-ray shows." And so the goal of that is at the end of the week, these families are armed with a regimen that they know by both their history and an x-ray that it's working for their child. And then, we follow them up in regular increments, one month, three months, six months, and a year after that boot camp.

So, long story short, I think the key for a care plan that's successful for chronic constipation is a consistent one, because our diets vary, so our poops will vary. So if we can keep the care plan consistent, that's the best recipe for success.

Host: Very good. I love what you've done there with the poop diary, calling it a poop log, no pun intended. I couldn't help but notice that.

Iris Lim-Beutel, MD: I was hoping, you'd catch it.

Host: Let's move on to inflammatory bowel disease. So, how do you distinguish that from just simply the common belly pains that might be longstanding, and sometimes even severe? How do you go about distinguishing what might just be more functional belly pain, for example, from truly IBD?

Iris Lim-Beutel, MD: And this is where I really struggle with this because with my pediatrics colleagues, they see all of the functional belly pain and they are tasked with trying to figure out what is functional and what is the one that I send to the surgeon and to GI, because I think it's harder in children than it is in adults. I came from the adult world. I trained in New York City, so there's a lot of IBD there, but these are adults. These are not children that you're like, "Well, does your stomach hurt because you haven't pooped or you haven't eaten? You can't tell me."

But I think when it comes to distinguishing in children, it's more than just belly pain. It's pain that's limiting their quality of life, that's leading to absences from school. And then, you'll see it, you know, in terms of their weight gain and their growth chart. If their pain is such that it's limiting what they're eating, you can see stunting of growth and often stunting of puberty.

There's a special subset of IBD called perianal Crohn's disease, and we studied that in Cincinnati where, you know, how often do these children that come in with perianal disease, like skin tags, fissures, perianal abscesses, how often is that IBD? And then when you look into it, it's usually young boys, at least age eight, who are coming in with a perianal abscess. And when you ask on history, do you have chronic abdominal pain that maybe your pediatrician never figured out? And there was a strong correlation with Crohn's disease in those children and we ended up diagnosing them. So, I think it's really pain that's persistent, and nobody has figured out the reason for it, and that's limited their quality of life.

Host: Very helpful. I wonder with the number of pharmaceutical therapeutics for IBD that have come out in, you know, the recent years, do we see that surgical intervention is now decreasing or no?

Iris Lim-Beutel, MD: I think it's more of the surgical interventions. Those children are often at a worse shape now when they need surgery than they were before. And I don't see this as a good or bad thing. I think it's because we have more tools medically to try to manage them before they go to surgery and often when I meet these families they've been battling with Crohn's or ulcerative colitis for many years. And they often see surgery as a failure of medical therapy and that's part of my kind of role with partnering with the GI team here at Children's Mercy, is making sure that families understand that surgery is not a failure. Surgery is just one of the many ways that we can treat this disease and that we shouldn't delay it just because we think It's a failure in the therapy.

Host: Yeah, very helpful. That's great. Dr. Lim-Beutel, I really appreciate your time and your expertise. It's been fantastic to hear your expertise on IBD and chronic constipation. I understand you have two kids and you bring them on these trips that you often travel. Is that correct?

Iris Lim-Beutel, MD: That's correct?

Rob Steele, MD (Host): Well, my kids like to play this game of would you rather, right? I don't know if your kids do this or not, but we're going to play a quick game of would you rather, right? And we'll focus on Tanzania. So, you only get to pick one, in your life, you can only pick one of these three. The other two, you don't get to do. So, would you rather go to the top of Kilimanjaro, go to the bottom of Ngorongoro crater, or just hang out on the beach of Zanzibar. You only get to pick one. What's your choice? And I'll also say, what would your kids rather do?

Iris Lim-Beutel, MD: Well, I think out of those three, that's very difficult. I've done two of those. I'd like to get to the top of Kilimanjaro. My kids are beach babies and they would rather be in Zanzibar.

Host: I think I'm with your kids on that one. I like a good beach trip. Well, Dr. Beutel, thank you again your time and expertise. And we look forward to following up with you sometime in future as we circle back. I want to thank the listeners. And as a reminder to claim your CME credit for listening to today's show, visit cmkc.link/cmepodcast and click the claim CME button. This has been another episode of Pediatrics in Practice, a CME podcast. See you next time.