Ivacaftor Dosage

Medically reviewed by Drugs.com. Last updated on Aug 21, 2023.

Applies to the following strengths: 150 mg; 50 mg; 75 mg; 25 mg; 13.4 mg; 5.8 mg

Usual Adult Dose for Cystic Fibrosis

150 mg orally every 12 hours with fat containing food

Comments:

Examples of fat-containing food include: eggs, butter, peanut butter, cheese pizza, and whole milk dairy products.
If the patient's genotype is unknown, an approved CF mutation test should be used to detect the presence of a CFTR mutation followed by verification with bi-directional sequencing when recommended by the mutation test instructions for use.

Uses:

For the treatment of cystic fibrosis in patients having one mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene that is responsive to ivacaftor potentiation based on clinical and/or in vitro assay data.

Usual Pediatric Dose for Cystic Fibrosis

Oral granules mixed with one teaspoon (5 mL) of soft food or liquid, administered orally with fat containing food.

1 month to less than 2 months (3 kg or greater): one 5.8 mg packet every 12 hours
2 months to less than 4 months (3 kg or greater): one 13.4 mg packet every 12 hours
4 months to less than 6 months (5 kg or greater): one 25 mg packet every 12 hours
6 months to less than 6 years:
5 kg to less than 7 kg: 25 mg packet every 12 hours
7 kg to less than 14 kg: 50 mg packet every 12 hours
14 kg or greater: 75 mg packet every 12 hours

Oral tablet:

6 years or older - one 150 mg tablet every 12 hours

Comments:

Doses should be taken just before or after fat-containing food; examples of fat-containing food include eggs, butter, peanut butter, cheese pizza, and whole milk dairy products.
Use is not recommended in patients less than 6 months with any level of hepatic impairment.
Use is not recommended in patients below 6 months of age taking concomitant moderate or strong CYP450 3A inhibitors. Food or drinks containing grapefruit should be avoided.
Not recommended for use in patients under 1 month of age.
The use of this drug in patients aged 1 to 6 months born at a gestational age less than 37 weeks has not been evaluated.
Dose adjustments are recommended for patients 6 months or older with hepatic impairment and/or taking concomitant moderate or strong CYP450 3A inhibitors.
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 bi-directional sequencing when recommended by the mutation test instructions for use.

Uses:

For the treatment of cystic fibrosis in patients one month and older having one mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene that is responsive to ivacaftor potentiation based on clinical and/or in vitro assay data.

Renal Dose Adjustments

Mild to moderate renal dysfunction: No adjustment recommended
Severe renal dysfunction (CrCl 30 mL/min or less) or ESRD: Caution is recommended

Liver Dose Adjustments

Age less than 6 months with any level of liver dysfunction: Not recommended

Mild liver dysfunction (Child-Pugh A), age 6 months or older: No adjustment recommended
Moderate liver dysfunction (Child-Pugh B):
6 months to less than 6 years: Reduce daily dose by 50% (i.e., reduce dose frequency to 1 packet once a day)
6 years of age and older: 150 mg orally once daily
Severe liver dysfunction (Child-Pugh C):
Patients 6 months or older: Use with caution after weighing the risks and benefits of treatment. Reduce dose by administering 1 packet once a day or less frequently.
6 years of age or older: Use with caution after weighing the risks and benefits of treatment. Reduce dose to 150 mg orally once daily or less frequently.

Dose Adjustments

Coadministration with Moderate CYP450 3A Inhibitors:
Age less than 6 months: Not recommended
Age 6 months to less than 6 years: Reduce daily dose by 50% (i.e., reduce dose frequency to 1 packet once a day)
6 years and older: 150 mg orally once daily

Coadministration with Strong CYP450 3A Inhibitors:
Age less than 6 months: Not recommended
Age 6 months to less than 6 years: one packet of oral granules twice a week based on dosing recommended for age and weight
Age 6 years and older: 150 mg orally twice weekly

Avoid food and liquids containing grapefruit.

Precautions

CONTRAINDICATIONS: None

Safety and efficacy have not been established in patients younger than 1 month.

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration advice:
ORAL TABLETS:

Take orally just before or just after fat-containing food; examples of fat-containing foods include eggs, butter, peanut butter, cheese pizza, etc.
Remove from blister at time of use and swallow whole.

ORAL GRANULES:

Entire packet should be mixed with 5 mL of age-appropriate soft food or liquid and completely consumed; food or liquid should be at or below room temperature; once mixed, must be consumed within 1 hour.
Each dose should be administered just before or just after fat-containing food; examples of fat-containing soft food or liquids include pureed fruits or vegetables, yogurt, applesauce, water, breastmilk, infant formula, milk, or juice.

Missed dose:

If a dose is missed within 6 hours of the time it is usually taken, take this dose as soon as possible and then take the next dose at the regularly scheduled time.
If more than 6 hours have passed since the time the dose is usually taken, skip the missed dose and resume dosing with the next scheduled dose.

General:

This drug is indicated for the treatment of cystic fibrosis in patients with 1 mutation in the CFTR gene that is responsive to ivacaftor potentiation based on clinical and/or in vitro assay data.
If patient genotype is unknown, an approved CF mutation test should be used to detect the presence of a CFTR mutation.

Monitoring:

Obtain transaminase levels (ALT and AST) prior to initiating therapy, every 3 months for the first year, and annually thereafter; more frequent monitoring should be considered in patients with a history of transaminase elevations; if increased transaminase levels develop, closely monitor until abnormalities resolve.
Ophthalmological exam should be performed at baseline and periodically thereafter in pediatric patients.

Patient advice:

Read the Patient Information and Instructions for Use.
Patients should avoid driving or operating hazardous machinery until it is known how this drug affects them; this drug may cause dizziness.
Patients should speak with their healthcare provider regarding all medications they are taking or plan to take including over the counter medicines, vitamins, and herbal supplements; there are major drug interactions that affect how well this drug may work or how other drugs may work.
Patients should avoid food or drink that contains grapefruit.