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.

Being a child with intellectual disabilities in hospital: The need for an individualised approach to care.

BACKGROUND: There is limited qualitative research focussed specifically on what it is like for children and young people with intellectual disabilities coming into hospital, with much of the evidence-base being about those with Autism Spectrum Condition or adults with intellectual disabilities. AIM: To share rich detail of the emotional and physical impact on children and young people with intellectual disabilities of attending hospital, from their own and their parent's perspective. METHODS: Talking Mats interviews, sticker survey and photography with children and young people with intellectual disabilities, and in-depth interviews, hospital diaries and photography with their parents. RESULTS AND CONCLUSIONS: The multiple and compounding layers of complexity surrounding hospital care of children and young people with intellectual disabilities resulted in challenges associated with loss of familiarity and routine, undergoing procedures, managing sensory overload, managing pain and having a lack of safety awareness. An individualised approach to their care is needed.

Talking with children and young people with 22q11DS about their mental health, behaviour, learning and communication.

BACKGROUND: 22q11DS11.2 deletion syndrome (22q11DS) is a complex multisystem syndrome characterized by physical abnormalities, psychiatric comorbidities and cognitive deficits. The views of children and young people (CYP) about the challenges associated with their mental health, behaviour, learning and communication difficulties have not been reported. The aim of this study was to address this gap and to understand whether they had help and support with these and their views of this. METHODS: A three-phase mixed-methods study was undertaken, involving interviews with CYP with 22q11DS, a follow-up survey for those aged 11-25 years and a stakeholder workshop at which CYP presented their views of living with 22q11DS to health professionals and parents. Interview transcripts were thematically analysed, and non-parametric statistics were used to analyse survey data. RESULTS: The interviews (n = 13) and survey (n = 32) indicated a mixed picture, with some CYP not reporting ongoing problems; others who had problems received help but a sizeable proportion had unmet needs and wanted to receive help. Two-thirds reported often experiencing negative feelings, and almost half had difficulties with social interactions. Family members were the main sources of support, with teaching assistants identified as an important support at school. CONCLUSIONS: The emotional impact of 22q11DS on CYP can be significant: They often do not understand the consequences of having 22q11DS and are frequently not given strategies to understand and manage their feelings, behaviour or problems. This leads to a range of emotions that manifest in different ways at home and at school. CYP are able to talk about the impact of different aspects of 22q11DS on them, facilitated by the use of creative methods, but they differ in how the condition affects them and their perceptions about that. It is imperative that CYP themselves are asked about their experiences, feelings and needs to ensure tailoring of interventions to their individual requirements.

'Wanting no regrets': Parental decision making around selective dorsal rhizotomy.

BACKGROUND: Selective dorsal rhizotomy (SDR) is an irreversible neurosurgical procedure used to reduce spasticity while aiming to improve gross motor function for children with cerebral palsy (CP). Little research has explored the complexity of parental decision-making experiences surrounding SDR surgery and required rehabilitation. The aim was to explore parental experiences and expectation in the decision-making process around the SDR operation, physiotherapy treatment and outcomes. METHOD: Qualitative methods using in-depth, semi-structured interviews were used. Eighteen parents (11 mothers and 7 fathers) participated whose child had SDR and completed 2-year post-operative rehabilitation. Data were analysed using thematic analysis. RESULTS: Decision making involved an interacting process between the 'parental information seeking experience', 'influence of professional encounters and relationships' and 'emotional and social aspects'. Despite underlying uncertainties about outcomes, parental drivers of expectations and aspirations for their child preceded and sustained this decision-making process. A foundational narrative of 'wanting no regrets' resolved their decision to undertake SDR. Outcomes did not always match expectations, and parents moved away from a position of idealism, which was driven in part by parental information seeking including overly optimistic media representation. CONCLUSION: Universally, parents expressed they had 'no regrets' on their decision, yet many felt a mismatch between expectation and outcomes of SDR surgery. A greater understanding of the complex nature of SDR decision making is required to help improve family preparedness and provide support from clinicians to enable balanced discussions in parental decision-making.

Acceptability of a novel device to improve child patient experience during venepuncture for blood sampling: Intervention with 'MyShield'.

