Specialist credits teamwork in treatment of inflammatory bowel disease

Host Amber Smith: Upstate Medical University in Syracuse New York invites you to be "The Informed Patient" with the podcast that features experts from Central New York's only academic medical center. I'm your host, Amber Smith. The remission rate for children with inflammatory bowel disease who are seen in the Karjoo Family Center for Pediatric Gastroenterology improved from 60% in 2013 to 87% in 2022. Here to explain how that happened is Dr. Prateek Wali, the director of the pediatric I B D program at the Upstate Golisano Children's Hospital. Welcome back to "The Informed Patient," Dr. Wali.

Prateek Wali, MD: Well, thank you for having me.

Host Amber Smith: A more than 20% improvement seems huge to me, but let's first start with a description of the inflammatory bowel diseases that you see in children.

Prateek Wali, MD: Inflammatory bowel disease is mainly two major disorders -- ulcerative colitis and Crohn's disease. Ulcerative colitis affects the colon and is more superficial, while Crohn's disease can involve any component of the GI tract and often affects the deeper layers of the gut. So it goes a little further into your muscle layers than ulcerative colitis does.

Compared to adults, children with inflammatory bowel disease tend to have more extensive disease and they tend to have aggressive progression, which means that they're sicker. This means that often they require more potent therapy. That's a good thing because kids need to grow, which is different than in adults. So if you get them into remission and feeling better, they also start growing again, and they don't miss those critical years of puberty. I think some of the typical symptoms that you'll typically see with inflammatory bowel disease are chronic diarrhea -- it can have blood or no blood --belly pain, with and without stooling.

Again, growth is such an important part of pediatrics. So weight loss, height stunting, delayed puberty. Other non GI symptoms are fever, joint pain, oral sores that can be sometimes seen with inflammatory bowel. So I think it's, a tricky thing at the beginning to put together this picture. The first step is to arrive at a diagnosis and figure out where the disease is, and we use different tools for that. Some of the tools are blood work, stool testing, endoscopy, which is a camera where we look at the upper part of the GI tract under anesthesia and a colonoscopy, which looks at the lower part of the GI tract with a camera under anesthesia.

Some of the newer things that we were going to talk about today are developments that have improved our ability to look at the small intestine, such as MRI technology and capsule endoscopy, which uses basically a camera that's in the shape of a pill to look at the small intestine.

Host Amber Smith: Do you see a lot of children with these diseases?

Prateek Wali, MD: Well, the peak incidence of inflammatory bowel disease is between the ages of 15 and 30. So if you think about it, you're going to see a lot of teenagers in that age group. We often see patients up to 19 to 21. The incidence of pediatric I B D is unfortunately increasing, around the world and especially in the United States, and the fastest rate of increase is children under 12, which is even more worrisome. There's probably about 50,000 children in the US currently with inflammatory bowel disease.

Host Amber Smith: What are the reasons for the increase in frequency, especially in the kids under 12?

Prateek Wali, MD: I don't think we know the answer to that question. I think there's an interplay between having predisposition genetically to our diets, our environment. You know, there's discussion about the microbiome and what the bacteria and viruses and fungi, what their effect in our GI tract is. And we're learning on a regular basis some of the things that might affect those protective layers we have in our intestine. And when those protective, layers are broken down, it puts us at risk for some of these inflammatory reactions to happen. And then once your body loses that tolerance, your immune system is unfortunately confused and starts to attack itself rather than the bacteria and the viruses and the fungi. So I don't think we have a clear understanding, but I think some of our more targeted therapy is looking at what might be, some of the reasoning behind it.

Host Amber Smith: So what does remission look like in inflammatory bowel diseases?

Prateek Wali, MD: That's a great question, but complicated.

There's clinical remission, which means you're feeling good. There's biochemical remission, which means your labs are looking better, your stool testing, which also looks at inflammation -- we have a specialized stool test called a calprotectin -- looks better. Over the last five to 10 years I think that gastroenterologists have pushed for what's called endoscopic remission, which means that not only are you feeling better and your labs are better, but we actually have healed the tissue. And so we're looking to see, have we healed the areas? And I think we're doing that more and more. We're looking to see how well patients are healed a year after they started therapy.

