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AimTo describe and evaluate the psychosocial impact of the COVID-19 pandemic and measures to reduce the risk of transmission on patients with early-onset neuromuscular and neurological disorders (NMDs) and their families.DesignA mixed-methods study in which data were collected between 17 September 2020 and 31 December 2020 using a semi-structured telephone questionnaire developed specifically to meet research aims, and were analysed using quantitative methods and qualitative inductive thematic analysis.ParticipantsForty questionnaires were completed by patients with NMDs (eg, muscular dystrophies, spinal muscular atrophy) or their parent. 70% (n=28) of patients were male, aged 2–48 years. 90% (n=36) were wheelchair users; 72.5% (n=29) required long-term non-invasive or tracheostomy ventilation.ResultsStrict adherence to risk mitigation strategies, for example, shielding, were reported at the start of the pandemic. Over half continued some or all measures after official limitations were relaxed. 67.5% (n=27) reported changes to personal care assistance arrangements including temporary cessation of outside carers. Three themes were identified: (1) Concern regarding the health impact of COVID-19; (2) Perceptions of strategies to prevent SARS-CoV-2 transmission; (3) Psychological impact of the COVID-19 pandemic. The level and pervasiveness of frequently reported negative psychological effects, for example, anxiety and fear fluctuated, and were related to the perceived risk of COVID-19, concern about attending hospital, and perceived lack of access to intensive care management if severe COVID-19 infection occurred. Support, particularly from family and healthcare services, were considered to have positive psychosocial effects.ConclusionsMeasures to reduce transmission of COVID-19 have greatly affected patients with NMDs and their families. For most, negative psychosocial impacts have and will continue to improve, but this may depend on the incidence of further pandemic waves. Consistent, up-to-date and accessible information on clinical outcomes and risk mitigation must be provided to support patients’ physical and mental well-being.

Introduction: Patients with early-onset neuromuscular diseases (NMDs) were considered clinically vulnerable to COVID-19 due to assumptions that treatments e.g. corticosteroids, or complications e.g. pre-existing respiratory failure or cardiac disease would increase risk. In the UK, most were advised to ‘shield’ and strictly limit social contact, however it is poorly understood how patients and families approached and responded to this. Methods: In-depth questionnaires specifically designed to meet research aims were completed by telephone between September 17 th and December 31 st 2020 by NMD patients or their parent. Open and closed questions enabled a) quantitative analysis and b) thematic analysis to evaluate reasoning and psychosocial impact. Results: 40 questionnaires were completed: patients were 70% male, aged 2 to 48 years with NMDs e.g. muscular dystrophies, spinal muscular atrophy. 80% (n=32) had long-term non-invasive or tracheostomy ventilation; 20% (n=8) had cardiac involvement. Strict adherence to shielding was initially reported: this included temporary cessation or reduction in external care support in 68% (n=27), leading to increased parental care. Shielding measures were often relaxed due to 1) official guidance, 2) evidence of less severe outcomes in children and NMDs, and 3) unsustainability of limited social contact. Anxiety, fear and worry were the most frequently reported emotions, but level and pervasiveness fluctuated. 75% (n=30) remained “extremely” or “very” worried about risk of COVID-19 to the patient’s health. Concern about hospital attendance during the pandemic and anxiety regarding perceived lack of capacity or access to Intensive care were common. Virtual healthcare access, experienced by 97.5% (n=39), was seen as positive, largely as an interim measure, but was less useful where examination or tests were required. Conclusions: Measures to reduce transmission of COVID-19 have disproportionately affected NMD patients and their families. For most, negative psychosocial impacts have and will continue to improve, particularly due to the success of the vaccination programme. Patients and carers require access to support and up-to-date information to minimise risk of infection and psychological burden.

Introduction Children with complex neurological and neuromuscular conditions often have respiratory involvement requiring ventilatory support and multidisciplinary expertise due to their complexity. To ensure patients’ needs are met whilst minimising repeated hospital visits, an Annual Review (A/R) pathway was created to combine sleep study and multi-professional review within one admission. Method Patients under our care with a scheduled in-patient sleep study were offered a full A/R as part of the pilot (August 2020-2021). Table 1 outlines investigations and reviews undertaken during A/R (tailored to the individual). The A/R team met regularly to review admissions. Continuous improvement methodology was used to assess issues and implement process changes. Key review findings were documented and shared with other teams involved. Patients and families were invited to provide feedback prior to discharge. Results 47 AR’s were performed for 46 patients. Mean age was 10.5 years (median of 11.9 years, range 1.3-17.6). 43% of patients were female. 33% (15/46 pts) had Spinal Muscular Atrophy (See table 2 for patient demographics) 54% of patients used nocturnal NIV, 11% using NIV for respiratory illnesses and 13% on oxygen. 19% had no respiratory support. A/R led to management changes in 59% of patients. These included microbacterial growths on sputum and commencement of treatment, airway clearance plan alterations, NIV requirement/establishment, identification of cardiomyopathy, vitamin D and Iron supplementation. Patients and families scored A/R’s as 9.5/10 median (8-10). Discussion This new model of individualised respiratory multidisciplinary review ensured a range of issues were identified, and treatment adjusted to optimise respiratory management for this cohort. Patients and families appeared receptive to the process. Further analysis is needed to determine whether A/R’s reduce the burden of travel by facilitating virtual reviews at other times. The A/R model is being expanded for children ventilated via tracheostomy and congenital central hypoventilation syndrome.

