AdultPediatricDosage Forms & Strengths copackaged tablets Copackaged as a fixed dose combination tablet of tezacaftor 100mg and ivacaftor 150mg PLUS a separate ivacaftor 150mg tablet Cystic Fibrosis Indicated for cystic fibrosis (CF) in patients who are homozygous for the F508del mutation or who have at least 1 mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene that is responsive to tezacaftor/ivacaftor based on in vitro data and/or clinical evidence Morning dose: One tezacaftor/ivacaftor 100-mg/150-mg fixed-dose tablet PO Evening dose: One ivacaftor 150-mg tablet PO Administer morning and evening doses ~12 hr apart Dosage Modifications Hepatic impairment Mild (Child-Pugh A): No dose adjustment required Moderate (Child-Pugh B) Morning dose: One tablet of tezacaftor 100 mg/ivacaftor 150 mg PO qDay Evening dose: Omit evening ivacaftor 150-mg dose Severe (Child-Pugh C) Morning dose: One tablet of tezacaftor 100 mg/ivacaftor 150 mg PO once daily or less frequently Evening dose: Omit evening ivacaftor 150-mg dose Renal impairment Mild or moderate: No dose adjustment required Severe or ESRD: Caution recommended Not studied in moderate/severe impairment or ESRD Coadministration with CYP3A inhibitors Avoid food or drink containing grapefruit or Seville oranges during treatment Moderate CYP3A inhibitors Morning dose: Alternate dosing schedule (ie, day 1, administer tezacaftor 100 mg/ivacaftor 150 mg qDay; on day 2, administer ivacaftor 150 mg qDay; continue dosing schedule with tezacaftor 100 mg/ivacaftor 150 mg or ivacaftor 150 mg on alternate days) Evening dose: Do not administer Strong CYP3A inhibitors Morning dose: Adjust dose to twice weekly, taken ~3-4 days apart (ie, on day 1, administer tezacaftor 100 mg/ivacaftor 150 mg qDay; do not administer any tablets on days 2 and 3; on day 4, administer tezacaftor 100 mg/ivacaftor 150 mg qDay; continue dosing with tezacaftor 100 mg/ivacaftor 150 mg twice a week, administered ~3 -4 days apart) Evening dose: Do not administer Dosing Considerations If the patient’s genotype is unknown, an FDA-cleared CF mutation test should be used to detect the presence of a CFTR mutation, followed by verification with bidirectional sequencing when recommended by the mutation test instructions for useDosage Forms & Strengths copackaged tablets Copackaged as a fixed-dose combination tablet of tezacaftor 50mg and ivacaftor 75mg PLUS a separate ivacaftor 75mg tablet Copackaged as a fixed dose combination tablet of tezacaftor 100mg and ivacaftor 150mg PLUS a separate ivacaftor 150mg tablet Cystic Fibrosis Indicated for cystic fibrosis in patients aged ≥6 yr who are homozygous for the F508del mutation or who have at least 1 mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene that is responsive to tezacaftor/ivacaftor based on in vitro data and/or clinical evidence <6 years: Safety and efficacy not established 6 to <12 years weighing <30 kg Morning dose: One tezacaftor/ivacaftor 50-mg/75-mg fixed-dose tablet PO Evening dose: One ivacaftor 75-mg tablet PO Administer morning and evening doses ~12 hr apart ≥6 years weighing ≥30 kg Morning dose: One tezacaftor/ivacaftor 100-mg/150-mg fixed-dose tablet PO Evening dose: One ivacaftor 150-mg tablet PO Administer morning and evening doses ~12 hr apart Dosage Modifications Hepatic impairment for patients aged 6 to <12 years weighing <30 kg Mild (Child-Pugh A): No dose adjustment required Moderate (Child-Pugh B) Morning dose: One tablet of tezacaftor 50 mg/ivacaftor 75 mg PO qDay Evening dose: Omit evening ivacaftor 75-mg dose Severe (Child-Pugh C) Morning dose: One tablet of tezacaftor 50 mg/ivacaftor 75 mg PO qDay or less frequently Evening dose: Omit evening ivacaftor 75-mg dose Hepatic impairment patients aged ≥6 years weighing ≥30 kg Mild (Child-Pugh A): No dose adjustment required Moderate (Child-Pugh B) Morning dose: One tablet of tezacaftor 100 mg/ivacaftor 150 mg PO qDay Evening dose: Omit evening ivacaftor 150-mg dose Severe (Child-Pugh C) Morning dose: One tablet of tezacaftor 100 mg/ivacaftor 150 mg PO qDay or less frequently Evening dose: Omit evening ivacaftor 150-mg dose Renal impairment Mild or moderate: No dose adjustment required Severe or ESRD: Not studied, caution recommended Not studied in moderate/severe impairment or ESRD Coadministration with CYP3A inhibitors for patients aged 6 to <12 years weighing <30 kg Avoid food or drink containing grapefruit or Seville oranges during treatment Moderate CYP3A inhibitors Morning dose: Alternate dosing schedule (ie, day 1, administer tezacaftor 50 mg/ivacaftor 75 mg qDay; on day 2, administer ivacaftor 75 mg qDay; continue dosing schedule with tezacaftor 50 mg/ivacaftor 75 mg or ivacaftor 75 mg on alternate days) Evening dose: Do not