This study aimed to explore the acceptability of a novel device ('MyShield'): a device used for distraction during clinical procedures. It is a cardboard cuff, designed to fit around the arm, either above the elbow or around the wrist and used to hide the procedure from view. This device was tested in practice, to establish acceptability to children, parents and clinical staff. Fifty-eight children tried 'MyShield' during a venepuncture procedure. Feedback from 54 children, 58 parents/carers and 16 clinical staff was collected using surveys and interviews. In 24 cases, observational data were also collected. A large majority of children (94%, n = 51) and parents (96%, n = 56) reported a positive experience when using 'MyShield'; saying they would likely use it again. Potential of 'MyShield' in promoting parent/clinician interaction with the child was highlighted. Data suggests that 'MyShield' may be a useful device for children undergoing venepuncture, when used in conjunction with standard care, and subject to individual preferences and choice. Further work is required to establish mechanism of action and whether use of 'MyShield' has any impact across a range of short- and long-term outcome measures relating to patient experience and effectiveness.

Carer preferences of route of administration of transmucosal diamorphine and willingness to take part in a randomised controlled trial: an interview study (DIPPER).

BACKGROUND: Children and young people are usually given liquid morphine by mouth for breakthrough pain, which can take thirty minutes to work. A faster-acting, quickly absorbed, needle-free pain medicine, that is easy to administer is needed such as transmucosal (sublingual, buccal, intranasal) diamorphine. Research evidence relating to the administration of medication for breakthrough pain in children and young people is limited. This study aims to describe the experiences and preferences of parents and/or children and young people regarding the route of administration of diamorphine, barriers and facilitators comparative to oral morphine, and participation in a randomised controlled trial. METHODS: In-depth, semi-structured interviews with parents and/or children and young people at home or hospital/hospice. RESULTS: Thirteen interviews with: nine mothers, one father, and three sets of parents jointly. No interviews took place with a child/young person. Most families had experience of the buccal route which was effective in ease of administration and time to control pain. The intranasal route was preferred by parents irrespective of experience. Parents' willingness for their child to take part in a trial depended on the time commitment, their child's pain trajectory and the stability of analgesic requirements. CONCLUSION: A randomised controlled trial of oral morphine versus transmucosal diamorphine would need to consider trial logistics, especially time commitment. Parents felt that the trial should be introduced initially by the clinical team, with written information from the research team, and sufficient time to ask questions. Patients who had discontinued oral morphine because of side effects, or those with gastrointestinal failure, should be excluded. Maintaining stability in pain management was essential to families, so the timing of the trial is a potential issue.

Diamorphine pharmacokinetics and conversion factor estimates for intranasal diamorphine in paediatric breakthrough pain:systematic review.

BACKGROUND: Intranasal diamorphine is a potential treatment for breakthrough pain but few paediatric data are available to assist dose estimation. AIM: To determine an intranasal diamorphine dose in children through an understanding of pharmacokinetics. DESIGN: A systematic review of the literature was undertaken to seek diamorphine pharmacokinetic parameters in neonates, children and adults. Parenteral and enteral diamorphine bioavailability were reviewed with respect to formation of the major metabolite, morphine. Clinical data quantifying equianalgesic effects of diamorphine and morphine were reviewed. REVIEW SOURCES: PubMed (1960-2020); EMBASE (1980-2020); IPA (1973-2020) and original human research studies that reported diacetylmorphine and metabolite after any dose or route of administration. RESULTS: The systematic review identified 19 studies: 16 in adults and 1 in children and 2 neonatal reports. Details of study participants were extracted. Age ranged from premature neonates to 67 years and weight 1.4-88 kg. Intranasal diamorphine bioavailability was predicted as 50%. The equianalgesic intravenous conversion ratio of morphine:diamorphine was 2:1. There was heterogeneity between pharmacokinetic parameter estimates attributed to routes of administration, lack of size standardisation, methodology and pharmacokinetic analysis. Estimates of the pharmacokinetic parameters clearance and volume of distribution were reduced in neonates. There were insufficient paediatric data to characterise clearance or volume maturation of either diamorphine or its metabolites. CONCLUSIONS: We estimate equianalgesic ratios of intravenous morphine:diamorphine 2:1, intravenous morphine:intranasal diamorphine 1:1 and oral morphine:intranasal diamorphine of 1:3. These ratios are based on adult literature, but are reasonable for deciding on an initial dose of 0.1 mg/kg in children 4-13 years.

