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.
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Acknowledgements
The researchers would like to thank caregivers who participated in this study and dietician colleagues and the NSPKU for their support with recruitment.
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Communicated by: Peter Burgard, PhD
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Synopsis
This study identified that for parents of children with phenylketonuria, parental psychological wellbeing was predicted by parenting stress and resilience, whereas treatment adherence was predicted by child age and perceived support from family.
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Emma Medford, Dougal Hare, Katie Carpenter, Stewart Rust, Simon Jones, and Anja Wittkowski declare that they have no conflict of interest.
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All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1975, as revised in 2000 (5). Informed consent was obtained from all participants for being included in the study.
Details of the Contributions of Individual Authors
Emma Medford contributed to identifying the research question, designing and planning the research, all aspects of the method section, and the majority of the writing of the article. She was the main researchers on this project.
Dougal Hare contributed to identifying the research question, designing and planning the research, and writing the article.
Katie Carpenter contributed to identifying the research question, deciding on the scope of the research and data collection.
Stewart Rust contributed to identifying the research question and designing and planning the research.
Simon Jones contributed to identifying the research question and designing and planning the research.
Anja Wittkowski contributed to the statistical analysis and writing the article. She oversaw the research process and the writing of the final manuscript.
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Medford, E., Hare, D.J., Carpenter, K., Rust, S., Jones, S., Wittkowski, A. (2017). Treatment Adherence and Psychological Wellbeing in Maternal Carers of Children with Phenylketonuria (PKU). In: Morava, E., Baumgartner, M., Patterson, M., Rahman, S., Zschocke, J., Peters, V. (eds) JIMD Reports, Volume 37. JIMD Reports, vol 37. Springer, Berlin, Heidelberg. https://doi.org/10.1007/8904_2017_23
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DOI: https://doi.org/10.1007/8904_2017_23
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