administer Strong CYP3A inhibitors Morning dose: Adjust dose to twice weekly, taken ~3-4 days apart (ie, on day 1, administer tezacaftor 50 mg/ivacaftor 75 mg qDay; do not administer any tablets on days 2 and 3; on day 4, administer tezacaftor 50 mg/ivacaftor 75 mg qDay; continue dosing with tezacaftor 50 mg/ivacaftor 75 mg twice a week, administered ~3 -4 days apart) Evening dose: Do not administer Coadministration with CYP3A inhibitors for patients aged ≥6 years weighing ≥30 kg Avoid food or drink containing grapefruit or Seville oranges during treatment Moderate CYP3A inhibitors Morning dose: Alternate dosing schedule (ie, day 1, administer tezacaftor 100 mg/ivacaftor 150 mg qDay; on day 2, administer ivacaftor 150 mg qDay; continue dosing schedule with tezacaftor 100 mg/ivacaftor 150 mg or ivacaftor 150 mg on alternate days) Evening dose: Do not administer Strong CYP3A inhibitors Morning dose: Adjust dose to twice weekly, taken ~3-4 days apart (ie, on day 1, administer tezacaftor 100 mg/ivacaftor 150 mg qDay; do not administer any tablets on days 2 and 3; on day 4, administer tezacaftor 100 mg/ivacaftor 150 mg qDay; continue dosing with tezacaftor 100 mg/ivacaftor 150 mg twice a week, administered ~3 -4 days apart) Evening dose: Do not administer Dosing Considerations If the patient’s genotype is unknown, an FDA-cleared cystic fibrosis mutation test should be used to detect the presence of a CFTR mutation, followed by verification with bidirectional sequencing when recommended by the mutation test instructions for useInteraction CheckerEnter a drug name and tezacaftor/ivacaftorNo Results No Interactions Found Interactions FoundContraindicatedSerious - Use AlternativeSignificant - Monitor CloselyMinorAll Interactions Sort By: SeverityName Contraindicated (1)lonafarnibivacaftor will increase the level or effect of lonafarnib by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Contraindicated. Lonafarnib is a sensitive CYP3A4 substrate. Coadministration with strong or moderate CYP3A4 inhibitors is contraindicated. Serious - Use Alternative (62)abametapirabametapir increases levels of ivacaftor by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug. For 2 weeks after abametapir application, avoid taking drugs that are CYP3A4 substrates. If not feasible, avoid use of abametapir.abametapir will increase the level or effect of tezacaftor by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug. For 2 weeks after abametapir application, avoid taking drugs that are CYP3A4 substrates. If not feasible, avoid use of abametapir.amobarbitalamobarbital will decrease the level or effect of tezacaftor by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug.apalutamideapalutamide will decrease the level or effect of ivacaftor by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug. Coadministration of apalutamide, a strong CYP3A4 inducer, with drugs that are CYP3A4 substrates can result in lower exposure to these medications. Avoid or substitute another drug for these medications when possible. Evaluate for loss of therapeutic effect if medication must be coadministered. Adjust dose according to prescribing information if needed.apalutamideapalutamide will decrease the level or effect of tezacaftor by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug. Coadministration of apalutamide, a strong CYP3A4 inducer, with drugs that are CYP3A4 substrates can result in lower exposure to these medications. Avoid or substitute another drug for these medications when possible. Evaluate for loss of therapeutic effect if medication must be coadministered. Adjust dose according to prescribing information if needed.avapritinibivacaftor will increase the level or effect of avapritinib by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug. Avoid coadministration of avapritinib with strong CYP3A4 inhibitors.bosentanbosentan will decrease the level or effect of tezacaftor by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug.bosentan decreases levels of ivacaftor by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug. Avoid coadministration with strong CYP3A4 inducers; systemic exposure of ivacaftor substantially reduced (ie, ~9-fold).butabarbitalbutabarbital will decrease the level or effect of tezacaftor by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug.carbamazepinecarbamazepine decreases levels of ivacaftor by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug. Avoid coadministration with strong CYP3A4 inducers; systemic exposure of ivacaftor substantially reduced (ie, ~9-fold).butalbitalbutalbital will decrease the level or effect of tezacaftor by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug.carbamazepinecarbamazepine will decrease the level