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 (e.g., eggs, butter, peanut butter, cheese pizza, whole milk, cheese, yogurt).
-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.
-The tablet formulation is recommended for patients 6 years and older.

Uses:
-For the treatment of cystic fibrosis (CF) in patients age 2 years and older who have one mutation in the CFTR gene that is responsive to ivacaftor potentiation based on clinical and/or in vitro assay

Usual Pediatric Dose for Cystic Fibrosis

2 to less than 6 years:
Less than 14 kg: 50 mg mixed with 5 mL (1 teaspoonful) of soft food or liquid orally every 12 hours
14 kg or greater: 75 mg mixed with 5 mL (1 teaspoonful) of soft food or liquid orally every 12 hours

6 years and older:
150 mg orally every 12 hours

Comments:
-This drug should be taken with fat-containing food (e.g., eggs, butter, peanut butter, cheese pizza, whole milk, cheese, yogurt).
-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.
-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.

Uses:
-For the treatment of cystic fibrosis (CF) in patients age 2 years and older who have one mutation in the CFTR gene that is responsive to ivacaftor potentiation based on clinical and/or in vitro assay

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

6 years and older:
-Mild liver dysfunction (Child-Pugh A): No adjustment recommended
-Moderate liver dysfunction (Child-Pugh B): 150 mg orally once a day
-Severe liver dysfunction (Child-Pugh C): 150 mg orally once a day or less frequently

2 to less than 6 years:
-Mild liver dysfunction (Child-Pugh A): No adjustment recommended
-Moderate liver dysfunction (Child-Pugh B):
1) Less than 14 kg: 50 mg mixed with 5 mL (1 teaspoonful) of soft food or liquid orally once a day
2) 14 kg or greater: 75 mg mixed with 5 mL (1 teaspoonful) of soft food or liquid orally once a day
-Severe liver dysfunction (Child-Pugh C):
1) Less than 14 kg: 50 mg mixed with 5 mL (1 teaspoonful) of soft food or liquid orally once a day or less frequently
2) 14 kg or greater: 75 mg mixed with 5 mL (1 teaspoonful) of soft food or liquid orally once a day or less frequently

Dose Adjustments

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
When coadministered with strong CYP450 3A inducers (e.g., rifampin, St. John's Wort): Not recommended

Precautions

Safety and efficacy have not been established in patients younger than 2 years.

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration advice:
-Tablets should be swallowed whole, not crushed, chewed, dissolved, or divided.
-This drug should be given with fat-containing food (e.g., eggs, butter, peanut butter, cheese pizza, etc.).
-Food containing grapefruit or Seville oranges should be avoided during therapy with this drug.
-The entire contents of each packet of oral granules should be mixed with 5 mL (1 teaspoonful) of soft food or liquid and completely consumed. Food or liquid should be at or below room temperature.
-After mixing, the dose is stable for one hour, and should be consumed during this period.

General:
-This drug should only be prescribed by physicians with experience in the treatment of cystic fibrosis.
-If patient genotype is unknown, an approved CF mutation test should be used to detect the presence of a CFTR mutation.

Monitoring:
Hepatic: Liver function tests (prior to starting treatment, every 3 months during the first year of treatment, and yearly thereafter).

Patient advice:
-This drug should be taken with a fat-containing meal or snack.
-If dizziness occurs, do not drive or operate machinery until symptoms abate.

Further information

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

Medical Disclaimer