Noninvasive Positive Pressure Ventilation in End-Stage Cystic Fibrosis: A Report of Seven Cases

Abstract

Noninvasive positive pressure ventilation (NIPPV) by nasal mask has been success-fully applied to the management of patients with chronic hypercapnic respiratory failure and to patients with neuromuscular disease. Although pediatric and young adult cystic fibrosis (CF) patients with end-stage disease exhibit abnormal gas ex-change while awake and further derangement in gas exchange during rapid eye movement sleep, little has been reported on results of NIPPV in such patients. We report our experience with 7 CF patients (age 9-30 years) in whom clinically impor-tant derangements in gas exchange were present despite conventional therapy. Application of nocturnal nasal bi-level intermittent positive pressure ventilation (BiPAP_TM) was well tolerated, improved respiratory acidosis and the subjective sen-sation of dyspnea, and was associated with decline in respiratory rates and im-proved quality of sleep and capacity to perform activities of daily living. On the basis of our experience, we believe that nocturnal NIPPV may be effective as a bridge between worsening of ventilation and transplantation for pediatric and young adult patients with cystic fibrosis.