Excited to be here today. My name is Jason Fisher. 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, Hirschprung's disease, spina bifida, patients with sacrococcygeal teratomas, patients who have sustaining 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 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 caughtinent 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 in an orderly fashion. First is something we call an appendicostomy or a Malone procedure. Others call it a MAC 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. And 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 the 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 Pisten. 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 Pisten 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 continence, and it's able to stimulate that nerve in a way that provides for 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 we implant that within the upper buttock area. 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 3 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 pattuless 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, uh, 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 can 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 gonna start with the question session, and I look forward to trying to answer 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 and thank you for sending it. When patients have To form 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, 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 starting at around age 5 years of age and older. I think most patients have the surgery performed between ages 5 and 10 or 12 years of age, but we do a broader spectrum than that, but typically not before age 5. And there's a comment about what causes vomiting after an enema. So I think Monica is probably more apt to answer this cause 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 100 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. I just think a couple of things is if you are giving an enema right after. After your child has eaten a full meal. Sometimes there's just too much distention 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 sycostomy, 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 kettle. 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. And what about the timing? How long you give the solution? Yeah, so we typically, um, give the solution over. At least 5 minutes, sometimes up to 10 to 15 minutes, so that it's not going in very rapidly because that also can cause fast distention 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 of, as, 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 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 a sacral nerve stimulator at that point. And sacral nerve stimulant is probably the least invasive of those options. Uh, the, typically, yeah, 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 the, 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 of 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 saccostomy or malone enemas. We're typically around 500 to the upper volume. On occasion a little bit more, but it's rare. Um, I think a question will flip over to, um, Uh, yeah. 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 Hirschprung'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 an artificial constipation situation that once it gets so backed up, either there's tremendous discomfort or overflow incontinence 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 1 or 2 or 3 redos for Hirschprung'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 wanna 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 an 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 um 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, uh, 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 of 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 we talked about. 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 an adult can absorb up to 1 L 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 colons removed, it becomes softer and looser, the stool. We have, like Monica just mentioned, we have ways to sort of 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, anal rectal, um, manometry or pressure readings in order to help with that. Um, 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 resection, um, maybe with or without sarcostomy? 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 Hirschberg's disease or interectal um interectal malformations is 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 removed 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 5 squares of X-lex. 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. is 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 anal rectal manometry, and the sphincter is 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 biofeedback 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 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. Dissect it and we kind of walk through all of 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 risks 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, cause I think there are a few steps before we go to that definitive step. Colons 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 that 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 wanna 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 ex-lax or senna laxatives, is it addicting and what are the long-term effects of that? I think that's a great question. Would you say we get asked that every, 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 in 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. Um, that what Monica is 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 made the analogy when I speak to families of, cause 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 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. Now, 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. Let me. With parathis, so in combination, in combination, so we typically