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ivacaftor and lumacaftor
R07AX30
Forms & Strengths
- Oral tablets: lumacaftor 100 mg / ivacaftor 125 mg
- Oral tablets: lumacaftor 200 mg / ivacaftor 125 mg
- Oral granules: lumacaftor 75 mg / ivacaftor 50 mg per packet
- Oral granules: lumacaftor 100 mg / ivacaftor 75 mg per packet
- Oral granules: lumacaftor 150 mg / ivacaftor 75 mg per packet
Adult Dosing
- Patients aged 12 years and older: Two tablets (totaling lumacaftor 400 mg / ivacaftor 250 mg) orally every 12 hours with fat-containing food
Pediatric Dosing
- Ages 6 to 11 years: Two tablets (totaling lumacaftor 200 mg / ivacaftor 250 mg) orally every 12 hours with fat-containing food
- Ages 2 to 5 years (weight-based granules): 14 kg or greater: lumacaftor 150 mg / ivacaftor 75 mg packet every 12 hours; Under 14 kg: lumacaftor 100 mg / ivacaftor 75 mg packet every 12 hours
Indications
- Treatment of cystic fibrosis in patients aged 2 years and older who are homozygous for the F508del mutation in the CFTR gene
Mechanism of Action
- Lumacaftor improves the conformational stability of F508del-CFTR, increasing the quantity of protein delivered to the cell surface
- Ivacaftor is a CFTR potentiator that facilitates increased chloride transport by augmenting the channel open probability of the protein at the cell surface
Contraindications
- Hypersensitivity to lumacaftor, ivacaftor, or any component of the formulation
Adverse Reactions
- Dyspnea
- Nasopharyngitis
- Nausea
- Diarrhea
- Upper respiratory tract infection
- Fatigue
- Respiration abnormal
- Blood creatine phosphokinase increased
- Alanine aminotransferase/aspartate aminotransferase increased
- Menstrual abnormalities (increased frequency, amenorrhea)
Drug Interactions
- Strong CYP3A inducers (e.g., rifampin, St. John's wort) significantly decrease exposure and efficacy of ivacaftor; coadministration is not recommended
- Hormonal contraceptives (estrogen/progestin): Lumacaftor is a strong CYP3A inducer and may decrease effectiveness of hormonal contraceptives; alternative non-hormonal methods should be used
- CYP3A inhibitors (e.g., ketoconazole): May increase exposure of ivacaftor; adjust dosage when coadministered with strong CYP3A inhibitors
- Sensitive CYP3A substrates (e.g., midazolam, digoxin): Lumacaftor may decrease systemic exposure of sensitive CYP3A substrates
Curated Content: Needs Vetting Before Put to Clinical Use