Ivacaftor can reduce common infections in patients with cystic fibrosis

Patients with cystic fibrosis who take ivacaftor seem to have fewer respiratory infections over time than those who do not take it, according to new research published online on the Internet. Annals of the American Thoracic Society.

Cystic fibrosis is caused by mutations in the transmembrane conductance regulator gene (CFTR) of cystic fibrosis. Ivacaftor (Kalydeco) is prescribed to patients whose cystic fibrosis is caused by a "gating" mutation. This group of mutations, responsible for about 4% of all cases of cystic fibrosis, prevents chlorides from entering and exiting cells. By restoring the functions of the door, ivacaftor has proven to improve lung function and quality of life.

In "Ivacaftor is badociated with reduced lung infection due to key pathogens of cystic fibrosis: cohort study using national registry data," British researchers said that ivacaftor could also reduce some of the lung infections the most common in patients with cystic fibrosis, including P. aeruginosa. If this is true, they say, it may mean that some patients taking ivacaftor may take fewer antibiotics in the long run while remaining clinically stable.

"People with CF experience improvement in lung function and quality of life shortly after starting ivacaftor treatment, but they still have to withstand a considerable treatment burden related to all other drugs that they have." they take, "said lead author Freddy Frost, BMBS, a fibrosis specialist at Liverpool Heart & Chest Hospital. "At the present time, we simply do not know if it is safe to stop some of these other treatments.The fact that we have seen a reduction in infections in this study suggests that some people can safely stop drugs targeting these infections. "

Using data from a cystic fibrosis registry in the United Kingdom, the authors compared patients (aged six years and older) with cystic fibrosis treated with ivacaftor to those with cystic fibrosis who had not taken the drug over a period of three years.

The study found that ivacaftor was badociated with a 32% reduction in the number of people infected with P. aeruginosa and a 15% reduction in Staphylococcus aureus. These reductions resulted in both increased clearance of infection in previously infected individuals and reduced acquisition in those who were not infected.

The authors stated that "with people starting ivacaftor and other drugs correcting the defects of the CF gene at an early age, the ability to prevent these infections is particularly interesting. "If these medications are taken before chronic infection begins, the risk of contracting an infection in the future can be significantly reduced," said Dr. Frost.

Ivacaftor was also badociated with a reduction of Aspergillus spp. infections. The drug did not appear to reduce infections to the Burkholderia cepacia complex.

Dr. Frost said the study adds to the growing evidence of long-term benefits of ivacaftor. "However, he added, randomized controlled trials will be needed to find out if it is safe to reduce the overall treatment burden of CF by decreasing the use of antibiotics in patients taking of ivacaftor or other drugs targeting defects of the CF gene ".