ivacaftor (Kalydeco) | strong CYP3A inhibitors (e.g., ketoconazole) | - Greater than or equal to 6 years of age:
- 6 months to less than 6 years of age:
- Greater than or equal to 14 kg:
- 75 mg granule packet twice weekly
- 7 kg to less than 14 kg:
- 50 mg granule packet twice weekly
- 5 kg to less than 7 kg:
- 25 mg granule packet twice weekly
- less than 6 months of age:
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ivacaftor (Kalydeco) | moderate CYP3A inhibitors (e.g., erythromycin, fluconazole) | - Greater than 6 years of age:
- 6 months to less than 6 years of age:
- Greater than or equal to 14 kg:
- 75 mg granule packet orally once daily
- 7 kg to less than 14 kg:
- 50 mg granule packet orally once daily
- 5 kg to less than 7 kg:
- 25 mg granule packet orally once daily
- less than 6 months of age:
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lumacaftor/ivacaftor (Orkambi) | strong CYP3A inhibitors (e.g., ketoconazole) | - Greater than or equal to 12 years:
- lumacaftor/ivacaftor dosages should be reduced to 200 mg/125 mg once daily for the first week, followed by 2 tablets (lumacaftor 400 mg/ivacaftor 250 mg) every 12 hours thereafter; no dosage adjustments are needed if CYP3A inhibitors are initiated in patients already taking lumacaftor/ivacaftor
- 6 to 11 years:
- lumacaftor/ivacaftor dosages should be reduced to 100 mg/125 mg once daily for the first week, followed by 2 tablets (lumacaftor 200 mg/ivacaftor 250 mg) every 12 hours thereafter; no dosage adjustments are needed if CYP3A inhibitors are initiated in patients already taking lumacaftor/ivacaftor
- 2 to 5 years (greater than or equal to 14 kg):
- lumacaftor/ivacaftor dosages should be reduced to 1 granule packet (150 mg/188 mg) every other day for the first week, followed by 1 granule packet (lumacaftor 150 mg/ivacaftor 188 mg) every 12 hours thereafter; no dosage adjustments are needed if CYP3A inhibitors are initiated in patients already taking lumacaftor/ivacaftor
- 2 to 5 years (less than 14 kg):
- lumacaftor/ivacaftor dosages should be reduced to 1 granule packet (100 mg/125 mg) every other day for the first week, followed by 1 granule packet (lumacaftor 100 mg/ivacaftor 125 mg) every 12 hours thereafter; no dosage adjustments are needed if CYP3A inhibitors are initiated in patients already taking lumacaftor/ivacaftor
- 1-2 years greater than or equal to 14 kg):
- lumacaftor/ivacaftor dosages should be reduced to 1 granule packet (150 mg/188 mg) every other day for the first week, followed by 1 granule packet (lumacaftor 150 mg/ivacaftor 188 mg) every 12 hours thereafter; no dosage adjustments are needed if CYP3A inhibitors are initiated in patients already taking lumacaftor/ivacaftor
- 1-2 years (9 to less than 14 kg):
- lumacaftor/ivacaftor dosages should be reduced to 1 granule packet (100 mg/125 mg) every other day for the first week, followed by 1 granule packet (lumacaftor 100 mg/ivacaftor 125 mg) every 12 hours thereafter; no dosage adjustments are needed if CYP3A inhibitors are initiated in patients already taking lumacaftor/ivacaftor
- 1-2 years (7 to less than 9 kg):
- lumacaftor/ivacaftor dosages should be reduced to 1 granule packet (75 mg/94 mg) every other day for the first week, followed by 1 granule packet (lumacaftor 75 mg/ivacaftor 94 mg) every 12 hours thereafter; no dosage adjustments are needed if CYP3A inhibitors are initiated in patients already taking lumacaftor/ivacaftor
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lumacaftor/ivacaftor (Orkambi) | moderate CYP3A inhibitors (e.g., erythromycin, fluconazole) | no dosage adjustments recommended |
tezacaftor/ ivacaftor (Symdeko) | strong CYP3A inhibitors (e.g., ketoconazole) | - greater than or equal to 6 years to less than 12 years (greater than or equal to 30 kg) & patients greater than or equal to12 years:
- Day 1: tezacaftor 100 mg/ivacaftor 150 mg in the morning
- Days 2 and 3: no dosages administered
- Day 4: tezacaftor 100 mg/ivacaftor 150 mg in the morning
- The evening ivacaftor dose should NOT be given
- Continue twice weekly (morning) administration schedule, with 3-4 days between dosages
