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Parent Coping and the Behavioural and Social Outcomes of Children Diagnosed with Inherited Metabolic Disorders

  • Research Report
  • Chapter
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JIMD Reports, Volume 31

Part of the book series: JIMD Reports ((JIMD,volume 31))

Abstract

Objective: To explore the level of coping and management of parents of children with inherited metabolic disorders (IMD) and the relationship with children’s cognitive, behavioural and social functioning.

Methods: Parents of children (n = 22) with confirmed IMD (glutaric aciduria type I, methylmalonic aciduria, propionic aciduria, isovaleric aciduria, glycogen storage disease, maple syrup urine disease, ornithine transcarbamylase or very long-chain acyl-CoA dehydrogenase deficiency) completed standardised questionnaires regarding psychological distress, coping and family management. Children completed cognitive assessments and parents rated their behavioural and social functioning on standardised questionnaires. Scores were compared with normative data.

Results: Most parents were coping well; 4/22 reported high levels of psychological distress. Exploratory analysis found that parent coping variables were correlated to the child’s internalising symptoms, whereas family management was related to children’s externalising behaviours and social skills. No relationship was found between parent variables and cognitive functioning.

Conclusions: Parental coping and family management impact on the child’s internalising symptoms and externalising behaviours, respectively. Early identification of issues in these domains may enhance referral for therapeutic interventions and family support programmes.

Competing interests: None declared

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Acknowledgements

This work was supported by the Victorian Government’s Operational Infrastructure Support Program.

Author information

Authors and Affiliations

  1. Murdoch Childrens Research Institute, Australian Centre for Child Neuropsychological Studies, Royal Children’s Hospital, Flemington Road, Parkville, Melbourne, VIC, 3052, Australia

    Amy Brown, Louise Crowe & Vicki Anderson

  2. Department of Paediatrics, The University of Melbourne, Melbourne, VIC, Australia

    Amy Brown, Avihu Boneh & Vicki Anderson

  3. Department of Psychology, Royal Children’s Hospital, Melbourne, VIC, Australia

    Louise Crowe & Vicki Anderson

  4. Metabolic Research, Murdoch Childrens Research Institute, Royal Childrens Hospital, Melbourne, VIC, Australia

    Avihu Boneh

Authors
  1. Amy Brown
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  2. Louise Crowe
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  3. Avihu Boneh
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  4. Vicki Anderson
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Corresponding author

Correspondence to Amy Brown .

Editor information

Editors and Affiliations

  1. Tulane University Medical School , New Orleans, Louisiana, USA

    Eva Morava

  2. Division of Metabolism and Children’s Research Centre, University Children's Hospital Zurich, Zurich, Switzerland

    Matthias Baumgartner

  3. Division of Child and Adolescent Neurology, Mayo Clinic, Rochester, Minnesota, USA

    Marc Patterson

  4. Clinical and Molecular Genetics Unit, UCL Institute of Child Health, London, United Kingdom

    Shamima Rahman

  5. Division of Human Genetics, Medical University Innsbruck, Innsbruck, Austria

    Johannes Zschocke

  6. Center for Child and Adolescent Medicine, Heidelberg University Hospital, Heidelberg, Germany

    Verena Peters

Additional information

Communicated by: Verena Peters

Appendices

Take-Home Message

Most parents report coping well with having a child with IMD. However, a small subset may not adapt as easily, potentially impacting on child behavioural outcomes.

Compliance with Ethics Guidelines

Conflict of Interest

Amy Brown, Louise Crowe, Avihu Boneh and Vicki Anderson declare no conflict of interest.

All procedures followed were in accordance with the ethical standards of responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1975, as advised in 2000 (5). Informed consent was obtained from all patients for being included in the study. Ethics approval was granted from the RCH Human Research Ethics Committee (HREC #32218A).

Details of the Contributions of Individual Authors

Amy Brown recruited patient subjects, performed neuropsychological assessments, devised a study protocol, collected data and wrote and edited the manuscript.

Louise Crowe participated in discussions about the manuscript and assisted in reviewing and editing the manuscript.

Avihu Boneh participated in devising a study protocol, discussions about the manuscript and reviewing and editing the manuscript.

Vicki Anderson participated in devising a study protocol, discussions about the manuscript and reviewing and editing the manuscript.

Details of Previous Publication

  • Abstract originating from this manuscript has been accepted for poster presentation at the SSIEM Annual Symposium, Lyon, France, September 2015.

  • Cognitive and behaviour scores of children with glutaric aciduria type I and very long-chain acyl-CoA dehydrogenase deficiency are reported in the following articles:

    • Brown A, Crowe L, Beauchamp MH, Anderson V, Boneh A (2014) Neurodevelopmental profiles of children with glutaric aciduria type I diagnosed by newborn screening: a follow-up case series. JIMD Reports 18:125–134

    • Brown A, Crowe L, Andresen BS, Anderson V, Boneh A (2014) Neurodevelopmental profiles of children with very long-chain acyl-CoA dehydrogenase deficiency diagnosed by newborn screening. Mol Genet Metab 113(4):278–282

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Brown, A., Crowe, L., Boneh, A., Anderson, V. (2016). Parent Coping and the Behavioural and Social Outcomes of Children Diagnosed with Inherited Metabolic Disorders. In: Morava, E., Baumgartner, M., Patterson, M., Rahman, S., Zschocke, J., Peters, V. (eds) JIMD Reports, Volume 31. JIMD Reports, vol 31. Springer, Berlin, Heidelberg. https://doi.org/10.1007/8904_2016_544

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  • DOI: https://doi.org/10.1007/8904_2016_544

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  • Print ISBN: 978-3-662-54118-0

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