The epidemiology of epidermolysis bullosa in England and Wales: data from the national epidermolysis bullosa database.

BACKGROUND: The National Health Service (NHS) epidermolysis bullosa (EB) service, established in 2002, offers comprehensive, free care to all patients in England and Wales. OBJECTIVES: To quantify prevalence, incidence and mortality of EB in England and Wales. METHODS: Demographic data for patients in England and Wales were collected on a secure electronic database, prospectively from January 2002 to April 2021 and retrospectively for cases prior to 2002. Vital status was verified using central NHS data. RESULTS: By March 2021, 2594 individuals were registered, of whom 2361 were living, which yielded a prevalence of 34·8 per million of the population for all EB types [EB simplex (EBS) 17 per million, dystrophic EB (DEB) 10·7 per million, junctional EB (JEB) 1 per million and Kindler EB 0·3 per million]. We recorded 1200 babies with EB born since 2002. The average incidence per million live births for EBS, DEB, JEB and Kindler EB was 32·5, 26·1, 8·9 and 0·9, respectively (total incidence for all types of EB was 67·8 per million). Birth rates fell progressively over the 19-year period for JEB-severe (JEB-S) (r = -0·56) and recessive DEB-severe (r = -0·44) and also for milder types of EB. We observed longer survival in JEB-S over the 19-year period (r2 = 0·18) with a median survival of 12·7 months over the past 5 years. CONCLUSIONS: In this study, we provide the first accurate epidemiological data for EB in England and Wales. We believe the observed reduction in birth incidence of severe types of EB reflects an uptake of genetic counselling advice, whereas the reduction in milder types may be due to delayed presentation. A potential small trend towards longer survival of babies with JEB-S may reflect improved multidisciplinary care.

Exploring stakeholder views on intervening in hospital around childhood secondhand smoke exposure (Precedent): a protocol for a qualitative study.

INTRODUCTION: Secondhand smoke exposure (SHSe) in childhood is linked with increased morbidity and mortality. Hospital or secondary care contact may present a 'teachable moment' to provide parents with support to change their home smoking behaviours to reduce children's SHSe. There is a lack of robust qualitative evidence around parents and healthcare professionals (HCPs) views on using this teachable moment to successfully initiate behavioural change. We aim to identify and understand what is important to stakeholders with a view to informing the development of a support package to help parents change their home smoking behaviours. METHODS AND ANALYSIS: This qualitative study will be theoretically underpinned by the Capability, Opportunity and Motivation Behaviour (COM-B) model of behavioural change. It will involve semistructured interviews and/or discussion groups with up to 20 parents who smoke and up to 25 HCPs. Stakeholders will be recruited from a single National Health Service children's hospital in England. Interviews and/or discussion groups will be audio recorded, transcribed and anonymised. The transcripts and any field notes will be analysed using the framework method. Initially, we will apply COM-B to the data deductively and will then code inductively within each domain. ETHICS AND DISSEMINATION: The protocol for this study received a favourable outcome from the East Midlands Leicester Central Research Ethics Committee (19/EM/0171). Results will be written up as part of a PhD thesis, submitted for publication in peer-reviewed journals and presentation at conferences. TRIAL REGISTRATION NUMBER: ISRCTN40084089.

A Mixed-Methods Systematic Review and Synthesis of Secondary Care Interventions to Reduce Secondhand Smoke Exposure Among Children and Young People.

INTRODUCTION: Childhood secondhand smoke exposure (SHSe) is linked with increased morbidity and mortality. Hospital or secondary care contact presents a "teachable moment" to support parents to change their home smoking behaviors to reduce children's SHSe. AIMS AND METHODS: This mixed-methods review explores: (1) if existing interventions in this context are effective, (2) if they are reported in sufficient detail to be replicated, (3) the experiences of health care professionals delivering such interventions, and (4) the experiences of parents receiving such interventions. Five electronic databases and the gray literature were searched for relevant literature published and indexed January 1980 to February 2020. Fourteen papers reporting 12 studies (nine quantitative and five qualitative) were included. Aligned with the Joanna Briggs Institute method, a segregated approach was used involving independent syntheses of the quantitative and qualitative data followed by an overall mixed-methods synthesis. RESULTS: There was some evidence of effective interventions that resulted in a short-term (<6 months) reduction in children's SHSe when SHSe was subjectively measured. This was not seen in longer-term follow-up (>6 months) or when SHSe was measured objectively. Inconsistencies with reporting make replication challenging. Experiential evidence suggests a mismatch between stakeholder preferences and interventions being offered. CONCLUSIONS: The pediatric secondary care interventions included in this analysis failed to show statistically significant evidence of longer-term effectiveness to reduce children's SHSe in all but one low-quality study. There was also inadequate reporting of interventions limiting assessment of effectiveness. It offers further insights into areas to target to develop effective interventions. IMPLICATIONS: This review used rigorous methods to explore the current, global literature on how children's exposure to secondhand smoke is being tackled in secondary care. This review identified only one low-quality intervention study showing a statistically significant reduction in children's SHSe beyond 6 months. Synthesis with qualitative research identifies a mismatch between what parents want in an intervention and what has been delivered to date. Reporting quality needs to be improved to ensure that interventions can be replicated and studies conducted within the National Health Service to ensure suitability to this setting.