The experiences and perceptions of users of an electronic patient record system in a pediatric hospital setting: a systematic review.

CONTEXT: With the ever-increasing need for digital health innovations, the transition to electronic patient records (EPR) is an integral part of the digital health revolution. There is an increasing body of literature on EPR use in the adult hospital setting, particularly related to adoption as a result of financial incentives in the United States (US). The experiences and perceptions of EPR users within the pediatric hospital setting are less well understood, despite the advent of patient portals accessible by children and young people (CYP) and their parents, which bring new benefits and challenges for them and healthcare professionals alike. OBJECTIVES: The aim of this review was to understand the experiences and perceptions of all relevant stakeholders using an EPR system in the pediatric hospital setting, including the use of an EPR-linked patient portal. METHODS: Studies were identified through electronic database and citation searching, following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. A systematic search was conducted in EMBASE, EMCARE, MEDLINE, Cochrane Library, Web of Science, Scopus, CINAHL, and PsycINFO electronic databases to identify literature published 2010-present, in addition to manual searching of conference abstracts, and research reports searched via the Health Research Authority website. Inclusion criteria were studies reporting on an EPR system in use in hospital setting where child patients (from 0 to 17.9 years) are cared for, with or without an EPR-linked patient portal. RESULTS: Thirty-six out of 42,946 articles were eligible for inclusion, almost two-thirds were studies conducted in the US. A wide range of benefits, challenges, and information and support needs were reported. Strategies for successful implementation and design improvements were suggested, as was desirable portal functionality and parental intention to use, or reasons for not using the portal. Several ethical and legal issues were raised. CONCLUSIONS: Experiences of using EPRs and patient portals were wide-ranging with challenges more prevalent soon after implementation. Although tailoring information and support to users' individual needs and practice context can be complex, this is essential to enable prolonged utility, user satisfaction and engagement, which, in turn, will promote effective care provision. Disease-specific portals may increase utility, and taking into consideration children's and young people's needs and preferences is essential.

Pain assessment tools in paediatric palliative care: A systematic review of psychometric properties and recommendations for clinical practice.

BACKGROUND: Assessing pain in infants, children and young people with life-limiting conditions remains a challenge due to diverse patient conditions, types of pain and often a reduced ability or inability of patients to communicate verbally. AIM: To systematically identify pain assessment tools that are currently used in paediatric palliative care and examine their psychometric properties and feasibility and make recommendations for clinical practice. DESIGN: A systematic literature review and evaluation of psychometric properties of pain assessment tools of original peer-reviewed research published from inception of data sources to April 2021. DATA SOURCES: PsycINFO via ProQuest, Web of Science Core, Medline via Ovid, EMBASE, BIOSIS and CINAHL were searched from inception to April 2021. Hand searches of reference lists of included studies and relevant reviews were performed. RESULTS: From 1168 articles identified, 201 papers were selected for full-text assessment. Thirty-four articles met the eligibility criteria and we examined the psychometric properties of 22 pain assessment tools. Overall, the Faces Pain Scale-Revised (FPS-R) had high cross-cultural validity, construct validity (hypothesis testing) and responsiveness; while the Faces, Legs, Activity, Cry and Consolability (FLACC) scale and Paediatric Pain Profile (PPP) had high internal consistency, criterion validity, reliability and responsiveness. The number of studies per psychometric property of each pain assessment tool was limited and the methodological quality of included studies was low. CONCLUSION: Balancing aspects of feasibility and psychometric properties, the FPS-R is recommended for self-assessment, and the FLACC scale/FLACC Revised and PPP are the recommended observational tools in their respective age groups.

Culture, cognisance, capacity and capability: The interrelationship of individual and organisational factors in developing a research hospital.

