Ivacaftor and sinonasal pathology in a cystic fibrosis patient with genotype deltaF508/S1215N

In patients with Cystic Fibrosis and a type III mutation, ivacaftor (Kalydeco (R), Vertex) can increase the opening time of the CFTR channel and improve chloride transport. Research showed significant improvement of lung function and increase in weight following ivacaftor use. However, ivacaftor showed to have adverse events on the sinonasal system as well, such as upper respiratory tract infections, nasal congestion and headaches. This case report showed a positive effect of ivacaftor on the sinonasal pathology in a 17 year old patient with CF. After 5 months of ivacaftor use, the CT-sinus showed complete resolution of the pacification of the paranasal sinuses and a decrease in symptoms of sinonasal disease. This positive effect of ivacaftor on sinonasal pathology seems promising, therefore more research is needed to evaluate the effect of ivacaftor on the upper airways in CF. (C) 2014 Published by Elsevier B.V. on behalf of European Cystic Fibrosis Society.