Along with that goes histologic remission, where we look at the biopsies under a microscope to see if they're better. The gold standard would be that on imaging, not only is the endoscopy better, but the finer layers of the gut are healed. That's called transmural remission. And the goal of all of this is that if you're in remission, not only feeling better but actually healed, that you prevent long-term complications, which we know is the natural history of the disorder.

Host Amber Smith: This is Upstate's "The Informed Patient" podcast with your host, Amber Smith. I'm talking with Dr. Prateek Wali. He's the director of the pediatric inflammatory bowel disease program at the Upstate Golisano Children's Hospital.

Let's talk about how you and your team have been able to improve the remission rates so much. What can you tell us about Improve Care Now?

Prateek Wali, MD: Improve Care Now is a really novel, collaborative community where clinicians, researchers, patients, parents, dieticians, social workers are empowered to learn and continuously improve the care of children with inflammatory bowel disease. It's dynamic I B D care, and I think that there are multiple things that are involved in this collaborative. But the key is to share. One of their key mottos is that they steal shamelessly and share seamlessly. And that's a really wonderful statement because we use resources from all these different centers, and they use our resources. It's a great way to provide care all around the country and actually internationally for this group of patients.

And it's become a really a model for what modern medicine should look like. It's also been awarded the Drucker Prize for quality improvement in medicine.

Host Amber Smith: So this is like a real time database?

Prateek Wali, MD: Yeah. You got it. One of the components is a realtime database. So when a patient comes to clinic, we consent them and ask them if they're willing to be in it, which I have to tell you it's hardly anybody that doesn't want to be in it, when they see the numbers of what it can provide. And we input their symptoms, their labs, their imaging, endoscopic details, their treatment into this database each time they actually come to the clinic.

And then we can actually in real time look at how that patient with that disease process and location and age is doing compared to a similar patient around the country or world with the same disease. And we can make fine changes that maybe will help us. And it gives us, like, almost like a report card for how we're doing as a center.

Host Amber Smith: So you might see how a fellow doctor is treating someone in another part of the world that's having some success, and it might give you an idea of something that might work with one of your patients?

Prateek Wali, MD: Yeah, exactly.

Host Amber Smith: Have any new discoveries about pediatric I B D come out of that database?

Prateek Wali, MD: Another component of the database or the collaborative is, if you can imagine with 30,000 children in the database, that we are able to have much better luck with looking at research. And one of the research studies that's recently been completed that we were a part of... We were one of 28 centers looking at whether methotrexate -- which is an immunosuppressive drug, would help one of the biologics, which is another treatment, and actually biologic therapy or biologic drugs are what have kind of revolutionized I B D care over the last 10 to 15 years -- whether that methotrexate would be helpful as an adjunct drug to these biologic therapies. And it just completed, and there were over 400 kids in the study, which is one of the largest pediatric I B D studies ever completed, and it had great results that we could really answer that question. And that's the kind of thing we're looking for is, if you have a really good question that can really change the care, you have a way of doing that because you have this collaborative.

Host Amber Smith: Interesting. Well, what can you tell us about the personalized approach that you use at Upstate? I'm curious, too, about the team members that are part of this.

Prateek Wali, MD: Yeah. Well, we have a wonderful team. So we try to meet every week to every two weeks to go over the patients that are going to be coming into our clinic, and we also go over the patients that might be ill, in other words, patients that have not been doing well recently. We call that care stratification. And, at these meetings, you have a physician lead, which is usually myself. We have an IBD nurse. We have an IBD nurse practitioner. Our IBD nurse practitioner is also in charge of infusion care, and so some of these biological medicines are given by IV so she coordinates that.

And actually one thing we've recognized over the last five to 10 years is that it's important to monitor the drug levels of these biological therapies so that you get the most optimal remission. And so she's also in charge of what's called therapeutic drug monitoring, or looking at these levels.

Another component obviously is growth and nutrition, so we have a pediatric dietician at our meetings. And we have a clinical research coordinator. That's who kind of pulls everything together for us to have these meetings. When we meet, we talk about what monitoring looks like in those patients that are coming in in the next two weeks, how they're doing, what their labs look like, what their levels look like, what their growth looks like.

