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Trikafta
elexacaftor/ tezacaftor/ ivacaftor and ivacaftor
Black Box Warnings .
Hepatotoxicity
can cause serious and potentially fatal drug-induced hepatic injury; hepatic failure leading to death or transplantation reported in pts w/ or w/o hepatic dz hx; hepatic injury reported w/in 1-15mo of tx start; monitor LFTs before tx start, then qmo x6mo, then q3mo x12mo, then at least q12mo or consider more frequently if hepatic dz hx or baseline LFTs elevated; interrupt tx if significant LFT elevations or if hepatic injury s/sx occur, consider hepatologist referral, and monitor closely until resolution; if abnormalities resolve, only restart tx if benefit outweighs risk w/ closer monitoring; avoid use in pts w/ severe hepatic impairment; in pts w/ moderate hepatic impairment, use not recommended or may use at reduced dose w/ close monitoring
Adult Dosing .
Dosage forms: convenience pack
Special Note
- [convenience pack components]
- Info: 7-day pack contains elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg tab x14 and ivacaftor 150 mg tab x7
cystic fibrosis
- [AM dose]
- Dose: 2 tabs (elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg) PO qam (1 dose = elexacaftor 200 mg/tezacaftor 100 mg/ivacaftor 150 mg); Info: for pts w/ at least one F508del-CFTR mutation or elexacaftor/tezacaftor/ivacaftor-responsive CFTR gene mutation; give 12h apart from evening dose; give w/ fat-containing food
- [PM dose]
- Dose: 1 tab (ivacaftor 150 mg) PO qpm; Info: for pts w/ at least one F508del-CFTR mutation or elexacaftor/tezacaftor/ivacaftor-responsive CFTR gene mutation; give 12h apart from morning dose; give w/ fat-containing food
renal dosing
- [see below]
- CrCl >30: no adjustment; CrCl <30: not defined, caution advised
- HD/PD: not defined
hepatic dosing
- [see below]
- Child-Pugh Class B: alternate 2 tabs elexacaftor/tezacaftor/ivacaftor w/ 1 tab elexacaftor/tezacaftor/ivacaftor at each morning dose, no ivacaftor evening doses; Child-Pugh Class C: avoid use
Peds Dosing .
- Dosage forms: convenience pack
Special Note
- [2-5 yo, <14 kg convenience pack components]
- Info: 7-day pack contains elexacaftor 80 mg/tezacaftor 40 mg/ivacaftor 60 mg pkt x7 and ivacaftor 59.5 mg pkt x7
- [2-5 yo, >14 kg convenience pack components]
- Info: 7-day pack contains elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg pkt x7 and ivacaftor 75 mg pkt x7
- [6-11 yo, <30 kg convenience pack components]
- Info: 7-day pack contains elexacaftor 50 mg/tezacaftor 25 mg/ivacaftor 37.5 mg tab x14 and ivacaftor 75 mg tab x7
- [6-11 yo, >30 kg convenience pack components]
- Info: 7-day pack contains elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg tab x14 and ivacaftor 150 mg tab x7
- [12 yo and older convenience pack components]
- Info: 7-day pack contains elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg tab x14 and ivacaftor 150 mg tab x7
cystic fibrosis
- [2-5 yo, <14 kg, AM dose]
- Dose: 1 pkt (elexacaftor 80 mg/tezacaftor 40 mg/ivacaftor 60 mg) PO qam; Info: for pts w/ at least one F508del-CFTR mutation or elexacaftor/tezacaftor/ivacaftor-responsive CFTR gene mutation; give 12h apart from evening dose; give w/ fat-containing food
- [2-5 yo, <14 kg, PM dose]
- Dose: 1 pkt (ivacaftor 59.5 mg) PO qpm; Info: for pts w/ at least one F508del-CFTR mutation or elexacaftor/tezacaftor/ivacaftor-responsive CFTR gene mutation; give 12h apart from morning dose; give w/ fat-containing food
- [2-5 yo, >14 kg, AM dose]
- Dose: 1 pkt (elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg) PO qam; Info: for pts w/ at least one F508del-CFTR mutation or elexacaftor/tezacaftor/ivacaftor-responsive CFTR gene mutation; give 12h apart from evening dose; give w/ fat-containing food
- [2-5 yo, >14 kg, PM dose]
- Dose: 1 pkt (ivacaftor 75 mg) PO qpm; Info: for pts w/ at least one F508del-CFTR mutation or elexacaftor/tezacaftor/ivacaftor-responsive CFTR gene mutation; give 12h apart from morning dose; give w/ fat-containing food
- [6-11 yo, <30 kg, AM dose]
- Dose: 2 tabs (elexacaftor 50 mg/tezacaftor 25 mg/ivacaftor 37.5 mg) PO qam (1 dose = elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg); Info: for pts w/ at least one F508del-CFTR mutation or elexacaftor/tezacaftor/ivacaftor-responsive CFTR gene mutation; give 12h apart from evening dose; give w/ fat-containing food
- [6-11 yo, <30 kg, PM dose]
- Dose: 1 tab (ivacaftor 75 mg) PO qpm; Info: for pts w/ at least one F508del-CFTR mutation or elexacaftor/tezacaftor/ivacaftor-responsive CFTR gene mutation; give 12h apart from morning dose; give w/ fat-containing food
- [6-11 yo, >30 kg, AM dose]
- Dose: 2 tabs (elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg) PO qam (1 dose = elexacaftor 200 mg/tezacaftor 100 mg/ivacaftor 150 mg); Info: for pts w/ at least one F508del-CFTR mutation or elexacaftor/tezacaftor/ivacaftor-responsive CFTR gene mutation; give 12h apart from evening dose; give w/ fat-containing food
- [6-11 yo, >30 kg, PM dose]
- Dose: 1 tab (ivacaftor 150 mg) PO qpm; Info: for pts w/ at least one F508del-CFTR mutation or elexacaftor/tezacaftor/ivacaftor-responsive CFTR gene mutation; give 12h apart from morning dose; give w/ fat-containing food
- [12 yo and older, AM dose]
- Dose: 2 tabs (elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg) PO qam (1 dose = elexacaftor 200 mg/tezacaftor 100 mg/ivacaftor 150 mg); Info: for pts w/ at least one F508del-CFTR mutation or elexacaftor/tezacaftor/ivacaftor-responsive CFTR gene mutation; give 12h apart from evening dose; give w/ fat-containing food
- [12 yo and older, PM dose]
- Dose: 1 tab (ivacaftor 150 mg) PO qpm; Info: for pts w/ at least one F508del-CFTR mutation or elexacaftor/tezacaftor/ivacaftor-responsive CFTR gene mutation; give 12h apart from morning dose; give w/ fat-containing food
renal dosing
- [see below]
- CrCl >30: no adjustment; CrCl <30: not defined, caution advised
- HD/PD: not defined
hepatic dosing
- [2-5 yo]
- Child-Pugh Class B: 1 pkt elexacaftor/tezacaftor/ivacaftor qam on days 1-3 and 5-6 of weekly dosing schedule, no dose on days 4 or 7; no ivacaftor evening doses; Child-Pugh Class C: avoid use
- [6 yo and older]
- Child-Pugh Class B: alternate 2 tabs elexacaftor/tezacaftor/ivacaftor w/ 1 tab elexacaftor/tezacaftor/ivacaftor at each morning dose, no ivacaftor evening doses; Child-Pugh Class C: avoid use