Elexacaftor / Ivacaftor / Tezacaftor Dosage

Medically reviewed by Drugs.com. Last updated on Jun 28, 2021.

Usual Adult Dose for Cystic Fibrosis

Morning dose: 2 (elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg) tablets orally each morning
Evening dose: 1 (ivacaftor 150 mg) tablet orally each evening

Comments:
-Doses should be taken approximately 12 hours apart; swallow tablets whole; tablets should be taken with fat-containing food.
-If genotype is unknown, an FDA-cleared cystic fibrosis (CF) mutation test should be used to confirm the presence of at least one F508del mutation or a mutation that is responsive based on in vitro data.

Use: For the treatment of CF in patients who have at least one F508del mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene or a mutation in the CFTR gene that is responsive based on in vitro data.

Usual Pediatric Dose for Cystic Fibrosis

Age: 6 to 12 years; weight: Less than 30 kg:
-Morning dose: 2 (elexacaftor 50 mg/tezacaftor 25 mg/ivacaftor 37.5 mg) tablets orally each morning
-Evening dose: 1 (ivacaftor 75 mg) tablet orally each evening
Age: 6 to 12 years; weight: 30 kg or more:
-Morning dose: 2 (elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg) tablets orally each morning
-Evening dose: 1 (ivacaftor 150 mg) tablet orally each evening

Age: 12 years or older:
Morning dose: 2 (elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg) tablets orally each morning
Evening dose: 1 (ivacaftor 150 mg) tablet orally each evening

Comments:
-Doses should be taken approximately 12 hours apart; swallow tablets whole; tablets should be taken with fat-containing food.
-If genotype is unknown, an FDA-cleared cystic fibrosis (CF) mutation test should be used to confirm the presence of at least one F508del mutation or a mutation that is responsive based on in vitro data.

Use: For the treatment of CF in patients aged 6 years or older who have at least one F508del mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene or a mutation in the CFTR gene that is responsive based on in vitro data.

Renal Dose Adjustments

Mild to moderate renal impairment (CrCl 30 mL/min/1.73 m2 or greater): No adjustment recommended
Severe renal impairment (CrCl less than 30 mL/min/1.73 m2) or ESRD: Use with caution

Liver Dose Adjustments

MILD hepatic impairment (Child-Pugh Class A): No adjustment recommended
MODERATE hepatic impairment (Child-Pugh Class B): Not recommended unless clear medical need and benefit exceeds risks; if used, reduce dose and increase liver function test monitoring
-Dose reduction for moderate hepatic impairment:
--Day 1: Two elexacaftor/tezacaftor/ivacaftor tablets in the morning; no evening dose of ivacaftor
--Day 2: One elexacaftor/tezacaftor/ivacaftor tablet in the morning; no evening dose of ivacaftor
--Continue alternating Day 1 and Day 2; no evening doses of ivacaftor are to be taken
SEVERE hepatic impairment (Child-Pugh Class C): Not recommended

DURING THERAPY:
-If significant transaminase elevations occur (e.g., ALT or AST elevations to greater than 5 times the upper limit of normal [5 x ULN], or ALT or AST elevations to greater than 3 x ULN with bilirubin elevations greater than 2 x ULN): Interrupt dosing and follow laboratory tests closely until abnormalities resolve
-Once transaminase elevations resolve, the benefits and risks of resuming treatment should be considered

Dose Adjustments

Concomitant use of Moderate CYP450 3A Inhibitors:
-Omit evening ivacaftor dose and alternate morning dose as follows: Two fixed dose tablets (elexacaftor/tezacaftor/ivacaftor) orally every other morning alternating with one ivacaftor tablet on alternate mornings

Concomitant use of Strong CYP450 3A Inhibitors:
-Omit evening dose and take two fixed dose tablets (elexacaftor/tezacaftor/ivacaftor) orally twice a week, approximately 3 to 4 days apart (i.e., 2 fixed dose tablets on day 1 and day 4; no doses on day 2 and day 3)

Avoid food or drink containing grapefruit during therapy

Concomitant Administration with Strong CYP450 3A Inducers: Not Recommended

Precautions

CONTRAINDICATIONS:
-None

Safety and efficacy have not been established in patients younger than 6 years.

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration advice:
-Take orally twice a day approximately 12 hours apart
-Swallow tablet(s) whole
-Take tablets with fat-containing food such as butter, peanut butter, cheeses, nuts, whole milk, or meats
-Co-packaged regimen consists fixed dose combination tablet (elexacaftor/tezacaftor/ivacaftor) and a single ingredient tablet (ivacaftor):
--Elexacaftor 50 mg/tezacaftor 25 mg/ivacaftor 37.5 mg tablets are light orange debossed with T50 on one side and plain on the other side
--Ivacaftor 75 mg tablets are light blue with V75 debossed on one side and plain on the other side
--Elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg tablets are orange debossed with T100 on one side and plain on the other side
--Ivacaftor 150 mg tablets are light blue with V150 on one side and plain on the other side

Missed dose:
-If 6 hours or LESS has passed since missed morning or evening dose: Take as soon as remembered with fat containing food and continue on normal schedule
-If MORE than 6 hours has passed since missing the morning dose: Take the morning dose as soon as possible and skip the evening dose; resume normal dosing the following morning
-If MORE than 6 hours has passed since missing evening dose: Skip the evening dose; resume normal dosing the following morning
-Patients should be instructed not to double up and take morning and evening doses at the same time; patients should be instructed to contact their healthcare provided if they have additional questions

General:
-Supplied as co-packaged blister pack; elexacaftor/tezacaftor/ivacaftor are orange tablets and the ivacaftor tablets are light blue.
-This combination drug is for treatment of patients with cystic fibrosis (CF) who have at least one F508del mutation in the CF transmembrane conductance regulator (CFTR) gene; this gene mutation is estimated to represent 90% of the CF population.
-The presence of at least one F508del mutation should be confirmed with an FDA-cleared genotyping assay prior to initiating therapy.

Monitoring:
-Perform liver function tests (ALT, AST, and bilirubin) prior to therapy, every 3 months during the first year, and annually thereafter; more frequent testing should be considered in patients with a history of hepatobiliary disease or liver function test elevations
-Pediatric patients should undergo baseline and follow-up ophthalmological examinations

Patient advice:
-Patients should be instructed to read the US FDA-approved patient labeling (Patient Information).
-Patients should understand that there are a number of drug interactions that may reduce the efficacy of therapy; they should report all medications, herbal supplements, and vitamins they are starting/stopping to their healthcare provider; they should avoid food or drink containing grapefruit during therapy.
-Patients should be instructed to take tablets with fat containing foods approximately 12 hours apart; they should understand what do in case of a missed dose.
-Patients should be instructed to speak with their healthcare provider if they are pregnant or planning to become pregnant or breastfeeding.

Further information

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.

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