Clinical and cost-effectiveness of an adapted intervention for preschoolers with moderate to severe intellectual disabilities displaying behaviours that challenge: the EPICC-ID RCT.

Background: Stepping Stones Triple P is an adapted intervention for parents of young children with developmental disabilities who display behaviours that challenge, aiming at teaching positive parenting techniques and promoting a positive parent-child relationship. Objective: To evaluate the clinical and cost-effectiveness of level 4 Stepping Stones Triple P in reducing behaviours that challenge in children with moderate to severe intellectual disabilities. Design, setting, participants: A parallel two-arm pragmatic multisite single-blind randomised controlled trial recruited a total of 261 dyads (parent and child). The children were aged 30-59 months and had moderate to severe intellectual disabilities. Participants were randomised, using a 3 : 2 allocation ratio, into the intervention arm (Stepping Stones Triple P; n = 155) or treatment as usual arm (n = 106). Participants were recruited from four study sites in Blackpool, North and South London and Newcastle. Intervention: Level 4 Stepping Stones Triple P consists of six group sessions and three individual phone or face-to-face contacts over 9 weeks. These were changed to remote sessions after 16 March 2020 due to the coronavirus disease 2019 pandemic. Main outcome measure: The primary outcome measure was the parent-reported Child Behaviour Checklist, which assesses the severity of behaviours that challenge. Results: We found a small non-significant difference in the mean Child Behaviour Checklist scores (-4.23, 95% CI -9.98 to 1.52, p = 0.146) in the intervention arm compared to treatment as usual at 12 months. Per protocol and complier average causal effect sensitivity analyses, which took into consideration the number of sessions attended, showed the Child Behaviour Checklist mean score difference at 12 months was lower in the intervention arm by -10.77 (95% CI -19.12 to -2.42, p = 0.014) and -11.53 (95% CI -26.97 to 3.91, p = 0.143), respectively. The Child Behaviour Checklist mean score difference between participants who were recruited before and after the coronavirus disease 2019 pandemic was estimated as -7.12 (95% CI -13.44 to -0.81) and 7.61 (95% CI -5.43 to 20.64), respectively (p = 0.046), suggesting that any effect pre-pandemic may have reversed during the pandemic. There were no differences in all secondary measures. Stepping Stones Triple P is probably value for money to deliver (-£1057.88; 95% CI -£3218.6 to -£46.67), but decisions to roll this out as an alternative to existing parenting interventions or treatment as usual may be dependent on policymaker willingness to invest in early interventions to reduce behaviours that challenge. Parents reported the intervention boosted their confidence and skills, and the group format enabled them to learn from others and benefit from peer support. There were 20 serious adverse events reported during the study, but none were associated with the intervention. Limitations: There were low attendance rates in the Stepping Stones Triple P arm, as well as the coronavirus disease 2019-related challenges with recruitment and delivery of the intervention. Conclusions: Level 4 Stepping Stones Triple P did not reduce early onset behaviours that challenge in very young children with moderate to severe intellectual disabilities. However, there was an effect on child behaviours for those who received a sufficient dose of the intervention. There is a high probability of Stepping Stones Triple P being at least cost neutral and therefore worth considering as an early therapeutic option given the long-term consequences of behaviours that challenge on people and their social networks. Future work: Further research should investigate the implementation of parenting groups for behaviours that challenge in this population, as well as the optimal mode of delivery to maximise engagement and subsequent outcomes. Study registration: This study is registered as NCT03086876 (https://www.clinicaltrials.gov/ct2/show/NCT03086876?term=Hassiotis±Angela&draw=1&rank=1). Funding: This award was funded by the National Institute for Health and Care Research (NIHR) Health Technology Assessment programme (NIHR award ref: HTA 15/162/02) and is published in full in Health Technology Assessment; Vol. 28, No. 6. See the NIHR Funding and Awards website for further award information.

