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• For use in patients with G551D mutation in CFTR gene • Dose should be taken with a fat-containing meal • Avoid food containing grapefruit or Seville oranges
• Reduce dose to 150mg twice weekly with concomitant administration of potent inhibitors of CYP3A, and once daily with moderate inhibitors of CYP3A • Reduce dose in moderate-to-severe hepatic impairment (150mg OD and 150mg alt days, respectively) • Sweat chloride may be used as a surrogate marker for bioavailability, particularly if ivacaftor dose is reduced due to concomitant administration of interacting drugs (changes occur within 15 days)