Sabina Ali, MD, an expert in pediatric IBD, provides a short yet thorough rundown on this progressive disease, including very-early-onset IBD. She covers risk factors, diagnostic tools, confirmatory tests, referral tips, and preventive care to protect patients’ overall physical and psychosocial health.
Yeah so uh welcome everyone. Thank you for joining us during your lunch time. You know some few good things during uh you know these pandemic as we can do these zoom events which has been great to connect with everyone. Um So today I'm going to uh talk about uh pediatric inflammatory bowel disease and more kind of as for at a level of you know kind of when you see in a clinic and what uh things that make you more suspicious of. But this is possibly I. D. D. And even after the diagnosis how we um from G. I. To primary care connection how do we um kind of co manage these patients? Yeah. Okay so I have no disclosures related to this topic so we'll just cover briefly uh things about IBT when and who to refer. Understanding a little bit of the tools when we make a diagnosis. And how do you explain to your patients when sending them to the first gi appointment? What to anticipate primary health supervision for these patients? And then a little bit just talking about our IBD program. So I'll present as three scenarios of uh possibly how these participations present. Um I be can be very variable and presentation at times. Um So we have a 12 year old caucasian male presented to our E. R. With a six week history of worsening diarrhea. Uh With stool consistency is being loose, no bleeding report, no seen blood in the school reported to have a 7 to £8 weight loss during the six week period. Um There is a right lower quadrant tenderness on his exam and a low grade temperature. Um So of course the concern is is this possibly appendicitis on the er visit. But then there's a family history of Crohn's disease. Another scenario can be six year old female of Asian American decent with 4-5 month history of jointing. On further questioning she talks the mom talks about that. There is some McDonald cramping at times or foul smelling flatulence. Her appetite has gone down and also noted with big claws possibly due to the poor appetite. Looking at the joint, it's a single joint. Me and some ankle swelling along with pain on movement. Um Go ahead and do a peri anal exam. And there you notice uh these multiple carrying out skin tax. Um There is no family history of autoimmune diseases. There is only Hyperloop Idina reported in the family history. Then you have a 13 year old female with history of more constipation and abdominal pain which you have been following in your clinic has been prescribed me relax in the past. And this time and she returns to clinic a mom reports that there has been some abdominal pain recently which is not getting better with user may relax but then she is also known as this rash on her shin area which is new. It is non blanching bread and a little bit pal capable. Um And then you do a period of exam and there is a noted anal skin tag and you know if you remember from previous rashes, I mean this rash is possibly concerning for Ricky mendoza. So inflammatory bowel disease is a chronic disease. It is limiting and it primarily involves a G. A. Track but it also has some extra intestinal manifestations some patients may present and is to leave with the extra intestinal manifestations and not initially with the G. I. Manifestations. It is a systemic disease. So primary site of environment is your G. I. Tract. There are three big types uh Crohn's disease, ulcerative colitis. And then there is um one which we call ivy D. Unclassified. We do when we put a diagnosis. Um It is and it is called indeterminant colitis. Um So it is the one which is kind of in that gray zone of some features that are concerning for you see. But then there are other features and that patients who are possibly close disease. So that's when we have a difficult time to make a diagnosis or patient has not proven themselves which diagnosis they fall under. So they may be called IBD unclassified or indeterminant colitis. Um That is um the other newer discussion when the past several years of IBD which is very early onset and this is the IBD in six years or less patient. Um And then there is the infantile IBD which is in our patients who are less than two years of age. Talking about very early onset, it does quite behave differently than what we see on our adolescent and adult patient. We all said it's been a report of a rapid increase in incidents. Um And this type of IBD, the majority of the early early onset IBD has a mono genic type which is there is some kind of a genetic defect in the background. Um The disease is also primarily more steam which is restricted to the colon and so it is more of a colitis as a presentation. Um The thing to note is that these patients do not respond are resistant to our standard medication of um that we use an I. D. 25% of these patients, not only have I. D. D. But also have an underlying immunodeficiency. So we do have a very early onset IBD program which we call uh have with our immunology colleague that we see these patients in That is also a much stronger family history. Uh 40 50% almost. And there are reported um genetic mutations already reported in this population and some of them are important to know and diagnosed early because bone marrow transplantation center transplantation is the treatment so uh epidemiology wide. It's the prevalence is around 1.4 million americans, peak ages 15 to 30 years, 25% of IBD is diagnosed and during childhood and the mean age we see in the pediatric population is 11 to 13. And that has been an increasing incidents uh diagnosis um incidents is increasing worldwide. We are getting reports in the literature of increasing incidents in the developing countries now in china. It has