use, so this is a great question. I think we could start 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 is 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 as ex-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 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. Continence or control of their stool. So if the stool is 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 tethered cords, 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 you usually correct me. OK, um, kind of leads us to, um, the question is, after a colostomy or the, the patient is given a colostomy because of motility issues, um, will they still suffer from constipation? And I, I kind of am thinking of kind of two options when I read this question. Um, one of the questions or one of my answers is, can a patient with a colostomy still have constipation? Absolutely. So we do have patients that have colostomies that do still suffer from constipation. So they are still on some sort of a stool softener or laxative to have the stool come out, the colostomy on a daily basis. Um, I think maybe the other thing I think about is, um, talking about the patients who have had motility testing, um, colonic manometry in collaboration with our GI motility specialist, where a portion of the lower bowel is proven not to have good contraction, and we sometimes will give a colostomy on the upper end of the colon. To allow the lower colon to rest with the thought that with bowel rest, that the colon could potentially recover. Um, and then we usually wait 6 months to maybe a year and then repeat that colonic manometry with our GI team to see if that bowel has been able to recover at all. We've done that on a handful or so patients I've had good success. Typically those that are patients that have other issues, meaning they've had either a procedure to correct an anorectal malformation or a Hirschberg's pull through and something went a little wrong. They have a narrowing or stenosis or it's a little tight, and then the colon behind that or rectum gets dilated and we give that dilated piece of colon or rectum a rest with a colostomy or an ileostomy. Retest if that colon's working 6 months a year. We've even gone 2 years in one patient where the colon then regained some motility and we're able to reconnect that or close that colostomy. The other thing is, if a colostomy is not working because like Monica said, you could still have a colostomy and be constipated, then one has to consider an ileostomy. Totally exclude the colon and divert the stool before it gets there with an ileostomy or that's the end of the small intestine. Um, the next enema question, um, is patients, um, with spina bifida using cone enemas, having pain and discomfort, and what other enema options are there. So we do not, um, Use, we do not administer the cone enemas through our program. Just it's not the, the delivery type that we use a lot. With that said, we have had a couple of patients coming to us who have been using it. And if it works for you and it's something you're comfortable with, we have no reason not to use it. Um, and so we have had a couple of patients come to us and we will leave you using that piece and we will then just adjust the enema, um, Ingredients within that enema, that cone enema to get you to a better, better place. If you are having pain and discomfort, I think the first recommendation would be to work with your physician on the ingredients within the enema, um, to see if the ingredients that you're using needs to be changed and if there are any other options. So we have to be very careful when patients contact us saying they're having pain and discomfort. Um, especially when they're very, very, very focused on constipation, that there are many reasons for abdominal pain, and you don't wanna miss out on those evaluations to make sure that there are no other things going on in the abdomen that might be causing pain or discomfort. Um, You that I think you have to make sure, depending on where the pain is, to make sure there is no injury just on the administration of the enema, the enema device. And so that's where your physician needs to make sure they're examining the anal region to make sure there's no fissures or cuts or little ulcers that may be developing to make sure that the device is being used properly and not causing any minor injury to that area that can cause. Extreme discomfort. But then also not forgetting about the other organs within the belly as well, um, appendix or, you know, gallbladder or anything like that, that could also be causing pain to not be so overly focused on the constipation, to forget about those things. Um, and the, the last part of that are what other enema options are available. Um, we kind of touched on, I don't know if you need any more elaboration of malone and saccostomies, but then I think here for spina bifida. Specifically would be the peristene um that we showed the picture of at the beginning. Um, this is actually a product that was designed originally for patients with spina bifida. The design of it is meant to be used for patients that might have a little bit more problems with, um, the dexterity of their fingertips or not having full use of their extremities and their arms as well. Um, so the product is very much designed for that type of patient. Um, it does mean that it has to be ordered through a physician and also needs to be, um, you need to have education on how to use it, but it is meant for a patient to be able to become independent with it. Um, with that said, we have tried it on a range of different age groups. And it seems like we've had more success with patients that have been uh older than 8 or 8 or above. Um, the patients we have tried it on that were a little bit younger, we just had a harder time with getting the balloon device to stay um in the rectum. So we've had much more success with age 8 or older. Um, there are some challenges with getting the Pistine product as far as like insurance coverage and um having a supplier. So that is something that you will