News

The use of the approved medication Trikafta (elexacaftor/tezacaftor/ivacaftor) was shown to significantly increase weight in people with cystic fibrosis-related diabetes (CFRD) and a low body mass index, or BMI, a measure of body fat, in a new study. The researchers say the study’s findings, which also showed…

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,…

Gut bacteria called Bacteroides had anti-inflammatory effects in the blood and airways of a mouse model of cystic fibrosis (CF) via its production of an immune-modulating metabolite called propionate, a study finds. The findings add to evidence of a so-called gut-lung axis, where microbes in the gut can influence…

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…

Sotagliflozin, an oral medication approved in the U.S. under the brand name Inpefa as a broadly used treatment for heart failure, may ease symptoms of liver disease related to cystic fibrosis (CF), according to a study in rabbits. While it’s still too early to tell if sotagliflozin will…

Some people with cystic fibrosis (CF) have levels of sweat chloride above the cut-off for a diagnosis of the disease despite being on CFTR modulators, suggesting there is room for improving treatment, a study found. The study, “Heterogeneity of CFTR modulator-induced sweat chloride concentrations in people with…

BiomX and Adaptive Phage Therapeutics (APT) are entering into a merger agreement with a goal of pooling resources to advance phage therapies such as BX004 — an experimental cystic fibrosis (CF) treatment that aims to clear up lung infections caused by Pseudomonas aeruginosa bacteria. At the same time as…

Sionna Therapeutics has raised $182 million in financing to advance the development of small molecules designed to restore CFTR protein function by binding to a specific part of the protein called the first nucleotide-binding domain, or NBD1. Cystic fibrosis (CF) is caused by mutations in the gene…

A New York State assistance program re-launched last year to provide financial aid to adults with cystic fibrosis (CF) for medical costs has a new administrator. The HealthWell Foundation‘s subsidiary HWF Direct has been tapped to administer the state’s Adult Cystic Fibrosis Assistance Program, known as ACFAP.

Stopping treatment with inhaled supportive therapies hypertonic saline (HS) or Pulmozyme (dornase alfa) didn’t negatively affect airway mucus clearance among cystic fibrosis (CF) patients with mild lung disease who were being also treated with Trikafta (elexacaftor/tezacaftor/ivacaftor), a report suggests. Indeed, the findings from the six-week study…