Ivacaftor Stada Nordic (Ivacaftor)

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

• Cystic fibrosis with G551D mutation: Improves CFTR channel opening and respiratory outcomes.
• Cystic fibrosis with other class III gating mutations: Enhances chloride transport and clinical parameters.
• Cystic fibrosis in patients aged 12 years and older with at least one approved gating mutation: Increases lung function and reduces pulmonary exacerbations.

• Ivacaftor binds to the cystic fibrosis transmembrane conductance regulator (CFTR) protein at the nucleotide‑binding domain, stabilizing the open channel conformation. This potentiation increases the probability that the CFTR chloride channel remains open, thereby enhancing chloride ion transport across epithelial cell membranes and improving hydration of airway surfaces.

Common Side Effects

• Headache: mild to moderate intensity.
• Diarrhea: usually transient.
• Nausea: may occur after dosing.
• Abdominal pain: occasional discomfort.
• Upper respiratory tract infection: mild symptoms.
• Rash: mild skin irritation.

Serious or Rare Side Effects

• Elevated liver enzymes: monitor hepatic function.
• Cataracts: periodic eye examinations recommended.
• Severe rash or hypersensitivity reactions: seek immediate medical attention.
• Arrhythmia: rare cardiac rhythm disturbances.
• Anaphylaxis: life‑threatening allergic reaction requiring emergency care.

Before initiating therapy, consider the following precautions:

• Monitor liver function: baseline and periodic tests are recommended.
• Assess for drug interactions: review all concomitant medications.
• Evaluate renal function: adjust therapy if significant impairment exists.
• Avoid use in patients with known hypersensitivity to ivacaftor.
• Pregnancy and breastfeeding: discuss potential risks with a healthcare professional.
• Store at ambient temperature: keep in the original container, protect from moisture and light.

Ivacaftor may interact with several medicines that affect its metabolism.

Major Interactions (Avoid)

• Strong CYP3A inducers (e.g., rifampin, carbamazepine): may markedly reduce ivacaftor exposure.
• Strong CYP3A inhibitors (e.g., ketoconazole, itraconazole): may increase ivacaftor levels and risk toxicity.

Moderate Interactions (Monitor Closely)

• