Home ventilation in cystic ﬁbrosis, bronchiectasis and diffuse interstitial lung disease A K Simonds
NIV in cystic ﬁbrosis 221
NIV in bronchiectasis 225
NIV in diffuse interstitial lung disease 226
Unlike neuromuscular and chest wall disorders, advanced diffuse interstitial lung disease, bronchiectasis and cystic ﬁbrosis are characterized by marked ventilation-perfusion mismatch and diffusion problems, although the work of breathing is also increased by airﬂow obstruction and hyperinﬂation in cystic ﬁbrosis and bronchiectasis, and reduced lung compliance in interstitial lung disease patients. Chronic CO2 retention is an endstage phenomenon. Any response to non-invasive ventilation (NIV) is therefore likely to be less impressive than in individuals with predominant alveolar hyopoventilation, and the goals of therapy should be clearly established before a trial.