Alina Morawska1, Amy E Mitchell1, Evren Etel1, Grace Kirby1, James McGill2,3, David Coman2,3, Anita Inwood2,4. 1. Parenting and Family Support Centre, School of Psychology, The University of Queensland, Brisbane, Queensland, Australia. 2. Queensland Lifespan Metabolic Medicine Service, Queensland Children's Hospital, Brisbane, Queensland, Australia. 3. School of Medicine, The University of Queensland, Brisbane, Queensland, Australia. 4. School of Nursing and Social Work, The University of Queensland, Brisbane, Queensland, Australia.
Abstract
OBJECTIVE: This study aimed to assess the impact of phenylketonuria (PKU) and its treatment on parent and child health-related quality of life (HRQoL) and to identify the parenting-related correlates of parent and child HRQoL, as well as metabolic control. METHODS: Eighteen mothers of 2- to 12-year-old children with PKU participated and completed a series of self-report questionnaires including the PKU Impact and Treatment Quality of Life Questionnaire (PKU-QOL). RESULTS: Mothers reported that the most significant impact of PKU on HRQoL was in relation to the impact of their child's anxiety during blood tests on their own HRQoL and guilt related to poor adherence to dietary restrictions and supplementation regimens. Higher reported intensity of child emotional and behavioural difficulties and parenting stress were associated with higher scores for PKU symptoms on the PKU-QOL, higher scores for emotional, social, and overall impact of PKU, and higher scores for the impact of dietary restriction. Where mothers reported greater use of overreactivity as a parenting strategy, children tended to have better lifetime phenylalanine levels; however, the overall impact of PKU and the impact of supplement administration on mothers' HRQoL were worse for these families. CONCLUSIONS: These findings have implications for a holistic family-centred approach to the care of children with PKU and their families.
OBJECTIVE: This study aimed to assess the impact of phenylketonuria (PKU) and its treatment on parent and child health-related quality of life (HRQoL) and to identify the parenting-related correlates of parent and child HRQoL, as well as metabolic control. METHODS: Eighteen mothers of 2- to 12-year-old children with PKU participated and completed a series of self-report questionnaires including the PKU Impact and Treatment Quality of Life Questionnaire (PKU-QOL). RESULTS: Mothers reported that the most significant impact of PKU on HRQoL was in relation to the impact of their child's anxiety during blood tests on their own HRQoL and guilt related to poor adherence to dietary restrictions and supplementation regimens. Higher reported intensity of child emotional and behavioural difficulties and parenting stress were associated with higher scores for PKU symptoms on the PKU-QOL, higher scores for emotional, social, and overall impact of PKU, and higher scores for the impact of dietary restriction. Where mothers reported greater use of overreactivity as a parenting strategy, children tended to have better lifetime phenylalanine levels; however, the overall impact of PKU and the impact of supplement administration on mothers' HRQoL were worse for these families. CONCLUSIONS: These findings have implications for a holistic family-centred approach to the care of children with PKU and their families.