Transitional Care In Patients With Hirschsprung Disease: Those Left Behind.

BACKGROUND: The long term effects of Hirschsprung disease are clinically variable. Improved understanding of challenges patients may face as adults can help inform transitional care management.

OBJECTIVE: To explore the outcomes and transitional care experiences in adult patients with Hirschsprung.

DESIGN: Cohort study.

SETTING: Single center.

PATIENTS: All patients treated for Hirschsprung 1977-2001 (aged >18 at time of survey July 2018-2019). Eligible patients were sent validated multi-domain surveys as well as qualitative questions regarding their transitional care.

MAIN OUTCOME MEASURES: Status of transitional care, bowel function and quality of life assessment. Qualitative analysis of transitional care experience.

RESULTS: Of 139 patients, 20 had received transition care (10 had at least 1 visit but had been discharged and 10 were receiving ongoing follow-up). These patients had inferior bowel function and quality of life scores at follow-up. Twenty-three (17%) patients had issues with soiling at time of discharge, 7 received transitional care. Of these, 9/23 (39%) had a normal bowel function score (≥17), 5/23 (22%) had a poor score (<12) and one patient had since had a stoma formation. Eighteen (13%) patients had active moderate-severe issues related to bowel function, only 5 had been transitioned, and just 2 remained under ongoing care. Importantly, when these patients were discharged from our pediatric center, at a median age of 14 (IQR 12-16) years, 10/17 had no perceptible bowel issues, suggesting a worsening of function after discharge.

LIMITATIONS: The retrospective design and reliance on clinical notes to gather information of discharge status as well as patient recall of events.

CONCLUSION: There remains a small but significant proportion of Hirschsprung patients for whom bowel function either remains or becomes a major burden. These results support a need to better stratify patients requiring transitional care, and ensure a clear route to care if their status changes after discharge. See Video Abstract.