Patient and Caregiver Perceptions of Airway Clearance Methods Used for Cystic Fibrosis.

Introduction: Cystic Fibrosis Foundation guidelines recommend people with CF perform daily airway clearance. This can be difficult for patients, as some find it time consuming or uncomfortable. Data comparing airway clearance methods are limited.

Pathogen acquisition in patients with cystic fibrosis receiving ivacaftor or lumacaftor/ivacaftor.

Background: The cystic fibrosis transmembrane conductance regulator (CFTR) modulators ivacaftor and lumacaftor/ivacaftor improve the status of existing infections in patients with cystic fibrosis (CF). It is unknown how well these drugs protect patients against incident infections. We hypothesized that CFTR modulator treatment would decrease new infections with Pseudomonas aeruginosa or Staphylococcus aureus.

Airway epithelial anion secretion and barrier function following exposure to fungal aeroallergens: role of oxidative stress.

Aeroallergens produced by can elicit life-threatening exacerbations of asthma in patients sensitized to this fungus. In this study, the effect of on ion transport mechanisms underlying mucociliary clearance and airway epithelial barrier function was investigated in human airway epithelial cells. Apical exposure to induced an increase in anion secretion that was inhibited by blockers of CFTR and Ca-activated Cl channels.