News

Orkambi eased airway inflammation, boosted bacterial diversity

Treatment with Orkambi (lumacaftor/ivacaftor) for six months lessened airway inflammation and enhanced airway bacterial diversity in people with cystic fibrosis (CF), but only when administered before patients were chronically infected with the bacterium Pseudomonas aeruginosa, a study has found. The findings suggest that “CFTR modulators [such as Orkambi] should…

Neutrophil cell subtype tied to lung function decline in stable CF

Lung function decline in adults with stable cystic fibrosis (CF) was tied to high numbers of mature low-density neutrophils, a white blood cell subtype associated with inflammatory disorders, a study suggests. While previous research showed that low-density neutrophils in CF children correlated with better lung function, the changes found…

Complete antibiotic coverage used mostly for polymicrobial infections

Pulmonary exacerbations in children with cystic fibrosis (CF) caused by multiple bacteria (polymicrobial) are generally treated with antibiotics that cover all the bacteria detected, a study reported. Whether this strategy is associated with better clinical outcomes than using antibiotics that cover only some of the detected bacteria remains to…

Orkambi seems to reduce CF lung damage in preschool-age children

In a small clinical trial, Orkambi (lumacaftor/ivacaftor) appeared to reduce structural damage in the lungs, as measured by a chest MRI, for preschool-age children with cystic fibrosis (CF) who are homozygous for the F508del mutation. “This study suggests that [Orkambi] may modify CF disease progression when administered early…

New nanoparticles may correct genetic defects in lungs

Researchers are developing new inhalable nanoparticles designed to deliver RNA-based gene-editing therapeutics directly to the lungs, a study reports. Now shown to be effective in mice, the ultimate goal is to develop inhaled therapeutics to correct genetic defects in conditions affecting the lungs, such as cystic fibrosis (CF). “This…