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Non-invasive ventilation (NIV) is assisted respiratory support delivered via facemask for people with chronic respiratory failure. Commercial NIV masks are available but masks that fit well are difficult to find for children who have small or asymmetrical facial features. Compromised ventilation can have significant health and quality of life impacts for patients and their families. The overarching aim of the project was to improve comfort, fit and performance of NIV masks for children using 3D technology. The needs of patients, parents/carers and healthcare professionals from 6 UK centres was ensured through events advising on mask design and research procedures. 3D scanning technologies were compared in vitro using workflows and deviation analysis and with 3 healthy and 19 patient volunteers. Early design concepts were tested and the final prototype selected using a test mannequin and adult volunteers. Prototype masks were manufactured using biocompatible materials to IOS 13485 quality standards and evaluated with 19 patients. Feedback on fit, comfort and facial marking and oxicapnography data to demonstrate ventilator effectiveness were collected. The potential cost-effectiveness of the customised masks was assessed using an economic model. A handheld structured light 3D scanner was selected due to superior resolution, ease of use, availability and cost. The final design was a semi-bespoke hybrid approach. 11/15 (73%) evaluation participants reported comfort and fit that was as good or better than their usual mask. 5/11 (45%) were unable to wear the mask due to poorly fitting headgear despite a well-fitting mask. All physiological ventilation parameters were improved (table 1). Preliminary health economics analysis demonstrated that to achieve a 5% improvement in fit and ventilation, a custom-made mask would be cost effective at a price below £500. A series of in vitro, user-perspective, healthy volunteer and patient evaluations informed the development of custom-made masks for children using NIV.

Background Long term ventilation (LTV) refers to mechanical support for breathing either at home or in hospital, for all or part of the day, for at least 3 months. Two national surveys carried out in 1998 and 2008, highlighted the increasing numbers of ventilator dependent children throughout the UK . Our objective was to collect current information about children receiving LTV in the UK, 10 years after the last national survey. Methods All LTV centres in the UK completed a single time point census survey on 30th September 2019 using an electronic questionnaire. Data included the child’s location, underlying diagnosis, interface and type of respiratory support, and whether disease-modifying drugs affected the decision to initiate LTV in specific groups within this target population. Results Data was collected from 25 LTV centres. The total study population was 2383 children and young people. The median age of the overall sample was x (range x-x). 40.3% female; 57.2% of those recorded were male. Diagnoses were 417 (17.5%) central nervous system, 692 (29%) musculoskeletal and 1274 (53.5%) a respiratory, of which 31.8% of the total had upper airway obstruction. Notable changes since 1998 were the decline in the use of 24-hour ventilation, negative pressure ventilation and tracheostomy as an interface, and the increase in the proportion of patients treated at home. 115 children had received a disease-modifying drug. The use of Ataluren and Myozme did not influence the decision to treat with LTV, but in 35% of the children treated with Nusinersin the clinician stated that the use of this drug had or may have influenced their decision to initiate LTV. Conclusion The number of children being treated with LTV has increased by 250% in the last 10 years with notable changes in practice over the last 20 years.

Introduction Poor sleep is associated with many adverse outcomes in childhood. The most common sleep difficulty experienced by children is behavioural insomnia which responds to behavioural therapy. The Coronavirus disease 2019 (COVID-19) global pandemic in 2020 has had a profound effect on children’s sleep patterns. This project aimed to evaluate a one-to-one sleep service delivered via on-line clinics by community sleep practitioners in the UK. Methods The study was an observational pre- and post- evaluation in a 12-month period. The intervention is derived from the behavioural aspects of Cognitive Behavioural Therapy for insomnia. The evaluation was questionnaire-based and assessed sleep parameters and wellbeing. Results 104 parents returned completed questionnaires. Average sleep onset was 1 hour, 39 minutes pre-intervention and 20 minutes post-intervention. Average nights that the child woke per week was 3.9 pre-intervention and 0.9 post-intervention; night awakenings fell from 1.9 to 0.5 and time awake after sleep onset fell from 66. 8 minutes to 5.8 minutes. The average time slept asleep per night was 8.0 hours pre-intervention and 10.2 hours post-intervention. Improvement in all sleep measures was statistically significant (p Discussion We believe that this is the first report of a video-based clinic developed for children’s behavioural insomnia during the COVID-19 pandemic in the UK. The COVID-19 pandemic has accelerated the evolution of telehealth and, in the case of children’s sleep clinics, has provided a mode of intervention delivery that is as effective as face-to-face delivery, acceptable and accessible.

