Elexacaftor / Ivacaftor / Tezacaftor Dosage

Medically reviewed by Drugs.com. Last updated on Dec 1, 2023.

Applies to the following strengths: 50 mg-37.5 mg-25 mg and ivacaftor 75 mg; 100 mg-75 mg-50 mg and ivacaftor 150 mg; 80 mg-60 mg-40 mg and ivacaftor 59.5 mg; 100 mg-75 mg-50 mg and ivacaftor 75 mg

Usual Adult Dose for Cystic Fibrosis

Morning dose: 2 tablets (of elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg) orally once a day
Evening dose: 1 tablet (of ivacaftor 150 mg) orally once a day

Comments:

Doses should be taken approximately 12 hours apart and administered with fat-containing food.
If genotype is unknown, a US 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

2 TO LESS THAN 6 YEARS:
Weight less than 14 kg:

Morning dose: 1 packet of granules (containing elexacaftor 80 mg/tezacaftor 40 mg/ivacaftor 60 mg) orally once a day
Evening dose: 1 packet of granules (containing ivacaftor 59.5 mg) orally once a day

Weight 14 kg or greater:

Morning dose: 1 packet of granules (containing elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg) orally once a day
Evening dose: 1 packet of granules (containing ivacaftor 75 mg) orally once a day

6 TO LESS THAN 12 YEARS:
Weight less than 30 kg:

Morning dose: 2 tablets (of elexacaftor 50 mg/tezacaftor 25 mg/ivacaftor 37.5 mg) orally once a day
Evening dose: 1 tablet (of ivacaftor 75 mg) orally once a day

Weight 30 kg or greater:

Morning dose: 2 tablets (of elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg) orally once a day
Evening dose: 1 tablet (of ivacaftor 150 mg) orally once a day

12 YEARS AND OLDER:

Morning dose: 2 tablets (of elexacaftor 100 mg/ tezacaftor 50 mg/ivacaftor 75 mg) orally once a day
Evening dose: 1 tablet (of ivacaftor 150 mg) orally once a day

Comments:

Doses should be taken approximately 12 hours apart and administered with fat-containing food; oral granules should be premixed in soft foods or liquids.
If genotype is unknown, a US 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 CFTR gene or a mutation in the CFTR gene that is responsive based on in vitro data

Renal Dose Adjustments

MILD TO MODERATE RENAL DYSFUNCTION (CrCl 30 mL/min/1.73 m2 or greater): No adjustment recommended
SEVERE RENAL DYSFUNCTION (CrCl less than 30 mL/min/1.73 m2) or ESRD: Use with caution; data not available

Liver Dose Adjustments

MILD LIVER DYSFUNCTION (Child-Pugh A): No adjustment recommended

MODERATE LIVER DYSFUNCTION (Child-Pugh B): Not recommended unless clear medical need and benefit exceeds risk. If administered, use with caution at a reduced dose based on the following age/weight parameters:
AGE 2 TO LESS THAN 6 YEARS:
Weight less than 14 kg:

Days 1 to 3: One packet of granules (containing elexacaftor 80 mg/tezacaftor 40 mg/ivacaftor 60 mg) orally once a day
Day 4: No dose
Days 5 and 6: One packet of granules (containing elexacaftor 80 mg/tezacaftor 40 mg/ivacaftor 60 mg) orally once a day
Day 7: No dose
Repeat weekly dosing schedule; no evening dose of ivacaftor oral granules should be taken on any day.

Weight 14 kg or greater:

Days 1 to 3: One packet of granules (containing elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg) orally once a day
Day 4: No dose
Days 5 and 6: One packet of granules (containing elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg) orally once a day
Day 7: No dose
Repeat weekly dosing schedule; no evening dose of ivacaftor oral granules should be taken on any day.

AGE 6 TO LESS THAN 12 YEARS:
Weight less than 30 kg:

Day 1: Two tablets (of elexacaftor 50 mg/tezacaftor 25 mg/ivacaftor 37.5 mg) orally in the morning
Day 2: One tablet (of elexacaftor 50 mg/tezacaftor 25 mg/ivacaftor 37.5 mg) orally in the morning
Repeat alternating daily dosing schedule; no evening dose of ivacaftor tablets should be taken on any day.

Weight 30 Kg Or More:

Day 1: Two tablets (of elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg) orally in the morning
Day 2: One tablet (of elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg) orally in the morning
Repeat alternating daily dosing schedule; no evening dose of ivacaftor tablets should be taken on any day.

AGE 12 YEARS OR OLDER:

Day 1: Two tablets (of elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg) orally in the morning
Day 2: One tablet (of elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg) orally in the morning
Repeat alternating daily dosing schedule; no evening dose of ivacaftor tablets should be taken on any day.

SEVERE LIVER DYSFUNCTION (Child-Pugh C): Not recommended; drug exposure is expected to be higher.

RECOMMENDATIONS FOR TRANSAMINASE ELEVATIONS DURING THERAPY:

For ALT or AST elevations 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 greater than 2 x ULN): Interrupt dosing of this drug and closely follow laboratory tests until abnormalities resolve.
Once transaminase elevations resolve, the benefits and risks of resuming treatment should be considered.

Comments:

Closely monitor liver function tests in patients with mild to moderate liver dysfunction who use this drug.

Dose Adjustments

COADMINISTRATION WITH MODERATE CYP450 3A INHIBITORS (e.g., fluconazole, erythromycin):
AGE 2 TO LESS THAN 6 YEARS:
Weight less than 14 kg:

Day 1: One packet of granules (containing elexacaftor 80 mg/tezacaftor 40 mg/ivacaftor 60 mg) orally in the morning
Day 2: One packet of granules (containing ivacaftor 59.5 mg) orally in the morning
Repeat alternating daily dosing schedule; no evening dose of ivacaftor oral granules should be taken.

