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ivacaftor
R07AX02
Forms & Strengths
- Oral granules: 5.8 mg, 13.4 mg, 25 mg, 50 mg, 75 mg
- Tablets: 150 mg
Adult Dosing
- Cystic fibrosis: 150 mg orally every 12 hours with fat-containing food
Pediatric Dosing
- Ages 1 to <6 months (weight-based): 5.8 mg to 25 mg granules every 12 hours
- Ages 6 months to <6 years (weight-based): 25 mg to 75 mg granules every 12 hours
- Ages 6 years and older: 150 mg tablet every 12 hours
Indications
- Treatment of cystic fibrosis in patients age 1 month and older who have at least one mutation in the CFTR gene responsive to ivacaftor
Mechanism of Action
- CFTR potentiator that increases the channel open probability (or gating activity) of the CFTR protein on the cell surface, enhancing chloride transport
Contraindications
- Hypersensitivity to ivacaftor or any component of the formulation
Adverse Reactions
- Headache
- Upper respiratory tract infection
- Nasopharyngitis
- Nausea
- Dizziness
- Elevated transaminases
- Cataracts in pediatric patients
Drug Interactions
- Strong CYP3A4/5 inhibitors (e.g., ketoconazole): significantly increase ivacaftor exposure; reduce dose
- Strong CYP3A4/5 inducers (e.g., rifampin): significantly decrease ivacaftor exposure; concomitant use is not recommended
Curated Content: Needs Vetting Before Put to Clinical Use