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What’s So Special about Pediatric IBD ?
David Tuchman, MD Division of Pediatric Gastroenterology and Nutrition Herman and Walter Samuelson Children’s Hospital at Sinai CSGNA Fall Educational Program November 8, 2014
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Educational Objectives
Review the different types of IBD Discuss etiology of IBD Learn the differences between Pediatric IBD and Adult IBD Learn the effects of IBD on growth and development Discuss the effects of IBD on bone development Learn about the transition of care in IBD
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Inflammatory Bowel Disease (IBD)
IBD is an term for a group of diseases which Crohn’s disease and Ulcerative colitis Chronic, debilitating conditions Distinctly different diseases but are grouped together as IBD Produce similar signs and symptoms Intestinal inflammation, abdominal pain and diarrhea
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IBD – Type and Anatomic Distribution
Ulcerative Colitis Indeterminate Colitis Crohn’s Diseae
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IBD – Facts and Figures Health care costs: $1.7 billion dollars annually 1.4 million Americans have been diagnosed with IBD About 10% of these individuals are children and adolescents under the age of 17 years Peak age incidence is between 15 and 25 years
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IBD in Children Impact on children Incidence and prevalence
25% of IBD occurs in childhood Incidence and prevalence 1.4 million people in the US have IBD Crohns disease is diagnosed in 5000 children each year It is estimated that 50,000 – 100,000 children have IBD NASPGHAN 2nd Edition
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Burril Crohn, Leon Ginzburg and Gordon Oppenhiemer Regional ileitis
Burril Crohn, Leon Ginzburg and Gordon Oppenhiemer Regional ileitis. Journal of American Medical Association (October 1932) … to describe, in pathologic and clinical details, a disease of the terminal ileum, affecting mainly young adults, characterized by subacute or chronic necrotizing and cicatrizing inflammation. The ulceration of the mucosa is accompanied by a disproportionate connective tissue reaction of the remaining walls of the involved intestine… (which) leads to stenosis … with formation of multiple fistulas. Aufses, Surgical Clinics of North America, 2001; 81(1) Feb 2001.
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IBD Presentation Symptom/Sign Crohns Disease Ulcerative Colitis
Abdominal Pain ++ ++++ Diarrhea +++ Rectal bleeding Weight loss Growth Failure + Perianal disease Mouth ulcers Fever Erythema nodosum Anemia Arthritis
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Crohns Disease vs. Ulcerative Colitis
Any portion of GI tract Colon only Skip areas Continuous Rectal Sparing No rectal sparing Non-caseating granulomas No granulomas Transmural inflammation Mucosal inflammation Fistulae and abscesses Abscesses rare Stictures commom Strictures rare Ileum and cecum commonly involved Perianal disease
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IBD – Diagnostic Approach
Suspect diagnosis History (“red flags”), Family History Labs: Iron deficiency anemia, elevated ESR, CRP, low serum albumin Exclude other etiologies Stools studies Enteric pathogens, C. difficile, amebiasis, TB skin test Classify disease Crohns, UC Determine extent of disease – “stage” the disease Evaluate for extra-intestinal manifestations Evaluate growth and development
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Laboratory Studies in the Initial Evaluation for IBD
CBC with differential ESR/CRP Comprehensive Metabolic Panel Serum albumin Liver chemistries Stool studies Enteric pathogens Fecal calprotectin Stool for occult blood
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Imaging Studies Upper GI series and small bowel follow through
Abdominal and pelvic CT scan Magnetic Resonance Imaging
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Imaging Studies in IBD Abdominal CT Scan MR enterography
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Endoscopic appearance of normal terminal ileum and colon
Normal vascular pattern No friability Smooth and shiny Normal folds Smooth and shiny Villi seen Lymphoid follicles (Peyer’s patches)
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Endoscopic Appearance of Crohns Disease
Deep fissures Cobblestoning Segmental distribution Relative rectal sparing Terminal ileal involvement Granulomas on biopsy
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Endoscopic Appearance of Ulcerative Colitis
Loss of vascular pattern Granularity Exudates Diffuse continuous disease No ileal involvement
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IBD Histology