Background Children and young people (CYP) with special educational needs (SEN) are more likely to experience disturbed sleep and poorer mental wellbeing. This study explored the differential impact of the COVID-19 pandemic on the sleep and mental wellbeing of CYP with and without SEN. Methods NIHR Children and Young People MedTech Co-operative, Sheffield Children’s NHS Foundation Trust, and The Sleep Charity carried out an online survey between 23 June 2020 and 17 August 2020. The 77-item survey was shared via social media platforms. Results 559 participants were included in the analyses, 15.74% of whom reported having a CYP with SEN. While sleep changes due to the pandemic were largely similar for both groups, CYP with SEN (40.91%) were more likely to get or wake up during the night than CYP without SEN (40.91% vs 27.18%). CYP with SEN were significantly more likely than children without SEN to be demotivated (61.44% vs 31.57%), sad and tearful (36.15% vs 19.35%) or anxious and stressed (43.48% vs 14.82%) during the pandemic, as well as to report that increased anxiety was more likely to contribute to poorer sleep (43.48% vs 14.82%). Conclusion While the majority of CYP in both groups reported sleep changes due to the pandemic, CYP with SEN experienced more sleep disturbance. The findings provide initial evidence to suggest that the pandemic may have had a greater impact on the sleep and mental wellbeing of CYP with SEN compared to CYP without SEN.

Introduction Paper sleep diaries and actigraphy are useful tools in Sleep Medicine to evaluate sleep wake patterns in the home environment over the course of 7–14 days. In our service, the demand for actigraphy far outweighs the available resources. As such the team are investigating using an electronic sleep diary, integrating with actigraphy and simplifying the whole process. The first stage of this process was to ask the stakeholders their opinions of sleep diaries and actigraphy. Methods Stakeholders were asked about sleep diaries and actigraphy considering all aspects of the process from when to request a referral and organising collection, completing a diary and wearing actigraphy, through to analysing and interpreting results. Questions asked were appropriate to the different stakeholder groups involved. Families and local hospital staff were interviewed and British Paediatric Sleep Society (BPSA) members and families undergoing actigraphy were sent appropriate brief questionnaires relevant to their part of the pathway [Service evaluation SE1347]. Results 47 stakeholders provided feedback. Interviews were conducted with 14 staff, 9 parents and 3 young people. Additional questionnaires were completed by 8 families undergoing actigraphy and 13 BPSA responders. A summary of the most common responses is shown in table 1. Most institutions were issuing sleep diaries and actigraphy to specific patient groups due to limited resources. Discussion This stakeholder engagement provides useful information to improve the use of sleep diaries and actigraphy within our paediatric clinical setting. If the process can be simplified then more sleep diary and actigraphy usage can occur at the triage stage of the sleep referral process and prevent unnecessary sleep studies, benefitting both families and limited resources. Feedback received confirms the current solution for paediatric sleep diaries and actigraphy is not ideal and there is a need to identify new approaches to increase usability, acceptability and stakeholder’s satisfaction.

Introduction Many children with sleep difficulties are prescribed melatonin. However, melatonin is costly and evidence for its efficacy is limited. The aim of this study was to evaluate a nurse-led sleep clinic to look at outcomes in terms of melatonin prescribing. Methods All new referrals attending a nurse-led sleep clinic were examined from June 2016 to March 2017. Patients/parents attending the clinic were given an individualised sleep programme to implement with their child at home, with ongoing follow-up support. Families received an average of 3 face-to-face clinic visits and 3 follow-up telephone calls from first visit to discharge. Retrospective data was gathered from clinic records [Clinical Audit approval number CA1309]. Results 69 patients aged 1–17 years (31 males) were analysed. The primary complaint was ‘problem with sleep initiation and maintenance’ (81%), ‘problem with sleep initiation’ (13%) and ‘problem with sleep maintenance’ (6%). 84% of patients had medical co-morbidities; 54% of which were neuro-disability, the commonest being ASD and/or ADHD. 40/69 patients were successfully discharged from the clinic during the evaluation period. 65% were discharged without melatonin (23% with neurodisability; 42% without neurodisability), of which 35% were weaned off melatonin and 30% avoided melatonin prescriptions. 12% felt that the sleep intervention had been successful but still required melatonin, 15% were referred to medical/psychology clinics, and 8% were non-compliant with the behavioural sleep programme and were discharged still taking melatonin. Discussion In this cohort of paediatric insomnia patients, a brief but intensive behavioural programme with support from a specialist sleep nurse was effective in resolving sleep difficulties and reducing melatonin usage. We recommend that standardised sleep support from trained practitioners should be available prior to prescribing melatonin for children with sleep difficulties. This approach is not only beneficial in effectively resolving sleep problems but is cost-effective when compared with melatonin prescribing.