often have to um work through with your insurance company to find a provider. Um, we often have to do appeal letters and it takes, uh, you know, a fair amount of work with you to try to do that. And it does take a fair amount. of paperwork just as caution, what we see is typically the insurance companies will deny it the first time just because it's a product that is not used a ton through the United States yet. And so I think it's very easy to kind of give it a denial initially and then um your healthcare provider will have to work and, and give letters and such to get through the appeal process. Um, This one's touching on research, any research toward tissue regeneration for anal sphincter, talking about innovation. So I can tell you here at Cincinnati Children's, Michael Helmrath, one of our surgeons, is heavily in the lab growing colons. So he is growing colons from single cells and able to lengthen the colon now in mice, and I think he's expanding on larger primates. Um, but as far as the actual sphincter, there's not, there's many people working on it, but it's never been successful. People have tried taking muscles, and there are some reports of muscles being placed as the sphincter. Um, people have tried taking Other sphincters from other areas where you have a sphincter in your esophagus or your stomach or other areas and replacing it. None have been really proven to work, unfortunately. Trust me, I wish I could invent this uh procedure because then I'd probably retire in the next few weeks. But um there are people out there doing it, but none are really working right now. So it's not something we offered. There's none proven at this point to be successful. This question is touching back on the sacral nerve stimulator. Um, it says, my daughter was one of the first ones to receive a sacral nerve, which was about 2 years ago. What have you learned about how children respond to it? I'll kind of read through the questions and then we'll come back and kind of answer each one. what are you learning about how children respond to it and how effective, if it, is it in children? What things have surprised you or what have you not expected? So, um, Any, any initial through this. So yes, we've been doing it. We're approaching 3 years of sacral nerve implantations now. Um, the sacral nerve stimulator was initially designed. For urology or urinary incontinence and control issues, when they were testing it in patients, mostly postpartum women who were having urinary incontinence, they found it also helped with fecal constipation and incontinence. And so then it got approved years later for the control of fecal incontinence and constipation. When we extrapolate that and use it in children, it becomes much more complicated because in the adult population, it's typically used in patients who have normal anatomy and normal development, whereas in the pediatric population, it's typically used in patients who have had Operations on their pelvic region were born with malformations or different disease processes. And so it's very hard to study because every patient is different. We have found that it works certainly in patients who have refractory constipation. We found that it works really well in patients who have urinary and stool issues in combination. We have found that it works in patients who have anal rectal malformations and patients who have Hirschberg's disease, but it's really an individualized um evaluation because every patient has a different reason why they're having issues. And depending on those issues is when we really can try to figure out. If the sacral nerve stimulator works. That being said, no one has published a paper in the pediatric literature right now that really details which patients it works in and which patients it does not work in. And that's because there are so many variables in our patient population. It's going to take quite some time. I think some of us who do a lot of these are starting to have more of a feel of which patients, but I can honestly tell you, I put it in a patient where I really didn't think it was going to work, and it works beautifully. So, that's one of the beauties of this is that there is a temporary stage to the procedure, so you could try it out for 1 to 2 weeks, and if it doesn't work, we remove it and don't implant the permanent battery. I think one of the things um from the nursing side is being the one who gets the calls um beyond is the part that I was surprised about is um how well the urinary symptoms have improved. And I do think that, um, you know, the manufacturer says 50% improvement is the goal based on manufacturer recommendations, but I feel that, and, and we don't have it like yet written all out, that we usually have more than 50% improvement. On our device. We are currently studying. One of our nurses, Patty Kern, who's fielding your questions right now, has been studying this and finding, um, hopefully that we'll have results soon in those patterns that you're talking about and or asking about, um, because it would be great if we knew which patients it would work in and which it doesn't. But that's the beauty of the procedure is that we do have a test phase. It is a procedure, but if it doesn't work, we can remove it. Um, and the other thing I think to note about it is, um, most patients, the way it's implanted, there are, um, about 4 different programs on the device as well. So, um, it's set on an initial program and over time, if symptoms return, then we do have the option of changing the program within the implant to get some gain success on that as well. Um, I think this kind of goes, it says, um, how does bowel management help with enuresis as well. Um, and I think I think it, I think we all believe that when you're having issues stooling, it can certainly affect the urinary tract and especially if you're constipated, people don't realize the, the colon and rectum, how intimate they are, intimately they're related to the urinary tract. And so if you have a large stool bowl or constipation or a fecal impaction, that really