A Slice of Food Protein-Induced Enterocolitis Syndrome (FPIES): Insights From 441 Children With FPIES as Provided by Caregivers in the International FPIES Association

Abstract

To characterize the demographics, food triggers, risk factors, and management in children with food protein-induced enterocolitis syndrome (FPIES).In the study, researchers included information about 441 children from 403 families with caregiver-reported FPIES among members of the lay organization, the International Food Protein-Induced Enterocolitis Syndrome Association.This is a retrospective analysis of surveys completed at an FPIES educational conference (n = 42) and online (n = 368).Most children were female (51%), White (86%), and atopic (55%), and the median age was 2 years (range: 2 months to 19 years). Avoided food groups included grains (60%; primarily, oat [37%] and rice [34%]), cow’s milk (52%), vegetables (43%; most common: sweet potato [17%]), and fruits (38%). Avocado was the most commonly avoided fruit (13%) and was associated with banana avoidance (P < .001). The median number of food groups avoided was 3. Having a first-degree relative with FPIES was significantly associated with avoiding multiple food groups (P = .035). Caregiver-reported history of their child’s worst acute FPIES reaction included vomiting (94%) within 1 to 4 hours of ingestion, lethargy (91%), pallor (86%), decreased activity (76%), diarrhea (70%), hypotension (33%), and hypothermia (32%). Overall, 56% of reactions were evaluated in a hospital setting. Treatment most commonly included ondansetron (40%), intravenous hydration (36%), and steroids (16%).Grains were the most common food group avoided. Avocado avoidance was higher than expected and associated with banana avoidance. Avoiding multiple food groups was more common that previously reported, and having a first-degree relative with FPIES was associated with avoidance of multiple food groups.The cardinal feature of FPIES is vomiting that occurs ∼2 hours after ingestion of the food. As noted above, the reaction can include severe and multiple symptoms. This type of food allergy is not IgE-mediated, so skin-prick tests and serum food-specific IgE are typically negative. Many FPIES symptoms mimic viral infections or sepsis, and, indeed, the white blood cell count is often elevated with a “left shift.” This cohort from a lay organization may represent patients with a more severe presentation than typical, but the lessons are clear: suspect FPIES when the cardinal features are noted and no additional explanation arises, and do not be fooled if typically “innocent” foods like rice, oat, avocado, banana, or sweet potato are implicated!