Transitional Care In Patients With Hirschsprung Disease: Those Left Behind

David S Thompson; Joseph R Davidson; Kathryne Ford; Stavros Loukogeorgakis; Simon Eaton; Simon Blackburn; Joseph Curry

doi:10.1097/DCR.0000000000003208

Transitional Care In Patients With Hirschsprung Disease: Those Left Behind

Dis Colon Rectum. 2024 Apr 23. doi: 10.1097/DCR.0000000000003208. Online ahead of print.

Authors

David S Thompson¹, Joseph R Davidson¹, Kathryne Ford¹, Stavros Loukogeorgakis¹, Simon Eaton², Simon Blackburn¹, Joseph Curry¹

Affiliations

¹ Neonatal and Paediatric Surgery, Great Ormond Street Hospital for Children, London, United Kingdom.
² Stem Cells and Regenerative Medicine Section, UCL-GOS Institute of Child Health, London, United Kingdom.

PMID: 38653495
DOI: 10.1097/DCR.0000000000003208

Abstract

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.