Research shows that in children without learning disabilities, parenting groups which support parents to develop skills to manage behaviours that challenge in their child can be helpful. The National Institute of Health and Care Excellence recommended that more research was needed to strengthen the evidence for such interventions for children with moderate to severe learning disability who are more likely to display behaviours that challenge in England. In this study, we tested in real-world conditions a programme called level 4 Stepping Stones Triple P, which has shown positive results in trials outside of the United Kingdom. Trained therapists delivered six groups and three individual sessions over 9 weeks to parents of children aged 30­59 months with moderate to severe learning disabilities. Two hundred and sixty-one parents were allocated to one of two arms by chance (randomisation): one received Stepping Stones Triple P and treatment as usual and the other treatment as usual only. Treatment as usual included support and advice by general practitioners or community child development teams. Our primary outcome was parent-reported child behaviour at 12 months after randomisation. We also collected data on other outcomes and carried out interviews with parents, service managers and therapists to find out their views about Stepping Stones Triple P. We did not find that Stepping Stones Triple P reduces behaviours that challenge in the child more than treatment as usual at 12 months. However, when we looked at people who received more than half of the sessions, there was a larger reduction in behaviours which suggests that Stepping Stones Triple P works for families if they attend the full programme. Stepping Stones Triple P seems to be good value for money, as we found that at 12 months (covering 10 months of costs), the Stepping Stones Triple P cost £1058 less than treatment as usual from a health and social care perspective. As such, Stepping Stones Triple P is fairly cheap to deliver and a suitable early intervention for behaviours that challenge especially because of positive feedback from parents. Throughout the trial, we included a Parent Advisory Group that oversaw study materials, interview topic guides and promotion of the study.

The effectiveness of parent-Child observation in parent-Mediated programmes for children with developmental disabilities and externalizing disorders: A systematic review and meta-analysis.

This systematic review evaluated the treatment effects of communication-focused parent-mediated interventions (CF-PMT), a form of intervention that involves therapists observing parent-child interactions and giving feedback to parents on how they can practice positive parenting strategies to prevent or reduce externalizing behaviours in children with developmental disabilities. A literature search was conducted on three electronic databases. To be included in the review, studies had to: evaluate CF-PMT where therapists give feedback after observing parent-child interactions; examine changes in externalizing behaviours amongst children with any forms of developmental disability; and adopt a randomised controlled trial study design. Fifteen studies met eligible criteria for the literature review, of those, 13 studies had available data on changes in the primary (child externalizing behaviours) and secondary outcomes (parental stress, child linguistic abilities and child social responsiveness). We found significant treatment effects for CF-PMT in reducing child externalizing behaviours (d = -.60) but not for any of the secondary outcomes. A sensitivity analysis showed a small but significant treatment effect for parental stress (d = -.18). Considerable bias was observed due to the lack of available information reported by studies on aspects measured by the Mixed Methods Appraisal Tool. Overall, we found evidence to support the benefits of complex interventions which incorporate direct parent-child observations and feedback to improve behavioural outcomes amongst children with developmental disabilities.

Stakeholder views on the barriers and facilitators of psychosocial interventions to address reduction in aggressive challenging behaviour in adults with intellectual disabilities.

Background: Success of psychosocial interventions in reducing aggressive challenging behaviour is likely to be related not only to mechanistic aspects, but also to therapeutic and system factors. The study aims to examine the facilitators and barriers that influence whether psychosocial interventions for aggressive challenging behaviour in adults with intellectual disabilities lead to positive change. Methods: We conducted 42 semi-structured interviews with adults with intellectual disabilities and aggressive challenging behaviour, family/paid carers, and professionals engaged in or delivering a psychosocial intervention across the UK. Data were analysed thematically using a framework approach. Results: Stakeholders considered therapeutic and supportive relationships and personalised care as facilitating factors of psychosocial interventions to address aggressive challenging behaviour. The operational structure of community intellectual disability services and conflicting expectations of professionals and carers were the main contextual barriers that impeded the implementation of psychosocial interventions addressing aggressive challenging behaviour in adults with intellectual disabilities. Conclusions: Findings highlight the valued components that maximise positive change in adults with intellectual disabilities who display aggressive challenging behaviour. Several operational adjustments including referral criteria, roles of professionals and workforce issues need to be addressed in services to maximise the implementation of psychosocial interventions to reduce aggressive challenging behaviour in adults with intellectual disabilities.

