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:
Age: 4 months to less than 6 months:
Weight: 5 kg or greater: 25 mg orally every 12 hours

Use is not recommended in the presence of hepatic impairment or in those taking concomitant moderate or strong CYP450 3A inhibitors

Age: 6 months or older:
Weight: 5 to less than 7 kg: 25 mg orally every 12 hours
Weight: 7 to less than 14 kg: 50 mg orally every 12 hours
Weight: 14 kg or greater: 75 mg orally every 12 hours

Oral Tablet:
Age: 6 years and older: 150 mg orally 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.
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 4 months or 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: 4 to less than 6 months: Not recommended

Age: 6 months or older:

Mild liver dysfunction (Child-Pugh A): No adjustment recommended
Moderate liver dysfunction (Child-Pugh B): Reduce daily dose by 50% (i.e., reduce dose frequency to 1 tablet or packet once a day)
Severe liver dysfunction (Child-Pugh C): Reduce daily dose by administering 1 tablet or packet once a day or less frequently

If increased transaminase levels develop, closely monitor until abnormalities resolve; dosing should be interrupted if ALT or AST are greater than 5 times the upper limit of normal; following resolution, consider the risk and benefits of resuming therapy

Dose Adjustments

Coadministration with Strong CYP450 3A Inducers: Not recommended

Coadministration with CYP450 3A Inhibitors:
Age: 4 to less than 6 months: Not recommended
Age: 6 moths or older:

When coadministered with moderate CYP450 3A inhibitors: Reduce dose frequency to 1 tablet or packet once a day
When coadministered with strong CYP450 3A inhibitors: Reduce dose frequency to 1 tablet or packet twice a week

Avoid food containing grapefruit

Precautions

CONTRAINDICATIONS: None

Safety and efficacy have not been established in patients younger than 4 months.

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

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 monitored until abnormalities resolve
Ophthalmological exam should be performed at baseline and periodically thereafter in pediatric patients

Patient advice:

Patients/caregivers should be instructed to read the US FDA-approved patient labeling (Patient Information).
Patients should be instructed to avoid driving or operating hazardous machinery until it is known how this drug affects them; this drug may cause dizziness.
Patients should be instructed to 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 be instructed to avoid food or drink that contains grapefruit.