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IBD – Perianal Disease Perianal abscesses, fistulae and fissures
Perianal disease is noted in about 10 % of children with newly diagnosed Crohn’s disease 1 1 Keljo et al. Inflamm Bowel Dis. 2009;15 :
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IBD - Extraintestinal Manifestations Eye
UVEITIS EPISCLERITIS
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IBD - Extraintestinal Manifestations Skin
Erythema Nodosum Pyoderma Gangrenosum
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IBD - Extraintestinal Manifestations
Hepatobiliary Disease Oral Disease
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Inflammatory Bowel Disease
Etiology – Unknown IBD occurs in genetically susceptible individuals whose immune systems react abnormally to environmental agents in the gastrointestinal tract
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Pathogenesis of IBD - Multifactorial
Environment Mucosal Immune System IBD
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Nature Reviews Immunology 8, 458-466 (June 2008)
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IBD - Genetics High degree of concordance among monozygotic twins
Increased susceptibility to IBD among first- or second-degree relatives of affected individuals Linkage between IBD and several genomic regions Several mutations/single-nuceotide polymorphisms (SNPs) have been found to be associated with increased susceptibility to IBD Age of onset: younger the onset, more likely a family history of IBD Bousvaros et. Inflamm Bowel Dis 2006; 12(9),
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Pediatric IBD Severity of Disease Growth (Weight and height gain)
Sexual maturation Health Supervision Psycho-social well being Healthcare Provider Transitioning
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Children with IBD are not just small adults with IBD
Adolescents with IBD have more extensive involvement 69% of adolescents present with ileo-colonic disease vs. 28% of adults1 23% of adolescents with Crohn’s present with upper tract involvement – uncommon in adults1 Adolescents more likely to have ulcerative pancolitis compared to adults (67 % vs. 44%)1 Childhood-onset Crohn’s – more extensive involvement than than adult- onset Crohns (43% vs. 3%)2 1Goodhand et al. Inflammatory Bowel Disease 2010:16: 2 VanLimbergen et al. Gastroenterology 2008;135: Abraham and Kahn Gastro and Hepatol 2014;10:
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“Idiopathic “ IBD is rare in the young child
Pediatric Reference Cutoff Proportion of young children Gupta 2008 (n=989) 5 years 10% (Crohn’s only) Kugathasan 2003 (n=199) 10 years 20 % Cannioto 2007 (n=184) 2 years 9% Bousvaros Boston Children’s Hospital Symposium 2014
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IBD in Younger Children (< 5 years)
Chronic granulomatous disease Glycogen storage disease 1b Hermansky – Pudlak syndrome NEMO Wiskott-Aldrich syndrome IPEX Hyper IgM syndrome Common Variable Immune Deficiency CGD – primary immunodeficiency characterized by inability of cells to kill bacteria and fungi Autosomal or x linked Colitis peri anal disease gastric outlet obstruction GSD 1 b Fasting hypoglycemia, hepatomegaly, growth retardation, neutrophil dysfunction, GI complications ileitis colitis NEMO is nuclear factor kappa B essential modifier mutation – x linked ectodermal dysplasia, males, sparse teeth, alopecia, hyperhidrosis, crohns like colitis Therapy is stem cell transplant WAS – eczema, thrombocytopenia, recurrent infections IPEX – X linked immunodysregulation, polyendocrinopathy, enteropathy – watery or bloody diarrhea Bousvaros Boston Children’s Hospital Symposium 2014
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IBD in young children Immunodeficiencies frequently involve the gi tract and have IBD like symptoms Most idiopathic IBD in children under the age of 5 years involves the colon Caution using immunosuppression in patients with immune deficiency Optimal treatment is determined after multidisciplinary consultation Bousvaros Boston Children’s Hospital Symposium 2014
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Growth Failure Definition Higher incidence at diagnosis in CD vs. UC
Height < 5th percentile Decrease in height velocity below 5th percentile Fall off of the child’s growth curve Higher incidence at diagnosis in CD vs. UC Corticosteroids Inadequate calorie intake Malabsorption Increased energy expenditure from chronic inflammation – Pro-inflammatory cytokines, decreased IGF -1 Sawczenko et al Pediatrics 2006;118:124-9 Tigas et al J Pediatr Gastroenterol Nutr 1993;16:373-80 Kocoshis - Presention
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Growth Failure in IBD Patients Occurrence (%) Pediatric IBD 35
Prepubertal CD 60-85 Children with UC 6-12 Kirschner in Kirsner, ed. IBD 5th ed. 2000 NASPGHAN
