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Home mechanical ventilation in slowly progressive neuromuscular disease: challenging perceptions of health-related quality of life
  1. Anita K Simonds1,2
  1. 1 Sleep & Ventilation Unit, NIHR CRF, Royal Brompton and Harefield Hospital, Guys and St Thomas' NHS Foundation Trust, London, UK
  2. 2 National Heart and Lung Institute, Imperial College, London, UK
  1. Correspondence to Professor Anita K Simonds, Sleep & Ventilation Unit, Royal Brompton and Harefield Hospitals, London SW3 6JH, UK; a.simonds{at}rbht.nhs.uk

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The neuromuscular disease (NMD) population receiving home mechanical ventilation (HMV) comprises a heterogeneous group, with a wide variety of disorders, genotypes, phenotypes and disease trajectories. 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 effective multidisciplinary input, cough assist devices, management of systemic complications, anticipatory care plans and trained home care teams.1

Timely initiation of HMV in NMD saves lives, improves symptoms and physiological measures, reduces hospital admissions 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) in HMV users has been assessed with increasing sophistication using generic tools such as SF-36 to allow comparison with other chronic disorders and also tools specific to ventilatory support such as the Severe Respiratory Insufficiency Questionnaire.6 Most studies of HRQoL have focused on before and after the introduction of HMV or short-term outcomes over 6–12 months. An exception is Norwegian Long Term Mechanical Ventilation registry study7 which looked at outcomes after a 6-year period of HMV and showed continued improvement in HRQoL in many patients, other than those with chronic obstructive pulmonary disease (COPD). Neuromuscular diagnostic groups were pooled, but it is unlikely those with rapidly progressive disease were included.

The report by Delorme et al 8 is therefore valuable in that it takes a subgroup of patients with slowly progressive NMD and assesses HRQoL and the factors that influenced this long term—on average after 17 (10–21) years of HMV. ‘Slowly progressive’ disease is not defined but in practice the authors included those with Duchenne …

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  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Commissioned; externally peer reviewed.

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