Bowel Management Updates & Innovations with Live Q&A: April 2018

excited to be here today. My name is Jason Frischer. I'm the director of the Colorectal Center here at Cincinnati Children's. And I'm Monica Holder, the clinical lead nurse at the Colorectal Center. And we are excited to have this hour with you today. The type of patients we take care of include anorectal malformations or patients with imperforate anus, Hirschsprung's disease, spina bifida, patients with sacrococcygeal teratomas, patients who have sustained trauma, and patients with refractory constipation. We see patients from all over the world and all 50 states. We work in a very unique collaboration with a number of partners. The colorectal center is a hub for multiple teams that we collaborate with in a coordinated fashion in order to provide individualized and specific care. We work with our urology and gynecology partners in order to help care for our anorectal malformation patients when necessary. We work with our inflammatory bowel disease partners in the gastroenterology department. We work very closely with our GI motility partners who help us with motility testing and caring for patients with motility disorders. And then we have a specific group of collaboratives within this including behavioral medicine, physical therapy, nutrition and radiology. And we've set up a collaborative clinic called the MDMC or multidisciplinary motility clinic where we present a coordinated treatment plan for all of our patients using many of these modalities. So our program, um, we have a very specific program for bowel management, which is um, very popular and probably a lot of what tonight will be about. Um, our patients are evaluated by the surgeon and testing on the appropriate evaluation for them and are usually categorized into patients who need enemas, laxatives or other oral treatment and then patients who may have already been on enemas that want to try to stop and see if they can be continent without enemas. The next few slides will review some of the treatments that are available, some old, some new. And then if there are questions about these, please chime in and either text them in and we'll be able to answer them, uh, in an orderly fashion. First is something we call an appendicostomy or a Malone procedure, others call it a Mace or an Ace procedure. And this is where we take the appendix, which is at the very beginning part of the colon, bring it up usually to the belly button. We make a little wrap or valve-like mechanism to prevent reflux into that appendicostomy. And then instead of doing rectal enemas, the patient is able to independently perform flushes through their colon through the belly button. I'll show a picture on the next slide here of a patient after the surgery where they have small incisions and you just place a tube within your belly button and administer a flush over a 10 or 15 minute period and then sit on the toilet for the remaining approximately 45 minutes to empty the colon. Another option we have on giving rectal enemas and being independent is a um device called Peristeen. So for patients who are wanting to be independent, a lot of the pieces needed are in front of you and we can use this Peristeen device. A newer device, which is not FDA approved for children under the age of 18, although we do perform this surgery on patients under the age of 18, is called the sacral nerve stimulator. It acts similar to a heart pacemaker and it's actually made by the same company, where we put a lead, an electric lead over the third sacral nerve, which controls both your bowel movements and urinary continents and it's able to stimulate that nerve in a way that provides for um, better outcomes, whether it be using enemas or laxatives or coming off of enemas to laxatives or even maybe not needing any medication at all. It involves using a battery device that's the same battery used for cardiac pacemakers and uh, we implant that uh, within the upper buttock area, uh, and it's a two-stage procedure where we do a test phase and a more permanent phase. Uh, and it's newer and we've been doing it here for almost three years now. Finally a newer device or or um treatment modality is something called Celesta. It's an actually injection of a hyaluronic acid or a polymer that fills the space in the anal canal. And if some patients and we've used this sometimes in patients who have anuses that are more patulous or more open, this fills that space and helps some patients gain control. And I think it's important to share that um the treatment that we offer is given by a a huge team of people. You will often um in clinic see your physician, nurse practitioner or maybe nurse, but in this picture you can see the array of people who are also behind the scenes and in front of you taking care of your child or you as an older patient to make sure the things that you need are taken care of. Um, one of the most important people on the team is you as a parent, a caregiver and as the patient as well. So we very much include you in all of the work that we do. This session that we set up is really for you. And so we're hoping over the next 50 minutes or so