The early years: hirschsprung disease and health-related quality of life

Purpose Chronic diseases are notorious in the way that they interfere with many aspects of a child’s development, and this holds true for children with Hirschsprung disease (HD). The present research aims to (1) determine whether the health-related quality of life (HRQoL) of HD children differs from healthy paediatric populations; and (2) explore the relationship between HD children’s HRQoL and psychosocial outcomes of parents. Methods Using a cross-sectional survey study design, children’s HRQoL was assessed using the Pediatric Quality of Life Inventory (PedsQL), while parental psychosocial outcomes were measured using the Patient-Reported Outcomes Measurement Information System (PROMIS) anxiety and depression short-forms, Family Management Measure (FaMM), and Parent Experience of Child Illness. Surveys were administered over telephone to parents of 48 Australian children treated for HD (87.5% male, median age 4.5 years) during the period May to November 2021. Results While postoperative HRQoL of HD children was comparable to that of healthy age-matched controls, psychosocial quality of life of HD children was significantly poorer (mean difference = 3.40, CI [0.05, 6.76]). All parental outcome measures were significantly correlated with the PedsQL ( r = − 0.77–0.67, p < 0.05) in expected directions, with FaMM subscales (except parent mutuality) demonstrating the most variation ( R 2 = 0.41–0.59). Of note, 31.3% of parents reported moderate to severe symptoms of anxiety on the PROMIS. Conclusion Despite overall positive results for children, parents reported elevated symptoms of anxiety. This study highlights the importance of long-term follow-up care for HD patients and their families.