Weight 14 kg or more:

Day 1: One packet of granules (containing elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg) orally in the morning
Day 2: One packet of granules (containing ivacaftor 75 mg) orally in the morning
Repeat alternating daily dosing schedule; no evening dose of ivacaftor oral granules should be taken.

AGE 6 TO LESS THAN 12 YEARS:
Weight less than 30 kg:

Day 1: Two tablets (of elexacaftor 50 mg/tezacaftor 25 mg/ivacaftor 37.5 mg) orally in the morning
Day 2: One tablet (of ivacaftor 75 mg) orally in the morning
Repeat alternating daily dosing schedule; no evening dose of ivacaftor tablets should be taken.

Weight 30 kg or more:

Day 1: Two tablets (of elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg) orally in the morning
Day 2: One tablet (of ivacaftor 150 mg) orally in the morning
Repeat alternating daily dosing schedule; no evening dose of ivacaftor tablets should be taken.

AGE 12 YEARS OR OLDER:

Day 1: Two tablets (of elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg) orally in the morning
Day 2: One tablet (of ivacaftor 150 mg) orally in the morning
Repeat alternating daily dosing schedule; no evening dose of ivacaftor tablets should be taken.

COADMINISTRATION WITH STRONG CYP450 3A INHIBITORS (e.g., ketoconazole, itraconazole, posaconazole, voriconazole, telithromycin, and clarithromycin):
AGE 2 TO LESS THAN 6 YEARS:
Weight less than 14 kg:

Morning dose: One packet of granules (containing elexacaftor 80 mg/tezacaftor 40 mg/ivacaftor 60 mg) orally in the morning twice a week, about 3 to 4 days apart.
Evening dose: None

Weight 14 kg or more:

Morning dose: One packet of granules (containing elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg) orally in the morning twice a week, about 3 to 4 days apart.
Evening dose: None

AGE 6 TO LESS THAN 12 YEARS:
Weight less than 30 kg:

Morning dose: Two tablets (of elexacaftor 50 mg/tezacaftor 25 mg/ivacaftor 37.5 mg) orally in the morning twice a week, about 3 to 4 days apart.
Evening dose: None

Weight 30 kg or more:

Morning dose: Two tablets (of elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg) orally in the morning twice a week, about 3 to 4 days apart.
Evening dose: None

AGE 12 YEARS OR OLDER:

Morning dose: Two tablets (of elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg) orally in the morning twice a week, about 3 to 4 days apart.
Evening dose: None

CONCOMITANT ADMINISTRATION WITH STRONG CYP450 3A INDUCERS: Not Recommended

Comments:

Avoid food or drink containing grapefruit during therapy.

Precautions

CONTRAINDICATIONS: None

Safety and efficacy have not been established in patients younger than 2 years.

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration advice:

For oral use; morning and evening doses should be administered approximately 12 hours apart.
Each dose should be taken with fat-containing food (e.g., meals or snacks prepared with butter/oils, or containing eggs, peanut butter, cheeses, nuts, whole milk, or meats).
TABLETS: Swallow whole.
ORAL GRANULES: Premix with soft food or liquid; administer immediately before or after ingestion of fat-containing food.

Missed dose:

If 6 hours or LESS have passed since the missed morning or evening dose: Take missed dose as soon as possible and continue on original schedule.
If MORE than 6 hours have passed since the missed morning dose: Take the missed morning dose as soon as possible and skip the evening dose; resume usual dosing schedule the following morning.
If MORE than 6 hours have passed since the missed evening dose: Skip the missed evening dose; resume usual dosing schedule the following morning.
The morning and evening doses should not be taken at the same time.

Storage requirements:

Store at 20C to 25C (68F to 77F); excursions permitted to 15C to 30C (59F to 86F).

Reconstitution/preparation:
PACKET OF ORAL GRANULES:

Mix entire contents of each packet of with one teaspoon (5 mL) of age-appropriate soft food or liquid at room temperature or below.
Examples of soft food or liquids include pureed fruits/vegetables, yogurt, applesauce, water, milk, or juice.
Once mixed, the product should be consumed completely within 1 hour.
Consult the manufacturer product information for detailed preparation instructions.

General:

This 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 OR a mutation that is responsive based on in vitro data; if genotype is unknown, use a US FDA-cleared CF mutation test prior to initiating therapy.
This combination product is supplied as fixed-dose combination (elexacaftor/tezacaftor/ivacaftor) tablets OR oral granule packets, co-packaged with single ingredient (ivacaftor) tablets OR oral granule packets.
Food or drink containing grapefruit should be avoided during treatment.

Monitoring:

Hepatic: Liver function tests, including 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 elevated liver function tests)
Ocular: Ophthalmological examinations in pediatric patients (at baseline and follow-up)

Patient advice:

Read the US FDA-approved patient labeling (Patient Information).
Take doses with fat containing foods approximately 12 hours apart; understand what do in case of a missed dose.
Inform your health care provider of any history of liver disease or liver test abnormalities.
Discontinue use of this drug immediately if signs/symptoms of a hypersensitivity reactions occur.
Understand that there are a number of drug interactions that may affect the efficacy of therapy; report all medications, herbal supplements, and vitamins to your healthcare provider.
Avoid food or drink containing grapefruit during therapy.
Speak with your healthcare provider if you are pregnant/planning to become pregnant, or breastfeeding.
Pediatric patients: Ensure baseline and follow-up eye exams are performed.