Gastrointestinal Eosinophil Diseases Program
New Multidisciplinary Center Links Children's Hospital Colorado and National Jewish Health
Glenn T. Furuta, MD
Director, Gastrointestinal Eosinophil Diseases Program
Associate Professor, Department of Pediatrics, Gastroenterology, Hepatology and Nutrition, University of Colorado Denver
Over the last decade, an increasing number of children and adults have received a diagnosis of eosinophilic gastrointestinal disease (EGID). This broad category of diseases includes eosinophilic esophagitis (EE), eosinophilic gastritis (EG), eosinophilic gastroenteritis (EGE) and eosinophilic colitis (EC) with the clinical diagnosis being based primarily on which organ contains increased numbers of mucosal eosinophils.
Children with EGID present with non-descript symptoms such as vomiting, regurgitation, feeding intolerance, abdominal pain, heartburn, diarrhea, bleeding and growth delay. With this constellation of commonplace symptoms, it is easy to see why there is confusion with other GI diseases and often a long delay in diagnosis. In fact, it is not unusual for there to be a four to six year delay in the diagnosis of EE.
Earlier last year, a collaborative program between Children's Hospital Colorado, National Jewish Health, and the University of Colorado School of Medicine was developed to care for patients with EGIDs and to initiate cutting-edge research to improve our understanding of this new group of diseases.
Demographics and Diagnosis – Recent Publication Established Diagnostic Criteria
To date, the most common EGID is eosinophilic esophagitis. A recent study from the Midwest suggests a prevalence of 1:10,000 children. EE is a disease that occurs primarily in boys and affects all races but formal statistics are still not available.
The diagnosis of EGID is made when an increased number of eosinophils are observed in the intestinal biopsies of patients with gastrointestinal diseases. EE is the most common, and most studied EGID, but until recently, diagnostic criteria were not available. In October 2007, as a result of an international collaboration initiated by the Gastrointestinal Eosinophil Diseases Program (GEDP), definitive diagnostic criteria were published. When the esophageal biopsy of a patient with upper gastrointestinal symptoms is found to have eosinophilia with greater than or equal to 15 eosinophils/high power field, and normal gastric and intestinal biopsies, and other causes of esophageal eosinophilia (such as gastroesophageal reflux) have been ruled out, one can safely make the diagnosis of EE. Since the number of eosinophils that populate the normal gastrointestinal tract is less clear and may vary according to season, geographic location and allergenic exposure, the exact diagnostic criteria for the other EGIDs are less clear.
Clinical Spectrum – Eosinophilic Esophagitis Often Confused with GERD
Younger children with EE present with non-specific symptoms, often confused with gastro esophageal reflux disease (GERD), that include regurgitation and vomiting, heartburn, abdominal pain and feeding refusal. Older children and adults complain of dysphagia and esophageal food impaction. In a previous study in which Dr. Glenn Furuta was the senior author, it was found that over half of patients presenting with food impaction to a private practice gastroenterologist ultimately were found to have EE. Thus, if a patient has GERD-like symptoms that are unresponsive to rigorous antacid blockade or surgical treatment of GERD, one should strongly consider EE. To date, the long-term outcome of EE is uncertain. A subset of children with EE may develop esophageal strictures. What defines this group of patients is not clear. Collaborative studies between the GEDP and other academic institutions will address these issues with natural history studies.
Symptoms suggestive of other EGIDs are non-specific and are representative of the part of the GI tract that is involved. Abdominal pain, diarrhea, bleeding, hypoalbuminemia and slow growth can all be seen in any of the EGIDs.
Treatments Include Corticosteroids and Nutritional Management
Present day medical management of EGIDs focuses on the use of corticosteroids. Both systemic and topical steroids bring prompt relief of symptoms and histopathology. Topical steroids are delivered with a metered dose inhaler (MDI) to the mouth. When patients spray an actuation from an MDI, they are instructed to not use a spacer, inhale, eat or drink for 30 minutes, nor rinse their mouth. This theoretically delivers a coating of steroid to the esophageal mucosa which has been shown in a number of studies to be effective treatment in most patients. Potential complications include Candida esophagitis and detrimental impact on growth. The latter effect is minimized by the first pass effect from hepatic metabolism that occurs because the medication is swallowed.
Nutritional management with the use of dietary elimination or an elemental diet is also effective treatment. This form of treatment includes the elimination of allergenic foods identified during skin testing. This form of treatment has also been shown by a number of studies to be effective. Potential side effects include non-compliance and nutritional insufficiencies when a dietitian does not monitor the diet.
The Gastrointestinal Eosinophil Diseases Program
The Gastrointestinal Eosinophil Diseases Program has both a clinical program and a research component. The Center’s clinical arm represents a joint multidisciplinary clinical program linking Children's Hospital Colorado and National Jewish Health and is directed by Glenn T. Furuta, MD from the Section of Pediatric Gastroenterology, Hepatology and Nutrition and Dan Atkins, MD from the Section of Pediatric Allergy and Pulmonary Medicine. The GEDP research program is primarily composed of a core group of basic and translational researchers (Glenn T. Furuta, MD; Sophie Fillon, PhD; Vince Mukkada, MD; Samantha Woodruff, MD; and Zachary Robinson, MA) from Children’s and clinical researchers (Dan Atkins, MD; David Fleischer, MD; Donald Y.M. Leung, MD, PhD; and Erwin W. Gelfand, MD) from National Jewish Health. The center is also a member of the Mucosal Inflammation Program at the University of Colorado Denver School of Medicine that is headed by Sean Colgan, PhD.
Together, this dedicated group of physicians and scientists are working together to understand the cause of EGIDs, to improve diagnostic techniques, develop novel treatments and identify the natural history for children, adolescents and young adults with EGIDs. The GEDP has already taken a national leadership role including the group’s successful international effort to establish diagnostic criteria for EE that was published in the October 2007 edition of the journal Gastroenterology and a recent edition of the Gastrointestinal Endoscopy Clinics of North America that focused solely on EE. A number of basic and clinical research studies have already been initiated at Children’s and National Jewish Health including those determining the impact of eosinophils on the intestinal epithelium, identifying the natural history of EE, seeking novel biomarkers and participating in novel treatment trials.
Since the fall of 2007, providers of the GEDP have been seeing patients at both Children's Hospital Colorado and National Jewish Health. This multidisciplinary program is composed of a pediatric gastroenterologist (Dr. Furuta), pediatric allergists (Drs. Atkins and Fleischer), feeding specialists (Angela Haas, MA, CCC-SLP and Nancy Maune, OTR), dietitians (Catherine Santangelo, MS, RD; Kristin Brown, MS, RD; and Emily McCloud, MS, RD), a social worker (Hallie Johannisson, MSW), nurses (Kathy Spomer, RN, BSN; Audra Swies-Gladysh, RN; and Roxann Rupp, RN, BSN), physician assistant (Stephanie Petersburg, PA) and program administrators (Felicia Timoteo and Allie Spain).
Each patient receives a comprehensive evaluation by designated members of the team. Further evaluations are then performed depending on the clinical circumstances. These tests may include upper endoscopy, colonoscopy, pH/impedance monitoring of the esophagus and allergy skin testing. After testing is completed, treatment options are discussed and instituted, with the expertise of dietitians and nursing staff. Close follow up and communication with the primary care provider are maintained to ensure proper growth and nutrition and to properly communicate the newest information available on this group of diseases.
The GEDP brings national expertise to Colorado and the Rocky Mountain region for this emerging set of disorders, and through its research programs, will offer novel therapies to improve the lives of children and adults with EGIDs.
For more information about the GEDP clinic, please call (720) 777-7457.