Chronic Respiratory Failure and the Roles of Noninvasive Ventilation and Lung Transplantation

I. Introduction Progressive loss of lung function in cystic fibrosis (CF) ultimately leads to gas exchange failure, manifested by hypoxemia with or without hypercapnia. Concurrently, the increased load placed on the respiratory muscles from airway obstruction and parenchymal damage, together with pulmonary hyperinflation and poor nutrition, place the respiratory muscles at a disadvantage and predispose the patient with advanced CF lung disease to respiratory muscle fatigue and failure.