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Growth Failure in Pediatric IBD
Suboptimal intake of calories Increased energy needs Malabsorption of nutrients Increased GI losses Malnutrition Growth Failure Corticosteroids Inflammation
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Growth Problems in Children with IBD
Increased cytokines act on Brain affecting appetite and calorie intake Hepatic expression of IGF 1 Act on chondrocytes of the growth plate of the long Growth hormone insensitiviy Sanderson Nature Reviews Gastroenterology & Hepatology 11, 601–610 (2014)
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Growth Velocity Curves
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Evaluating Growth
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IBD Treatment Goals Maximize therapeutic response Maximize adherence
Minimize toxicity Improve quality of life Promote physical growth and pubertal development Promote psychological growth Prevent disease complications NASPHGAN slide set
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IBD – Treatment Approach
Follow up appointment very soon after procedure Stress that IBD is not rare Famous people with IBD President Eisenhower Review the proposed etiologies State what IBD is not allergy, stress, diet What are the limits? None, except sky diving and bungee jumping Introduce the team Provide literature – CCFA, NASPGHAN
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Treatment of Crohn’s Disease
Mild to moderate CD Aminosalicylates Topical and oral Antibiotics Enteral feeds Corticosteroids Budesonide Prednisone Moderate to severe CD Enteral feeds (induction) Corticosteroids (induction) Budesonide vs. prednisone Immunomodulators (maintenance) 6-mercaptopurine Azathioprine Methotrexate Biologics (Induction and maintenance) Infliximab Adalimumab Certolizumab
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Aminosalicylates (Different formulations)
Aminosalicylic acid is a locally active, anti-inflammatory agent. In addition to oral delivery systems, this agent is commercially available as rectal suppositories and enemas, as well. There are several oral preparations of aminosalicylic acid. Each of these employs a different delivery mechanism to protect the active drug from intra-gastric degradation with release of the active agent at varying levels throughout the gastrointestinal tract.
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The use of aminosalicylates to maintain remission has also been an area of intense interest and varying results. Again, various sites of original disease activity, drug delivery systems and doses used has resulted in an array of findings. Camma performed a meta-analysis of controlled trials that looked at the use of aminosalicylates to maintain remission in patients who had undergone medical induction (as opposed to surgical resection). The findings of this meta-analysis were weakly positive in favor of treatment with aminosalicylates over control for Crohn’s maintenance therapy. References: Camma C, Cottone M. Mesalamine and relapse prevention in Crohn's disease. Gastroenterology 2000;119:597
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Crohn’s Disease – Antibiotic Therapy
Effect on luminal bacterial concentrations and subsequent down regulation of the local inflammatory response Selectively eliminate bacterial subsets Bacterial tissue invasion and microabscess formation Bacterial translocation and systemic dissemination Sartor; Gastroenterology 2004; 126:
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Enteral Nutrition – IBD
Improves nutrition for all IBD Effective therapy for pediatric Crohn’s UC – not shown to be effective 80-100% calories by formula NG tube vs. oral Proposed mechanism - Modulatoin of intestinal bacteria Baldassano and Kim
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IBD and Corticosteroid Therapy
Steroids are rarely used as monotherapy If clinical response to initial therapy is inadequate, add corticosteroids early Steroids are not maintenance drugs Many side effects including growth impairment But, use of steroids can “get you out of trouble quickly”
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Immunomodulators and IBD
6 MP, Azathioprine, Methotrexate Measure TPMT phenotype Closely monitor CBC, LFT’s Other adverse effects: Pancreatitis Increased risk of lymphoma Slight increased risk for EBV associated lymphoma Minimal if any risk of non-Hodgkin’s lymphoma
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Mercaptopurine in Maintaining Remission in CD
Markowitz, et al; Gastroenterology 2000: 119:
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Biologic Agents – Adverse Events
Infusion reactions Acute/delayed Rare complications HSTCL TB and increased risk of infection Lupus – like reaction Monitor PPD CXR Skin examnations Labs: CBC, LFT’s
