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.
Reasons for a lower recognition of ASD, ADHD and mental health conditions in clinical practice compared with research studies may include 'diagnostic overshadowing' due to presence of intellectual disability and a genetic syndrome. However, there may also be a lack of belief in the utility of such diagnoses in neurogenetic syndromes among relevant professionals and/or lack of access to professionals with sufficient expertise in the recognition of such diagnoses in those with neurogenetic syndromes. The low rates of prescribing of medication for behavioural/psychiatric reasons may reflect the low level of clinical diagnoses or lack of belief in the utility of psychopharmacology in this population.
This paper describes a study of retention of school-acquired Irish among school leavers. The initial group selected represented final year secondary school students from three different instructional categories: ordinary level Irish, advanced level Irish and immersion school students. Participants were interviewed and their language skills assessed as they completed their study of Irish and again 18 months after they had left secondary school. Proficiency was measured in terms of scores on a communicative test of spoken Irish and a C-test in Irish. Background information collected included participants' self-assessed ability in spoken Irish, attitude/motivation in relation to learning Irish and their out-of-school use of Irish. Test results did not indicate any attrition of Irish language skills over time, despite participants' general feelings of loss. This outcome matches findings from similar studies of second language attrition elsewhere, for example, Weltens, 1989. The results also indicated a general decline in opportunity to use Irish among participants from all three instructional backgrounds. The ex-immersion school participants, however, were the most likely to continue speaking the language. A small but significant gain on one particular Irish speaking subtest which was noted in this ex-immersion group may be attributed, in part at least, to these participants' greater access to Irish language-speaking networks.
This article outlines the impact of an eight-week Precision Teaching (PT) intervention on the sight vocabulary, reading fluency and reading attainment scores of a group of Irish primary school pupils. This study also sought to evaluate the role of PT in facilitating formative assessment of pupils' progress. Employing a mixed-method approach, this study comprised a quasi-experimental design and qualitative interviews involving 40 Irish primary school pupils and seven Learning Support Teachers. Following intervention, a mixed between-within subjects analysis of variance confirmed the differential growth in sight vocabulary scores across the control and intervention groups. Notably, the latter group displayed a greater increase in magnitude in sight vocabulary following intervention, paralleled by statistically significant increases in reading accuracy, fluency and comprehension standard scores and overall reading ability composite scores. Semi-structured interviews highlighted PT as an effective strategy in facilitating formative assessment and a highly motivational tool in education. Results are interpreted in light of relevant literature, with implications for practice and future research discussed.
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