puts a lot of pressure on the urinary. Tract and can cause a tremendous amount of issues whether it be urinary infections, improper emptying, issues with emptying, feelings of urgency, a number of symptoms, and so they are clearly related and often when you see a urologist, if they suspect. Constipation or issues with stool, that they'll refer to us first to treat that and then only if then the symptoms persist will they go and investigate further. And from our standpoint, if we have a patient who's been um well on bowel management, whether it's enemas or oral laxatives, if you contact me and the only symptom is you are starting to have bedtime wetting that you have been clean for a while, the first thing we're gonna do is suspect constipation as well. So we would check for that um in that aspect. So the question kinda leads to should patients with anorectal malformation be followed by urology? I think we strongly recommend and in our collaboration, like I mentioned at the beginning, that all patients be seen by a urologists and followed by them long term. So patients, there's a whole spectrum of anorectal malformations, and the more complex, the more. Um, associated malformations there might be, especially with the urinary tract, but even in the patients with the most minor malformations, Follow up in intervals, and it doesn't always have to be every year, but follow up in intervals that make sure the kidney is functioning well and that the urinary tract is emptying properly is very important, even if you don't see signs of a problem. It is very important to have checkups with your urologist at times because that is the one area where you could really get diminished health, and it really comes late before the signs and symptoms show up. And we really stress that it is important. Usually, at a minimum, they'll do an exam, see you, and usually get a renal ultrasound or a kidney ultrasound. They may recommend further testing, but it is really important to follow up with the urologist. I think this is a, a good point to kind of um advocate for our female inter ectal malformation patients to also introduce the concept of them seeing a gynecologist, um, kind of for the same reason to have them evaluated and examined so that you know what the reproductive health is, um, for your little girl with anorectal malformation. Great point. You know, I think, and that's why we have a weekly meeting here at Cincinnati Children's where we meet the colorectal surgeons and team, the urology team and the gynecology team, reviewing all the patients, um, to make sure that they're getting a comprehensive evaluation and not just a focused evaluation. Um, so we have some questions about long-term use of items. So we'll kind of go through, um, the long-term effects of MiraLax is the first one. Um, and long-term use of glycerin or castile soap on the colon. So, long-term effects of MiraLax, like I said, we do not use MiraLax that often. Um, we do use it on some patients. There is growing, growing literature and reports and articles in the lay press about, um, I think it was about a year or two ago, a class action lawsuit against MiraLax. I've not heard much about it lately. The, um, the literature on the use of long-term MiraLax is scant. And so, many, many patients are treated with MiraLax and do just fine. And so we have to stress, we, at the colorectal center, we see the patients who fail simple treatment algorithms. Usually, most pediatricians or gastroenterologists start with MiraLax, and I think that's just fine. If it's not working, then we have to find the other cause, other treatment options. But long-term effects of Miralax, as far as I know, um, have not been documented. Um, In multiple studies, there are a couple of studies that came out that there are uh developmental and neuro um developmental outcomes that might be um less than ideal. They haven't been reproduced in any way that we know, but it is out there and something to be thoughtful about when prescribing that. Uh, and then long term effects of glycerin and castile on the colon, I think we, we see. I think any irritant that you add to the colon, you have to monitor. Um, so if we have patients on glycerin or castile soap, typically we don't see long-term effects, but on occasion, we might have a patient who is starting to produce mucus when they stool, and then you have to consider if the bowel is over irritated from the additives. So then we would for sure see if we can kind of dial back on some of the additives or maybe change the product. Um, I think it's important here to talk about um what we do see the most effect when we have products for enemas is the um fleet dissocotyl or I'm sorry, the fleet phosphate. Um, we have seen some irritation to the long-term use of that product, so we try to use that. We can, we use it intermittently, but we try to use it less for long term use. We have a few patients, a number of patients actually that have been on that long term, and it really makes their colon so irritated that we call it like a lead pipe. It looks very stiff, doesn't have the mobility or, or, or, um, pliability that other colons have. And so. Um, we do use, um, phosphate enemas, more of a rescue enema than as a long-term maintenance enema because of this finding. You do also have to be careful with that product. If there's any renal injury to a patient, that is a product that you want to stay away from if there's any kidney, um, trouble. Um, I wanted to talk a little bit about since we are talking about new innovations. The question is what exactly is Celesta used for or when, when would you use it? Great question. What age? So again, a product that is not FDA approved for children. We use it typically in patients who have what we call have a pattuous anus or a more of an opening. So what, when you look at a healthy anal canal in a patient that hasn't had surgery or born with a malformation, it's tight. And you can't see an opening at rest. Some patients though, after surgery or born with certain malformations, the anus is a little