Ivacaftor Dosage

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.