Steve Bryson, PhD, science writer —

After starting Trikafta, people with cystic fibrosis (CF) significantly reduced their use of other CF-related medications, suggesting that many people with CF on Trikafta “may prioritize reducing treatment burden over maximizing lung function,” researchers said. Still, lung function continued to decline after Trikafta initiation, they reported in a real-world…

Pulmonary ionocytes, a rare airway cell type that produces high levels of CFTR — the protein that’s defective in cystic fibrosis (CF) — were found to regulate the acidity of the liquid coating the surface of the airways, a cell-based study showed. Results also showed that club cells, another…

The European Commission (EC) has expanded the approval of Kalydeco (ivacaftor) for the treatment of infants with cystic fibrosis (CF) as young as 1 month with at least one CFTR gating mutation. The approval makes Kalydeco, marketed by Vertex Pharmaceuticals, the first and only CFTR modulator…

A grant of $500,000 in Australian dollars (around $326,000) will support researchers there in the development of antibiotics formulated with liquid crystalline nanoparticles (LCNPs), the better to eliminate hard-to-treat lung infections in people with cystic fibrosis (CF). “By overcoming the processes that cause drug resistance and uncontrollable infection, this…

Elevated levels of the pro-inflammatory signaling protein galectin-3 in the bloodstream of children with clinically stable cystic fibrosis (CF) significantly correlated with worse lung function, according to a study. The findings suggest galectin-3 may serve as a noninvasive biomarker to predict lung disease outcomes in children with CF, the…

A protein secreted into the airways, called SPLUNC1, may be a sensitive biomarker of both mild pulmonary exacerbations and treatment response in children with cystic fibrosis (CF), a study suggested. Data showed that SPLUNC1 levels in patients’ sputum were low during an exacerbation, then significantly rose after oral and,…

Children with cystic fibrosis (CF) who began taking Orkambi (lumacaftor/ivacaftor) between ages 2 and 5 showed improved height, weight, and body fat content compared with matched patients who hadn’t yet received modulator therapies, a real-world study reports. There was also a 50% decline in the number of pulmonary…

Zinc may be key to reducing bacterial infections in people with cystic fibrosis (CF), researchers at the University of Queensland have found. They discovered that defects in the CFTR protein, the underlying cause of CF, interfered with the bacteria-killing function of immune cells known as macrophages. They were able…

A European Medicines Agency (EMA) committee recommends extending the label for Kalydeco (ivacaftor) to children with cystic fibrosis (CF) as young as a month who carry at least one CFTR gating mutation. If the European Commission (EC) accepts the endorsement by the Committee for Medicinal Products for Human…

A pooled analysis of data from multiple clinical trials of more than 1,100 people with cystic fibrosis (CF) confirms Trikafta effectively improves lung function, sweat chloride levels, and health-related quality of life. “Further monitoring and assessment of the safety profile of [Trikafta]…