Kalydeco

Ivacaftor, the active ingredient in Kalydeco, is a CFTR potentiator presented as a tablet for oral use. It is indicated for cystic fibrosis in patients who have specific CFTR gating mutations, helping to improve chloride transport in epithelial cells.

GNH USA supplies Kalydeco as a licensed, GDP‑compliant international pharmaceutical supplier serving hospitals, pharmacies and clinics worldwide.

Kalydeco is a CFTR potentiator used in the management of cystic fibrosis caused by specific gating mutations.

Cystic fibrosis (gating mutations): improves lung function and reduces the frequency of pulmonary exacerbations. Cystic fibrosis (G551D mutation): enhances chloride transport, leading to better respiratory outcomes. Cystic fibrosis (other responsive mutations): supports nutritional status by improving pancreatic function.

Adverse reactions to ivacaftor may vary in frequency and severity.

Common Side Effects: - Headache - Diarrhea - Upper respiratory tract infection - Nausea - Abdominal pain

Serious or Rare Side Effects: - Elevated liver enzymes (ALT, AST) - Pulmonary hemorrhage - Severe skin reactions such as rash or Stevens‑Johnson syndrome

When prescribing ivacaftor, clinicians should consider several safety measures.

Liver function monitoring: assess transaminases before initiating therapy and periodically thereafter. Drug interaction review: evaluate concomitant use of strong CYP3A4 inhibitors. Pregnancy risk: safety data are limited; discuss potential risks with the patient. Breastfeeding considerations: unknown safety; weigh benefits against possible infant exposure. Adherence advice: take with a fat‑containing meal to maximize absorption. Store at ambient temperature: keep below 25°C, retain in the original packaging, and protect from moisture.

Ivacaftor may interact with other medicines, requiring careful management.

Major Interactions (Avoid): - Ketoconazole: potent CYP3A4 inhibitor that can increase ivacaftor exposure. - Itraconazole: strong CYP3A4 inhibitor, may raise plasma levels of ivacaftor. - Clarithromycin: CYP3A4 inhibitor that can lead to toxicity. - HIV protease inhibitors (e.g., ritonavir): significantly elevate ivacaftor concentrations.

Moderate Interactions (Monitor Closely): - Rifampin: CYP3A4 inducer that may reduce ivacaftor effectiveness. - Oral contraceptives: ivacaftor can decrease hormonal efficacy, consider alternative contraception.