Hypoventilation in cystic fibrosis

Abstract

Cystic fibrosis (CF) is the most common life-shortening genetic disorder in Caucasians. With the improved treatments that have become available, the median survival for patients with this disorder has increased to 37.4 years of age. Unfortunately, the overwhelming majority of patients still die from respiratory failure. Hypoventilation, arising from a variety of etiologies, may occur as part of the disease process and causes increased morbidity and mortality. Although inspiratory muscles training, oxygen therapy, and noninvasive ventilation are used in the treatment of hypoventilation in CF, more data are needed to guide their optimal use.