that we answer questions that you have for us. And so please send your questions in via Facebook or via the chat lines and we will answer as many questions as we can. Finally, if you're interested in contacting the Colorectal Center, some of the information is posted now on your screen, whether it be phone number, um, email, you could like us on Facebook, um, or international phone numbers if needed. We will take all the questions that are sent if we do not get to them all, we will group them together and we will in the next few days post on our Colorectal Center Facebook page many answers to the questions sent in if we can't get to them all this evening. So now I think we're going to start with the question session and I look forward to trying to answer as many of the questions with Monica, uh, to help, uh, help you understand more about bowel management and other related colorectal uh processes. So one of the questions um given to us is um a child with a deformed sacrum currently on enemas and has failed um bowel management programs and at what age is a Malone indicated or any other procedures? So that's a great question. Thank you for sending it. When patients have deformed sacrums as mentioned, they the the likelihood of being successful with bowel management with medication alone, not on an enema program is lower. That being said, a Malone as described a few minutes ago, allows for the administration of an enema through the belly button in a more independent fashion. We typically start discussing this when they're, when a patient has already gone through a bowel management program and has been successful with enemas. You have to be successful with enemas because really what the Malone does is it allows for a different route of administration. It's still the same process. So we don't want to perform a surgical procedure on someone who's not successful on an enema program. That being said, we typically perform this surgery between starting at around age five years of age and older. I think most patients have this surgery performed between ages five and 10 or 12 years of age, but we do a broader spectrum than that, but typically not before age five. And there's a comment about what causes vomiting after an enema? So I think Monica is probably more apt to answer this because she takes all the parent phone calls during the day. I'll mention a couple and she'll correct me and and add to it. I think vomiting from an enema, when you give a high volume enema, a few hundred MLS along with an irritant, that could really irritate the colon and stretching the colon can cause discomfort. So I think there are a few tricks to do to try to limit that possibility, and maybe you want to comment on a few. So I I just think a couple things is if you are giving an enema right after your child has eaten a full meal, sometimes there's just too much distension of the stomach and then you're filling the bowel and that can make them feel nauseated. So we recommend either doing the enema prior to meals or giving about an hour after a meal to digest before giving an enema. Um, I think we also see patients who have maybe a sacostomy, um, when they're given the enema, if it's too fast or if the solution is kind of going into the small bowel instead of to the colon, sometimes that can cause vomiting. And if we suspect that, then we have a study that we can do to kind of track the way the solution flows into it. I think other things are warming the solution. Warming the solution helps and making sure that the irritant is not too much for that kiddo. Um, you know, you have to really treat the symptoms. So if it's, if it's too irritating, then we often have to change the additive we're using and adjust the volume of the solution to to treat that symptom. What about the timing, how long you give the solution? Yeah, so we typically um give the solution over at least five minutes, sometimes up to 10 to 15 minutes so that it's not going in very rapidly because that also can cause fast distension and um vomiting. Um, we can talk a little bit about sacral nerve. The question is, how can I have my son evaluated for sacral nerve? Excellent question. So one as I mentioned earlier, sacral nerve stimulation stimulators are not FDA approved for children under 18 in the United States. That being said, we perform these surgeries off label in a number of patients under the age of 18. The indication is according to the manufacturer, that you have to get 50% improvement on whatever symptoms you have. So you must have already gone through a bowel management program and tried non-surgical or invasive ways of treating the symptom uh, that is of concern. And then the evaluation is really the biggest issue is having a proper or normal sacrum. Because we use the sacral nerve stimulator in an algorithm of we do bowel management and then if things are working or we want to try to improve the situation, we can either do a Malone, a Celesta injection or sacral nerve stimulator at that point. And sacral nerve stimulation is probably the least invasive of those options. Uh, the typically most people would say you have to have a normal sacrum to do that. That being