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

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Abstract

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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Acknowledgements

The authors would like to acknowledge Dr Penny Hartmann, Research Officer at Murdoch Children’s Research Institute, for administrative, database, and statistical support. We also acknowledge A/Prof Susan Donath, Senior Consultant Statistician at the Clinical Epidemiology and Biostatistics Unit at Murdoch Children’s Research Institute, for statistical advice. We also thank the families who generously provided their time to complete the survey.

Funding

This study did not receive any specific grant from funding agencies in the public, commercial or not-for-profit sectors. Professor Sebastian King’s position as Academic Paediatric Surgeon is possible due to a generous grant from The Royal Children’s Hospital Foundation.

Author information

Authors and Affiliations

  1. Melbourne Graduate School of Education, The University of Melbourne, Parkville, VIC, Australia

    Fern Ee Caryn Koo & Man Ching Esther Chan

  2. Clinical Sciences, Murdoch Children’s Research Institute, 50 Flemington Road, Parkville, VIC, Australia

    Fern Ee Caryn Koo, Sebastian K. King & Misel Trajanovska

  3. Department of Paediatrics, The University of Melbourne, Parkville, VIC, Australia

    Sebastian K. King & Misel Trajanovska

  4. Department of Paediatric Surgery, The Royal Children’s Hospital, Parkville, VIC, Australia

    Sebastian K. King

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  1. Fern Ee Caryn Koo
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Contributions

Conceptualisation of the initial project idea, data collection, analysis, and interpretation were performed by FECK. MT sourced the data collection instruments and contributed to data analysis and interpretation. This study is part of a larger longitudinal project, which has the aim of determining long-term quality of life outcomes for children with anorectal malformations and Hirschsprung disease, as well as assessing parent wellbeing and impact of illness for families with a child born with anorectal malformations and Hirschsprung disease. The larger project was conceptualised by SKK. All authors contributed to the overall conception and design of the study. The first draft of the manuscript was written by FECK and revised by MT, SKK, and MCEC. All authors contributed to revisions and approved the final manuscript.

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Correspondence to Misel Trajanovska.

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Koo, F.E.C., Chan, M.C.E., King, S.K. et al. The early years: hirschsprung disease and health-related quality of life. Qual Life Res 32, 3327–3337 (2023). https://doi.org/10.1007/s11136-023-03482-2

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