AIM: To share our experience of implementing a programme of interventions aimed at building research capacity and capability of nurses and allied health professionals in a specialist children's hospital. BACKGROUND: Clinicians at the forefront of care are well positioned to lead on research to improve outcomes and experiences of patients but some professional groups continue to be underrepresented. Inequities persist alongside robust national infrastructures to support Clinical Academic Careers for non-medical health professionals, further highlighting the need to address local infrastructure and leadership to successfully build research capacity. DESIGN: An evolving programme of inquiry and analysis was established in one organisation, this included targeted interventions to mitigate barriers and enable research capacity and capability. METHODS: An all-staff survey was conducted in 2015 to understand the existing research culture. Interventions were put in place, evaluated through a second survey (2018), and focus group interviews with staff who had accessed interventions. RESULTS: Respondents demonstrated high levels of interest and commitment to research at the individual level which were not always harnessed at the organisational level. Inequities between professional groups existed in terms of training, time to undertake research and opportunities and outputs. Follow-up revealed continuing structural barriers at an organisational level, however at an individual level, interventions were reflected in >30 fellowship awards; major concerns were reported about sustaining these research ambitions. CONCLUSIONS: Success in building a research-active clinical workforce is multifactorial and all professional groups report increasing challenges to undertake research alongside clinical responsibilities. Individuals report concerns about the depth and pace of cultural change to sustain Clinical Academic Careers and build a truly organisation-wide research hospital ethos to benefit patients. RELEVANCE TO CLINICAL PRACTICE: The achievements of individual nurses and allied health professionals indicate that with supportive infrastructure, capacity, cognisance and capability are not insurmountable barriers for determined clinicians. We use the standards for reporting organisational case studies to report our findings (Rodgers et al., 2016 Health Services and Delivery Research, 4 and 1).

Oral morphine versus transmucosal diamorphine for breakthrough pain in children: methods and outcomes: UK (DIPPER study) consensus.

OBJECTIVES: No randomised controlled trials have been conducted for breakthrough pain in paediatric palliative care and there are currently no standardised outcome measures. The DIPPER study aims to establish the feasibility of conducting a prospective randomised controlled trial comparing oral and transmucosal administration of opioids for breakthrough pain. The aim of the current study was to achieve consensus on design aspects for a small-scale prospective study to inform a future randomised controlled trial of oral morphine, the current first-line treatment, versus transmucosal diamorphine. METHODS: The nominal group technique was used to achieve consensus on best practice for mode of administration, dose regimen and a range of suitable pain intensity outcome measures for transmucosal diamorphine in children and young people with breakthrough pain. An expert panel of ten clinicians in paediatric palliative care and three parent representatives participated. Consensus was achieved when agreement was reached and no further comments from participants were forthcoming. RESULTS: The panel favoured the buccal route of administration, with dosing according to the recommendations in the Association for Paediatric Palliative Medicine formulary (fifth Edition, 2020). The verbal Numerical Rating Scale was selected to measure pain in children 8 years old and older, the Faces Pain Scale-Revised for children between 4 and 8 years old, and Face, Legs, Activity, Cry and Consolability (FLACC)/FLACC-Revised as the observational tools. CONCLUSIONS: The nominal group technique allowed consensus to be reached for a small-scale, prospective, cohort study and provided information to inform the design of a randomised controlled trial.

Healthcare professionals' views of the use of oral morphine and transmucosal diamorphine in the management of paediatric breakthrough pain and the feasibility of a randomised controlled trial: A focus group study (DIPPER).