Introduction Narcolepsy occurs due to an inability to regulate the sleep-wake cycle, causing disturbed night-time sleep and excessive daytime sleepiness. Children and young people with narcolepsy have increased psychiatric illness, compared to healthy controls. A recent study of 31 paediatric narcolepsy patients found 43% had psychiatric comorbidity, compared with 10% in the general population.1 The aim of this service evaluation was to determine whether Sheffield Children’s Hospital narcolepsy patients have increased psychological morbidity in order to inform service development. Methods 43 patients, aged 4–16 years, and their parents were given Revised Children’s Anxiety and Depression Scale (RCADS) questionnaires in clinic [Service Evaulation SE1473]. Software produced by the developer was used to analyse the results, using data from a cohort of children and parents separated by gender and American school grade. Scores are divided into: Separation Anxiety, General Anxiety, Panic, Social Phobia, Obsessive Compulsive Disorder and Depression. Results 34 patients and parents completed their questionnaires. A positive score was defined by a T score>65, indicating clinically significant anxiety or depression. 16 patients scored positively based on their questionnaires and 25 scored positively based on their parent’s questionnaires, giving psychological morbidity rates of 47% and 74% respectively. The category scored positively in most frequently was depression: 15 patients and 23 parents’ questionnaires scored positively here. Scoring for anxiety was lower: 13 in the parent and 2 in the patient questionnaires. Discussion This service evaluation showed raised levels of anxiety and depression in Sheffield Children’s Hospital narcolepsy children and young people, compared with national averages. Regular psychological assessment and early intervention for patients would be appropriate, given the results shown here and these results will be put forward as part of a future business case. Reference Szakacs, et al. Psychiatric comorbidity and cognitive profile in children with narcolepsy with or without association to the H1N1 influenza vaccination. Sleep 2015.

Introduction Narcolepsy is a disabling neurological sleep disorder characterised by excessive daytime sleepiness and attacks of muscle weakness precipitated by strong emotions, known as cataplexy. A previous exploratory technology workshop with children from the Sheffield Children’s Hospital narcolepsy clinic identified ‘Head and neck support in the car’ as their most important unmet need in terms of aids to daily living (32/39 participants). There is currently no suitable car seat or effective support on the market for these children. Therefore, this project aimed to design, develop and evaluate concepts for a neck stabilising aid for children with narcolepsy. Methods Detailed ‘needs capture’ through a co-design workshop with children with narcolepsy and their parents to map and discuss their travel experiences resulted in an initial specification list. A second creative workshop for idea generation using existing products and early design concepts informed further development. Results A detailed design specification list has been produced. Seven concept designs have been developed for further evaluation and selection at an upcoming ‘dragon’s den’-style workshop. Concepts will not reach prototype stage within the scope of the project so worksheets and interactive design activities will be used to capture early subjective user opinions. Discussion The use of creative, co-design methods have proven effective in capturing the voices of children and families to ensure the project is generating meaningful solutions to the core issues in this area. The project is currently ongoing with a number of possible concepts being proposed and evaluated by children and families. The preliminary concepts and supporting evaluation data will be used to apply for future funding to develop the chosen concept to prototype level and beyond.

