ABSTRACT
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 and December 31 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.
ABSTRACT
IntroductionMost patients with early-onset neuromuscular disorders (NMDs) were advised to shield during the pandemic due to assumptions that treatments e.g. corticosteroids, or complications e.g. pre-existing respiratory failure would increase COVID-19 risk. It remains poorly understood how those with NMDs and their families approached and responded to risk mitigating strategies e.g. shielding. We aimed to determine themes describing the psychosocial impact of the COVID-19 pandemic and measures taken to reduce risk in this population.MethodsIn-depth questionnaires specifically designed to meet research aims were completed by telephone between September 17th and December 31st 2020 by patients with NMDs or their parent. Inductive thematic analysis was performed to first code transcriptions of audio recordings of questionnaires then develop candidate themes by exploring coded data. Candidate themes were evaluated against the original data set before defining final themes: participant validation was sought to provide additional confirmation of accuracy.Results40 questionnaires were completed: patients were 70% male, aged 2 to 48 years with NMDs e.g. muscular dystrophies, spinal muscular atrophy. 80% required long-term non-invasive or tracheostomy ventilation. 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. Anxiety, fear and worry were the most frequently reported emotions, particularly in relation to health risk of COVID-19, but level and pervasiveness fluctuated during the pandemic. Strict adherence to shielding was reported at the start of the pandemic but was often relaxed due to 1) official guidance, 2) emerging evidence of less severe outcomes in children and NMD cohorts, and 3) unsustainability of limited social contact including cessation or reduction in personal care. Concern about hospital attendance during the pandemic, and anxiety regarding perceived lack of access to Intensive Care were common.ConclusionsMeasures to reduce transmission of COVID-19 have disproportionally affected patients with NMDs and their families. For most, negative psychosocial impacts have and will continue to improve, particularly due to the success of the vaccination programme. These aspects should be considered when advising patients and families on risk and risk-mitigating strategies during the current and future pandemics.