BACKGROUND: Oral morphine is frequently used for breakthrough pain but the oral route is not always available and absorption is slow. Transmucosal diamorphine is administered by buccal, sublingual or intranasal routes, and rapidly absorbed. AIM: To explore the perspectives of healthcare professionals in the UK caring for children with life-limiting conditions concerning the assessment and management of breakthrough pain; prescribing and administration of transmucosal diamorphine compared with oral morphine; and the feasibility of a comparative clinical trial. DESIGN/ PARTICIPANTS: Three focus groups, analysed using a Framework approach. Doctors, nurses and pharmacists (n = 28), caring for children with life-limiting illnesses receiving palliative care, participated. RESULTS: Oral morphine is frequently used for breakthrough pain across all settings; with transmucosal diamorphine largely limited to use in hospices or given by community nurses, predominantly buccally. Perceived advantages of oral morphine included confidence in its use with no requirement for specific training; disadvantages included tolerability issues, slow onset, unpredictable response and unsuitability for patients with gastrointestinal failure. Perceived advantages of transmucosal diamorphine were quick onset and easy administration; barriers included lack of licensed preparations and prescribing guidance with fears over accountability of prescribers, and potential issues with availability, preparation and palatability. Factors potentially affecting recruitment to a trial were patient suitability and onerousness for families, trial design and logistics, staff time and clinician engagement. CONCLUSIONS: There were perceived advantages to transmucosal diamorphine, but there is a need for access to a safe preparation. A clinical trial would be feasible provided barriers were overcome.

Protocol for The Toxin Study: Understanding clinical and patient reported response of children and young people with cerebral palsy to intramuscular lower limb Botulinum neurotoxin-A injections, exploring all domains of the ICF. A pragmatic longitudinal observational study using a prospective one-group repeated measures design.

INTRODUCTION: Botulinum neurotoxin-A (BoNT-A) is an accepted treatment modality for the management of hypertonia in children and young people with cerebral palsy (CYPwCP). Nevertheless, there are concerns about the long-term effects of BoNT-A, with a lack of consensus regarding the most meaningful outcome measures to guide its use. Most evidence to date is based on short-term outcomes, related to changes at impairment level (restrictions of body functions and structures), rather than changes in adaptive skills (enabling both activity and participation). The proposed study aims to evaluate clinical and patient reported outcomes in ambulant CYPwCP receiving lower limb BoNT-A injections over a 12-month period within all domains of the WHO's International Classification of Functioning, Disability and Health and health-related quality of life (HRQoL). METHODS AND ANALYSIS: This pragmatic prospective longitudinal observational study will use a one-group repeated measures design. Sixty CYPwCP, classified as Gross Motor Function Classification System (GMFCS) levels I-III, aged between 4 and 18 years, will be recruited from an established movement disorder service in London, UK. Standardised clinical and patient reported outcome measures within all ICF domains; body structures and function, activity (including quality of movement), goal attainment, participation and HRQoL, will be collected preinjection and at 6 weeks, 6 months and up to 12 months postinjection. A representative subgroup of children and carers will participate in a qualitative component of the study, exploring how their experience of BoNT-A treatment relates to clinical outcome measures. ETHICS AND DISSEMINATION: Central London Research Ethics Committee has granted ethics approval (#IRAS 211617 #REC 17/LO/0579). Findings will be disseminated in peer-reviewed publications, conferences and via networks to participants and relevant stakeholders using a variety of accessible formats including social media.

'We don't know what tomorrow will bring': Parents' experiences of caring for a child with an undiagnosed genetic condition.

BACKGROUND: Families and healthcare professionals caring for a sick or disabled child without a definitive diagnosis face unique challenges, particularly in relation to managing uncertainty, access to healthcare and coordination of care. There has been little research exploring the impact this has on families, their support needs or their experience of health services. METHODS: This qualitative interview study included interviews with 14 mothers of children with undiagnosed genetic conditions. Transcripts were analysed using thematic analysis. RESULTS: Four themes emerged, uncovering overlapping patterns in the data: (1) living with complexity amidst uncertainty-'We don't know what tomorrow will bring'; (2) parental role-'I do everything I can'; (3) parental role-'Not coping is not an option'; and (4) support needs-'There's lots of help that just isn't out there'. CONCLUSIONS: The results clearly demonstrate the stresses faced when caring for a child with an undiagnosed genetic condition. Some themes are shared with the experience of other families caring for children with complex needs. However, parents were doing all they could for their child in the context of a life of uncertainty, with the absence of a clear diagnosis clearly causing additional stress that impacted on the whole family. Impact on their emotional and physical well-being was evident; they described times of feeling stressed, worried and anxious. They were confused due to being overloaded with information and frustrated by a lack of care coordination. Parents did not appear to prioritize their own well-being and held back their emotions to protect themselves and others. As a result, they had many unmet needs, particularly relating to emotional support.