been going up in India but it is still higher in the western world. The most important question we always come across in our clinics with uh with families is I've you know I have no have IBD and my anyone in my family and why I my childhood diabetes. So there's been a lot of studies looking at immigrant populations of patients who are second generations of first generations. Um And seeing diabetes that is also literature coming from Canada um that patients who are immigrants um to the western world we are seeing uh finding of IBD in that population. What are the causes? So it's a multifactorial um that can be a genetic predisposition. There are 200 genes identified. Do not do genetic testing at this time in every patient. Um as we don't know um yet that if there is a connection of genetic testing and medication options uh environmental causes have been looked at and you know there is a role of diet exposure to smoking whether it be passive or um smoking. Early use of antibiotics. Um uh latitude where the where the patient is in the so northern hemisphere that maybe more I. V. D. Vs. In sub Saharan african nation that is less report of I. V. D. Um that is underlying uh possibility of a particular barrier dysfunction or immune dis regulation. Um Also there is literature on microbiome patients with IBT versus non IBD. There are certain bacteria that we see higher in our IBD patients versus the ones who do not have IBD. It is a progressive disease and it's very important to know that and understand for the family and the patient because the more disease progresses it's increased risk of structuring fistulas and abscesses and surgery Because patients with all this come and say well I want to wait and not try any treatment yet. Or you know I'm doing well and I would like to stop my treatment. Um and those are important things to talk about at that time that this is a progressive disease. Even if you stop medication when you were doing well. The disease is a relapsing remitting conditions so it will return. It may take months but it can also take sometimes years. But the literature has shown that 45% of times the disease does re occur within a year. Uh So big difference between christians and you see just a quick overview. Crohn's is a patchy patchy inflammation. It also is transferred ural um colitis is uh is in continuous fashion. It involves the colon only in our own skin uh is present from anywhere to mouth to china. So patient presenting with oral ulcers or recurrent ulcers as the only finding operation of skin tags of Patiala as an initial finding symptoms can be variable. We have seen patients with complaints of this. Freesia, vomiting, diarrhea, blood in stool, abdominal pain, urgency or 10 isthmus cramping. Some patients we have also seen with complain of constipation but are losing weight or feeling of fullness or our eating and early satiety. Um So the big difference between Crohn's and also to collide, it is also is the depth of the disease uh alteration within the mucosa is seen and I'm sort of colitis. Crohn's disease is a trance mural inflammation. Um And that's the one big difference between the when it's how it involves the new culture. Mhm. And that also it's important to understand the complications we see in Crohn's disease as we see fistulas and structures and apps disease and Crohn's disease and not. And you see because of the uh with the condition of Crohn's disease, the inflammation is trans mural um and when that is transmitted inflammation that can develop a demon mucosal at the mucosal lining and then narrowing of the lumen which can lead to some structuring of fibrosis and then the and then um that is the development of fistulas can happen. The fistulas can be entra and Terek but the most commonly what we see are the peri anal fistula. And that's very important to do a period of examine your patient who complains who has a complaint of chronic abdominal pain of other features that are worrisome um that maybe for an inflammatory bowel disease conditions. So um when to refer a patient if you have a suspicion or are worried about um diagnostic delays. We have seen if the patient is not referred early on and there is a delay in diagnosis. What we have seen is medical therapies do become less effective at times. Or they were already may see a patient who already have a structuring uh happened um And now they're needing more surgery first and before starting medical therapy. Um There is increased risk of bowel obstruction. Um And then those patients with delay diagnosis, there is more report of needing surgery earlier on, more more commonly needing Ostuni. Um And even with the delay diagnosis that can be poor nutritional status and increased risk of surgical complications or increased risk of vascular complications. Um What possibly can be the reason for diagnostic delay for sometimes, you know, there are very a difficult symptoms. 44% of your patients may not have diarrhea, 45% may not have abdominal pain. And this has been looked at the literature that patients may have very variable symptoms, which can be confusing at times initially. Um Some patients may only have extra intestinal manifestations, almost 8% in the literature that patients have no reporter gi symptoms and that's how we see sometimes patients being referred to us from our rheumatology, colleagues because they were initially seen for joint manifestations and very subtle or possibly no gi symptoms or patients coming from our dermatology colleagues saying seen by them for psoriasis uh initially or other autoimmune conditions like hydrogen itis. And being a further evaluation of questioning. They have some settled G. I. Symptoms. Anemia was seen. Anemia is a very very common extra intestinal manifestations present can be present in 3% of patients without having over G. I. Symptoms. We've lost. Our growth failure can be an initial um some