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

Abstract

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. To explore the outcomes and transitional care experiences in adult patients with Hirschsprung. Cohort study. Single center. 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. Status of transitional care, bowel function and quality of life assessment. Qualitative analysis of transitional care experience. 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. The retrospective design and reliance on clinical notes to gather information of discharge status as well as patient recall of events. 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.