The European respiratory journal : official journal of the European Society for Clinical Respiratory Physiology
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Respiratory muscle weakness in neuromuscular disease causes significant morbidity and mortality. The published data on respiratory muscle activity and breathing during sleep in normal subjects, the impact of respiratory muscle weakness on sleep and breathing and the relations to daytime respiratory function in neuromuscular disease are reviewed here. In normal subjects during sleep upper airway resistance increases, chemosensitivity is reduced and the wakefulness drive to breathe is lost, resulting in a fall in ventilation. ⋯ Correlations between daytime respiratory function and nocturnal desaturation are moderate or weak, but daytime respiratory function has greater prognostic value than nocturnal measurements. Noninvasive ventilation improves sleep quality and breathing in subjects with respiratory muscle weakness. However, the optimal criteria for initiation of ventilation and its role in rapidly progressive neuromuscular diseases are unclear.
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Noninvasive positive-pressure ventilation (NPPV) has been shown to be a means of reducing the need for endotracheal intubation, which when effective reduces the complication rate and improves outcome. Because paralysis and sedation are not needed and because the patient is not necessarily dependent upon a machine for respiration, ventilation outside the intensive care unit (ICU) is an option. A number of studies have shown that NPPV for acute exacerbations of chronic obstructive pulmonary disease (COPD) can be effective in the non-ICU environment, though usually in patients with less severe exacerbations. ⋯ A dedicated intermediate care unit with particular expertise in noninvasive modes of ventilation may provide the best environment, both in terms of outcome, but also cost effectiveness. The ideal location for noninvasive positive-pressure ventilation will vary from country to country and indeed from hospital to hospital, depending upon local factors. However, the most important factor is that staff be adequately trained in the technique and be available throughout the 24-h period.