patients only present with petty criminal manifestation and not have uh complaint of diarrhea or even blood in stool initially. So it's very very important. I keep bringing up the mention of perino evaluation which I think it's very important to do when you are seeing patients with chronic abdominal pain. Or even though it's some of the times patients feel you know the kids maybe like why am I getting a periodical exam? It's uh it's embarrassing for them but it's also something to explain that it's important. Um Things that you may see on a periodical exam is hemorrhoids, fissures, Skin times or periods, fistulas are so large or large. Deep fissures. Um Skin tags can be small. Um A small skin tag at a certain location which we can sometimes see and sometimes skin tax may not always be abnormal. You know commonly skin tags are also seen and normally in which can be benign and can be seen sometimes in patients who are constipated. But the appearance of skin tag is important if you see a skin tag which are large and multiple and um in unusual locations. Um and that is important to keep in my mindful of that. These may not be the normal benign skin tags. Um You know, skin tags which looks like a large elephant ear size. That's not a normal skin tag. Uh huh. In those figures are also important in those figures. If they are deep or multiple, that can be also something to think about is an inflammatory condition as an underlying. Um Of course some patients can have a small fisher they have is to have constipation but they should also heal normally if you have a non healing figure or a deeper fissure. Uh keep in mind that may be an underlying inflammatory about disease, hemorrhoids are rare in kids. These are vascular religions. When you see hemorrhoids, think about constipation as a possibility of prostatitis, which is inflammation of the recto, signal rectal region or even considered. Think about total hypertension as a finding of a hemorrhoid. Um think about sending your patient to a surgeon. If you see um a fistula or anal skin tag because if this is a tax that is possibly due to a Crohn's legion and if it gets if it's referred to a surgeon and sometimes in situations it gets removed and it's an underlying inflammatory condition. These skin tags may lead to poor healing and can also develop fistulas or fecal incontinence. So having a skin tag is not an immediate referral to a surgeon. I think it's also important to keep in mind that skin tags or fistulas or these lesions can be an underlying inflammatory bowel disease. Yeah, growth failure is another important extra intestinal feature or manifestation of inflammatory bowel disease, which we see a much more commonly in our pediatric population with IBD, it's almost 35%. And Crohn's disease can be as high as 85% and you see it's less than Crohn's disease. You also can have a decrease in height velocity before patients starts complaining or have G. I. Symptoms. So, a kid who has started flattening on the height growth curve is another thing. Another indication to think about it. That is possibly an underlying inflammatory condition or something of a malabsorption state Up to 25% patient. We not even achieve adults high potential um even after their diagnosis and treatment. So it's important to make an earlier diagnosis. We are very mindful of using less steroids when possible, because we you know, we know that that can also person the impairment of growth. Um and um important to uh not also kind of make them get into a optimal terribly stage to get their potential. Uh we kind of mentioned the extreme manifestation as can be the only initial presentation before they have gi symptoms. So there are a lot of place organ system that IBD can get uh can be involved in skin can be almost 10% at times. Can be initial finding of psoriasis or eczema. Nardoza. Organdy unknowns. And as the initial presentation in your eye finding a patient with you be itis as florida's um from arthritis. Um delayed puberty um Iron deficiency anemia of B. 12 anemia. Patients with finding of transfeminine uh increased liver enzymes or trans emanates levels being up can be seen in with our autoimmune liver conditions that can be associated with IBT like autoimmune hepatitis patient with schools and cholangitis also are having association with IBD. So if you suspect diabetes uh you know look at the growth charts carefully. If you're patient seems like it's falling off the growth chart that is a flattening of the growth. Something to definitely keep in consideration what screening labs can you order initially? So start with a complete blood count to look at anemia thrombosis, psychosis. Trumbo psychosis can be due to an inflammatory responses. Um All versus anemia can also be to elevation of your platelet count. Um You do a metabolic panel not just look at the L. T. S. D. But also look at the albumin model patients with malabsorption or G. Ay manifestations can also develop dropping out doing levels um increase in elevated S. R. R. C. R. P. Are both. Um We have moved on a little bit more doing crp. S. In our I. D. D. Population but you also have to keep in mind that is a subset of the population and patients when both S. R. N. C. R. B. May appear normal. Uh And then uh stool infection testing if they are reporting of diarrhea. So stool culture or if there is a concern for c diff possibly with a recent antibiotic exposure. Uh Those are the labs that can be ordered. Fickle cal protecting its been used a lot. It's a great screening test. It's a stool inflammation marker and then can be done. The other one that is available is locked affair in which we commonly end up doing cow protecting because of it's stability uh To get to the lab. Different is a little more unstable. So the patient has to drop it to the lab earlier on and cannot be um you know stored in a refrigerator or fees are for a long period of