Eosinophilic Esophagitis Speech-Language Pathologist s Role in Diagnosis and Management Stephanie J Olive, MS, CCC-SLP Richard J Noel, MD, PhD
Disclosure In the past 12 months, I have had no relevant financial relationships with the manufacturer(s) of any commercial product(s) and/or provider(s) of commercial services discussed in this CME activity.
Children s Hospital of Wisconsin Feeding and Swallowing Center Ellen Blank, MD Richard Noel, MD Joan Arvedson, PhD, CCC-SLP Amy Delaney, PhD, CCC-SLP Dorie Mendell, PhD, CCC-SLP Stephanie Olive, MS, CCC-SLP Mary Beth Feuling, MS, RD Catherine Karls, MS, RD Megan Van Hoorn, RD Andrea Begotka, PhD Elizabeth Fischer, PhD Beth Long, PsyD Alan Silverman, PhD Mary Jean Hubert, RN Charlene Rambadt-Hersh, RN Mary Witzlib, RN
Overview Background and history of eosinophilic esophagitis (EoE) Clinical Presentation and Demographics of EoE Diagnosis of EoE Treatment of EoE Natural History SLP Perspective
BACKGROUND AND HISTORY OF EOE
Eosinophils in the esophagus! Normal esophageal intraepithelial eosinophil count approaches zero Cadaver study Lowichik et al. (1996) Review of normal biopsies DeBrosse et al. (2006) Esophageal intraepithelial eosinophils associated with peptic esophagitis. Winter et al. (1982) Idea that high-level intraepithelial eosinophils may be something other than GERD proposed Attwood et al. (1993), Kelly et al. (1995)
Papillary lengthening Intraepithelial eosinophils, ± microabscess formation and degranulation Basal layer expansion
CLINICAL PRESENTATION AND DEMOGRAPHICS
Normal Esophagus
Population-based study Hamilton County, OH Pediatric population (0-19 years) 242,076 in 2000 U.S. Census Single pediatric GI and pathology providers 103 EoE cases diagnosed in years 2000-2003 (age 0-19 years) 71% Male Noel et al., NEJM 2004
Presenting Symptoms Vary by Age Noel et al., NEJM 351; 2004
Ethnicity and Geography of EoE Ethnic distribution of U.S. EoE study populations Ethnicity (U.S.) Spergel et al 1 Assa ad et al 2 White / Caucasian 90% 94.4% African-American 4% 4.5% Asian 3% 0% Canada Mexico United States Argentina Brazil Mexico Belgium Denmark England France Greece Italy Netherlands Spain Sweden 1 Spergel et al, JPGN, 2009 2 Assa ad et al, JACI, 2007 International distribution of reported EoE cases China Iran Israel Japan Turkey Australia New Zealand
Atopy in pediatric EoE study population Feature Percentage Rhinoconjunctivitis 57.4 Wheezing 36.8 Food allergy* 46 FH atopy 73.5 FH eosinophilic esophagitis 6.8 * H/O positive skin-prick, RAST, or clinical response Noel et al., NEJM 351; 2004
Atopy in EoE study populations Author / Population Cases Asthma Allergic rhinitis Atopic dermatitis Atopy in general population (U.S.) Spergel et al, Philadelphia Assa ad et al, Cincinnati Sugnanam et al, Australia Guajardo et al, WWW Registry 8.5% 25% 10% 620 50% 61% 21% 89 39% 30% 19% 45 66% 93% 55% 39 38% 64% 26%
Frequency of EoE in a single county 2000 2001 2002 2003 Cases 22 24 24 31 Incidence* 0.909 0.991 1.033 1.281 Prevalence* 0.991 1.983 3.016 4.296 Hamilton County, OH * per 10,000 population age 0-19 years Chi-square test for trend NS Noel et al., NEJM 351; 2004
Etiology of EoE Blanchard et al, JACI, 2006
DIAGNOSIS OF EOE
Consensus Statement 2007 systematic literature review and consensus recommendations defined EoE according to : Min. peak eosinophil density of 15 / HPF No evidence of GERD by either... Normal ph probe study Failure to resolve disease by high-dose PPI Furuta et al., Gastroenterology 133, 2007
Most current consensus statement Conceptual definition Eosinophilic esophagitis represents a chronic, immune/antigen-mediated esophageal disease characterized clinically by symptoms related to esophageal dysfunction and histologically by eosinophil-predominant inflammation. Chronic Immune/antigen-mediated Other... 15 eos per HPF...but allow for patients with <15 eos per HPF EoE, not EE No pathognomonic elements remains a clinicopathologic diagnosis Liacouras et al., JACI, 2011
