IDEAS home Printed from https://ideas.repec.org/a/gam/jijerp/v19y2022i10p5895-d814235.html
   My bibliography  Save this article

Towards Understanding Behaviour and Emotions of Children with CLN3 Disease (Batten Disease): Patterns, Problems and Support for Child and Family

Author

Listed:
  • Aline K. Honingh

    (Faculty of Behavioural and Movement Science, Vrije Universiteit Amsterdam, 1081 BT Amsterdam, The Netherlands
    These authors contributed equally to this work.)

  • Yvonne L. Kruithof

    (Special Education Visually Impaired Children, Bartiméus, 3703 AJ Zeist, The Netherlands
    These authors contributed equally to this work.)

  • Willemijn F. E. Kuper

    (Department of Metabolic Diseases, Wilhelmina Children’s Hospital, University Medical Centre Utrecht, Utrecht University, 3508 AB Utrecht, The Netherlands)

  • Peter M. van Hasselt

    (Department of Metabolic Diseases, Wilhelmina Children’s Hospital, University Medical Centre Utrecht, Utrecht University, 3508 AB Utrecht, The Netherlands)

  • Paula S. Sterkenburg

    (Faculty of Behavioural and Movement Science, Vrije Universiteit Amsterdam, 1081 BT Amsterdam, The Netherlands
    Special Education Visually Impaired Children, Bartiméus, 3703 AJ Zeist, The Netherlands)

Abstract

The juvenile variant of Neuronal Ceroid Lipofuscinosis (CLN3 disease/Batten disease) is a rare progressive brain disease in children and young adults, characterized by vision loss, decline in cognitive and motor capacities and epilepsy. Children with CLN3 disease often show disturbed behaviour and emotions. The aim of this study is to gain a better understanding of the behaviour and emotions of children with CLN3 disease and to examine the support that the children and their parents are receiving. A combination of qualitative and quantitative analysis was used to analyse patient files and parent interviews. Using a framework analysis approach a codebook was developed, the sources were coded and the data were analysed. The analysis resulted in overviews of (1) typical behaviour and emotions of children as a consequence of CLN3 disease, (2) the support children with CLN3 disease receive, (3) the support parents of these children receive, and (4) the problems these parents face. For a few children their visual, physical or cognitive deterioration was found to lead to specific emotions and behaviour. The quantitative analysis showed that anxiety was reported for all children. The presented overviews on support contain tacit knowledge of health care professionals that has been made explicit by this study. The overviews may provide a lead to adaptable support-modules for children with CLN3 disease and their parents.

Suggested Citation

  • Aline K. Honingh & Yvonne L. Kruithof & Willemijn F. E. Kuper & Peter M. van Hasselt & Paula S. Sterkenburg, 2022. "Towards Understanding Behaviour and Emotions of Children with CLN3 Disease (Batten Disease): Patterns, Problems and Support for Child and Family," IJERPH, MDPI, vol. 19(10), pages 1-21, May.
  • Handle: RePEc:gam:jijerp:v:19:y:2022:i:10:p:5895-:d:814235
    as

    Download full text from publisher

    File URL: https://www.mdpi.com/1660-4601/19/10/5895/pdf
    Download Restriction: no

    File URL: https://www.mdpi.com/1660-4601/19/10/5895/
    Download Restriction: no
    ---><---

    References listed on IDEAS

    as
    1. Brehaut, J.C. & Kohen, D.E. & Garner, R.E. & Miller, A.R. & Lach, L.M. & Klassen, A.F. & Rosenbaum, P.L., 2009. "Health among caregivers of children with health problems: Findings from a Canadian population-based study," American Journal of Public Health, American Public Health Association, vol. 99(7), pages 1254-1262.
    Full references (including those not matched with items on IDEAS)

    Most related items

    These are the items that most often cite the same works as this one and are cited by the same works as this one.
    1. Ragnhild Nes & Lars Hauge & Tom Kornstad & Petter Kristensen & Markus Landolt & Leif Eskedal & Lorentz Irgens & Margarete Vollrath, 2014. "The Impact of Child Behaviour Problems on Maternal Employment: A Longitudinal Cohort Study," Journal of Family and Economic Issues, Springer, vol. 35(3), pages 351-361, September.
    2. Eun-Young Park, 2021. "Validity and Reliability of the Caregiving Difficulty Scale in Mothers of Children with Cerebral Palsy," IJERPH, MDPI, vol. 18(11), pages 1-9, May.
    3. Eun-Young Park, 2021. "Validity of the Friedrich Short Form of the Questionnaire on Resources and Stress in Parents of Individuals with Autism Spectrum Disorder," IJERPH, MDPI, vol. 18(22), pages 1-9, November.
    4. Jackson, Dylan B. & Testa, Alexander & Turney, Kristin, 2022. "Unpacking the connection between parental incarceration and parenting stress: The mediating role of child health and health care strains," Journal of Criminal Justice, Elsevier, vol. 81(C).
    5. Lisbeth Gravdal Kvarme & Elena Albertini‐Früh & Idunn Brekke & Ragnhild Gardsjord & Liv Halvorsrud & Hilde Liden, 2016. "On duty all the time: health and quality of life among immigrant parents caring for a child with complex health needs," Journal of Clinical Nursing, John Wiley & Sons, vol. 25(3-4), pages 362-371, February.
    6. Monika Novak-Pavlic & Peter Rosenbaum & Briano Di Rezze, 2023. "Changing Directions and Expanding Horizons: Moving towards More Inclusive Healthcare for Parents of Children with Developmental Disabilities," IJERPH, MDPI, vol. 20(21), pages 1-11, October.

    Corrections

    All material on this site has been provided by the respective publishers and authors. You can help correct errors and omissions. When requesting a correction, please mention this item's handle: RePEc:gam:jijerp:v:19:y:2022:i:10:p:5895-:d:814235. See general information about how to correct material in RePEc.

    If you have authored this item and are not yet registered with RePEc, we encourage you to do it here. This allows to link your profile to this item. It also allows you to accept potential citations to this item that we are uncertain about.

    If CitEc recognized a bibliographic reference but did not link an item in RePEc to it, you can help with this form .

    If you know of missing items citing this one, you can help us creating those links by adding the relevant references in the same way as above, for each refering item. If you are a registered author of this item, you may also want to check the "citations" tab in your RePEc Author Service profile, as there may be some citations waiting for confirmation.

    For technical questions regarding this item, or to correct its authors, title, abstract, bibliographic or download information, contact: MDPI Indexing Manager (email available below). General contact details of provider: https://www.mdpi.com .

    Please note that corrections may take a couple of weeks to filter through the various RePEc services.

    IDEAS is a RePEc service. RePEc uses bibliographic data supplied by the respective publishers.