time. So we tend to do more cal production but that is sometimes insurance um issues and laughter friend may be covered and tell production may not be covered by the insurance but if you have any of these concerns in any of these labs please give us a call or refer to the gi clinic talking about cal protecting. So it's a neutral Felix apostolic uh protein. It's shedding the stool. If that is new coastal information it's a great surrogate marker for endoscopic assessment of mucosal inflammation. It is pretty cost effective if you look at it comparing to patient needing you know, initial colonoscopy, endoscopy assessment doing a production first um and normal less than 50 value is an excellent non productive value and level less than that, it's pretty much saying that the patient may cause, it's the symptoms may not need to do uh inflammatory condition. Um There are a subset of patients. If they have primarily small bowel inflammation can protect, it may not be very effective in screening as it may appear normal or very low in that patient. So you have to look at the whole picture and if there is still a suspicion that your patient may have an inflammatory conditional malabsorption with with a really local production. I think that patients also needs a further assessment. Uh Other by using other modalities to look for an inflammatory bowel disease. Um there are some limitations um other than what else can increase your health protecting if the patient has been on nonsteroidal anti inflammatory drug whether they had a injury recently and they were put on pain medications with inset that can also drive your car protecting up recent infection of the gi tract can also increase your child protective. Also it's not a good data for using infants. Most of the literature that was looked at was in um Older patients are not less than 3-4 year old. So a lot of time protecting literature is still limiting on how to use it in a younger patients. Uh I see this sometimes is uh these being ordered. Its uh the IBD serology. Um There is no role of using IBT serology as an initial diagnostic evaluation for a child with suspected diabetes. There's a lot of false positives and there can be lots of false negatives. It's a very expensive test and that is um no use of ordering this if you're suspecting IBD um inefficient. Um So when you are sending a patient to the G. I. Clinic uh you know what to talk to your patient about is you know these are the labs that were concerning and the further evaluation is needed that your child may need. Uh an upper endoscopy, colonoscopy. The biopsies will be reviewed and what we see on the initial scope patients, we need other imaging studies like an MRI or a cT scan depending on what the findings are on the initial scope. And then some patient. A subset of patients may need further evaluation with the capsule endoscopy. If we already suspicious of only of a small value information. Um And our upper and lower endoscopy is our normal and we are still very uh worried about that. This patient has inflammatory values in those scenarios. Capsule endoscopy are uh used. The caveat the limitation of a capsule is that we don't we cannot obtain biopsies. But if we see findings that are suspicious of alteration or I. D. D. With these other labs and clinical symptoms. Uh We are HIV medicine cabinet has gone really big and large which has been really great for our patients. They have a lot more options. Uh We off we do that is too big categories. That is a non immune uh suppressive treatment options. And then there is the immune suppressive treatment options. And we sometimes combine both or we depending on what the patient will fit best. Um So non immune suppressive is the dietary therapies. That is the whole talk on its own. Um patients can have a lot a couple more options of trying dietary therapies more specific for Crohn's disease. So you can use formula diets or special diets like the names are specific carbohydrate diets or Crohn's disease exclusion diet. We have really good dieticians who are very well versed in our IBD clinic to help patients understand more about these diets if that's what their interests in uh is in as a part of their treatment. Um Then the other is um you know solicit late your missile amines or yourself facility. These are in the category of non immune suppressive medication. They are primarily useful colonic inflammation or alter the clients. I mean you're you're immune suppressive category are your steroids and then you're immuno modulators like methotrexate cyclosporin. Um to bring these are the names you may see um when you see your patients in your clinic who have I. D. D. And then you you have a long list of biologic therapies and the list is growing longer. Um Which is great. Uh It's inflexible mob or Remicade Humira or a dilemma mob uh interview or idealism. And some of them are infusions and they come to our infusion center um to get infusions. Some of them are injectable therapies with the patient are trained by our nurses and our team to how to give injections by the parents or the patient themselves at home. Mhm. Our experience with biologic we have approximately 50% of our IBD population on a biologic agent. It's very important to approach therapy and stratosphere y and treat the patient. Um Depending on what the extent of the disease is, what the fino type of the disease is, how severe the growth failure is. Um And how complicated the diseases. Um if there is a patient with severe growth failure and a very complicated disease, it's important to think about the biologic therapy early on and not wait longer with other medications. Um Oral medicines are non immune therapies. We have to infusion sites um uh currently in East Bay which is the main hospital at Oakland. And then we also have an infusion uh like in our wonder quick where we do infusions. And then we also do infusions at