Overview of advances in educational and social supports for young persons with NCL disorders

Elmerskog, Bengt; Tøssebro, Anne-Grethe; Atkinson, Rebecca; Rokne, Svein; Cole, Barbara; Ockelford, Adam; Adams, Heather

doi:10.1016/j.bbadis.2019.05.016

Cited by 5 publications

(5 citation statements)

References 20 publications

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“…While educational and social support modules for children with CLN3 disease have been developed and focus for example on music therapy and braille [33], no list of advice on direct contact with these children has been formalised. With the list of seven categories of specific CLN3 support, tacit knowledge of professionals working with children with CLN3 disease has been made explicit.…”

Section: Discussionmentioning

confidence: 99%

“…Children with a decline in memory and attention may have limited abilities to benefit from behaviour management programs [32]. An educational and social support program has been developed for children with CLN3 disease, including for example music therapy and braille education [33]. However, no behavioural support or a formalisation on manners or contact with respect to these children has been formalised.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

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

Honingh

Kruithof

Kuper

et al. 2022

IJERPH

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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.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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

Honingh

Kruithof

Kuper

et al. 2022

IJERPH

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“…[72][73][74][75] A published approach to educational and social supports for patients with CLN3 disease may be applicable to individuals with the juvenile CLN1 disease phenotype. 76 Caregiver Survey Insights Two thirds of respondents completely or somewhat agree with the statement, "I feel my child is welcomed at his/her school, and that the school has partnered with me to accommodate his/her needs. "…”

Section: Neurocognitive Assessment and Educational Strategiesmentioning

confidence: 99%

Management of CLN1 Disease: International Clinical Consensus

Augustine

Adams

Reyes

et al. 2021

Pediatric Neurology

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“…Finally, this Special Issue has contributions from those working directly with NCL patients and their families. This includes a review from Elmerskog et al on the challenges and recommendations around education and social support for children with Juvenile NCL [9]. Band et al also discuss the NCLs from the patient organisation perspective with their views on hurdles that they face, research and interactions with the pharmaceutical industry [10].…”

Section: J O U R N a L P R E -P R O O Fmentioning

confidence: 99%