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Submitted
July 28, 2017
Accepted
October 18, 2017
Published
November 17, 2017
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Abstract

Background: The neuronal ceroid lipofuscinoses, collectively known as Batten disease, are rare pediatric neurodegenerative disorders resulting in blindness, progressive motor impairment, dementia, and premature death. This study identified services and support accessed by parents of children with Batten disease and assessed parental marital quality. Methods: Parents completed a survey to identify services their affected children received, and their own uses of social support, information, and online resources. We used the Dyadic Adjustment Scale to evaluate parental marital quality. Results: 29 parents of 23 Batten-affected children completed the survey. 18 children (78%) received receiving in-home nursing or paraprofessional care.  Over 90% of parents used the internet and social media to learn or talk about Batten disease; their preferred learning method was from other parents of children with Batten disease.  Batten parent marital quality was significantly lower than that reported in studies of the general population or parents of children with chronic, but non-neurodegenerative, disease. Conclusions: Parents expressed an interest in and preference for parent-to-parent communication for support and information about Batten disease. Further work is needed to understand factors associated with the significantly lower marital quality among Batten parents.

Keywords

Genetics and genetic disorders critically ill children family functioning

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Copyright (c) 2017 Elizabeth J Cozart, Erika F Augustine, Jonathan W Mink, Alyssa R Thatcher, Heather R Adams

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Author Biographies

Elizabeth J Cozart, University of Vermont Medical Center

Resident in Neurology

Erika F Augustine, University of Rochester Medical Center

Assistant Professor, Neurology and Pediatrics

Jonathan W Mink, University of Rochester Medical Center

Frederick A. Horner, MD Endowed Professor in Pediatric Neurology

Professor of Neurology, Neuroscience, and Pediatrics

Chief, Division of Child Neurology

Vice Chair, Department of Neurology

Alyssa R Thatcher, University of Rochester Medical Center

Department of Neurology

Heather R Adams, University of Rochester Medical Center

Associate Professor, Neurology and Pediatrics
How to Cite
Cozart, E. J., Augustine, E. F., Mink, J. W., Thatcher, A. R., & Adams, H. R. (2017). Batten disease and parents: marital quality, support, and communication. Journal of the International Child Neurology Association, 1(1). https://doi.org/10.17724/jicna.2017.114
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