Colin Reilly

The Impact on the Family of Four Neurogenetic Syndromes: A Comparative Study of Parental Views

Research on behavioral phenotypes in neurogenetic syndromes has primarily focused on aspects of the affected child’s behavior. However, the impact of having a child with a neurogenetic syndrome on aspects of family functioning can be significant and differ across syndromes. Parents (N = 381) of school-aged children with one of four neurogenetic syndromes: fragile X Syndrome (FXS), Prader Willi Syndrome (PWS), Williams Syndrome (WS) and 22q11.2 (22qDEL) were asked about challenges, restrictions, future concerns and positive aspects arising from having an affected child. Factors associated with these aspects were analysed via logistic regression. Restrictions were most often reported by parents of children with FXS. The factors significantly (p < .01) associated with the parent reported restrictions were the presence of a behavioral/psychiatric condition and a lower level of receptive communication. The challenges endorsed most often were the child’s learning and social skills difficulties. Significant differences (p < .01) between the syndromes were noted for many of the challenges. Ninety-six percent of parents endorsed at least one positive aspect but most aspects endorsed did not differ between the genetic syndromes. Having a behavioral/psychiatric condition was the only factor significantly associated (p < .01) with endorsing a lower number of positive aspects and a higher number of challenges. There are some commonalities but also significant differences across syndromes regarding views on how a child’s syndrome affects family functioning. These differences may be important with regard to how to counsel and support affected families. The presence of behavioral/psychiatric conditions is an important predictor of negative family outcome across the syndromes.

A comparative study of educational provision for children with neurogenetic syndromes: parent and teacher survey

BACKGROUND A number of neurogenetic syndromes have a high association with special educational needs including fragile X syndrome (FXS), Prader-Willi syndrome (PWS), Williams syndrome (WS) and Velo-Cardio-Facial syndrome (VCFS). There is a paucity of research on educational provision for children affected by these syndromes. METHOD Parents (n = 381) and teachers (n = 204) of school-aged children with one of the four syndromes in the UK and Ireland were surveyed in a range of areas concerning the childs educational provision. Areas surveyed included school placement, views on the needs of children with the syndromes, desired changes to current provision and perceived teacher knowledge. RESULTS School placement in mainstream settings decreased with age in all of the syndromes. Males with the syndromes were more likely to be in specialised educational settings with the exception of WS. Teachers reported limited input on initial or subsequent training for all of the syndromes. The majority of teachers did not view the needs of children with syndromes as different from other children with intellectual disability (ID) although there were significant differences between the syndromes. Changes deemed necessary to provision by parents and teachers differed between the syndromes indicating the existence of perceptions of syndrome specific needs. The lowest perceived level of teacher knowledge was in the VCFS group. CONCLUSION The majority of teachers of children with neurogenetic syndromes report limited knowledge of the syndromes, but also a lack of belief that the childrens needs are different from the majority of children with ID. Differences between the syndromes in some areas of provision suggest that a childs syndrome does impact on educational provision in some areas.

Screening for Asperger syndrome in school‐age children: issues and instruments

Many children with Asperger syndrome are not identified prior to school entry, and difficulties associated with the condition may only become evident when a child enters school. Failure to identify children with the syndrome may lead to increased risk for psychopathology, and lack of understanding of the reasons for social and communicative impairments, and the display of atypical behaviours. Therefore, there is a great need for effective screening instruments to be used with at risk children. This article reviews a number of screening instruments for Asperger syndrome and higher functioning autism spectrum disorders, and highlights the need for surveillance for the conditions among professionals who work in educational settings.

Children with epilepsy: the role of the educational psychologist

Childhood epilepsy is the most common paediatric neurological disorder. It is a condition with a well-documented association with cognitive, behavioural and emotional difficulties. Children with epilepsy are at increased risk of global and specific cognitive impairments. They are also at increased risk for symptoms associated with attention deficit hyperactivity disorder (ADHD), autism spectrum disorder (ASD), depression and anxiety. The cognitive, behavioural and emotional difficulties associated with childhood epilepsy are often overshadowed by the need to treat epileptic seizures. Psychologists working in schools can provide valuable insight into the cognitive, behavioural and emotional issues faced by children with epilepsy through appropriate assessment and the recommendation of interventions which will lessen the impact of such difficulties. Educational psychologists can also help the children’s families cope with a condition with challenging psychosocial implications and provide a valuable link between school staff and supporting health professionals.

Behavioural phenotypes in the classroom: a qualitative study of parental and teacher knowledge of classroom guidelines and teacher views on best practices

Research suggests that genetic syndromes associated with intellectual disability often have specific cognitive and behavioural profiles. It has been suggested that educational approaches need to reflect these profiles. Parents (n = 381) and teachers (n = 204) of children with one of four syndromes, fragile X syndrome, Prader‐Willi syndrome, Williams syndrome and velo-cardio-facial syndrome were surveyed. Syndromes were compared with respect to what parents and teachers had researched or been told with respect to appropriate educational approaches. Parent and teacher reports were subsequently compared with actual published guidelines on the syndromes. Teachers were also surveyed on what they felt were successful approaches based on actual experiences of teaching the children. Parent and teacher reports of efficacious strategies for supporting their children based on what they had been researched or been told differed across syndromes as might be anticipated. However, differences between parents and teachers and between parent/teacher reports and the published guidelines suggest that parent and teacher knowledge of guidelines is deficient and/or that parents and teachers are accessing knowledge elsewhere. With respect to teaching practices, there were much less differences between the syndromes than in the published guidelines indicating that differences between syndromes in guidelines may not be as evident in practice.