Generic and gastrointestinal health-related quality of life in children and adolescents with hirschsprung disease: a national cross-sectional study
Gustav Axelsson, Linnea Söderström, Johan Danielsson, Michaela Dellenmark, Matilda Bräutigam, Helena Borg, Pernilla Stenström, Anna Löf, Tomas WesterAbstract
Introduction
Persistent bowel dysfunction in Hirschsprung disease (HSCR) may impair health-related quality of life (HRQoL), yet data on the effects of disease burden on generic and disease-specific HRQoL are limited. This study evaluated generic and gastrointestinal HRQoL in children and adolescents with HSCR and explored risk factors for reduced HRQoL.
Methods
This national cross-sectional study recruited children and adolescents aged 8–18 years surgically treated for HSCR at pediatric surgery departments in Sweden between 2004 and 2018. Patients and parents completed the Pediatric Quality of Life Inventory Generic Core Scales version 4.0 (PedsQLv4.0), the PedsQL Gastrointestinal Symptoms Module (PedsQL GI), and the Rintala Bowel Function Score (BFS). Demographic and clinical data were obtained from medical records.
Results
Seventy patients were included; 31 were aged 8–12 years and 39 were aged 13–18 years (median age 13 years, IQR 11–16), and 16 had associated malformations or syndromes. Children with normal bowel function (BFS ≥17) reported higher PedsQLv4.0 and PedsQL GI scores than those with impaired bowel function in both child- and parent-reported outcomes (PedsQLv4.0: 93 versus 85, P = 0.010; 90 versus 80, P = 0.014; PedsQL GI: 90 versus 83, P = 0.002; 89 versus 79, P = 0.004). Parent- and child-reported scores showed strong correlations for PedsQLv4.0 (ρ = 0.79) and PedsQL GI (ρ = 0.86).
Discussion
Despite generally high HRQoL in patients with HSCR, impaired bowel function was associated with reduced generic and gastrointestinal HRQoL, highlighting the importance of long-term bowel function management and psychosocial support.