The current study looked at what factors that lead to good outcomes from therapies that aim to reduce aggressive challenging behaviour in adults with learning disabilities. We also looked at the factors that may prevent positive change ( i.e., reduction in aggressive challenging behaviour). We interviewed 42 people including 14 adults with learning disabilities, 13 carers (6 family; 7 paid) and 13 professionals across the UK. All stakeholders agreed that supportive relationships between the person with a learning disability and their therapist, staff consistent, personalised therapy and involvement of the person with a learning disability and their carer(s) in care planning facilitate the reduction of aggressive challenging behaviour. On the other hand, differences in expectations between professionals and carers, personal reasons ( e.g. being the right timing for a person), and the way community learning disability services currently run may stop people from having the opportunity to take part in therapy are barriers to positive change such as reduction of aggressive challenging behaviour. The study findings highlight the valued components that maximise the positive change in adults with intellectual disabilities who display aggressive challenging behaviour. Several operational adjustments including referral criteria, roles of professionals and workforce issues need to be addressed in services to maximise the implementation of psychosocial interventions to reduce aggressive challenging behaviour in adults with intellectual disabilities.

Complex interventions for aggressive challenging behaviour in adults with intellectual disability: A rapid realist review informed by multiple populations.

OBJECTIVES: Approximately 10% of people with intellectual disability display aggressive challenging behaviour, usually due to unmet needs. There are a variety of interventions available, yet a scarcity of understanding about what mechanisms contribute to successful interventions. We explored how complex interventions for aggressive challenging behaviour work in practice and what works for whom by developing programme theories through contexts-mechanism-outcome configurations. METHODS: This review followed modified rapid realist review methodology and RAMESES-II standards. Eligible papers reported on a range of population groups (intellectual disability, mental health, dementia, young people and adults) and settings (community and inpatient) to broaden the scope and available data for review. RESULTS: Five databases and grey literature were searched and a total of 59 studies were included. We developed three overarching domains comprising of 11 contexts-mechanism-outcome configurations; 1. Working with the person displaying aggressive challenging behaviour, 2. Relationships and team focused approaches and 3. Sustaining and embedding facilitating factors at team and systems levels. Mechanisms underlying the successful application of interventions included improving understanding, addressing unmet need, developing positive skills, enhancing carer compassion, and boosting staff self-efficacy and motivation. CONCLUSION: The review emphasises how interventions for aggressive challenging behaviour should be personalised and tailored to suit individual needs. Effective communication and trusting relationships between service users, carers, professionals, and within staff teams is essential to facilitate effective intervention delivery. Carer inclusion and service level buy-in supports the attainment of desired outcomes. Implications for policy, clinical practice and future directions are discussed. PROSPERO REGISTRATION NUMBER: CRD42020203055.

Divergent presentation of anxiety in high-risk groups within the intellectual disability population.

BACKGROUND: Anxiety symptomatology is common in individuals with intellectual disability (ID). Symptomatology includes both traditional Diagnostic and Statistical Manual of Mental Disorders, 5th Edition (DSM-5) anxiety disorders and autism spectrum disorder (ASD)-related anxiety traits. Some genetic disorders such as Cornelia de Lange (CdLS) and fragile X syndromes (FXS) are at very high risk of anxiety and afford the opportunity to examine prevalence, profiles and associated person characteristics. However, prevalence and associated characteristics of anxiety in these high-risk groups remain poorly described and understood. The aim of the current study was to examine the prevalence and profile of DSM-5 and ASD-related anxiety symptomatology in individuals with CdLS and FXS and associated behavioural and cognitive characteristics. METHODS: Questionnaires and interviews assessing DSM-5 and ASD-related anxiety were conducted with caregivers of individuals with CdLS (n = 49) and FXS (n = 36). RESULTS: DSM-5 anxiety symptomatology was present in both groups with high co-morbidity across anxiety diagnoses. ASD-related anxiety was also prevalent with specific difficulties related to intolerance of uncertainty identified in both groups. Symptomatology was persistent over the lifespan for both groups. Anxiety type was partially associated with repetitive behaviour but not measures of overall ASD phenomenology in CdLS. CONCLUSIONS: DSM-5 and ASD-related anxiety are common in these high-risk syndromes associated with ID. Prospective syndrome specific presentations and associations, which may implicate specific underlying mechanisms, are discussed. Clinicians should be aware of the risk and difficulties involved in assessment of anxiety in individuals with ID, including atypical types, to ensure these individuals do not "miss" diagnoses and support in general clinical practice.