- greater than or equal to6 years to less than 12 years (less than 30 kg):
- Day 1: tezacaftor 50 mg/ivacaftor 75 mg in the morning
- Days 2 and 3: no dosages administered
- Day 4: tezacaftor 50 mg/ivacaftor 75 mg in the morning
- The evening ivacaftor dose should NOT be given
- Continue twice weekly (morning) administration schedule, with 3-4 days between dosages
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tezacaftor/ivacaftor (Symdeko) | moderate CYP3A inhibitors (e.g., erythromycin, fluconazole) | - greater than or equal to 6 years to less than 12 years (greater than or equal to30 kg) and patients greater than or equal to12 years:
- Day 1: tezacaftor 100 mg/ivacaftor 150 mg in the morning
- Day 2: ivacaftor 150 mg in the morning
- The evening ivacaftor dose should NOT be given
- Continue alternate day (morning) administration schedule
- greater than or equal to 6 years to less than 12 years (less than 30 kg):
- Day 1: tezacaftor 50 mg/ivacaftor 75 mg in the morning
- Day 2: ivacaftor 75 mg in the morning
- The evening ivacaftor dose should NOT be given
- Continue alternate day (morning) administration schedule
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elexacaftor/tezacaftor/ivacaftor (Trikafta) | strong CYP3A inhibitors (e.g., ketoconazole) | - greater than or equal to12 years of age:
- 2 tablets (elexacaftor 100 mg/ tezacaftor 50 mg/ ivacaftor 75 mg) in the morning twice weekly, with 3-4 days between dosages
The evening ivacaftor dose should NOT be given
- 6-11 years of age:
- weight greater than or equal to30 kg:
- 2 tablets (elexacaftor 100 mg/ tezacaftor 50 mg/ ivacaftor 75 mg) in the morning twice weekly, with 3-4 days between doses
The evening ivacaftor dose should NOT be given
- 6-11 years of age:
- weight less than 30 kg:
- 2 tablets (elexacaftor 50 mg/ tezacaftor 25 mg/ ivacaftor 37.5 mg) in the morning twice weekly, with 3-4 days between doses
The evening ivacaftor dose should NOT be given
- 2-5 years of age:
- weight greater than or equal to14 kg:
- one packet (elexacaftor 100 mg/ tezacaftor 50 mg/ ivacaftor 75 mg) in the morning twice weekly, with 3-4 days between doses
The evening ivacaftor dose should NOT be given
- 2-5 years of age:
- weight less than 14 kg:
- one packet (elexacaftor 80 mg/ tezacaftor 40 mg/ ivacaftor 60 mg) in the morning twice weekly, with 3-4 days between doses
The evening ivacaftor dose should NOT be given
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elexacaftor/tezacaftor/ivacaftor (Trikafta) | moderate CYP3A inhibitors (e.g., erythromycin, fluconazole) | - greater than or equal to 12 years of age:
- Day 1: 2 tablets (elexacaftor 100 mg/ tezacaftor 50 mg/ ivacaftor 75 mg) in the morning
- Day 2: ivacaftor 150 mg in the morning
- The evening ivacaftor dose should NOT be given
- Continue alternate day (morning) administration schedule
- 6-11 years of age:
- Weight greater than or equal to 30 kg:
- Day 1: 2 tablets (elexacaftor 100 mg/ tezacaftor 50 mg/ ivacaftor 75 mg) in the morning
- Day 2: ivacaftor 150 mg in the morning
- The evening ivacaftor dose should NOT be given
- Continue alternate day (morning) administration schedule
- 6-11 years of age:
- Weight less than 30 kg:
- Day 1: 2 tablets (elexacaftor 50 mg/ tezacaftor 25 mg/ ivacaftor 37.5 mg) in the morning
- Day 2: ivacaftor 75 mg in the morning
- The evening ivacaftor dose should NOT be given
- Continue alternate day (morning) administration schedule
- 2-5 years of age:
- weight greater than or equal to 14 kg:
- Day 1: one packet (elexacaftor 100 mg/ tezacaftor 50 mg/ ivacaftor 75 mg) in the morning
- Day 2: one packet (ivacaftor 75 mg) in the morning
- The evening ivacaftor dose should NOT be given
- Continue alternate day (morning) administration schedule
- 2-5 years of age:
- weight less than 14 kg:
- Day 1: one packet (elexacaftor 80 mg/ tezacaftor 40 mg/ ivacaftor 60 mg) in the morning
- Day 2: one packet (ivacaftor 59.5 mg) in the morning
- The evening ivacaftor dose should NOT be given
- Continue alternate day (morning) administration schedule
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