Ivacaftor Dosage

Medically reviewed on February 13, 2017.

Usual Adult Dose for Cystic Fibrosis

150 mg orally every 12 hours

Comments:
-This drug should be taken with fat-containing food, examples 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

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

Age: 6 years and older:
150 mg orally every 12 hours

Comments:
-This drug should be taken with fat-containing food, examples include: eggs, butter, peanut butter, cheese pizza, and whole milk dairy products.
-The oral granule packets are recommended for patients less than 6 years old and the tablet formulation is recommended for patients 6 years and older.
-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 12 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 end-stage renal disease: Caution is recommended

Liver Dose Adjustments

-Mild liver dysfunction (Child-Pugh A): No adjustment recommended
-Moderate liver dysfunction (Child-Pugh B): Recommended dose should be reduced to once a day
-Severe liver dysfunction (Child-Pugh C): Recommended dose should be reduced to once a day or less frequently

Dose Adjustments

Concomitant administration with CYP450 3A Inhibitors/Inducers:
-When coadministered with moderate CYP450 3A inhibitors (e.g., fluconazole): 150 mg orally once a day
-When coadministered with strong CYP450 3A inhibitors (e.g., ketoconazole, itraconazole, posaconazole, voriconazole, telithromycin, clarithromycin): 150 mg orally twice a week
-Coadministration with strong CYP450 3A inducers (e.g., rifampin, St. John's Wort): Not recommended

Food containing grapefruit or Seville oranges should be avoided

Precautions

CONTRAINDICATIONS: None

Safety and efficacy have not been established in patients younger than 12 months.

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration advice:
-Take orally just before or just after fat-containing food
-Examples of fat-containing include eggs, butter, peanut butter, cheese pizza, etc.

TABLETS: 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; examples of soft food or liquids include pureed fruits or vegetables, yogurt, applesauce, water, 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
-Ophthalmological exam should be at baseline and periodically in pediatric patients

Patient advice:
-This drug should be taken with a fat-containing meal or snack.
-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 or Seville oranges.

Further information

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

Medical Disclaimer