Prevalence of anxiety symptomatology and diagnosis in syndromic intellectual disability: A systematic review and meta-analysis.

Individuals with syndromic intellectual disability are at increased risk of experiencing anxiety. Comparing prevalence estimates of anxiety will allow the identification of at-risk groups and inform causal pathways of anxiety. No known study has explored estimates of anxiety symptomatology and diagnosis, including specific anxiety profiles, across groups whilst accounting for methodological quality of studies. This systematic review and meta-analysis aimed to fill this gap. Prior to review completion, methodology and analysis plans were registered and documented in a protocol (CRD42019123561). Data from 83 papers, involving a pooled sample of 13,708 across eight syndromes were synthesised using a random effects model. Anxiety prevalence ranged from 9 % (95 % CI: 4-14) in Down syndrome to 73% in Rett syndrome (95 % CI: 70-77). Anxiety prevalence across syndromic intellectual disability was higher than for intellectual disability of mixed aetiology and general population estimates. Substantial variability between syndromes identified groups at higher risk than others. The identification of high-risk groups is crucial for early intervention, allowing us to refine models of risk and identify divergent profiles.

Anxiety characteristics in individuals with Williams syndrome.

BACKGROUND: Williams syndrome anxiety research predominantly focuses on disorder prevalence and symptomatology, categorised using standardised mental health classifications. However, the use of these assessments may not fully capture the phenotypic features of anxiety in Williams syndrome. In this study, we examined characteristics of anxiety using a formulation framework. METHOD: A semi-structured interview was conducted with thirteen parents of individuals with Williams syndrome (median age: 19, age range: 12-45, 8 females). RESULTS: Various anxiety triggers were reported, including anxiety triggered by phobias, uncertainty and negative emotions in others. The range of described behaviours was diverse with both avoidant and active coping strategies for anxiety management reported. CONCLUSIONS: Many of the characteristics described were consistent with findings in the intellectual disability and typically developing literature, although novel information was identified. The study demonstrates the utility of a formulation framework to explore anxiety characteristics in atypical populations and has outlined new avenues for research.

Clinical and cost effectiveness of a parent mediated intervention to reduce challenging behaviour in pre-schoolers with moderate to severe intellectual disability (EPICC-ID) study protocol: a multi-centre, parallel-group randomised controlled trial.

BACKGROUND: Children with intellectual disabilities are likely to present with challenging behaviour. Parent mediated interventions have shown utility in influencing child behaviour, although there is a paucity of UK research into challenging behaviour interventions in this population. NICE guidelines favour Stepping Stones Triple P (SSTP) as a challenging behaviour intervention and this trial aims to evaluate its clinical and cost effectiveness in preschool children with moderate to severe intellectual disabilities. METHODS: This trial launched in 2017 at four sites across England, with the aim of recruiting 258 participants (aged 30-59 months). The Intervention Group receive nine weeks of SSTP parenting therapy (six group sessions and three individualised face to face or telephone sessions) in addition to Treatment as Usual, whilst the Treatment as Usual only group receive other available services in each location. Both study groups undergo the study measurements at baseline and at four and twelve months. Outcome measures include parent reports and structured observations of behaviour. Service use and health related quality of life data will also be collected to carry out a cost effectiveness and utility evaluation. DISCUSSION: Findings from this study will inform policy regarding interventions for challenging behaviour in young children with moderate to severe intellectual disabilities. TRIAL REGISTRATION NUMBER: Clinicaltrials.gov, NCT03086876. Registered 22nd March 2017, https://clinicaltrials.gov/ct2/show/NCT03086876.

Communication in Angelman syndrome: a scoping review.

