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Ivacaftor / Lumacaftor Dosage

Applies to the following strength(s): 125 mg-100 mg ; 125 mg-200 mg

The information at Drugs.com is not a substitute for medical advice. Always consult your doctor or pharmacist.

Usual Adult Dose for:

Usual Pediatric Dose for:

Additional dosage information:

Usual Adult Dose for Cystic Fibrosis

Two tablets (ivacaftor 125 mg-lumacaftor 200 mg) orally every 12 hours with fat-containing food

Comments:
-If a dose is missed within 6 hours of the usual scheduled time, take the dose immediately with a fat-containing meal; if more than 6 hours have elapsed, skip that dose and resume the normal schedule for the following dose.

Use: For the treatment of cystic fibrosis (CF) in patients who are homozygous for the F508del mutation in the CFTR gene; if the patient's genotype is unknown, an FDA-cleared CF mutation test should be used to detect the presence of the F508del mutation on both alleles of the CFTR gene

Usual Pediatric Dose for Cystic Fibrosis

6 to 11 years: Two tablets (ivacaftor 125 mg-lumacaftor 100 mg) orally every 12 hours with fat-containing food

12 years or older: Two tablets (ivacaftor 125 mg-lumacaftor 200 mg) orally every 12 hours with fat-containing food

Comments:
-If a dose is missed within 6 hours of the usual scheduled time, take the dose immediately with a fat-containing meal; if more than 6 hours have elapsed, skip that dose and resume the normal schedule for the following dose.

Use: For the treatment of CF in patients age 6 years and older who are homozygous for the F508del mutation in the CFTR gene; if the patient's genotype is unknown, an FDA-cleared CF mutation test should be used to detect the presence of the F508del mutation on both alleles of the CFTR gene

Renal Dose Adjustments

-Mild to moderate renal disease: No adjustment recommended
-Severe renal impairment (CrCl less than or equal to 30 mL/min) or end-stage renal disease: Caution is recommended

Liver Dose Adjustments

Mild liver dysfunction (Child-Pugh Class A): No adjustment recommended.
Moderate liver dysfunction (Child-Pugh Class B): Reduce to 2 tablets orally once in the morning and 1 tablet once in the evening
Severe liver dysfunction (Child-Pugh Class C): Caution is recommended after weighing risk versus benefit; if therapy is decided, reduce to 1 tablet in the morning and 1 tablet in the evening, or less

Dose Adjustments

Concomitant use of CYP450 3A Inhibitors:
-No dose adjustment is necessary when CYP450 3A inhibitors are initiated in patients already taking this combination drug.
-When initiating this combination drug in patients currently taking strong CYP450 3A inhibitors (e.g., itraconazole), reduce dose to 1 tablet orally once a day for the first week of treatment; after this period, continue with the recommended daily dose.
-If this combination drug is interrupted for more than 1 week and then reinitiated while taking strong CYP450 3A inhibitors, patients should reduce this combination drug dose to 1 tablet orally once a day for the first week of treatment reinitiation; after this period, continue with the recommended daily dose.

Precautions

Safety and efficacy have not been established in patients younger than 6 years.

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration advice:
-Take with fat-containing foods (e.g., eggs, avocados, nuts, butter, peanut butter, cheese pizza, whole-milk dairy products, etc.)

General:
-Clinical studies did not include a sufficient number of patients 65 years and older and it is therefore unknown if they respond differently than younger patients.
-Safety and efficacy have not been established in patients with CF other than those homozygous for the F508del mutation.

Monitoring:
-Cardiovascular: Monitor blood pressure periodically throughout treatment.
-Hepatic: Measure ALT, AST, and bilirubin prior to initiation, every 3 months during the first year of therapy, and annually thereafter.

Patient advice:
-If a dose is missed within 6 hours of the usual scheduled time, take the dose immediately with a fat-containing meal; if more than 6 hours have elapsed, skip that dose and resume the normal schedule for the following dose; a double dose should not be taken to make up for a missed dose.

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