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Eosinophilic Esophagitis Now, how do you say that? Presenters: Sean Jameson, BA Jennifer Jacob, RN, BSN Annette Ahrens, BA Cincinnati Center for Eosinophilic Disorders Division of Gastroenterology, Hepatology, and Nutrition and The Division of Allergy and Immunology

Eosinophilic Esophagitis: Now, how do you say that?

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Eosinophilic EsophagitisNow, how do you say that?

Presenters:Sean Jameson, BA

Jennifer Jacob, RN, BSNAnnette Ahrens, BA

Cincinnati Center for Eosinophilic DisordersDivision of Gastroenterology, Hepatology, and Nutrition

and The Division of Allergy and Immunology

Objectives

• Define Eosinophilic Esophagitis (EE)

• Recognize the psycho-social aspects of Eosinophilic Esophagitis in the pediatric population.

• Discuss research findings related to EE.

Magnet Forces

• Force 6: Quality Care

• Force 7: Quality Improvement

• Force 8: Consultation & Resources

• Force 13: Interprofessional Relationships

The Cincinnati Center for Eosinophilic Disorders (CCED)

The CCED is a:

Fast Growing

High Volume

Multidisciplinary Care Center

The mission of the CCED is to provide the best personal care for each patient and to learn from each patient through research.

LeadershipDirectorMarc Rothenberg M.D., Ph.DDirector, Division of Allergy and ImmunologyDirector, Cincinnati Center for Eosinophilic DisordersProfessor of Pediatrics

Medical DirectorPhilip Putnam, M.D.Associate Professor of PediatricsDivision of Gastroenterology, Hepatology and Nutrition

Allergy/Immunology Research CoordinatorsMarc Rothenberg, MD, PhD Bridget Buckmeier, BA, CCRPPablo Abonia, MD Annette Ahrens, BA Kimberly Risma, MD, PhD Maggie Palazzolo, MSSean Jameson, BABarb Hanlon, RNDeborah Seider, RN NutritionChristine Steinmetz, RN Mandy Clemons, RD, LD

PathologyMargaret Collins, MD Social Work

Maleshia Neugebauer, MSW, LSWENT

Alessandro D’Alarcon, MD Speech Pathology

Gastroenterology Drew Gerwin, MA-CCC/SLPJames Franciosi, MDPhilip Putnam, MDJennifer Jacob, RN, BSN PsychologyAisha Williams Wendi Lopez, PsyDTerryll Richardson, LPNBrandy Snyder, LPNAnn Weaver, RN

CCED Members

What We Do

Diagnose the disorder

Provide medical care

Conduct significant research about Eosinophilic Gastrointestinal Disorder (EGID)

Link patients to the latest treatments

How We Do It Coordinated care is critical to managing eosinophilic disorders. The C.C.E.D has members from eight divisions and has 35 clinical and research professionals. The center utilizes a number of ancillary services within the hospital during the process of treating patients.

The C.C.E.D Model (Research Driven Patient Centered Care)

Allergy

GI

Nutrition

PathologySocialwork

Psychology Speech Pathology

ENT

Week Long EvaluationCCED New Patient Evaluation Week:

Monday Tuesday Wednesday Thursday Friday

GI clinic visit X XEndoscopy/Colonoscopy XAllergy skinprick test XAllergy patch

test XSocial work

visit XEducation

Class XTreatment planpresented XDietary consult XPatch testreading XResearch X X X

EE at CCHMC

• Over 40 new diagnoses of EE from Hamilton County presented to GI clinic last year

• Over 100 additional patients referred to the CCED per year nationally

• The CCED treats over 300 patients each year

• The CCED performs greater than 25% of endoscopies at CCHMC

Patient Distribution through the CCED 2004 to 2007

The CCED Impact

The CCED was the first center in the country dedicated to research and treatment of eosinophilic disorders

Three other Centers now exist since the CCED’s conception following a similar model. ( CHOP, Denver Children’s, San Diego Children’s)

EE in the Literature

01020304050607080

1998 2001 2004 2007

Number of Articles

CCED Publications

FIGERSFirst International Gastrointestinal Eosinophil Research Symposium

Eosinophilic Esophagitis (EE)

Who has Eosinophilic Esophagitis?

• 70% are males

• Predominantly Caucasian

• Personal/family history of allergic conditions

• Familial tendency

What is an Eosinophil?

• Type of white blood cell produced in the bone

• Serves as a protection against infections

• Found in the GI tract, spleen, thymus and lymph nodes

• Within the GI tract eosinophils are found in the lining of the– Stomach– Small intestine– Cecum– Colon

Definition of Eosinophilic Esophagitis

Clinico-pathologic disorder

– Histologic changes

– Clinical symptoms

**Present with adequate proton pump inhibitor (PPI) treatment**

Esophagogastroduodenoscopy (EGD)

Histologic Changes

• Greater than 15 eos/hpf• Basal cell hyperplasia• Thickened epithelium• Fibrotic lamina propria• Long papillae

Abnormal

Normal

Eosinophilic infiltration

EE

White exudate

Basal layer hyperplasia

Degranulation

Endoscopic Appearance Normal Esophagus

Endoscopic Appearance Abnormal Esophagus

Furrows Rings

Exudate

Normal

But…

Abnormal histology

Clinical SymptomsPain

Heartburn / refluxSubsternal pressureEpigastric pain

Vomiting

Associated with specific foodsNon-specific

Dysphagia

Feeding disordersDifficulty getting food downFood impaction

Clinical Symptoms

Noel, et al, 2004

Associated Conditions

• Eczema• Chronic rhinitis• Food allergies• Reactive airway problems• Developmental delays• Failure to thrive

So, what now?

Types of EE

Allergic• responds to elimination of foods• reoccurs when offending foods are put back into

diet.

Non-allergic• no response to food elimination

Treatment Allergic type

Elemental diet—amino acid-based, hypo-allergenic formula– Elecare– Neocate

Elimination diet– Directed– “Six Food” ( Milk, egg, wheat, soy, seafood, peanut)

Food Trials

If biopsy confirms resolution of esophagitisReintroduce dietary antigens• 1 new food every 2 weeks• 1-4 new foods between EGDs

EGD every 2-3 months• Until there is a nutritionally adequate diet acceptable to the

family and child

**Recurrent esophagitis requires withdrawal of the recently added foods**

Non- Allergic Treatment Pharmacotherapy

SteroidsTopical

Fluticasone (Flovent)Budesonide (Pulmicort)

SystemicPrednisone

BiologicAnti-IL5 ( Monoclonal antibody)

Immodulators6MP

Treatment flow chart

Complications of EE

• Food impactions

• Strictures

Psycho-Social Issues

Financial• Foods• Formula• Frequent surgeries• Travel (hotel, meals,

airfare)• Work/ school absence

Social• Speech and feeding• Poor adjustment• Self image• School interactions • Meal times

Nursing Considerations

• EE is a REAL disease

• EE is a chronic disease

• Off label use of medications

• Children often have food restrictions

• Parents under stress

• Parents advocates for children

Support for Families

• “The Eddy project”

• Family education day

• Family Education and Enrichment for Eosinophilic Disorders

Support for Families• The American Partnership for Eosinophilic

Disorders (APFED)www.apfed.org

• Campaign Urging Research for Eosinophilic Disorders (CURED)www.curedfoundation.org

• The Cincinnati Center for Eosinophilic Disorderswww.cincinnatichildrens.org/cced

Research

How it works What we find How it helps

Research Process

Clinical Visit

Clinical Research

Coordinator

Lab Personnel

Patient Participation in Research

The quality of DNA extracted from saliva is comparable to DNA from blood!

Patient

Blood&

Tissue Biopsy

Questionnaires&

AssessmentsSaliva DNAfrom Parents

Our Studies 19 Active Eosinophilic Studies

Live sample• Quality of Life and Treatment Adherence • Longitudinal Sample Collection of blood, tissue, & saliva

Drug• Flovent Dosing • Anti-IL 5

• Anti-IL 13 (coming soon)

Data• EE Long Term Follow-up • Prevalence and Incidence of EE

• EE and Celiac (coming soon)

Findings

We have:

• Identified 500 specific genes increased in EE. (Biopsy)

• Identified Eotaxin 3, a key gene in EE. (Biopsy & Blood)

• Implemented Anti-IL 5 in patients with EGID

• Created several EE mouse models for experimentation.

The EE transcript signature

EE NL

Eotaxin-3 is the number one gene over expressed

EE Normal

Difficulty swallowing

Reflux

Difficulty swallowing & frequent dilatations

Eosinophilic Esophagitis (EE)

Real EE Patient Pedigree

Recruitment Data of EE Patients From 2005-Current

203

337

6015

Adult Tissue & Blood (UC Affiliate)

Clinical SymptomScore

Pediatric Tissue &Blood

DNA, Saliva Study

The Impact of Research

• Application of Flovent for treatment

• Positive outcome from utilization of Anti-IL 5

• Understanding the difference in quality of life

• Familial inheritance identified, 1 in 1,000 individuals have EE

How we make it happen

Research Staff (27)11 Research Assistants3 Clinical Research Coordinators6 Post-Docs5 Graduate Students2 Visiting Research Fellows

Representation of 11 Countries in our LabIsrael Chile AustraliaEgypt Japan IndiaCroatia Spain FranceKazekstan China