AIM: A scoping review was conducted to examine and evaluate empirical data on the communication profile of Angelman syndrome beyond the described dissociation between receptive language and speech. METHOD: Three databases (PsycINFO, Embase, and Web of Science) were searched to retrieve articles investigating communication in Angelman syndrome. Seventeen articles investigating the broader communication profile were found; their methodology was evaluated against quality criteria. RESULTS: Despite the absence of speech, individuals with Angelman syndrome have a wide repertoire of non-verbal communicative behaviours, mainly characterized by gestures, although advanced forms such as symbolic communication are used by some individuals. The use of communicative forms differs between the genetic aetiologies of Angelman syndrome; individuals with non-deletion aetiologies typically have greater communicative abilities. INTERPRETATION: The broader communication profile of Angelman syndrome is characterized by diverse and multimodal abilities, including some use of symbolic forms of communication that appears atypical given the absence of speech. This is suggestive of a probable dissociation between speech and other expressive forms of communication, indicating an isolated speech production impairment. This highlights a need in this population for alternative communication and specific input from services tailored to support the nuances of the communication profile of Angelman syndrome. WHAT THIS PAPER ADDS: Although absent speech is near universal, a diverse profile of other communicative abilities has been reported. Parental reporting has been predominantly used to assess the communication profile of Angelman syndrome. Literature that investigates the specificities and possible dissociations in such a communication profile is limited.

Williams syndrome: recent advances in our understanding of cognitive, social and psychological functioning.

PURPOSE OF REVIEW: Since the last review of Williams syndrome in Current Opinion (2001) there have been many advances in knowledge about the cognitive, social and psychological impairments that characterize the disorder. The present review focuses on current research in these areas. RECENT FINDINGS: Williams syndrome is associated with a wide range of cognitive, linguistic, social and other difficulties. When young, these deficits may appear relatively mild - for example, many children are highly sociable and talkative - but with age the impact of these difficulties becomes more evident. Thus, inappropriate social behaviours can significantly increase the risk of social exclusion and vulnerability to abuse. Their superficially good speech can lead to educational and other services failing to understand the true extent of impairments or the need for specialist support. Mental health problems, especially related to anxiety, often become an increasing challenge from adolescence onwards. SUMMARY: The core difficulties associated with Williams syndrome have a cascading effect on many areas of development over time. However, specialist provision is rare and intervention trials are almost nonexistent. Longitudinal research is needed to identify factors associated with cognitive, social and emotional problems and to develop more effective ways of minimizing and treating difficulties.

A Multifaceted Quality Improvement Programme to Improve Acute Kidney Injury Care and Outcomes in a Large Teaching Hospital.

Acute kidney injury (AKI) is now widely recognised as a serious health care issue, occurring in up to 25% of hospital in-patients, often with worsening of outcomes. There have been several reports of substandard care in AKI. This quality improvement (QI) programme aimed to improve AKI care and outcomes in a large teaching hospital. Areas of documented poor AKI care were identified and specific improvement activities implemented through sequential Plan-Do-Study-Act (PDSA) cycles. An electronic alert system (e-alert) for AKI was developed, a Priority Care Checklist (PCC) was tested with the aid of specialist nurses whilst targeted education activities were carried out and data on care processes and outcomes monitored. The e-alert had a sensitivity of 99% for the detection of new cases of AKI. Key aspects of the PCC saw significant improvements in their attainment: Detection of AKI within 24 hours from 53% to 100%, fluid assessment from 42% to 90%, drug review 48% to 95% and adherence to nine key aspects of care from 40% to 90%. There was a significant reduction in variability of delivered AKI care. AKI incidence reduced from 9% of all hospitalisations at baseline to 6.5% (28% reduction), AKI related length of stay reduced from 22.1 days to 17 days (23% reduction) and time to recovery (AKI days) 15.5 to 9.8 days (36% reduction). AKI related deaths also showed a trend towards reduction, from an average of 38 deaths to 34 (10.5%). The number of cases of hospital acquired AKI were reduced by 28% from 120 to 86 per month. This study demonstrates significant improvements related to a QI programme combining e-alerts, a checklist implemented by a nurse and education in improving key processes of care. This resulted in sustained improvement in key patient outcomes.

Palliative care for patients with mesothelioma.

Mesothelioma is a highly symptomatic aggressive cancer affecting quantity and quality of life. Excellent palliative care is essential from diagnosis. This article discusses commonly encountered issues and management strategies including the rationale for early referral to specialist palliative care.