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Immunomodulators and Biologics for Ulcerative Colitis
6 MP and AZA used to reduce steroid exposure Started soon after induction with other agents Not indicated for acute treatment of fulminant UC Role of biologics still being defined
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Immuno-compromised patient
No live virus vaccines Intra-nasal influenza MMR, OPV, Varicella Killed vaccines should be given accroding to recommended schedule Influenza Pneumococcus Hepatitis B Meningococcus HPV Melmed. Inflamm Bowel Dis 2009;15:
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Bone Health in children with IBD
Bone mineral density if often reduced in children with IBD Pathogenesis is multifactorial Decreased bone turnover more likely than increased resorption Vertebral compression fractures can occur Sylvester et al J Pediatr 2006; 148: NASPGHAN slide set
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Therapy for Decreased Bone Density
Control the underlying disease Optimize nutrition Calories/protein Calcium/Vitamin D Promote physical activity Consider referral to a specialist in bone health
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Nutritional Complications of IBD
Osteopenia and osteoporosis Anemia Micronutrient deficiencies Iron Folate B12 Zinc
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Depression and Anxiety in Children with IBD
25 -30% of children with IBD have symptoms of depression and/or anxiety 10-30% meet criteria for clinical depression or an anxiety disorder Predictors of depression Stressful life events; maternal depression Family dysfunction; steroid treatment; older age These rates are similar to children with other chronic illnesses Mackner et al . Inflamm Bowel Dis 2006;12:
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MESSAGE Acronym for Depression Screening
Mood (depressed or irritable) and Motor (hyper or hypo) E Energy (fatigue) S Sleep (insomnia or hypersomnia) Suicide or Self-Esteem A Anhedonia (lack of pleasure) G Guilt Eating (change of appetite) Rufo et al. NASPGHAN monograph - June
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Pediatric and Adult IBD - Transitions
Compared with adults, adolescents with IBD had Fewer clinic visits More documented non-compliance More active IBD on endoscopic evaluation Higher Crohn’s disease activity1 1 Bollegala et al. J Crohns Colitis 2013; 7:e55-e60
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Pediatric vs. Adult Healthcare
Pediatric Care Adult Care Family centered Multi-disciplinary Parent primary caregiver and decision-maker May ignore growing independence and increasingly adlt behavior Patient centered Single physician Acknowledges patient autonomy and independence May neglect family concerns
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Healthcare Provider Transitioning Checklist from Digestive Health for Life, CDHNF, NASPGHAN
Age Patient Health Care Team 12-14 Early Adolescence – New knowledge and responsibilities I can describe my GI condition I can name my medications, the amount and times I take them I can describe the common side effects of my medications I know my doctors and nurses names and roles Discuss the idea of visiting the office without parents or guardians in the future Encourage independence by performing part of the exam with the parents or guardians out of the examining room 14-17 Mid Adolescence – Building knowledge and practicing independence I know the names and purposes of the tests that are done I know what can trigger a flare of my disease I know my medical history Always focus on the patient instead of the parents or guardians when providing any explanations Allow the patientto select when the parent or guardian is in the room for the exam 17 + Late Adolescence – Taking Charge I can describe what medications I should not take because thety might interact with the medication I am taking for my health condition I carry insurance information (card) with me in my wallet/purse/backpack Remind patient and family that at age 18 the patient has the right to make his or her own health choices Develop specific plans for self-management outside the home (work/school)
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Resources and Tools for Successful Transition
Educational Resources for Providers Transition in IBD Transition Guidelines for Providers NASPGHAN guidelines (Baldassano et al J Pediatr Gastroenterol Nutr 2002;34: Transition Readiness Assessment and Tools Resources and Tools for Adolescents and Parents Crohns and Colitis Foundation of America (CCFA) Transition Advocacy and Support for Patients, Parents and Providers Society for Adolescent Health and Medicine Abraham and Kahn Gasto and Hepatol 2014:10:
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