Hirschsprung-associated Enterocolitis Action Plan: A Pictographic Tool for Caregivers of Children With Hirschsprung Disease

Background: Hirschsprung-associated enterocolitis (HAEC) is a life-threatening complication of Hirschsprung disease (HD), with significant morbidity and mortality rates. Early diagnosis and treatment are critical, and caregivers play a key role in detecting HAEC, performing colonic irrigations, and seeking timely medical care. This study validates an HAEC action plan designed to assist caregivers in managing HAEC at home. Methods: A caregiver-focused HAEC Action Plan was developed following the National Institute of Health (NIH) 5-step “Clear & Simple” approach. It included patient-specific information, symptom pictographs, and a stepwise HAEC management protocol. Previously validated pictograms were used with the permission of the original institution. Additionally, 6 unvalidated pictograms were used. Readability was assessed using multiple formulas. Caregivers and the community evaluated the newly used pictograms' validity and action plan's comprehension, quality, and usefulness using the Consumer Information Rating Form (CIRF). Understandability and actionability were evaluated by librarians using the Patient Education Materials Assessment Tool. Clinicians validated its suitability using the modified Suitability Assessment of Materials (SAM) instrument. Results: The 6 new pictograms were validated. Readability was acceptably below the 6th-grade level. Overall CIRF score reached 91.5 % (superior). Understandability, actionability, and suitability were rated at 91 %, 91 %, and 82.5 %, respectively. Suggested improvements included using clearer pictograms, unit adjustments, additional details on irrigation duration, improved text visibility, and translation of the tool into other languages. Conclusions: The HAEC Action Plan is a clear, user-friendly tool for guiding caregivers in the early recognition and management of HAEC. Once employed in clinical practice, it has the potential to reduce HAEC-associated morbidity and mortality in children with HD. Level of Evidence: N/A.