Diana Campelo Delgado,  —

      Disease progression and the overall health of young people with cystic fibrosis (CF) can be evaluated by putting them through the modified shuttle test (MST) once, as its repeated use showed no significant differences in exercise capacity, a study reports. This test involves repeated movement along…

The Cystic Fibrosis Foundation (CFF) is awarding Life Edit Therapeutics up to $400,000 to explore its new gene-editing technology to develop treatments for certain patients with cystic fibrosis (CF) who cannot benefit from existing medicines. “We’re looking forward to working with the CF Foundation to leverage the unique…

Lonodelestat (also known as POL6014), Santhera Pharmaceuticals’ investigational therapy to treat chronic lung inflammation, was safe and effective at blocking the activity of a pro-inflammatory biomarker in patients with cystic fibrosis (CF), according to data from a Phase 1b trial. “We are very much encouraged by the…

Female adolescents with cystic fibrosis (CF) should be offered contraceptive counseling and informed about pregnancy risks, a long-term study shows. Adding a gynecologist to the medical team following female patients with CF could improve their quality of care, the researchers suggested. The study, “Reproductive Health Counseling and Contraceptive…

A rare and disease-causing mutation for cystic fibrosis (CF), called the F1099L mutation and characterized by a protein maturation defect, was identified in a recent study. Treatment with lumacaftor was also seen to effectively rescue function of the defective CFTR protein that results from this mutation, found in two infants…

The Cystic Fibrosis Foundation (CFF) is awarding Eloxx Pharmaceuticals up to $2 million in additional funding to support the clinical program of ELX-02, a potential therapy for cystic fibrosis (CF) caused by nonsense mutations. “The Cystic Fibrosis Foundation is committed to accelerating treatments for the underlying cause of…

The metabolism of cholesterol is impaired in a mouse model of cystic fibrosis, a preliminary study suggests. In the model, there seems to be an altered production of bile salts (those that help with the digestion of fats) that may reduce the digestion and/or absorption of cholesterol. This increases cholesterol’s…

Project GAIA, establishing a large-scale manufacturing process for Pyocin S5, an investigational antibiotic for the treatment of lung disease in people who have cystic fibrosis, is now complete. The scale-up process for Pyocin S5 manufacturing was performed by CPI, an independent technology innovation center in the U.K. This work will…

A modified version of Pulmozyme (dornase alfa), a widely-used cystic fibrosis (CF) therapy, lasts longer and reduces the need for treatment from once a day to once a week, a study in mice suggests. The study, “PEGylation of Recombinant Human Deoxyribonuclease I Provides a Long‐Acting Version of…

Pseudomonas aeruginosa mutate into a mucoid form in the lungs of people with cystic fibrosis (CF), creating biofilms that render these bacteria more resistant and more damaging, a study reported. Its researchers suggest a way of studying P. aeruginosa cells to identify treatments…