said, we performed today in our operating room here, we have a special operating room called the hybrid OR, which combines surgery and interventional radiology and we can get three-dimensional imaging of the sacrum, of which we did today on a patient who had a very abnormal sacrum and we were able to place the sacral nerve in this patient today and hopefully they'll have a good outcome. Only time will tell. Um, but that's how to get the evaluation is we would need to see some prior records of what was done and then usually a consultation and in order to um discuss the option of sacral nerve stimulation. Um, and and going back to um enema use, the question is what is the maximum volume that we would use for enemas? Um, I think we have we can answer that in a in a couple different ways. We really try to tailor the volume based on the size of the colon. So prior to any bowel management program, we do a contrast enema study, which is a study in radiology where they fill the bowel with a contrast solution and gives us a good roadmap of how distended or non-distended the colon may be. And we kind of use that to gauge the volumes that we use. Um, with that said, I think we on average use um, at the upper end of about 500. Um, we may on occasion go a little bit higher. Um, and this is for uh rectal enemas and sacostomy and Malone enemas, we're typically around 500 to the upper volume on occasion a little bit more, but it's rare. Um, think a question will flip over to um. Sure. Here's a question, for a failed redo pull through, what other options are available? A Mace question mark or just go back to ileostomy? This is probably a Hirschsprung's patient, but for any patient, we try our best to allow for the family to make decisions on what is best for the child. And that being said, it's important that there are options to still have your use your rectum and your anus as a way of evacuating stool, even if you are maybe incontinent. And so that's where bowel management comes in in that we can mechanic, bowel management is truly a mechanical way of emptying the colon and trying to get the patient to stay clean. Both clean in normal underwear and clean so they're not backing up with stool and really making a artificial constipation situation that once it gets so backed up, either there's tremendous discomfort or overflowing continents where loose stool leaks around the hard stool. So that being said, we can always or almost always redo a pull through to at least make a channel so that stool could be evacuated. We've done many patients where they've had one or two or three redos for Hirschsprung's disease or other disease processes and we're able to redo the operation in order to make it a more functional outcome. We there are very few patients that end up with an ileostomy. I don't want to say never, but if that if the family's goal is to try to um family and patient's goal is to try to end up with a patient that is um evacuating via the rectum and the anus, then we typically can set up a situation where that's possible. It's not always true though. Um, just to add to that, there are certain patients if um, depending on the amount of colon that they have left or maybe almost all of the large bowel has been removed, we may be able to get them clean on a enema program or um, the challenge for some of them without colon is that the stool can be either liquid or frequent and we may be able to keep them from having accidents, but some of those patients have to be uh, mindful of their diet. They may not be able to eat foods that are very laxative in nature, they might not be able to have certain drinks that are high in sugar. Um, so we may be able to find a very regimented routine with specific diet and medication. Um, they might be taking a an imodium type of medication multiple times a day, but it might be such that their quality of life is altered so much with their diet and the medication that they then choose if this is a good quality of life for them. And on occasion, we've had those patients say, I know I can be clean, but it takes so much of a diet regimen and medication that I might prefer the um stoma. So that is on a on a couple occasions we've had patients who have chosen to keep the stoma even if we have been able to get them clean in the underwear without it. I just want to stop for a second and say, we are getting tons of questions and we thank you very much. Keep them coming. We will try to answer as many as we can now and we will hit the rest on our Facebook page uh over the next few days. Um, do you want to add anything about what to expect when part of the large colon is removed? I think that goes a little bit with what. Sure. So the large intestine or colon is an organ that its primary function is to absorb water. So when your stool or or food material enters the colon, it's liquid and by the time it gets to the end of the colon, it's supposed to be solid. The colon in adult can absorb up to 1 liter of water a day. Uh, so if a portion of the colon is removed, then the stool will be softer or less water is absorbed. As more and more colon is removed, it becomes softer and looser the stool. We have, like