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Taking Orkambi (lumacaftor/ivacaftor) for more than two years helped clear trapped air from the lungs of children with cystic fibrosis (CF), ages 6-11, but didn’t prevent an abnormal widening of the airways, a real-world study finds. The findings indicate the medication may not completely stop lung damage from…

Treatment with Kaftrio, a triple combo of elexacaftor, tezacaftor, and ivacaftor sold as Trikafta in the U.S., may help reduce the number of medications people with cystic fibrosis (CF) take each day, a study found. Patients may have to take multiple medications to manage all symptoms of…

Both genetics and environmental factors, such as attending daycare, family income, and maternal education, may determine how early some lung problems develop in children with cystic fibrosis (CF), a study finds. This is important because while variations in a person’s genes can’t be modified, “some extrinsic exposures can and…

Surrogate markers of nutritional status, including body mass index (BMI) and fat-free mass index, only weakly associated with lung function among children and adolescents with cystic fibrosis (CF) in a recent study. These young patients had different trajectories in nutritional status throughout childhood, with girls tending to maintain a…

Taking high-dose vitamin D supplements during acute lung infections may help to improve bone health for people with cystic fibrosis (CF). “These findings suggest that use of high-dose vitamin D supplementation during an APE [acute pulmonary exacerbation] may mitigate the bone loss suffered by people with CF with each…

An international team of scientists has published new guidelines for how best to perform exercise testing in people with cystic fibrosis (CF). “Guidance and standard operating procedures for functional exercise testing in cystic fibrosis” was published in European Respiratory Review. “Our goal is that all people…

The U.S. Food and Drug Administration (FDA) has granted fast track designation to BX004, a virus-based therapy that BiomX is developing to treat Pseudomonas aeruginosa bacterial infections in people with cystic fibrosis (CF). The FDA gives this designation to therapies that are designed to address unmet medical…

Note: This story has been updated Aug. 11, 2023, to correct the definition of a rare disease, which is defined as one affecting fewer than 200,000 people in the U.S., and to reflect that researchers estimate cystic fibrosis may affect about 160,000 people worldwide. Of the more than 100 investigational…

Treatment with Trikafta (ivacaftor/tezacaftor/elexacaftor) may help to ease a particular type of lung inflammation found in people with cystic fibrosis (CF), called a type 2 inflammatory response, that’s often associated with asthma and allergies. As asthma is reported to affect 30.8% of CF patients, a research team wondered…

A year of treatment with Kaftrio (elexacaftor/tezacaftor/ivacaftor) led to gains in lung function and nutritional status for cystic fibrosis (CF) patients in Germany, a real-world study in over 2,600 people reported. Sold as Trikafta in the U.S. and Canada, Kaftrio’s use also resulted in fewer in pulmonary exacerbations…