When diet is wrong medicine is of no use. When diet is correct medicine is of no need. Ancient Ayurvedic Proverb He that takes medicine and neglects diet, wastes the skill of the physician. Chinese Proverb TREATMENT OF EOE
Elemental Diet Empiric Elimination Guided Elimination Topical Steroids Systemic Steroids Biologics Dilatation Mucosal Healing >95% 80% Variable, Up to 70% >65% 99%? 0% Cost, labor High High Low-Moderate Low Low High (?) High Side Effects (Psychosocial) High* High* Variable Low Moderate-High* Low (?) Low Side Effects (Medical) None Low* Low* Rare High? Moderate (?) Acceptance by sick patients High High High High High High (?) High Acceptance by well patients Low Low Moderate High Low?? *Depending on patient factors and on difficulty of request
Elemental Diet Empiric Elimination Guided Elimination Topical Steroids Systemic Steroids Biologics Dilatation Mucosal Healing >95% 80% Variable, Up to 70% >65% 99%? 0% Cost, labor High High Low-Moderate Low Low High (?) High Side Effects (Psychosocial) High* High* Variable Low Moderate-High* Low (?) Low Side Effects (Medical) None Low* Low* Rare High? Moderate (?) Acceptance by sick patients High High High High High High (?) High Acceptance by well patients Low Low Moderate High Low?? *Depending on patient factors and on difficulty of request
EE Diagnosis Comprehensive food allergy testing Discuss therapeutic options and begin plan of care Amino acid-based formula Unguided elimination diet Guided elimination diet Topical glucocorticoids Surveillance endoscopy Adapted from Putnam PE, Immunol All Clinics of NA 29; 2009
Persistent Inflammation Surveillance endoscopy Resolved Inflammation Remove foods toward elemental diet (guided or unguided) Add a medication Increase medication Add food in stepwise manner Attempt to reduce medication to maintenance dosing Do not make changes Compose long-term plan Adapted from Putnam PE, Immunol All Clinics of NA 29; 2009
NATURAL HISTORY
Presenting Symptoms Vary by Age Noel et al., NEJM 351; 2004
Presenting Symptoms Vary by Age Noel et al., NEJM 351; 2004
Friability of mucosa Straumann et al., Gastro. Endosc., 2003
IL-5 and role in tissue remodeling Mishra et al, Gastro, 2008
Kwiatek et al, Gastroenterology 2011
While distension of bag can cause esophageal contractions, abnormal plateau is maintained across periods of muscular contraction and quiescence. Defect appears to fix maximal distension without affecting ability to contract and relax Adults with EoE demonstrate decreased esophageal compliance when compared to normal controls Kwiatek et al, Gastroenterology 2011
SLP Perspective
Presenting Symptoms Vary by Age Noel et al., NEJM 351; 2004
Clinical presentation Feeding problem in an infant or toddler Feeding refusal / difficult feeder Difficult transition from breast feeding Vomiting or gagging Delayed advancement of diet interrupted motor skill development Restricted range of food accepted Haas & Maune, 2009
Clinical presentation Endorsement of other concerns Allergies Rashes/eczema Sleep Upper airway concerns Family history of feeding/swallowing problems Developmental differences
VFSS in EoE
Patient #1 11 year; 8 month old male Complaint of food getting stuck in throat with swallows worst with pills, bread products, burritos, and meat Asthma and possible seasonal allergies History of milk allergy, resolved Eczema Normally developing No feeding concerns until 5-6 years prior to VFSS Father also reports issues with food feeling stuck with swallows
Patient #2 2 year; 11 month old girl Difficulty with acceptance of age-appropriate solids Feeding issues since birth Low volume milk intake gradual increase with inconsistent vomit No issues with purees Solids needed to be very soft and finely diced gag and choke with solids Complaints of sore throat with and apart from eating Environmental allergies and intermittent asthma Sleep issues History of vomiting and constipation Eczema started 6 months prior to VFSS parents eliminated milk and issues resolved Normally developing
