Ivacaftor Dosage

Medically reviewed by Drugs.com. Last updated on Jan 5, 2021.

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.

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Further information

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