
Home Monitoring vs. Traditional Care in CF: Year-Long Study Results
A study of home monitoring vs. traditional cystic fibrosis care in 267 patients age fourteen and older showed that after a year, FEV1 decline is similar in both models, though exacerbations are detected earlier in the home monitoring group. Home monitoring consisted of twice weekly electronic logging of spirometry and …

Lung Clearance Index Improved in Children Ages 6-11 on Ivacaftor/Lumacaftor
Ivacaftor/Lumacaftor Improves Lung Clearance Index Score In Children A new phase 3 study, led by The Hospital for Sick Children at the University of Toronto, Canada, has been completed on the CF treatment ivacaftor/lumacaftor in children ages 6-11 with two F508-del CFTR mutations. In a group of 206 children from …

Outcomes and Tolerances in Cystic Fibrosis Treatment Lumacaftor/Ivacaftor
Last month, the results of a study were published that looked at the progress and side effects of the cystic fibrosis treatment lumacaftor/ivacaftor in 116 people with CF during a year after starting the medication. 46 (39.7%) people reported side effects related to lumacaftor/ivacaftor, and of the side effects, 82.2% …

Full-Fat Fermented Dairy May Cause Less Inflammation Than Regular Dairy
In the cystic fibrosis diet, dairy products are often used to reach high-calorie targets, and new research has shown that it may be beneficial to focus on full-fat fermented dairy rather than regular dairy, because full-fat fermented dairy may cause less cardiovascular inflammation. A study published in the British Journal …

Orkambi Study On Children Age 6-12 With DDF508: Cystic Fibrosis Treatment
Results are in from a 24-week study of Orkambi (lumacaftor/ivacaftor – a medication used in cystic fibrosis treatment) in 58 children with cystic fibrosis age 6-12 with two copies of the F508-delta mutation. Researchers were looking to examine the safety and efficacy of the CFTR potentiator on this age group and …

Risk of Hearing Loss from IV Aminoglycosides in Cystic Fibrosis
Cumulative exposure to IV tobramycin and amikacin, which are critical for treating cystic fibrosis infections, increases the risk of hearing loss. A study, “The cumulative effects of intravenous antibiotic treatments on hearing in patients with cystic fibrosis,” published last month in the Journal of Cystic Fibrosis discusses how IV tobramycin, …

Treatment for Cystic Fibrosis: Vertex Acquires Concert’s CFTR Potentiator CTP-656
Vertex Pharmaceuticals recently purchased the Phase 2 cystic fibrosis potentiator drug CTP-656 from Concert Pharmaceuticals. The deal is estimated to be worth $250 million dollars. CTP-656 is intended to be a longer-acting, once daily version of Vertex’s drug ivacaftor, which aims to restore CFTR function in treatment for Cystic Fibrosis. …

Respiratory Health and Air Pollution
The impact of small particle air pollution on the lower airways (lungs) has long been studied, but now scientists have evidence of its negative impact on the upper sinonasal airways as well. The World Health Organization’s recommendation for small particle air pollution (PM2.5) is that it not exceed an average …

Does Ivacaftor Affect Lung Bacteria in Cystic Fibrosis?
Ivacaftor has been shown to increase CFTR activity and lung function in people with Cystic Fibrosis and the G551D mutation, but until a recent study, scientists were not sure if ivacaftor changed the level of bacteria in the lungs. The results of a deeper study on ivacaftor have been published, …

Research on CF and Exercise in Cystic Fibrosis treatment
Significant research, both recent and decades old, shows that exercise helps people with CF. Yet it’s not always a part of a person’s Cystic Fibrosis treatment regimen. Here are some facts that may convince you to exercise more with the the guidance and approval of your CF team. “Exercise can …