High-flow oxygen therapy for the management of patients with acute exacerbation of cystic fibrosis
Cystic fibrosis is an autosomal recessive genetic disease that provokes dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR). The disease occurs most often from birth and affects mainly the lungs. This protein dysfunction directly provokes thick mucus into bronchi promoting pulmonary infections and leading to development of chronic obstructive pulmonary disease (COPD), followed by chronic respiratory failure. Cystic fibrosis also affects intestinal function, leading to pancreatic insufficiency and malnutrition that may alter muscular function and worsen respiratory failure. Its evolution is marked by iterative episodes of acute-on-chronic respiratory failure with hypercapnia.