But we also talk about preventative health. Are they taking their vitamin D? Have they gotten their eyes checked? Have they gotten their bone density? Have they gotten their vaccines, flu shot, Covid vaccines? And this is a great way to not miss any of their preventative health. Have they seen their pediatrician regularly? And then we go into patients that are maybe not doing so well -- that care stratification piece -- where we look at, can we suggest to the primary GI doctor ways that we could augment the care so that they have a better chance of getting into re mission.

Host Amber Smith: I understand your team has had success with a therapy for Crohn's disease in children. Can you tell us about that?

Prateek Wali, MD: We're really blessed to have a lot of therapies in inflammatory bowel disease at this current moment compared to 10 years ago. If you look at 15 to 20 years ago, it was pretty bleak as to what medical therapies you could provide, and a lot of patients went to surgery.

And now with all of the medical therapies we have, we really have the ability to look at each patient differently and say this patient with this disease in this location with this growth pattern would benefit from this drug better. Unfortunately, drugs have side effects. And one of the most common drugs that have side effects are oral steroids, and we try to avoid using them as much as we can. Unfortunately, oral steroids in the United States are often the drug of choice for what we call induction therapy, or getting you into feeling better. Because therapy is often divided into induction, which means you're going to get them better, which is usually about eight to 12 weeks. And then what's called maintenance therapy, or keeping them better.

And because these are chronic disorders, you have to be on some type of medication to keep that immune system quiet. So when I arrived in 2010, we started to use something called enteral therapy for Crohn's disease. Enteral therapy was something I learned in fellowship. And what it entails is using formula for 80% of your calories through the day, with 20% being either a snack or a small meal that is about 300 or 400 calories. And you do that for eight weeks. And it's quite challenging. If you think about eating as a very social activity. You sit down at the table with a family. And so this is difficult to explain and carry out to with families.

But what we found is that if you use enteral therapy for induction, it has the same rate of remission as steroids, about 80 to 85%, and the huge benefit is that you don't have the side effects of oral steroids. And on top of that, you have these wonderful nutritional recoveries. You know, these patients who have had malnutrition for quite some time now are feeling better, back to sports, back to school. And so we've had more than 30 kids do enteral therapy in this time period. And it's a wonderful alternative to oral steroids. And of course, you have to say that it's the standard of care outside the United States.

Host Amber Smith: Wow. So that has helped, also, improve the remission rates among your patient population, right?

Prateek Wali, MD: It absolutely has helped to improve the nutritional status of these patients dramatically compared to using oral steroids for induction.

Host Amber Smith: Now, you've also pioneered the use -- you mentioned it earlier -- capsule endoscopy. Can you describe what that is and when it's used?

Prateek Wali, MD: So when we use endoscopy, which is the camera under anesthesia, to take a look at your upper GI tract and lower GI tract, there's a large portion of the small intestine that we are not able to get to because your small intestine is very curvy. And therefore, we use other technologies.

And so we've been able to start two new programs at Upstate since 2010. One is called MR enterography, where we look at the small intestine using a specialized MRI (magnetic resonance imaging) that looks to see if you have inflammation or thickening in those areas in the small intestine, which is very important. And then if you have signs that there are areas that are inflamed or that are at least suspicious, then we can use something called capsule endoscopy, which sounds really interesting, from a technical aspect. But basically it's a pill cam. So it's a camera that looks like a pill, and it takes close to 50,000 pictures as it goes through your intestine. The kids, if they're older, can swallow it. If they're younger, we can place it endoscopically. And it basically brings all these pictures together into a movie, a video. It's a really long video, and it takes us about four hours to watch it all, but the advantage is we can see the entire small intestine, which we're not able to do during endoscopy. And those images can help us to, decide where the disease is located in the small intestine, and then that actually tailors your therapy plan as to what medicines would work better.

Host Amber Smith: I'm assuming that most of your patients are referred to you or the pediatric I B D program from their pediatrician. Can you walk us through what to expect at the first visit?

Prateek Wali, MD: That's a complex question because when pediatricians and family practice doctors and nurse practitioners send us patients, they suspect inflammatory bowel disease sometimes, but they're also just sending us patients for belly pain or blood in the stool and weight loss, without a clear understanding of whether that's the diagnosis. So at the first visit, often there's a discussion of a very good history and physical exam, blood work, sometimes stool testing, and then discussion about doing an endoscopy, so setting them up to do an endoscopy if we feel that this is suspicious for inflammatory bowel disease.

