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In a recent talk, researchers showed the enhanced benefits of two next-generation CFTR correctors in cystic fibrosis (CF) patients with specific mutations. The positive results support the clinical evaluation of both drugs in early-stage clinical trials. The talk, “Discovery and biological profile of next-generation CFTR correctors,” was recently presented at the…

A new database to improve the characterization of cystic fibrosis (CF) patients and more effectively identify variables that may impact patient success following lung transplant, was proposed recently at the 30th Annual North American Cystic Fibrosis Conference (NACFC), Oct. 27-29, in Orlando, by Dr. Elliott Dasenbrook, of the Cleveland Clinic. Dasenbrook proposed the database in…

Fewer than half of the cystic fibrosis (CF) patients eligible for treatment with Orkambi (lumacaftor/ivacaftor) used the medication at the end of its first year on the market, an especially low number when compared to the spread of Kalydeco (ivacaftor) use. The study, “Uptake of Lumacaftor-Ivacaftor Use…

Patients with very advanced cystic fibrosis (CF) lung disease who get a lung transplant have the same chances of survival as CF patients with more stable disease, according to a recent study. The findings suggest that lung transplants are not only appropriate for the sickest patients, but the approach is…

Differences in survival between adults with cystic fibrosis (CF) in Canada and the U.S. likely are explained by a delayed introduction of nutritional support for patients, according to a recent study. However, the findings, presented at the 30th Annual North American Cystic Fibrosis Conference (NACFC), held in Orlando, Florida…

A recent study revealed that many patients with cystic fibrosis (CF), as well as parents, are not aware of what the palliative care encompasses, and how it could be helpful to people with CF and their families. CF care providers, for this reason, need to be trained in discussing the topic of…

Two talks, presented today at the 30th Annual North American Cystic Fibrosis Conference (NACFC), focused on the role of nutrition in children with cystic fibrosis (CF). In a first presentation, “Have we journeyed to Junk?,” Tamarah Katz, MSc (Nutr&Diet), with the Sydney Children’s Hospital, quantified how much core (e.g., fruit, vegetables) and non-core foods (e.g., snacks, sweetened beverages) contribute to…

Kalydeco (ivacaftor), an approved therapy for cystic fibrosis (CF) patients with a specific mutation, improves insulin secretion in those with CF-related diabetes, researchers led by Ronald Rubenstein, MD, with the Children’s Hospital of Philadelphia, found. The mechanism underlying this benefit, however, remains unknown. The results were presented today at the 30th Annual North American…

In a talk presented today, Charles Esther Jr., MD, PhD, from UNC Chapel Hill, identified the loss of airway hydration as the first triggering factor in young children with cystic fibrosis (CF). The talk, titled “Mucus Dehydration as the Initiating Defect in Early CF Airways Disease,” was presented at the 30th Annual…

Among people with rare mutations in the CFTR gene, the gene defective in cystic fibrosis (CF), studies are needed to map down which patients may benefit from CFTR potentiators, such as Kalydeco (ivacaftor), or CFTR correctors. In this way, researchers will also be able to identify those in need…