A sibling-controlled, prospective study of outcomes at home and school in children with severe congenital heart disease

Objectives: The objectives of this study were to compare behaviour problems and competencies, at home and school, in 7-year-old children with congenital heart disease with a sibling control group, to examine the prospective determinants of outcome from infancy, and to explore whether any gains were maintained in our sub-group of children who had participated in a previous trial of psychological interventions in infancy.
Methods: A total of 40 children who had undergone surgery to correct or palliate a significant congenital heart defect in infancy were compared (Child Behavior Checklist) with a nearest-age sibling control group (18 participants). Comparisons were made between sub-groups of children and families who had and had not participated in an early intervention trial.
Results: Problems with attention, thought and social problems, and limitations in activity and school competencies, were found in comparison with siblings. Teacher reports were consistent with parents, although problems were of a lower magnitude. Disease, surgical, and neurodevelopmental functioning in infancy were related to competence outcomes but not behaviour problems. The latter were mediated by family and maternal mental health profiles from infancy. Limited, but encouraging, gains were maintained in the sub-group that had participated in the early intervention programme.
Conclusions: The present study is strengthened by its longitudinal design, use of teacher informants, and sibling control group. The patterns of problems and limitations discerned, and differential determinants thereof, have clear implications for interventions. We consider these in the light of our previously reported intervention trial with this sample and current outcomes at the 7-year follow-up.

A Randomized Controlled Trial of Interventions to Promote Adjustment in Children With Congenital Heart Disease Entering School and Their Families

Objective: To report on a randomized controlled trial of psychological interventions to promote adjustment in children with congenital heart disease and their families.
Method: Following baseline assessment, 90 children (aged 4–5 years) and their families were randomly assigned to an Intervention or Control group before entering school. 68 (76%) were retained at 10-month follow-up.
Results: Gains were observed on measures of maternal mental health and family functioning. Although no differences were found on measures of child behavior at home or school, children in the intervention group were perceived as “sick” less often by their mother and missed fewer days from school. A regression model, using baseline measures as predictors, highlighted the importance of maternal mental health, worry and child neurodevelopmental functioning for child behavioral outcomes almost a year later.
Conclusions: The intervention promoted clinically significant gains for the child and family. The program is of generalizable significance.

Examination of the physical and psychosocial determinants of health behaviour in 4-5-year-old children with congenital cardiac disease

To assess the general health and activity levels of 4- and 5-year-old children after intervention for congenital cardiac disease.

End-of-Life care and people with intellectual disabilities: A multi-media educational resource

Aim This study aimed to develop and evaluate a multi-media educational resource in palliative and end-of-life care for specialist palliative care and intellectual disability services which promoted collaborative working. Methods: A mixed methods design involving three phases was used. Qualitative data were obtained from semi-structured interviews with a purposive sample of professionals (n=30) and family carers (n=5) and from two focus groups with people with intellectual disabilities (n=17). Data were content analysed as outlined y Newell and Burnard (2006). This identified training needs and issues, in end-of-life care for this population which were confirmed through quantitative data from services in a regional scoping study analysed using descriptive statistics. A DVD and manual were developed and evaluated with twelve professionals. Data were collected using a solicited diary, the Readiness for Inter-professional Learning Scale, Likert Scales and an evaluation questionnaire. Thematic analysis and descriptive statistics appropriate to data were used.Results: Findings suggest that this resource demonstrates the need for and benefits of partnership working and transferability of this learning to practice could address issues at end-of-life for people with intellectual disabilities. Conclusions: Findings of this study have importance for partnership working and service provision in end-of-life care for this population.

Primary Reflex Persistence in Children With Partial Hearing

Persistence of the asymmetrical tonic neck reflex (ATNR) was examined in children with partial hearing (aged 6–12 years). Core literacy skills were also assessed. Three groups of children were selected from three schools with special units for children with partial hearing. All children completed an upright ATNR test protocol and standardized tests of reading and spelling. Children with partial hearing had significant levels of ATNR persistence, and significant reading and spelling difficulties. The findings suggest that persistence of an early sub-cortical reflex system may be associated with some of the motor and cognitive difficulties experienced by children with partial hearing.

A cross-sectional evaluation of factors influencing adherence in children with cystic fibrosis

Hawthorne N., Hawwa A., McElnay J., Shields M., Reid A (2013). A cross sectional evaluation of factors influencing adherence in children with cystic fibrosis. Journal of Cystic Fibrosis: 12 (Supplement 1); S11.

Task-switching deficits and repetitive behaviour in genetic neurodevelopmental disorders: Data from children with Prader-Willi syndrome chromosome 15 q11-q13 deletion and boys with Fragile X syndrome

Prader-Willi syndrome (PWS) and Fragile X syndrome (FraX) are associated with distinctive cognitive and behavioural profiles. We examined whether repetitive behaviours in the two syndromes were associated with deficits in specific executive functions. PWS, FraX, and typically developing (TD) children were assessed for executive functioning using the Test of Everyday Attention for Children and an adapted Simon spatial interference task. Relative to the TD children, children with PWS and FraX showed greater costs of attention switching on the Simon task, but after controlling for intellectual ability, these switching deficits were only significant in the PWS group. Children with PWS and FraX also showed significantly increased preference for routine and differing profiles of other specific types of repetitive behaviours. A measure of switch cost from the Simon task was positively correlated to scores on preference for routine questionnaire items and was strongly associated with scores on other items relating to a preference for predictability. It is proposed that a deficit in attention switching is a component of the endophenotypes of both PWS and FraX and is associated with specific behaviours. This proposal is discussed in the context of neurocognitive pathways between genes and behaviour.