The early years: hirschsprung disease and health-related quality of life
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Updated: Jul 22, 2025
Diagnosis of Hirschsprung's Disease by Immunostaining Rectal Suction Biopsies for Calretinin, S100 Protein and Protein Gene Product 9.5
Published on: April 26, 2019
Fern Ee Caryn Koo1,2, Man Ching Esther Chan1, Sebastian K King2,3,4
1Melbourne Graduate School of Education, The University of Melbourne, Parkville, VIC, Australia.
Insights
Children with Hirschsprung disease (HD) have comparable health-related quality of life (HRQoL) to healthy peers, but poorer psychosocial quality of life. Parents of HD children experience significant anxiety, underscoring the need for comprehensive family support.
Area of Science:
- Pediatric Health
- Quality of Life Research
- Psychosocial Outcomes
Background:
- Hirschsprung disease (HD) significantly impacts child development.
- Understanding the health-related quality of life (HRQoL) in children with HD is crucial.
- Parental psychosocial well-being is closely linked to pediatric chronic conditions.
Purpose of the Study:
- To compare the HRQoL of children with HD to healthy pediatric populations.
- To investigate the association between children's HRQoL and parental psychosocial outcomes.
- To identify specific areas of concern for families managing HD.
Main Methods:
- Cross-sectional survey design.
- Utilized the Pediatric Quality of Life Inventory (PedsQL) for children's HRQoL.
- Employed PROMIS anxiety and depression, FaMM, and Parent Experience of Child Illness for parental outcomes.
Main Results:
- Children with HD showed comparable postoperative HRQoL to healthy controls.
- Psychosocial quality of life was significantly poorer in children with HD.
- Parental anxiety symptoms were reported by 31.3% of parents, with significant correlations between parental outcomes and child HRQoL.
Conclusions:
- While children's physical HRQoL is comparable, psychosocial aspects require attention.
- Elevated parental anxiety highlights the need for integrated family care.
- Long-term follow-up and support are essential for HD patients and their families.
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 (R2 = 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.
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