Lung function and disability in neuromuscular patients at first admission to a respiratory clinic

SummaryBackgroundRespiratory failure is the most common cause of morbidity and mortality in patients with neuromuscular diseases (NMD). Non-invasive mechanical ventilation is considered highly effective for treating chronic respiratory failure. Perception and knowledge of risks associated with respiratory derangements may be underestimated.ObjectiveThe aim of our study was to evaluate the association among respiratory function, general clinical disability and need of home mechanical ventilation (HMV) in patients with slowly progressive NMD admitted for the first time to dedicated respiratory outpatient clinics.MethodsAnthropometrics, lung function, respiratory muscle function, daytime blood gases data, and general clinical disability assessed by means of a clinical interview were recorded. Indication for HMV was an arterial CO2 tension >45mmHg and/or a vital capacity <50% predicted, and/or maximal inspiratory pressure