Associations between behaviours that challenge in adults with intellectual disability, parent perceptions and parental mental health

Objectives. This study examined parental perceptions of behaviours that challenge (CB) in their adult children with intellectual disability (ID), and explored whether perceptions mediated associations between CB and parental psychological distress. Design. A within-group correlational design was employed. Methods. Sixty-five parents reported on individuals with genetic syndromes and ID who had chronic behaviours that challenge (CB). Parents completed the Illness Perception Questionnaire-Revised (IPQ-R) adapted to measure perceptions of self-injury, aggression or property destruction, alongside assessments of parental locus of control, attributions about behaviour, parental psychological distress, and CB. Results. A high proportion of parents evidenced anxiety and depression at clinically significant levels (56.9% and 30.8%, respectively). Contrary to predictions, psychological distress was not significantly associated with CB. The perception that the adult with ID exerted control over the parent’s life mediated the association between CB and parental psychological distress. Few parents endorsed operant reinforcement as a cause of CB (< 10%). Conclusions. The high levels of psychological distress in parents is notable and of concern. Further research should consider the reasons why parents have causal attributions that might be inconsistent with contemporary interventions. Key words: Adults; parents; attributions; self-regulatory model; challenging behaviour; intellectual disability

The importance of understanding the behavioural phenotypes of genetic syndromes associated with intellectual disability

Behavioural phenotype research is of benefit to a large number of children with genetic syndromes and associated developmental delay. This article presents an overview of this research area and demonstrates how understanding pathways between gene disorders and behaviour can inform our understanding of the difficulties individuals with genetic syndromes and developmental delay experience, including self-injurious behaviour, social exploitation, social anxiety, social skills deficits, sensory differences, temper outbursts and repetitive behaviours. In addition, physical health difficulties and their interaction with behaviour are considered. The article demonstrates the complexity involved in assessing a child with a rare genetic syndrome

An occupational therapy intervention for residents with stroke related disabilities in UK care homes (OTCH): cluster randomised controlled trial

Objective To evaluate the clinical efficacy of an established programme of occupational therapy in maintaining functional activity and reducing further health risks from inactivity in care home residents living with stroke sequelae. Design Pragmatic, parallel group, cluster randomised controlled trial. Setting 228 care homes (>10 beds each), both with and without the provision of nursing care, local to 11 trial administrative centres across the United Kingdom. Participants 1042 care home residents with a history of stroke or transient ischaemic attack, including those with language and cognitive impairments, not receiving end of life care. 114 homes (n=568 residents, 64% from homes providing nursing care) were allocated to the intervention arm and 114 homes (n=474 residents, 65% from homes providing nursing care) to standard care (control arm). Participating care homes were randomised between May 2010 and March 2012. Intervention Targeted three month programme of occupational therapy, delivered by qualified occupational therapists and assistants, involving patient centred goal setting, education of care home staff, and adaptations to the environment. Main outcome measures Primary outcome at the participant level: scores on the Barthel index of activities of daily living at three months post-randomisation. Secondary outcome measures at the participant level: Barthel index scores at six and 12 months post-randomisation, and scores on the Rivermead mobility index, geriatric depression scale-15, and EuroQol EQ-5D-3L questionnaire, at all time points. Results 64% of the participants were women and 93% were white, with a mean age of 82.9 years. Baseline characteristics were similar between groups for all measures, personal characteristics, and diagnostic tests. Overall, 2538 occupational therapy visits were made to 498 participants in the intervention arm (mean 5.1 visits per participant). No adverse events attributable to the intervention were recorded. 162 (11%) died before the primary outcome time point, and 313 (30%) died over the 12 months of the trial. The primary outcome measure did not differ significantly between the treatment arms. The adjusted mean difference in Barthel index score at three months was 0.19 points higher in the intervention arm (95% confidence interval −0.33 to 0.70, P=0.48). Secondary outcome measures also showed no significant differences at all time points. Conclusions This large phase III study provided no evidence of benefit for the provision of a routine occupational therapy service, including staff training, for care home residents living with stroke related disabilities. The established three month individualised course of occupational therapy targeting stroke related disabilities did not have an impact on measures of functional activity, mobility, mood, or health related quality of life, at all observational time points. Providing and targeting ameliorative care in this clinically complex population requires alternative strategies

Psychological Functioning in Pediatric Patients with Single Ventricle Congenital Heart Disease: A Meta-Analysis and Systematic Review Protocol

Mental health is an important yet understudied area of care for patients with congenital heart disease. Through limited studies, it is known that children and adults with congenital heart disease have increased incidence of mental health disorders when compared to their peers [1-3]. Some studies estimate that over half of adult patients with congenital heart disease have significant symptoms of a mood or anxiety disorder [3], although it is very likely that these symptoms are underrecognized. It is also known that in adults with congenital heart disease, depression is responsible for the variability of self-reported health status of patients, including physical functioning [4]. A prior review and meta-analysis of patient with complex congenital heart disease showed an increased risk of internalizing and externalizing behavior problems, however this review was not specific to the single ventricle population [5]. A recent review and meta-analysis of patients with children and adults with single ventricle physiology found worse health-related quality of life outcomes in this population [6], however currently less is known about psychological functioning specifically in the pediatric single ventricle population. The aim of this systematic review is to summarize and meta-analyze the existing literature of psychological outcomes in pediatric single ventricle patients. It is hypothesized that pediatric patients with single ventricle heart disease will have an increased risk of internalizing and externalizing problems as compared to their peers.No funding associated with this projecthttp://deepblue.lib.umich.edu/bitstream/2027.42/167623/3/SVSysRevProposal2021.pdfDescription of SVSysRevProposal2021.pdf : This is a protocol for an evidence sythesis project on Psychological Functioning in Pediatric Patients with Single Ventricle Congenital Heart DiseaseSEL