open or patchless. Well, sometimes we could use this agent, Celesta as almost like a filler. It acts like a filler. We place it. It's in adults, it's done in an outpatient, in an office setting. Here we do it under anesthesia. Um, but it's used to sort of fill that gap to try to help with fecal incontinence. So it's primarily used in patients who have fecal incontinence, and we use it as a filler to help fill that canal to make it a little bit smaller. Um, it could be used in any age patient. We've done as 34 years old and older. Um, mixed results. It's not a perfect agent, but it has little side effect. There is a small risk of infection. Um, but otherwise, there's really very little side effect and could be of benefit. Um, do you think that it could help with The transfer from someone on enemas to laxatives to gain more potential. Good question. I think that's an individual question that you'd have to see what the anal canal looks like to see if it's a potential um area where it could be improved. Another thing that could help that depending again on the situation, we use sacral nerve stimulators to get patients who have tried to transfer from enemas to laxatives and have failed. Then sometimes we use sacral nerve stimulator or Celesta as a potential agent or therapy that might improve those chances of doing better with a laxative, uh, treatment algorithm. Um, to kind of go back to sacral nerve, um, do you think it can work for patients with spina bifida? Great question. There is some literature on that in, um, And some research that has been done, and the answer is it's mixed. There are certainly patients with spina bifida that have responded positively to sacral nerve stimulation, and there are others that haven't. So it's a mix, it can work. This is where it's important to know what the sacrum looks like and is it actually even possible to do the procedure. Again, like I mentioned earlier, we now have a new operating room that has 3D technology where we can do this in patients where previously we were not able to do this, but it still depends on the anatomy of the sacrum. And again, also the benefit of the two-stage procedure and where you could trial it, see if it works. And then determine if you want to use it long term or not. Um, so this is kind of a specific question. Then what options are available for children with deformed sacrums? So, again, I think if the sacrum is Able we get dedicated imaging of the sacrum to see if it's even possible. If it is possible, you have to have a 3rd sacral foramina or 3rd sacral opening where the 3rd sacral nerve comes out of the sacrum and innervates the pelvis. And so if that's there, then typically we're able to do it, especially with this new. Technology. If that's not there, then unfortunately, sacral nerve stimulation at this point is not a therapy available for those patients. So then I think talking about what is the therapy, and I think um having a deformed sacrum, there, there are lots of ranges of what that really means. And we have patients that have You know, on paper, it'll say deformed sacrum, but we still evaluate them to see what their potential is. So we have some patients that still have the ability to be continent with a, with a deformed sacrum at some degree. Um, if it's a patient who has a very Deformed sacrum is not a candidate for sacral nerve, then probably the best option is uh enema of some fashion, whether it's rectal or malone, saccostomy, peristen. I think at this point, that's the, the range of options. Um, I just wanna say we have less than 10 minutes left. You guys are giving us great questions. We have a table here full of note cards with questions on them, which are exciting to answer, and we'll do as many as we can in the next few minutes, but we will work on answering these and posting them on our Facebook page, like I mentioned earlier. What is the difference between Celesta and Botox? Uh, great question. So Botox is a muscle relaxant. People use Botox on their foreheads to get rid of wrinkles. It relaxes the muscle. So Botox in the anal canal relaxes the anal sphincter. So you typically use in patients with Hirschberg's disease that are known to have higher anal, uh, higher resting pressures of the anal canal. That's different than Celesta. Celesta is hyaluronic acid. It's a polymer. It's an, it's a filling agent. It, it's like a, a gel that fills the anal canal that if it's very wide open or, or patchless, it'll help close it a little bit. Very different, different acting agents. Um, this question is at what age can you identify when, when a child with Hirschprung's disease can be continent or not? And maybe we can comment specifically about Hirschsprung's and then kiddos with continence in general. As a mother of 4 children, I'm gonna leave that to you. So, um, I think in general, um, and this can vary, I feel like among cultures and location of where patients come from. We are a referring center and patients come from all over the country in the world. So I think this can vary depending on what is Um, typical in your culture. For us, um, it is typical that patients or, or children, um, with normal anatomy, potty train anywhere between 2.5 to 3.5, um, and sometimes even 4. in general, I think boys tend to potty train a little bit later than girls. And again, you also have to evaluate kind of the maturity of the child. Um, so, our bowel management program, when a parent says to us, when do we start? What we basically say to them is when you feel like your child needs to be in normal underwear. So we don't really put in a hard age on it. Um, so that's typically when the kiddo is ready to start preschool or kindergarten, and they are becoming worried that their school-age friends or their neighbor friends of their age are in underwear and they are not. Um, for some that is 3, and for some that's 4, and if the kid's at home and they're not engaged with a lot of play activity yet and they're not worried about it, it can even be a little bit later than 4. So it's really when you feel like they need to be in