or effect of tezacaftor by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug.chloramphenicolchloramphenicol will increase the level or effect of tezacaftor by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug.cimetidineivacaftor increases levels of cimetidine by P-glycoprotein (MDR1) efflux transporter. Avoid or Use Alternate Drug. Ivacaftor and its M1 metabolite has the potential to inhibit P-gp; may significantly increase systemic exposure to sensitive P-gp substrates with a narrow therapeutic index.colchicineivacaftor increases levels of colchicine by P-glycoprotein (MDR1) efflux transporter. Avoid or Use Alternate Drug. Avoid use of colchicine with P-gp inhibitors. If coadministration is necessary, decrease colchicine dose or frequency as recommended in prescribing information. Use of any colchicine product in conjunction with P-gp inhibitors is contraindicated in patients with renal or hepatic impairment. .dabigatranivacaftor increases levels of dabigatran by P-glycoprotein (MDR1) efflux transporter. Avoid or Use Alternate Drug. Atrial fibrillation: Avoid coadministering dabigatran with P-gp inhibitors if CrCl <30 mL/min. DVT/PE treatment: Avoid coadministering dabigatran with P-gp inhibitors if CrCl <50 mL/min.dabrafenibdabrafenib will decrease the level or effect of tezacaftor by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug.dexamethasonedexamethasone will decrease the level or effect of tezacaftor by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug.dexamethasone decreases levels of ivacaftor by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug. Avoid coadministration with strong CYP3A4 inducers; systemic exposure of ivacaftor substantially reduced (ie, ~9-fold).efavirenzefavirenz will decrease the level or effect of tezacaftor by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug.efavirenz, ivacaftor. Other (see comment). Avoid or Use Alternate Drug. Comment: Efavirenz decreases levels of ivacaftor by CYP enzyme induction; whereas, ivacaftor increases levels of efavirenz by inhibiting CYP3A4 .elacestrantivacaftor will increase the level or effect of elacestrant by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug.enzalutamideenzalutamide will decrease the level or effect of tezacaftor by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug.enzalutamideenzalutamide will decrease the level or effect of ivacaftor by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug.eslicarbazepine acetateeslicarbazepine acetate will decrease the level or effect of tezacaftor by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug.eslicarbazepine acetate will decrease the level or effect of ivacaftor by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug. Avoid coadministration with strong CYP3A4 inducers; systemic exposure of ivacaftor substantially reduced (ie, ~9-fold)etravirineetravirine decreases levels of ivacaftor by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug. Avoid coadministration with strong CYP3A4 inducers; systemic exposure of ivacaftor substantially reduced (ie, ~9-fold).etravirine will decrease the level or effect of tezacaftor by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug.fexinidazolefexinidazole will increase the level or effect of ivacaftor by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug. Fexinidazole inhibits CYP3A4. Coadministration may increase risk for adverse effects of CYP3A4 substrates.fexinidazole will increase the level or effect of tezacaftor by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug. Fexinidazole inhibits CYP3A4. Coadministration may increase risk for adverse effects of CYP3A4 substrates.fosphenytoinfosphenytoin decreases levels of ivacaftor by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug. Avoid coadministration with strong CYP3A4 inducers; systemic exposure of ivacaftor substantially reduced (ie, ~9-fold).fosphenytoin will decrease the level or effect of tezacaftor by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug.grapefruitgrapefruit increases levels of ivacaftor by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug. Avoid grapefruit, grapefruit juice, or Seville oranges while taking ivacaftor.grapefruit will increase the level or effect of tezacaftor by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug. Coadministration of tezacaftor with grapefruit juice, which contains 1 or more components that moderately inhibit CYP3A, may increase exposure of tezacaftor. Avoid food containing grapefruit or Seville oranges.infigratinibivacaftor will increase the level or effect of infigratinib by affecting hepatic/intestinal enzyme CYP3A4 metabolism. Avoid or Use Alternate Drug.ivosidenibivosidenib will decrease the level or effect