our UCSF SAn Francisco um in campus at the infusion center there. Uh we have patients who also receive infusions at outside infusion center which primarily sometimes it's based on what their insurance is uh dictating at times or um uh And if they are further away from our infusion tonight we have patients who live six hours away. So we do coordinate and find infusion centers for them as close as possible. Uh Important to know about vaccinations and what is the role of How do you put vaccinations in and uh kind of put that in the uh in their treatment cycles. So usually for biologic therapies we do check hepatitis B. Uh better seller tighter. Especially quantify on a tv testing of some sort before we start patients on biologic therapy. That is a standard of care. Um You may be may ask uh most of the time sometimes before biologic therapy check hepatitis B typewriters and they may appear to be non immune. And we may reach out to you think uh we checked hepatitis B surface antibody and patient appears to be non immune and they will need a repeat uh hepatitis B vaccination. Um uh 333 inductions. Um We also do very seller tigers. Just sometimes if if there is the flexibility of giving them a live vaccine before we can start immuno suppressive therapy, we need um ask them to get their varicella vaccination completed. But most of the times unfortunately there is no flexibility to do that because the patient maybe two second. We need to start immuno suppressive therapy early on it's just important to know if they have immunity to very sell or not. Um And for the family if that is an exposure then how do we manage that? Um The on immuno suppressive therapy, they can receive all uh passive non live vaccines. That is no contraindications for that. Um The when they are on immuno suppressive therapy so that will include steroids more than 20 mg per day. All the biologic therapies at the at this time and also in Iran or methotrexate. Uh They are not able to receive live vaccines while they're on it. Um There is some uh information on when can they be given live vaccines if they are in between uh the immuno suppressive and there is time in between. So for varicella if you if they have received a varicella vaccination and uh today they can they have to wait four weeks before they can start any immunosuppressive therapy for NMR, it's like six weeks. So there is some guidelines which should we follow for the vaccination. Other important thing is we monitor their labs very carefully because of monitoring the disease itself and also the side effects of medication bone health is something very important that we monitor for our patients because of the history of malabsorption state or if they have been exposed to steroids or their history of multiple fractures, their calcium and vitamin D. Intake. We do X. A scans uh if there is a concern of poor bone health. Um um in this patient population eye exams is something we do recommend for our patients for an annual ice screaming to keep an eye unless you know also someone who has been on a long tapers or multiple exposure to steroids. Um Skin exam is important for IBD related skin conditions like psoriasis or also to monitor malignancy um in this patient population. Um And cancer prevention is sunscreen, HPV vaccination, hepatitis B vaccination and colon cancer screening with surveillance colonoscopies at intervals which is needed. Uh The other important is assessing mental health with chronic diseases as we know these patients are concerns for anxiety or depression with coping with them. And uh and it's important to assess that and review that we do depression screening uh with the health of our social workers and an I. D. D. Program and IBD clinic. And then to appropriate referrals or assessment if they need therapy. Uh 20 to 30 25 to 30% of Children diabetes have symptoms of depression, anxiety. 30% do meet the clinical criteria of the pressure on anxiety disorders and which we understand is because of stressful life events. Schools, friends dr appointments, hospitalizations, surgeries coming to infusions. Missing important events in their lives. Sometimes with closer to a long periods of steroids can remove changes. A lot of them have sleep issues for quality of sleep complaint of the teak. Um And these grades are very similar in Children which we see in other chronic illnesses. So what is our goal of therapy is the biggest one is to induce remission, not even clinical remission but also mucosal healing which are endoscopy remission and earlier as earlier as possible. So we can get them growing normal psychosocial development, improve their quality of life with school activities and friends and of course minimize their side effects of medication and reduce the number of flares if possible. Our IBD program is includes both our SAn Francisco and our Oakland. We have multiple satellites where we see patients are uh It includes also are immunologist for our very early onset IBD patients. Uh We have infectious disease and our surgeons and our IBD program also have a dedicated nurse practitioners and social workers and dietitian. Sure. Uh every program it's a very multidisciplinary. We also have IBD clinical trials for our patients who are failing the standardized, you know, what's available at the approved and they can also enroll in clinical trials. We also have a young adult clinic where we help patients transition through this process and transfer to an adult colleague of ours. Um And then our program improves, our research coordinators and our advanced imaging uh with our radiology colleagues and our adults gi colleagues for uh cases that are complicated and need other interventional endoscopy studies and how to refer your patient to UCSF. It's the one it gets 28 77 you see child, you can call that number. The fax number for both san Francisco outpatient in Oakland are on the screen mm.