The effect of ivacaftor (VX-770, registered in Australia as Kalydeco) on exocrine pancreatic function in patients 6 years and older with cystic fibrosis and at least one copy of the G551D CFTR mutation and who are naive for ivacaftor

Exclusion Criteria7

• known hypersensitivity to ivacaftor, its metabolites, or formulation excipients
• galactose intolerance, Lapp lactase deficiency or glucose-galactose malabsorption
• have undergone organ transplantation
• Use of any inhibitors (itraconazole, posaconazole, voriconazole, ketoconazole, telithromycin, clarithromycin) or inducers of (rifampicin, rifabutin, phenobarbital, carbamazepine, phenytoin, St Johns Wort) CYP450 3A, within 14 days prior to the first dose of ivacaftor or during the study period
• hepatic insufficiency defined as Child-Pugh Class C impairment (a score of 10)
• severe renal impairment, as defined by creatinine clearance less than or equal to 30mL/min or end stage renal disease
• Pregnant, planning a pregnancy during the course of the study , or breast-feeding