title: Treatment Adherence and Psychological Wellbeing in Maternal Carers of Children with Phenylketonuria (PKU).
abstract: Phenylketonuria (PKU), a rare metabolic disorder, causes cognitive impairment unless treated with a strict, protein-restricted diet, but few studies have examined the relationship between treatment compliance and parental wellbeing. In the present study, 46 primary caregivers of children with PKU completed measures of psychological distress, parenting stress (related to caring for a child with an illness), resilience, perceived social support and child dependency. Treatment adherence was assessed using the proportion of blood phenylalanine concentrations within target range in the preceding year. Results indicated that 59% of caregivers showed clinical levels of psychological distress, which was predicted by their parenting stress and resilience. Whilst the proportion of blood phenylalanine concentrations in range was not associated with parental distress, it was predicted by child age and caregiver's perceived support from family. Despite experiencing high levels of distress, the results indicated that caregivers' ability to adhere to treatment was not affected. Interventions to reduce parenting stress and boost caregiver resilience may have a positive effect on parental wellbeing. Additionally, interventions to promote treatment adherence benefit parents of older children, with a focus on promoting support from family members. Further research with larger sample sizes and longitudinal designs is needed to further establish causal mechanisms.
authors.affiliation: Division of Psychology and Mental Health, School of Health Sciences, University of Manchester, Manchester, UK.|Greater Manchester Mental Health NHS Foundation Trust, Prestwich, UK.
authors.forename: Emma
authors.initials: E
authors.lastname: Medford
authors.affiliation: School of Psychology, Cardiff University, Cardiff, UK.
authors.forename: Dougal Julian
authors.initials: DJ
authors.lastname: Hare
authors.affiliation: Division of Psychology and Mental Health, School of Health Sciences, University of Manchester, Manchester, UK.
authors.forename: Katie
authors.initials: K
authors.lastname: Carpenter
authors.affiliation: Paediatric Psychosocial Service, Royal Manchester Children's Hospital, Manchester, UK.
authors.forename: Stewart
authors.initials: S
authors.lastname: Rust
authors.affiliation: Central Manchester University Hospitals NHS Foundation Trust St Mary's Hospital, Manchester Centre for Genomic Medicine, Manchester, UK.
authors.forename: Simon
authors.initials: S
authors.lastname: Jones
authors.affiliation: Division of Psychology and Mental Health, School of Health Sciences, University of Manchester, Manchester, UK. Anja.Wittkowski@manchester.ac.uk.|Greater Manchester Mental Health NHS Foundation Trust, Prestwich, UK. Anja.Wittkowski@manchester.ac.uk.
authors.forename: Anja
authors.initials: A
authors.lastname: Wittkowski
keywords: Adherence; Children; Distress; Parent; Phenylketonuria; Wellbeing