Ivacaftor Dosage

Medically reviewed by Drugs.com. Last updated on Nov 9, 2019.

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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

Oral Granules:
Age: 6 months to less than 6 years:
Weight: 5 kg to less than 7 kg: 25 mg orally every 12 hours
Weight: 7 kg 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:
-The oral granules should be mixed with 5 mL of age-appropriate soft food or liquid and completely consumed; food or liquid should be at room temperature. See Administration Advice for further guidance.
-The oral tablet 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 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 6 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): Reduce daily dose by 50% by reducing 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 before resuming therapy

Dose Adjustments

Concomitant administration with CYP450 3A Inhibitors/Inducers:
-When coadministered with moderate CYP450 3A inhibitors (e.g., fluconazole): Reduce daily dose by 50% by reducing dose frequency to 1 tablet or packet once a day
-When coadministered with strong CYP450 3A inhibitors (e.g., ketoconazole, itraconazole, posaconazole, voriconazole, telithromycin, clarithromycin): Reduce dose by reducing dose frequency to 1 tablet or packet 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 6 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 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; examples of 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 at baseline and periodically 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 or Seville oranges.

Further information

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

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