Monica just mentioned, we have ways to sort of have sort of harden the stool or thicken the stool in a way that sometimes bowel management requires and it seems weird, but we have to use agents like Imodium to constipate the child and then use an enema to empty the child. So we thicken and harden the stool and then we have to use another method to empty the stool. And so depending on the amount of colon and how the colon's functioning, and this is where sometimes in conjunction with our GI motility specialists, we could do tests like motility testing, colonic motility, anorectal ummetry or pressure readings in order to help with that. Yeah. Um, and I think this um goes with this question as well. The question is, um what are your feelings about resections and the success rate of the resections? Um, maybe with or without sacostomy, does it work and how long before you decide they need an ileostomy? So, I'm assuming that's a patient without any other malformations or diseases like Hirschsprung's disease and anorectal mal um, anorectal malformations, this patient we call say has idiopathic constipation or refractory constipation, they've used multiple medications. So there have been multiple studies, studies done at Cincinnati Children's, studies done at other centers in pediatric patients. And what I tell patients, there's no perfect surgery for constipation. If we had it, we would all recommend it and do it. There are um, we've done procedures where we do transanal resections of the colon and rectum and remove large segments of colon that way. It's very minimally invasive. The problem is we found some of those patients become incontinent after that surgery and so there's some risk to that. We've done surgeries where we just remove the sigmoid colon. We've done surgeries where we remove the sigmoid colon and parts of the rectum or taper the rectum. So the plan really has to be individualized depending on the patient's anatomy and their situation. That being said, there's no proven surgery that absolutely works. What I often tell families is that the surgery may lessen the amount of treatment that the patient may need. So let's say they're on uh, a laxative or X-Lax and they're on 10 squares of X-Lax. Our hope would be that we can get you down to five squares of X-Lax. Unfortunately, that's not always true. Um, it really is individualized and we use colon resection as a last ditch effort. Before that, we typically will offer obviously the bowel management, but then sacral nerve stimulators, we certainly would do manometry to test, how does the colon work? Sometimes there's a segment of colon that doesn't work when we do our colonic manometry and that's where we will focus our surgical procedure on. So it really is a multidisciplinary approach or is the patient having an outlet problem where the colon works just fine, but they are not able to expel the stool because their their sphincter isn't working right. That's where sometimes we do anorectal manometry and the sphincter's working opposite of what you think it is. The child thinks they're squeezing and they're relaxing and the child thinks they're relaxing and squeezing. That's an opportunity where sometimes Botox works in younger children or physical therapy and bio feedback works in older children. We have all those modalities here and that's why we work as a team and that's that multidisciplinary motility team that I was speaking of earlier, where we pre-visit plan and and review all the patients to see what is the best modality and what's the cause? We don't always know the cause, but if we can find the cause, then we go ahead and try to treat it. I think it's important to say we do get many, many referrals with either the family or the physician referring to go directly to bowel resection. And just as we talked about, um, I think it's frustrating for a family who contacts us and says, we need to have the bowel resected. And we kind of walk through all these other treatment options. Um, and I think it's important to know and understand it's because there are many times we have these other options that are much less invasive. And since it's not a perfect outcome that we know of yet, then we do try these other options first. I think that's a huge point. It's not a perfect option. No one has proven that any surgical procedure is definitively curative for constipation. And so we do individualize it and sometimes we get to that level. But it's important to know that these other options are less invasive. A colon resection is a big surgery. Um, it requires an anastomosis or a connection of two ends of the bowel, um, which has risk to it. Uh, we do many of them here, so it's not like we don't do it, but I think it's important and that's why we entitled this new innovations in bowel management because I think there are a few steps before we go to that definitive step. Once you resect the colon, you can't go back. We can't place it back. All the other treatments we stated are reversible. Um, and I think the other thing is families often contact us feeling like they have tried some of these other options such as enemas and laxatives. And I think just to understand