chapter  17
8 Pages

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

References 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.