Psychosocial Outcomes of Parents of Children with Hirschsprung Disease Beyond Early Childhood

Purpose: The lifelong impact of Hirschsprung disease (HD) upon children and their families is increasingly well recognized. Parental psychosocial wellbeing and family functioning are determinants of psychological and health -related outcomes in children with chronic conditions. We performed a crosssectional cohort study to evaluate the psychosocial functioning of parents/caregivers of children with HD, beyond early childhood. Methods: Parents/caregivers of children with HD, aged 4-14 years, managed at a tertiary pediatric surgical center were surveyed. Parent psychosocial outcomes, including adjustment to illness and family response, were assessed using four validated measures: Family Management Measure (FaMM); Parent Experience of Child Illness (PECI); Patient Reported Outcomes Measurement Information System (PROMIS R ) anxiety; and PROMIS R depression. The Pediatric Quality of Life Inventory (PedsQL) was administered to assess child quality of life (proxy -report). Results: Forty parents (mean age 38.7 +/- 5.6 years) of children with HD (mean age 8.0 +/- 2.5) participated. Parents expressed greater long-term uncertainty (PECI) and poorer perceived condition management ability (FaMM) than comparator chronic disease cohorts. Other scores for parental adjustment to their child's condition (PECI) and family response (FaMM) were comparable to reference cohorts. Symptoms of anxiety and depression were prevalent in our cohort (52.5 % and 42.5 % respectively); however, the proportion with moderate - severe PROMIS R anxiety ( c 2 = 2.50, p = 0.114) and depression ( c 2 = 0.156, p = 0.693) scores did not signi ficantly differ from the expected population distribution. Proxy -reported child quality of life (PedsQL) was signi ficantly reduced relative to healthy children ( p = 0.0003), but comparable to those with physical health problems with special healthcare needs ( p = 0.624). Conclusions: Parents of children with HD experience long-term uncertainty and have poorer perceived condition management ability than parents of children with other chronic childhood illnesses. This work highlights the importance of targeted parental education and support beyond primary surgical management, and provides a benchmark for this cohort, against which subsequent intervention -based studies may be assessed. Level of evidence: II. Crown Copyright (c) 2023 Published by Elsevier Inc. All rights reserved.