that, you know, many patients have tried dosing um combinations that are either dosed to your age or your weight or the manufacturer recommendations, but many of the patients we see, their constipation is so severe, the treatment is much beyond what is kind of outlined. Um, and so we, you may come to us have already tried a lower dose that we may still be able to get you successful on something additional. Um, you want to kind of go to um, yeah, so we can kind of touch on laxatives a little bit. We haven't talked about that. Um, the question is um, for X-Lax or senna laxatives, is it addicting and what are the long-term effects of it? I think that's a great question. Would you say we get asked that question every day? I think we get asked that question every day. I'll let you answer it. So, um, what what we see is there is no research that kind of leads you to any sort of scary outcomes of Senna. Um, what we do see is that patients over time, we continue to monitor ongoing. You will not see us give you a dose of laxatives and send you out the door and discharge you on that dose. Um, we do see patients that need to increase the dose sometimes over time, but we also see patients that can decrease. Um, it also goes with kind of your your diet, your exercise, hormone changes. Um, as patients go through toddlerhood and school and adulthood, they they change their dietary stuff. Um, the one thing that you will be able to see is if they ever have like a colonoscopy, there's some markings on the colon. It kind of looks like a freckling type um visual, so you can see if a patient is somebody who's been on longer term uh laxative use, but there hasn't been anything connected to any scary outcomes. That's right. Um, that what Monica's alluding to is something called melanosis coli, it's sort of a freckling of the colon when we do colonoscopies, but other than seeing freckles, there is no long-term side effects to that. I like to, we get asked this question all the time and I make the analogy when I speak to families of because they ask, do you get addicted to it? Does it make you immune to letting your colon work in the future? And I sort of say it's like insulin for a diabetic. A diabetic needs insulin to lower their blood sh, blood glucose levels. A patient who has severe constipation needs a laxative stimulant to help move their bowels. Will it change over time? Yes, it very well might. You may need more, you may need less going through all those developments, um, growth developments throughout um, one's growth cycle, uh can definitely change it along with uh diet as you mentioned. But uh I I don't want to let out too much information, but I certainly know people who have been on laxatives for a very long period of time, well over 10 years without any side effects or being addicted to the laxative. No, I think the one more obvious side effect is if the patient is on too much laxatives for what they need, then they obviously can have diarrhea. So it does need to be adjusted and monitored by a healthcare person. Um, kind of on the same lines, the question is the recommendation for the use of laxatives, which we kind of touched on and Miralax, um, and the use of both of those. With Peristeen that's in combination. In combination. So we typically use, so this is a great question. I think we can talk from the beginning. So there are two stimulant laxatives, which means that these medications make the colon squeeze or push the stool forward. That does two things. One, the obvious, it pushes the stool forward, which is one of the things that we often need in our patient population. The other thing is the stool's moving quicker through the colon, it has less time to absorb, the colon has less time to absorb water, so the stool is a little softer. So that's why we prefer to use stimulant laxatives. There are only two stimulant laxatives that are available in the United States: Senna, some of which we use is X-Lax, those are the chocolate squares, but it comes in tablet form as well as liquid form and Dulcolax. And so those are the only two real stimulant laxatives available in the United States. That's different than Miralax, which has the word laxative in it, but is not a stimulant laxative. What Miralax does is it allows the colon to absorb water. And so the water stays in the colon and not absorbed by the colon, which makes the stool softer. We try to avoid that especially in patients who don't have all the mechanisms to have great continents or control of their stool. So if the stool's too liquidy and you have some of the nerves and some of the muscle but not all to have good control, then making a very loose bowel movement is very hard to control. And so we typically do not use Miralax in many of our patients. That being said, in patients who have spinal cord issues for a number of different reasons, whether it be tether cord, spina bifida or others, often the stool is so formed that we have to use a Miralax type agent to help soften the stool along with motility agents. Did I miss anything? I often do and usually correct me. Okay. Um, kind of leads us to um, the question is, after a colostomy for the the patient is given