Home mechanical ventilation in slowly progressive neuromuscular disease: challenging perceptions of health-related quality of life

The neuromuscular disease (NMD) popu-lation receiving home mechanical ventilation (HMV) comprises a heterogeneous group, with a wide variety of disorders, genotypes, phenotypes and disease trajec-tories. These span rapidly progressive conditions such as in amyotrophic lateral sclerosis/ motor neuron disease (ALS/ MND), to more slowly progressive myopathies, muscular dystrophies and neuropathies. HMV strategies have evolved over the last few decades with the increasing use of non- invasive rather than invasive ventilation, and the introduction of effec-tive multidisciplinary input, cough assist devices, management of systemic compli-cations, anticipatory care plans and trained home care teams. 1 Timely initiation of HMV in NMD saves lives, improves symptoms and physi-ological measures, reduces hospital admis-sions and changes the natural history of many conditions. 2 It is therefore an essential component of many evidence-based clinical guidelines. 3–5 Health- related quality of life (HRQoL