Supporting families of children with an undiagnosed genetic condition: Using co-design to ensure the right person is in the right post doing the right job.

BACKGROUND: Families and professionals caring for a child without a definitive diagnosis face unique challenges, particularly in relation to managing uncertainty; access to healthcare; obtaining relevant information and support; and trying to navigate a healthcare system that is often fragmented. We used co-design to inform the establishment of the first UK specialist nursing post dedicated to working with children with undiagnosed genetic conditions and their families. OBJECTIVES: (1) To understand what families and hospital staff want from the service; (2) To understand how the post should be operationalized in practice; (3) To develop the job description and person specification for the postholder. METHODS: A range of approaches were used to collect data: interviews (nine parents and 10 hospital staff); a focus group (three parents); a creative workshop (six patients and siblings); and an online forum (81 parents). Data were analyzed using framework and thematic analysis. The strands of data were brought together and reviewed as a whole to formulate the postholder's job description and person specification. RESULTS: Stakeholders identified nine key elements to the role which were incorporated into the job description: practical support; point of contact; community liaison; signposting to other services; care coordination; supporting families; advocacy; raising awareness; and emotional support. CONCLUSIONS: Highlighted in this paper are the practical aspects of engaging and involving all relevant stakeholders in the process of co-designing a new post and subsequent staff recruitment. The flexibility employed in the setting and methods of data collection were instrumental in ensuring that the views of a diverse range of participants were ascertained. A major consideration is the resources required to undertake co-design, in terms of time and finances. We believe that the resources required for the co-design are offset by the advantages of having the right person in the right post, doing the right job.

Hospitalized children with intellectual disability: Parents as partners in their care.

BACKGROUND: Limited evidence exists of what hospital care is like for parents of children and young people (CYP) with intellectual disability (ID). Effective collaboration is often missing, with parents lacking trust in professionals, with feelings of being expected to care and consequently unable to leave their child. This paper focuses on what parents want from their relationship with healthcare professionals. METHOD: An ethnographic approach was used, including in-depth interviews and informal discussions with nine parents of CYP with intellectual disability. Researcher voice poems are interspersed with quotations to illustrate key findings. RESULTS: Parents described the need for a genuine partnership with professionals. They identified seven elements that ideally characterize this partnership: Preparation, Accessibility, Reliability, Trust, Negotiation, Expertise and Respect (PARTNER). CONCLUSION: Children and young people with intellectual disability and their parents are frequent users of healthcare services. Getting it right from the outset is important to establish their trust in the system.

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.

Missed opportunities: incomplete and inaccurate recording of paediatric early warning scores.

BACKGROUND: Paediatric early warning scores (PEWS) are widely used as an adjunct to support staff in recognising deterioration in hospitalised children. Relatively little is known about how staff use these systems. OBJECTIVE: To examine the completeness and accuracy of PEWS recording in hospitalised children in a tertiary specialist children's hospital. DESIGN: This is a secondary analysis of retrospective, case-controlled study data. Case patients suffering from a critical deterioration event were matched with controls present on the same ward at the same time and matched for age. Data were extracted from the PEWS chart for the 48 hours before the critical deterioration event for case patients and the corresponding 48 hours period for the control. Observation sets were assessed for completeness and accuracy of PEWS scoring. RESULTS: In total 297 case events in 224 patients were available for analysis. Overall 13 816 observations sets were performed, 8543 on cases and 5273 on controls. Only 4958 (35.9%) of observation sets contained a complete set of vital sign parameters and a concurrent PEWS. Errors were more prevalent in the observation sets of case patients versus controls (19.5% vs 14.1%). More errors resulted in the PEWS value being underscored rather than overscored for all observation sets (p<0.0001). 9.1% of inaccuracies for case patients were clinically significant, as the accurately calculated PEWS would have prompted a different escalation from the documented value. CONCLUSION: Failure to record complete and accurate PEWS may jeopardise recognition of children who are deteriorating. Technology may offer an effective solution.