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

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.

Implications of COVID-19 Regulations for People With Visual and Intellectual Disabilities: Lessons to Learn From Visiting Restrictions.

Background: Due to the COVID-19 pandemic, governments of many countries announced regulations to prevent the virus from spreading. For people with a disability living in a sheltered care facility in the Netherlands, this meant that they were not able to receive any visitors for almost 3 months. Aim: This study examines how people with an intellectual and visual disability and their families experienced the period in which it was mandated not to have any physical contact. The aim is to examine the experiences of this target group and gain insight in the way measures were taken in order to be able to advise care organizations about adequate care with respect to possible restrictive measures in the future. Methods: In-depth interviews were conducted with two groups of people: (1) fourteen people with an intellectual and visual disability, living in sheltered care facilities and (2) twelve people being relatives of residents of these same sheltered care facilities. In the interviews, the participants were questioned about their experiences with respect to the adjusted visiting regulations and with respect to the relation with their family during this period. A thematic analysis was performed first separately and then combined. Results and Discussion: A number of themes resulted from the analysis that were related to (1) the instructed regulations of the sheltered care facilities and the government; (2) the relation with family and friends; and (3) the consequences of COVID-19 and the regulations. Both relatives and residents were understanding of the difficult situation, but also expressed criticism about the chosen regulations, the communication thereof, and the practical implementation. Both groups have experienced the interruption of close contact as emotional and difficult. However, also positive consequences of the restrictions due to COVID-19 were mentioned. The results provide a list of recommendations for sheltered care facilities.

An Exploratory Study among Intellectual Disability Physicians on the Care and Coercion Act and the Use of Psychotropic Drugs for Challenging Behaviour.

The new Dutch Care and Coercion Act aims to better regulate the use of psychotropic drugs for challenging behaviour in people with an intellectual disability. This study explores experiences of intellectual disability physicians (IDPs) in prescribing psychotropic drugs and investigates how the Act and the new multidisciplinary guideline on challenging behaviour affects their practice. A qualitative study was conducted, consisting of nine semi-structured in-depth interviews with IDPs, followed by a thematic analysis. It was found that IDPs experienced the new Act and guideline as supportive of their work as guardians of the appropriate use of psychotropic drugs. The multidisciplinary character of the guideline was experienced positively. However, IDPs are faced with organisational barriers and time constraints, as such, they question the feasibility of implementing the Act. Based on these findings, it can be concluded that the Care and Coercion Act may support the existing shift towards the appropriate use of psychotropic drugs if required conditions can be met.