After we come to a diagnosis -- which may be Crohn's disease, which may be ulcerative colitis -- then we can discuss starting therapy. And we go over all of the different therapies that are available, and we tailor that therapy to the patient and that's complicated. It sometimes is the age of the patient, the accessibility of services, the side effect profiles of the different medications, the growth potential of the patient... and so there's a lot of things that go into what therapy we might choose, but it's a discussion with the family. And I think it really is great when we have that discussion because you get buy-in from the family because they choose what they feel like is also going to be the next best step for them.

Host Amber Smith: You're listening to Upstate's "The Informed Patient" podcast with your host, Amber Smith. I'm talking with Dr. Prateek Wali, the director of the pediatric inflammatory bowel disease program at the Upstate Golisano Children's Hospital.Is there any difference in an adult who comes in with new symptoms versus an adult who has had I B D since childhood?

Prateek Wali, MD: There have been studies that show that the T-cells do change over time. So that means that. If you've had the disease for a long time, you're not actually having the same reaction of T-cell inflammation that you had at the beginning, which means that your ability to benefit from therapy changes with the amount of time you've had it. One of the big things that we're seeing in kids, and I think this is where pediatrics has kind of led the field: we're much more aggressive with our therapies than adults are. So we often start with very potent therapies versus adults, which will often wait until you failed a few therapies -- or I guess the the therapies have failed you -- to go to a more potent target. The issue with that is that often, then, you've caused damage already, and often now you're repairing damage -- and they don't do as well.

And actually the big adult studies now show that what we call "biologically naive" patients do better with new drugs. So the newer drugs that are coming out, if they haven't had those other drugs before, they're actually doing better in getting into remission, which probably means there really is a different pattern that's developing, whether you've had it for one year versus five years versus 15 years.

Host Amber Smith: Is the pediatric version something that you would grow out of as you mature, or once you have it, do you have it for life?

Prateek Wali, MD: You have it for life. It's like a switch that's turned on. We haven't figured out how you're going to turn that switch off.

Host Amber Smith: I know everyone's always hopeful for a cure. I'm curious. What do you expect a cure might look like, and how close do you think scientists are?

Prateek Wali, MD: We always are pushing for a cure, and this disease is really devastating for these children when they're ill. And it really affects their daily life. And often we have children that really push through it, and they'll go to school not feeling well on a daily basis because they want to get back to their lives and their sports and their activities. A cure, I think, will look like personalized medicine. I think at some point in the future we'll have a profile of what type of inflammatory bowel disease a patient has. I think Crohn's disease and ulcerative colitis is a very gross generalized approach. I think that in the future it'll be, "you have inflammatory bowel disease, 10 or 9, and your genetic profile fits this, and the best medication for you will be A, B, or C." And I think that personalized approach will be based on looking at your genetics, but also what proteins are involved and how your pharmacokinetics. In other words, how do you as an individual break down drugs and use drugs, and what effect that has. And if we are able to find a way to identify the cause, then of course prevention would be the best cure for this disease.

Host Amber Smith: Do you think there'll be a day where there'll be a test to see whether you're at high risk for a bowel disease of some sort, and that there might be a way to prevent it from developing it all?

Prateek Wali, MD: Yeah, I mean, I think it's challenging because there's over 200 genes identified that may be involved in that predisposition for inflammatory bowel disease. So, I hope that at some point there will be an ability to say you are at a little bit of a higher risk, and these are risks that you can avoid so that you may not develop inflammatory bowel disease. And so therefore we don't have to worry about personalized medicine and how to treat it. We can just prevent it all together.

Host Amber Smith: Well, this has been very interesting and Dr. Wali, I appreciate you making time for this interview and congratulations on the big improvements.

Prateek Wali, MD: Oh, thank you. We have a wonderful team, and we have wonderful support from the community, and we appreciate all of the families that really help us on a daily basis to learn from this.

Host Amber Smith: My guest has been pediatric gastroenterologist, Dr. Prateek Wali. He directs the pediatric inflammatory bowel disease program at the Upstate Golisano Children's Hospital. "The Informed Patient" is a podcast covering health, science and medicine, brought to you by Upstate Medical University in Syracuse, New York, and produced by Jim Howe. Find our archive of previous episodes at Upstate.edu/Informed. This is your host, Amber Smith, thanking you for listening.