Introduction The current gold standard sensors to measure airflow in apnoea and hypopnoea detection are the oronasal thermal airflow sensor and the nasal pressure transducer, respectively.1 Due to the contact nature of these sensors in the nasal region, they are poorly tolerated by children. In a recent audit, we found that 50% of children refused the nasal pressure sensor due to its invasiveness, and of those that accepted it, 64% removed the prongs over the course of the night. The aim of the current study was to evaluate a non-contact method to monitor respiration by developing infrared thermal imaging, whereby temperature fluctuations associated with respiration are measured and correlated with airflow. Methods 11 healthy adult volunteers participated following University Ethics. Respiratory signals were recorded over four simulated apnoea scenarios resembling normal respiration; central; obstructive and hypopnoeic pauses. Simulated apnoeas were measured via nasal pressure cannula and respiratory inductance bands [SOMNOtouchTM RESP; SOMNOmedics, Germany] versus thermal imaging techniques. Results 70% of the apnoea episodes correlated with airflow sensor readings (Example trace in figure 1). In 16% of recordings the subject’s head position did not allow correct identification of the region of interest (i.e. the nostrils). For the remaining 14% of cases there was partial agreement between the thermal measurements and nasal pressure readings. Discussion These results indicate thermal imaging may be valuable as a detection tool for sleep apnoea, particularly in the case of paediatric patients whose tolerance for contact nasal sensors is poor. Reference Berry RB, Albertario CL, Harding SM, et al. for the American Academy of Sleep Medicine. The AASM Manual for the Scoring of Sleep and Associated Events: Rules, Terminology and Technical Specifications. Version 2.5. Darien, IL: American Academy of Sleep Medicine, 2018.

Introduction Narcolepsy is a chronic neurological condition in which the brain’s ability to regulate the sleep-wake cycle is disturbed and can result in reduced quality of life (QOL). A positive association between exercise and QOL has been found in children. The aim of this study was to assess the feasibility of implementation of an exercise intervention with a mobile app to monitor outcomes in children with narcolepsy. Methods This feasibility study received service evaluation (SE1308), and University of Sheffield ethical approval (project number: 022995). A patient and parent engagement exercise to inform study design was carried out during the ‘Sheffield Children’s Hospital Annual Narcolepsy Family Day’. An exercise intervention was designed based upon NHS recommendations and tailored to the child’s activity levels.1 Weekly goals were set and progress was reviewed weekly, using an app specifically developed by Aparito Ltd (Wrexham, UK). QOL was monitored using the Pediatric Quality of Life Inventory (PedsQL). Patients were asked for feedback at the end of the intervention, to assess its feasibility and acceptability. Results 117 children and parents took part in the engagement exercise. 12 patients took part in the feasibility study (mean age 12.9 years). The focus group highlighted the importance of motivation, timing and tailoring exercise interventions to the individual. Children said exercise should be fun to help motivation. Average physical activity increased from 229 minutes to 254 minutes. Of those who had successfully increased their exercise, baseline average PedsQL score increased from 70.6 to 77.6 (figure 1). Feedback on the app and exercise intervention were both positive. Conclusion This feasibility study has shown that a targeted exercise intervention can improve QOL in children with narcolepsy. The Aparito app, it is being developed further for children with narcolepsy. Reference Health Survey for England 2015: Physical Activity for Children

Introduction Non-invasive ventilation (NIV) masks that fit well are difficult to find for children who are small or have atypical facial features. Poorly fitted masks create problems e.g. discomfort, non-adherence and facial deformity. Our project aims to design and produce masks that fit well. Children’s voices are vital, but not often heard, in respiratory research projects. Integral to the research, we aimed to construct a patient and public involvement and engagement (PPIE) program designed to: Understand the problems children and families experience with NIV and establish their wants and needs Provide an inclusive and creative environment for non– constrained thinking Get actionable feedback and ideas for improvements from a diverse patient group Method We created a method focussed on planning, innovation and participation (the PIP model). Session activities were designed to enable parents and children of all ages and abilities to participate. Examples include: Archery target activity – a method for realising the relative importance of patient’s requirement (prioritisation). Graphic scribe recording – to reflect back to the children that they had been heard/understood and stimulate creative ideas. Use of technology – making short videos to help families understand concepts. Results Our priorities and design brief changed as a result of the PPIE. The graphic scribe outputs formed part of the creative process whilst providing a unique and lasting resource. We are confident that we will produce NIV interfaces that are fit for real life purpose that people will want to trial. Discussion For respiratory research to be truly successful, PPIE should be woven throughout a project, from concept to completion. It needs to be genuine and aligned with research aims. Time and effort spent enabling participation and creatively planning for inclusivity is rewarded by generating richer and more valuable information.