Drug Index



Mechanism :

Lumacaftor improves the conformational stability of F508del-CFTR, resulting in increased processing and trafficking of mature protein to the cell surface.
Ivacaftor is a CFTR potentiator that facilitates increased chloride transport by potentiating the channel-open probability (or gating) of the CFTR protein at the cell surface.

Indication :

• Treatment of cystic fibrosis (CF) in patients age 2 years and older who are homozygous for the F508del mutation in the CFTR gene.

Contraindications :

Hypersensitivity to lumacaftor, ivacaftor or any component of the formulation.

Dosing :


Children 2 to 5 years:

<14 kg: Lumacaftor 100 mg/ivacaftor 125 mg every 12 hours.

≥14 kg: Lumacaftor 150 mg/ivacaftor 188 mg every 12 hours.

Children 6 to 11 years: Lumacaftor 200 mg/ivacaftor 250 mg every 12 hours.

Children ≥12 years and Adolescents: Lumacaftor 400 mg/ivacaftor 250 mg every 12 hours.

Adverse Effect :

Chest discomfort, headache, nausea, abdominal pain, diarrhea, increased AST & ALT, dyspnea, changes in respiration, nasal congestion, increased systolic blood pressure, fatigue, skin rash, menstrual disease, increased creatine phosphokinase.

Interaction :

CYP3A4 Inducers: May decrease the serum concentration of Ivacaftor.
CYP3A4 Inhibitors: May increase the serum concentration of Ivacaftor.
CYP3A4 Substrates: CYP3A4 Inducers (Strong) may increase the metabolism of CYP3A4 Substrates.
CYP2C19 Substrates: Lumacaftor may decrease the serum concentration of CYP2C19 Substrates.
CYP2C8 Substrates: Lumacaftor may increase the serum concentration of CYP2C8 Substrates.

03/03/2020 19:42:32 Lumacaftor/Ivacaftor
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