The behaviour and wellbeing of children and adults with severe intellectual disability and complex needs: the Be-Well checklist for carers and professionals

This is an accepted manuscript of an article published by Elsevier in Paediatrics and Child Health on 13/10/2020, available online: https://doi.org/10.1016/j.paed.2020.09.003 The accepted version of the publication may differ from the final published version.Children and adults with severe intellectual disability and complex needs often show behaviours and distress that carers and professionals find difficult to identify causes for, manage and decrease. The prevailing view is that these behaviours and distress are learned and consequently interventions focus on behavioural techniques. In this article we summarise the findings of research that indicate that behaviour and distress in this population are influenced by transient and stable characteristics or conditions that can interact with aspects of learning, be independent of learning, and interact with each other. These transient and stable characteristics or conditions are: pain and discomfort, sensory sensitivity, anxiety and low mood, sleep problems, atypical emotional regulation, specific cognitive difference, and differences in social behaviour. To aid carers and professionals, we present a checklist of the elements of an assessment process that covers these transient and stable characteristics or conditions and other relevant influences on behaviour and distress such as seizures, medication, learning and communication. We also draw attention to the benefit of identifying the cause of intellectual disability to inform the assessment process.Published versio

Profiles of autism characteristics in thirteen genetic syndromes : a machine learning approach

Background: Phenotypic studies have identified distinct patterns of autistic characteristics in genetic syndromes associated with intellectual disability (ID), leading to diagnostic uncertainty and compromised access to autism-related support. Previous research has tended to include small samples and diverse measures, which limits the generalisability of findings. In this study, we generated detailed profiles of autistic characteristics in a large sample of > 1500 individuals with rare genetic syndromes. Methods: Profiles of autistic characteristics based on the Social Communication Questionnaire (SCQ) scores were generated for thirteen genetic syndrome groups (Angelman n = 154, Cri du Chat n = 75, Cornelia de Lange n = 199, fragile X n = 297, Prader–Willi n = 278, Lowe n = 89, Smith–Magenis n = 54, Down n = 135, Sotos n = 40, Rubinstein–Taybi n = 102, 1p36 deletion n = 41, tuberous sclerosis complex n = 83 and Phelan–McDermid n = 35 syndromes). It was hypothesised that each syndrome group would evidence a degree of specificity in autistic characteristics. To test this hypothesis, a classification algorithm via support vector machine (SVM) learning was applied to scores from over 1500 individuals diagnosed with one of the thirteen genetic syndromes and autistic individuals who did not have a known genetic syndrome (ASD; n = 254). Self-help skills were included as an additional predictor. Results: Genetic syndromes were associated with different but overlapping autism-related profiles, indicated by the substantial accuracy of the entire, multiclass SVM model (55% correctly classified individuals). Syndrome groups such as Angelman, fragile X, Prader–Willi, Rubinstein–Taybi and Cornelia de Lange showed greater phenotypic specificity than groups such as Cri du Chat, Lowe, Smith–Magenis, tuberous sclerosis complex, Sotos and Phelan-McDermid. The inclusion of the ASD reference group and self-help skills did not change the model accuracy. Limitations: The key limitations of our study include a cross-sectional design, reliance on a screening tool which focuses primarily on social communication skills and imbalanced sample size across syndrome groups. Conclusions: These findings replicate and extend previous work, demonstrating syndrome-specific profiles of autistic characteristics in people with genetic syndromes compared to autistic individuals without a genetic syndrome. This work calls for greater precision of assessment of autistic characteristics in individuals with genetic syndromes associated with ID

Childbirth experience questionnaire: validating its use in the United Kingdom

BACKGROUND: The Childbirth Experience Questionnaire (CEQ) was developed in Sweden in 2010 and validated in 920 primiparous women. It has not been validated in the United Kingdom (UK). Measuring the impact of an intervention on a woman's childbirth experience is arguably as important as measuring its impact on outcomes such as caesarean delivery and perinatal morbidity or mortality and yet surprisingly it is rarely done. The lack of a robust validated tool for evaluating labour experience in the UK is a topical issue in the UK at present. Indeed NICE say 'A standardised method to measure and quantify women's psychological and emotional wellbeing and their birth experiences is urgently required to support any study investigating the effectiveness of interventions, techniques or strategies during birth.' METHODS: The Childbirth Experience Questionnaire and part of the Care Quality Commission Maternity Survey (2010) was sent to 350 women at one month postnatal. The CEQ was sent again two weeks later. The CEQ was tested for face validity among 25 postnatal mothers. Demographic data and delivery data was used to establish construct validity of the CEQ using the method of known-groups validation. The results of the scored CEQ sent out twice were used to measure test-retest reliability of the CEQ by calculating the quadratic weighted index of agreement between the two scores. Criterion validity was measured by calculating the Pearson correlation coefficient for the CEQ and Maternity Survey scores. RESULTS: Face validity of the CEQ in a UK population was demonstrated with all respondents stating it was easy to understand and complete. A statistically significantly higher CEQ score for subgroups of women known to report a better birth outcome demonstrated construct validity of the CEQ. A weighted kappa of 0.68 demonstrated test-retest reliability of the CEQ. A Pearson correlation co-efficient of 0.73 demonstrated a strong correlation between the results of the CEQ and the results of the 'gold standard' assessment of childbirth experience in the UK: the Maternity Survey and hence criterion validity of the CEQ. CONCLUSIONS: The Childbirth Experience Questionnaire is a valid and reliable measure of childbirth experience in the UK population