Ivacaftor
Absorption	Take tablet with fat-containing food
Elevated liver transaminases	Monitor alanine and aspartate transaminases prior to initiation, every 3 months in the first year, and annually thereafter
Cataracts	Eye exam prior to initiation and follow-up for patients younger than 18 years of age
Drug interactions (St. John’s wort, rifampin)	Decreases exposure to ivacaftor and co-adminstration is not recommended
Drug interactions (ketoconazole, fluconazole)	Increases exposure to ivacaftor. Dose adjustment of is needed.
Drug interactions (Seville oranges, grapefruit)	Increases exposure to ivacaftor. Avoid food containing grapefruit or Seville oranges.
Lumacaftor-Ivacaftor
Absorption	Take tablet with fat-containing food
Elevated liver transaminases	Monitor alanine and aspartate transaminases prior to initiation, every 3 months in the first year, and annually thereafter
Liver disease	Childs-Pugh score classification and dose adjustment may be necessary. Caution in patients with advanced liver disease.
Cataracts	Eye exam prior to initiation and follow-up for patients younger than 18 years of age
Chest discomfort, dyspnea, breathing difficulties	Additional monitoring recommended for patients with FEV1% predicted <40 during therapy initiation
Drug interactions (hormonal contraceptives)	Decreased efficacy of hormonal contraception. Alternative methods of contraception and anticipation of menstrual-related adverse reactions.
Drug interactions (benzodiazepines, immunosuppressants, digoxin, corticosteroids, antidepressants, proton pump inhibitors)	Decreases efficacy of these medications. Dose adjustment, level monitoring if applicable, or alternative agent recommended. Co-administration not recommended.
Drug interactions (St. John’s wort, rifampin, phenytoin)	Decreases exposure to lumacaftor-ivacaftor and co-adminstration is not recommended
Drug interactions (ketoconazole, itraconazole, voriconazole, clarithromycin)	Increases exposure to lumacaftor-ivacaftor. Dose adjustment may be needed when initiating therapy.