News

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…

Liver disease in cystic fibrosis (CF) patients needs to be detected much earlier, but early detection is increasingly likely as research interest in severe forms of the condition has grown. That was the message relayed during a talk by Dr. Daniel Leung of Baylor College Of Medicine in Texas, presented…

Recommendations by the Cystic Fibrosis Foundation (CFF) on enternal tube feeding for patients were supported by Jillian Sullivan, MD, from the University of Vermont Children’s Hospital today at the 30th Annual North American Cystic Fibrosis Conference in Orlando, Florida. Sullivan’s presentation,“Role of the Gastroenterologist in Enteral Tube Feedings,” was part of a symposium…