Abstract
Phenylketonuria (PKU) is an inherited metabolic condition that can lead to the onset of intellectual disabilities if not strictly managed through a low-protein diet. Parents are responsible for supervising their child’s treatment for PKU, which may impact on their experience of distress. This cross-sectional study aimed to identify the factors that contribute to distress in parents who care for a child with PKU, distinct from parents in the general population. Thirty-eight parents of children and adolescents with PKU and 32 parents in the general population completed the questionnaires measuring parental psychological resilience, child behaviour problems, perceived social support and distress. Parents of children with PKU also completed measures of their child’s care dependency and behaviour related to developmental and intellectual disabilities. The findings revealed no statistically significant differences in distress between the groups, but parents of children with PKU reported more child behaviour problems. Multiple regression analysis identified that parental psychological resilience and child anxious behaviour explained 35% of the variance in distress for parents of children with PKU. By comparison, parental psychological resilience and generic child behaviour only accounted for 19% of the variance in distress for parents in the general population. This has implications for developing interventions in clinical settings that aim to reduce parents’ distress by enhancing their psychological resilience and supporting them to manage child behaviour difficulties, particularly anxious behaviour. Future research should include larger, more diverse samples and use longitudinal study designs.
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Communicated by: John Christodoulou, MB BS PhD FRACP FRCPA
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Synopsis
This research study identified that child anxious behaviour and parents’ psychological resilience explained 35% of the variance in parents’ distress in families affected by PKU, whereas child behaviour problems and parents’ resilience explained 19% of the variance for parents in the general population.
Details of the Contributions of Individual Authors
Dr. Olivia Ambler contributed to planning the research design, carrying out all aspects of the method and statistical analysis and writing the majority of the manuscript.
Dr. Emma Medford contributed to planning the research design and planning the method.
Dr. Dougal Julian Hare contributed to planning the research design, method, interpretation of the findings and preparation of manuscript.
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Dr. Dougal Julian Hare.
Conflict of Interest
The authors declare that there was no conflict of interest.
Details of Funding and Ethics Approval
This study was funded by Cardiff University as part of the South Wales Doctorate Programme in Clinical Psychology.
Ethical approval was obtained from Cardiff University School of Psychology Ethics committee (EC.16.07.12.4554A2).
Informed Consent
Informed consent was obtained from the participants as part of the remit of ethical approval from Cardiff University School of Psychology Ethics Committee (EC.16.07.12.4554A2).
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© 2018 Society for the Study of Inborn Errors of Metabolism (SSIEM)
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Ambler, O., Medford, E., Hare, D.J. (2018). Parenting a Child with Phenylketonuria: An Investigation into the Factors That Contribute to Parental Distress. In: Morava, E., Baumgartner, M., Patterson, M., Rahman, S., Zschocke, J., Peters, V. (eds) JIMD Reports, Volume 41. JIMD Reports, vol 41. Springer, Berlin, Heidelberg. https://doi.org/10.1007/8904_2018_105
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DOI: https://doi.org/10.1007/8904_2018_105
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