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.
Int J Environ Res Public Health. 2022 May 12;19(10):5895. doi: 10.3390/ijerph19105895.
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.
神经元蜡样脂褐质沉积症（CLN3 病/巴滕病）的少年型是一种罕见的儿童和青年期进行性脑疾病，其特征是视力丧失、认知和运动能力下降和癫痫。CLN3 病患儿常表现出行为和情绪紊乱。本研究旨在更好地了解 CLN3 病患儿的行为和情绪，并检查患儿及其父母所接受的支持。采用定性和定量分析相结合的方法对病历和家长访谈进行分析。使用框架分析方法开发了一个代码本，对来源进行编码，并对数据进行分析。分析结果得出了以下几方面的概述：（1）CLN3 病患儿的典型行为和情绪；（2）CLN3 病患儿所获得的支持；（3）这些患儿的父母所获得的支持；（4）这些父母面临的问题。对于一些患儿，他们的视力、身体或认知能力的恶化被发现会导致特定的情绪和行为。定量分析显示，所有患儿都有焦虑报告。所呈现的支持概述包含了医疗保健专业人员的隐性知识，本研究使这些知识变得明确。这些概述可能为 CLN3 病患儿及其父母提供适应性支持模块的线索。