Patient #3 17 month old girl Successful acceptance of bottle; refused cup presentations Gag/vomit and refuse with textured purees and attempts at solids Eczema Hard bowel movements Delayed speech/expressive language extremely guarded about interactions with anyone other than her mother
VFSS findings Oral phase Normal Prolonged chew Delayed oral skills Piecemeal swallow Pharyngeal phase Normal Concerns related to upper airway obstruction Upper esophageal phase - screening pass down esophagus needed in this population Slow bolus movement in esophagus with or without retrograde movement Residue in esophagus Other findings Chin tuck or extra head movement at any point in swallow Patient report of discomfort or pain
SLP Treatment Occurs in consultation/collaboration with medical team Co-treatment or referral to other disciplines if indicated Delayed skills vs. disordered skills Positive exposure to safe/appropriate food considering: Diet restrictions Developmental level Skill levels Sensory characteristics of food (taste, temperature, texture, bite size, bite placement, etc ) Caregiver education Provide home programming to facilitate success Make recommendations that fit the family s goals
Case for discussion (1) 6 month-old girl seen by Birth-to-Three / Early Intervention speech language pathologist for feeding problems, including: Fussiness with bottle feeding Inefficiency with feeding Concerns for aspiration secondary to respiratory problems Born at term with a BW 3.8 kg but complicated course: Caesarian section Meconium aspiration ventilator and parenteral nutrition NICU for 2 months At 6 months of age, bottle feeding 30 ml each feed with pushing away and falling asleep; parents cut slit in nipple without any benefit. Frequent regurgitation.
Case for discussion (2) Frequent, round-the-clock feedings helped maintain healthy weight velocity. At 6 months of age, VFSS performed and was unremarkable. Referred to GI for evaluation: Focused on calorie-dense smooth foods together with SLP Used cyproheptadine to stimulate appetite Ultimately performed EGD and found changes consistent with reflux esophagitis. Despite efforts, feedings described as traumatic for both patient and parents. Force feeding was described.
What to do? Collaborate with social worker to facilitate access to care Consider different approach to therapies Parent:child interaction training, Sensory-based therapies, Occupational therapy Behavioral therapy Consider further developmental evaluation Consider further medical evaluation Interdisciplinary care Provided dietary guidance Continued skill acquisition with SLP Initiated behavioral management Sought further medical etiologies
Outcome: EGD performed and identified EoE Based on EoE diagnosis and behavioral concerns, a plan was made to place gastrostomy tube: Allows dietary treatment of EoE with maintenance of growth Allows a complete reestablishment of feeding with escalating goals and strong supervision of skill-acquisition and behaviors Eliminates parental stress, allowing focus on small positive gains Was able to add dietary elements, maintaining a dairy-free diet, but still had difficulty completely weaning off gastrostomy calories. Was able to wean fully off tube via intensive inpatient feeding program with SLP and psychologist co-treatment.
Conclusions EoE is a complex esophageal inflammatory disorder that can present with feeding problems in young children. An SLP may be the initial person to whom these children present for evaluation and management. If EoE suspected, early referral to GI is important. Resolution of inflammation does not guarantee that feeding issues will be resolved. Patients may require ongoing skill-based intervention or education regarding advancement of diet. Interventions may require collaboration with medical team, dietitians, and/or pediatric psychologist.-