Covid-19 related concerns and outcomes from shielding in clinically extremely vulnerable adults and children with neuromuscular disease

Abstract

Background: Many patients with neuromuscular disease (NMD) with or at risk of respiratory failure were advised to shield during the Covid-19 pandemic: This study describes clinical and psychosocial outcomes.

Methods: Semi-structured telephone questionnaires were conducted in UK patients or carers from September to December 2020. Analyses used quantitative and qualitative methods.

Results: 40 questionnaires were completed: Patients were 70% male, aged 2 to 48 years with NMD (e.g. muscular dystrophies and spinal muscular atrophy). 80% required long-term non-invasive or tracheostomy ventilation. All received advice to shield: >50% shielded before being advised and 73% considered themselves to be shielding. 25% reported having ≥1 symptom associated with Covid-19; no hospitalisations or positive PCR tests were reported. 75% remained “extremely” or “very” worried about the risk of Covid-19 due to anticipated medical consequences and uncertainty about disease impact in NMDs. >80% reported anxiety, depression and/or fear. If severely unwell, 93% wished to be considered for ICU care but 59% were unsure this wish would be respected due to ICU bed constraints, and/or a perception that disabled or vulnerable patients might have low priority for admission. 68% reported disruption to care support, leading to increased parental care. Access to and use of PPE by carers was variable. Virtual access to healthcare was seen as positive.

Conclusions: Shielding is likely to be effective in reducing Covid-19 transmission, but patients and carers require access to support and up-to-date information to minimise risk of infection and psychological burden.