Psychosocial impact of the COVID-19 pandemic and shielding in adults and children with early-onset neuromuscular and neurological disorders and their families: a mixed-methods study.

AIM: To 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. DESIGN: A 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. PARTICIPANTS: Forty 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. RESULTS: Strict 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. CONCLUSIONS: Measures 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.

Preference of neuromuscular patients regarding equipment for daytime mouthpiece ventilation: A randomized crossover study.

BACKGROUND: Patients with neuromuscular disorders (NMDs) are likely to develop respiratory failure which requires noninvasive ventilation (NIV). Ventilation via a mouthpiece (MPV) is an option to offer daytime NIV. OBJECTIVES: To determine the preferred equipment for MPV by patients with NMDs. METHODS: Two MPV equipment sets were compared in 20 patients with NMDs. Set 1, consisted of a non-dedicated ventilator for MPV (PB560, Covidien) with a plastic angled mouthpiece. Set 2, consisted of a dedicated MPV ventilator (Trilogy 100, Philips Respironics) without backup rate and kiss trigger combined with a silicone straw mouthpiece. The Borg dyspnea score, ventilator free time, transcutaneous oxygen saturation (SpO2) and carbon dioxide tension (TcCO2 ) were recorded with and without MPV. Patient perception was assessed by a 17-items list. RESULTS: Carbon dioxide tension measurements and total perception score were not different between the two MPV sets. Dyspnea score was lower with the non-dedicated versus dedicated equipment, 1 (0.5) versus 3 (1-6), P < 0.01. All patients with a ventilator free time lower than 6 hours preferred a set backup rate rather than a kiss trigger. Sixty five percent of patients preferred the commercial arm support and 55% preferred the plastic angled mouthpiece. CONCLUSIONS: Dedicated and non-dedicated MPV equipment are deemed effective and comfortable. Individualization of arm support and mouthpiece is advised to ensure success of MPV. A ventilator free time lower than 6 hours seems to be a useful indicator to a priori set a backup rate rather than a rate at zero associated to the kiss trigger.