A lack of stability in a specific region of the CFTR protein may explain why certain people with cystic fibrosis (CF) don’t respond to available modulator therapies, a new study suggests. The findings imply drugs to stabilize this specific region — some of which are already in clinical development…
Two experimental CFTR modulator therapies being developed by Sionna Therapeutics are able to improve the function of the most common mutated form of CFTR protein that causes cystic fibrosis (CF). That’s according to new data Sionna presented at the European Cystic Fibrosis Society (ECFS) 48th European Cystic Fibrosis…
Although infants with cystic fibrosis (CF) may have lung function abnormalities between the ages of 3 months and 2 years, these are predominantly mild and temporary, a real-world study reports. Tests that indicate abnormal lung function may influence decisions about clinical management of CF. Clinical findings, however, including evidence…
SION-719 and SION-451, two NBD1 stabilizers Sionna Therapeutics is developing to restore CFTR protein function in cystic fibrosis (CF), will advance to the next stages of clinical development after positive safety results in Phase 1 clinical trials. Sionna tested both compounds in healthy volunteers to assess safety and…
While most adults with cystic fibrosis (CF) view their health as very good or excellent while on Kalydeco (ivacaftor) or Trikafta (elexacaftor/tezacaftor/ivacaftor) — both approved CFTR modulator therapies for CF — about 1 in 7 still report their health as fair or poor, a survey study…
Using inhaled antibiotics can help control the growth of certain bacteria in the lungs, but it may increase the risk of fungal infections with Aspergillus fumigatus in people with cystic fibrosis (CF), according to a study. The study, “Association between inhaled antibiotic use and treatment-emergent organisms among Canadian people with cystic fibrosis,”…
The Cystic Fibrosis Foundation (CFF) has awarded $5 million to the Cleveland Clinic to continue as the biorepository coordinating center for the Cystic Fibrosis Lung Transplant Consortium until 2030, supporting research and other efforts to improve lung transplant outcomes. Created in 2017, the consortium has established a…
Scientists have discovered a potential therapy for cystic fibrosis (CF) patients who are infected with Mycobacterium abscessus, a bacterium that can cause serious lung infections. They found that a signaling molecule called PI5P can boost the antimicrobial activity of immune cells isolated from patients against the bacterium. This happens even…
In young children with cystic fibrosis (CF), infections with methicillin-resistant Staphylococcus aureus (MRSA) are linked to more antibiotic use, worse lung function, and more lung damage by early school age, according to a study led by researchers in Brazil. Because infections with MRSA appear to indicate more severe disease…
A personalized inhaled phage therapy targeting multidrug-resistant lung infections significantly reduces bacterial load and improves lung function in adults with cystic fibrosis (CF), according to a small study. The experimental therapy was personalized by selecting phages that worked best against Pseudomonas aeruginosa strains in patients’ sputum samples. It…
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