Improving health, wellbeing and parenting skills in parents of children with special health care needs and medical complexity - a scoping review.

BACKGROUND: Parenting children with special health care needs can be challenging particularly if children have complex conditions. Parents may struggle to manage their child's health and their own emotions, contributing to poorer health outcomes for the family. Frequent healthcare contact presents opportunities to intervene, but current evidence review is limited. This review scopes and synthesizes interventions to improve health, wellbeing and parenting skills. METHODS: Using formal scoping review methodology MEDLINE, EMBASE, PsycINFO, CINAHL, The Cochrane Library, ERIC, ASSIA, HMIC and OpenGrey were searched to February 2017. Citations were double screened according to predetermined eligibility criteria. Data were extracted and synthesized on study design, population, measurement tools, and results. RESULTS: Sixty-five studies from 10,154 citations were included spanning parenting programs, other parent behavior change interventions, peer support, support for hospital admission and discharge and others. Interventions for parents of children with a wide range of conditions were included. These targeted a broad selection of parent outcomes, delivered by a wide variety of professionals and lay workers. Most studies reported positive outcomes. No serious adverse events were noted but issues identified included group and peer relationship dynamics, timing of interventions in relation to the child's disease trajectory, the possibility of expectations not fulfilled, and parent's support needs following intervention. Children with medical complexity were not identified explicitly in any studies. CONCLUSIONS: The range of interventions identified in this review confirms that parents have significant and diverse support needs, and are likely to benefit from a number of interventions targeting specific issues and outcomes across their child's condition trajectory. There is much scope for these to be provided within existing multi-disciplinary teams during routine health care contacts. Careful tailoring is needed to ensure interventions are both feasible for delivery within routine care settings and relevant and accessible for parents of children across the complexity spectrum. Further review of the existing literature is needed to quantify the benefits for parents and assess the quality of the evidence. Further development of interventions to address issues that are relevant and meaningful to parents is needed to maximize intervention effectiveness in this context.

Impact of environmental tobacco smoke exposure on anaesthetic and surgical outcomes in children: a systematic review and meta-analysis.

BACKGROUND: Tobacco smoke exposure in adults is linked to adverse anaesthetic and surgical outcomes. Environmental tobacco smoke (ETS) exposure, including passive smoking, causes a number of known harms in children, but there is no established evidence review on its impact on intraoperative and postoperative outcomes. OBJECTIVES: To undertake a systematic review of the impact of ETS on the paediatric surgical pathway and to establish if there is evidence of anaesthetic, intraoperative and postoperative harm. ELIGIBILITY CRITERIA PARTICIPANTS: Children aged 0-18 years undergoing anaesthetic or surgical procedures, any country, English language papers. EXPOSURE: ETS exposure assessed via questioning, observation or biological marker. OUTCOME MEASURES: Frequency of respiratory and other adverse events during anaesthesia, surgery and recovery, and longer term surgical outcomes. RESULTS: 28 relevant studies were identified; 15 considered anaesthetic outcomes, 12 surgical outcomes, and 1 a secondary outcome. There was sufficient evidence to demonstrate that environmental smoke exposure significantly increased risk of perianaesthetic respiratory adverse events (Pooled risk ratio 2.52 CI 95% 1.68 to 3.77), and some evidence that ear and sinus surgery outcomes were poorer for children exposed to ETS. CONCLUSIONS: ETS exposure increases the risk of anaesthetic complications and some negative surgical outcomes in children, and this should be considered when planning surgery. Research is required to demonstrate whether changes in household smoking behaviour prior to surgery reduces risk of adverse outcomes, and to close the evidence gap around other outcomes such as wound healing and respiratory infections. TRIAL REGISTRATION NUMBER: Review registration number 42014014557.