Drug reference

Ivacaftor

CFTR Modulator · Cystic Fibrosis Therapy

CFTR ModulatorCystic Fibrosis Therapy

CDSCO approvedSchedule H

EXCRETION

—

not curated

INTERACTIONS

12 major

SEVERE in our sources

PREGNANCY

—

not curated

Top interactionssee all 12 →

AmprenavirSevereDatabaseDDInter
ApalutamideSevereDatabaseDDInter
AprepitantSevereDatabaseDDInter
AtazanavirSevereDatabaseDDInter

Mechanism

Ivacaftor is a small molecule potentiator that binds to the cystic fibrosis transmembrane regulator (CFTR) ion channel. It acts as a positive allosteric modulator, improving CFTR function in individuals with an abnormal gating mechanism of their CFTR protein, thereby increasing channel activity. This mechanism allows it to partially rescue hypoactive channels caused by certain mutations.

Indications

Cystic fibrosis in individuals who have an abnormal gating mechanism of their CFTR proteinCystic fibrosis (in patients who harbor the coding mutation CFTR-p.G551D)cystic fibrosis (specifically for patients with certain rare CFTR variants such as G551D)CF with G551D mutationCF with 96 other CFTR variants (US approval)CF with at least one F508del variant (in combination with tezacaftor and elexacaftor)

Dosing

Adult: Adult: 150 mg every 12 hours. ▶ Manufacturer advises reduce dose to 150 mg twice a. ▶ Manufacturer advises reduce dose to 150 mg once daily. 150 mg once daily in moderate impairment; in severe. impairment reduce starting dose to 150 mg on alternate

Pharmacokinetics

Metabolism

CYP3A4