normal underwear. Um, and that also is based on maturity. So with that said, we have, we talked about kind of having two options. We either evaluate and figure out that they can be potty trained, um, with an oral regimen, or we feel like with the diagnosis and the testing that they do not have good potential and we might start off with enemas, um, right away. With that said, we may be on enemas for a couple of years and then come back and see if they can then come off of enema enemas and potty train later when they have more maturity and more buy-in, and that is dependent on their anatomy. Um, uh, you wanna talk specifically about Hirstprung. I think Hirschprung's, we, we, I think all patients can potty train around the same age. Sometimes with Hirschprung's disease, depending on what the anal canal looks after surgery and how things have developed, some patients we know will have a harder time with continence or control. And so when we uh referred a patient with Hirschpru's disease, typically we need to evaluate a few things. We need to evaluate. How the surgery was completed and what the anatomy of the anal canal and the entire colon looks like, and that's why we get a contrast enema. During the initial evaluation, and we also repeat a biopsy because sometimes when we do Poulter procedures for Hirschmann's disease, we rely on our pathologists tremendously doing this procedure and sometimes in the initial surgery doing a frozen section, it looks like all the nerve cells are there and look good, but later on in life, those nerve cells might. Not be as abundantly present, and we call that a transition zone. And so we have to do a biopsy to check to make sure there isn't a transition zone there that might be causing some of the symptoms. Um, I think it's also important to note that if a patient has the right anatomy, um, or the right condition that will allow them to be able to potty train, um, sometimes they are not. potty trained because their treatment is not adequate. Meaning a patient that has normal anatomy, uh, may be so severely constipated that they are leaking, uh, stool around and they do not have the ability to feel that. And so sometimes it's just getting them on the Adequate treatment to then allow them to potty train like they normally would. And some of the. So we'll kind of go to anorectal malformation for a minute or so. Um, one, I think this is a very great, great question. Can you explain to me what an anorectal malformation is? So an anorectal malformation is an abnormal development of where the colon or rectum ends up. Where does it land in development? And so there's a wide spectrum of anal rectum malformations both in male patients and female patients. And briefly, the anus can, or the rectum can land outside of the anal canal or the sphincter complex or the muscle complex, and it can land just on the skin just above that area. It could land in the area just outside the vagina in girls. It could land anywhere along the urinary tract in boys um with a fistula to the urinary tract and. Boys could be born with stool coming out of their urinary tract, and that's usually the initial diagnosis is made that way along with seeing no anus or opening in the anal canal, or it could be connected to the bladder and even more complex patients beyond that. And so, an anorectal malformation is a large spectrum of malformations that really need a good physical exam to help try to make the diagnosis. And then after that, sometimes you require imaging as well. Um, we just have a minute, so I know there are a lot of questions, um, and it went so fast, and there are a couple questions also, um, it's more of like resource questions that I think we can make sure we get some information, um, online for you about how can families connect with other families. And maybe some financial resources. Um, so we do, we do have some options and there are lots of options out there. So, um, but we can get those things, um, posted for you. And we work with a social worker in our center that also will help us answer those questions and get the right resources and, um, areas where we can help in those ways. So I think we are 60 minutes into this. I want to thank Global Cat and the team working very hard to, uh, that put us, put this production together. It was great. We're excited. I think we'll do many more of these in the future. I, this is my favorite. We do Globalcast to help and educate surgeons and doctors around the country and around the world. This is the first one we've done here for patients, by far my most favorite. So thank you. To the Globalcast team. I'd like to thank Rob Morales, our program manager who helped coordinate and um organize this event. Patty Kern, who's offscreen, who's Monica's partner in crime in the nursing unit in our center. She's the one who wrote hundreds and hundreds of questions on, on, uh, index cards for us to answer. Um, and I'd like to thank Cincinnati Children's in the Telehealth Center for helping put this together. Um, it's been a great event. I promise you two things. One, we will do more of these, and two, we will get these questions online and be able to, um, answer them over the next few days or weeks, um, because we literally have hundreds of questions here. I'm gonna post up one more time our contact information. Uh, you should be able to see it now coming up, I believe. Maybe. Oh, there, so it should be up now. Our contact information, you'll see it there. We'll post on our Facebook answers to these questions. If you wanna get in touch with us, please do. We're happy to help and, uh, thank you, Monica. Who really is the backbone of the program and helped put this all together as well, and helps take care of thousands of patients with, uh, dealing with these types of situations and is probably the most skilled and, uh, I hate to say this, senior nurse, uh, in colorectal in the United States, having done this for many, many years. So, on behalf of all of us at Cincinnati Children's and the Colorectal Center here, thank you and have a great evening. Goodbye.
