Ivacaftor (Kalydeco) added to standard care for hard-to-treat chronic rhinosinusitis

You may qualify if

• 18 years of age
• Patient has provided informed consent
• Diagnosis of CRS made by one of the investigators
• Standard of care CT scan with definitive demonstration of isolated or diffuse mucosal thickening, bone changes, and air fluid levels, obtained within 30 days of treatment
• Positive culture of at least one gram negative bacteria (e.g. Pseudomonas, E. coli, Steenotrophomonas) within 30 days prior to testing
• Previous surgery with (at least) exposed maxillary and ethmoid sinuses
• Ability to perform EDSPD testing such that nasal cavity space and sinus openings are sufficient for catheter placement
• Negative pregnancy test for females of childbearing potential within 72 hours of testing and start of study treatment

You can't join if

• < 18 years of age
• Acute illness other than sinusitis within 2 weeks before start of study treatment that, in the opinion of the investigator, would preclude participation
• Currently taking medications that are moderate or strong CP3A inhibitors
• History of asthma attack requiring emergency room visit or treatment with oral steroids within 2 months prior to study treatment
• History of solid organ or hematological transplantation
• History of known immunodeficiency, autoimmune or granulomatous disorder
• Serum creatinine > 1.5x upper normal limit
• Abnormal liver function, as defined by serum AST > 2x upper normal limit, serum ALT > 2x upper normal limit, Alkaline phosphatase > 2x upper normal limit, Total bilirubin > 2x upper normal limit
• Women who are pregnant or breastfeeding