Towards Understanding Behaviour and Emotions of Children with CLN3 Disease (Batten Disease)

Patterns, Problems and Support for Child and Family

Bibliographic Data

ID15480809
AuthorsAline Honingh (0000-0002-6721-1422, Vrije Universiteit Amsterdam, corresponding author), Yvonne Kruithof (0000-0001-6877-0560, Bartiméus), Willemijn F E Kuper (0000-0003-1676-6759, Utrecht University), Peter M van Hasselt (0000-0001-9821-8398, Utrecht University), P S Sterkenburg (0000-0001-6014-7539, Bartiméus)
Year2022
Volume19
Issue10
Pages5895-5895
Publication date2022-05-12
Open AccessYes
TypeARTICLE
VenueInternational Journal of Environmental Research and Public Health (JOURNAL)
Journal identifiersISSN: 1661-7827 • E-ISSN: 1660-4601
PublisherMultidisciplinary Digital Publishing Institute (PUBLISHER • CH)
DOI10.3390/ijerph19105895
PMID35627432
OpenAlexW4280489381
LanguageEN
References cited38

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

Anxiety · Batten disease · Cognition · Developmental psychology · Disease · Epilepsy · Psychiatry · Clinical Psychology · Family and Disability Support Research · Glycogen Storage Diseases and Myoclonus · Lysosomal Storage Disorders Research · Medicine · Psychology

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