Introduction This study aimed to describe the cohort of obese children who were on respiratory support for obstructive sleep apnoea (OSA) or nocturnal hypoventilation in a tertiary respiratory centre and their adherence at 1 year. Methods Clinical and cardiorespiratory polygraphy data were reviewed for children with a body mass index (BMI) >25kg/m2 established on NIV for OSA (AHI>1ev/Hr) or OHS (BMI>30kg/m2 and criteria for nocturnal hypoventilation)1 from 2013–21. Children with neuro-disability were excluded. Nocturnal hypoventilation was defined as transcutaneous carbon dioxide (TCO2) >6.7kPa ≥ 25% total sleep time (TST).1 Studies less than 4 hours TST were excluded. Day time hypercapnia was defined as TCO2 >5.99kPa ≥10 minutes.1 NIV compliance was >4 hours/night use for >70% of nights.2 Results Data was obtained from 16 children (9 males, 7 females) of whom 2 children had a BMI of 25-30kg/m2, and 14 > 30Kg/m2. Baseline polygraphy data was available on 13/16 children, 8 children had OSA only, 3 had OSA and nocturnal hypoventilation and 2 had and isolated OHS. 5 children (3M, 2F, age 9.0 (4.5-16) years) had nocturnal hypoventilation and also had the highest BMI of the cohort, BMI median 36.2 (28.6-47.3) kg/m2. Daytime hypercapnia was present in 4/9 children with measurements, but only 2 had nocturnal hypoventilation. No child had a TCO2 increase >10mmHg from wake to sleep. Children were established on NIV continuous (n=11) and bilevel (n=5). Only 2/14 children with adherence data were adherent to respiratory support at 1 year. (Table 1). Discussion Assessment for complications of excess weight should include day and night-time TCO2 monitoring as OHS was evident from 5 years. Adherence to NIV is poor and intensive multi-disciplinary input may improve this. References Masa Eur Respir Rev 2019. Schwab Am J Respir Crit Care Med 2013.

SARS-CoV-2 has caused a global health crisis and mass vaccination programmes provide the best opportunity for controlling transmission and protecting populations. Despite the impressive clinical trial results of the BNT162b2 (Pfizer/BioNTech), ChAdOx1 nCoV-19 (Oxford/AstraZeneca), and mRNA-1273 (Moderna) vaccines, important unanswered questions remain, especially in patients with pre-existing conditions. In this position statement endorsed by the British Society of Gastroenterology Inflammatory Bowel Disease (IBD) section and IBD Clinical Research Group, we consider SARS-CoV-2 vaccination strategy in patients with IBD. The risks of SARS-CoV-2 vaccination are anticipated to be very low, and we strongly support SARS-CoV-2 vaccination in patients with IBD. Based on data from previous studies with other vaccines, there are conceptual concerns that protective immune responses to SARS-CoV-2 vaccination may be diminished in some patients with IBD, such as those taking anti-TNF drugs. However, the benefits of vaccination, even in patients treated with anti-TNF drugs, are likely to outweigh these theoretical concerns. Key areas for further research are discussed, including vaccine hesitancy and its effect in the IBD community, the effect of immunosuppression on vaccine efficacy, and the search for predictive biomarkers of vaccine success.

Pediatric physical therapy (physiotherapy) is an essential component in the management of many cardio respiratory presentations in infants and children of all ages. The input will vary considerably depending on assessment findings and underlying pathology; however, the general principles remain the same across the spectrum. This chapter covers the key areas of physical therapy for specific conditions such as cystic fibrosis and bronchiectasis regarding airway clearance techniques, exercise, inhalation therapies, ventilator support, and secondary complications. It also identifies the role of cough augmentation and noninvasive ventilation in the management of children with neuromuscular conditions and spinal cord injuries. The role of the physical therapist in the critical care environment is discussed, with strategies modified for the intubated child to assist in secretion clearance and promote early rehabilitation. The importance of postoperative input through mobilization for children that require intervention and adjuncts to support therapy is outlined. Conditions that cause airway or structural abnormalities and may respond effectively to physiotherapy management are discussed. The interventions, as always, are tailored to the individual and monitored for safety and efficacy. Conditions and clinical presentations usually not amenable to physiotherapy are also reviewed. The main focus of the chapter is to highlight the essential elements of pediatric physical therapy for respiratory disease, reviewing the growing evidence base behind the many interventions available in the therapist's “toolbox.” Physiotherapy is not only a science but also an art. It requires the therapist to constantly review, modify, and adapt to a potentially changing presentation as well as the ability to communicate with the child and family for engagement and best outcomes.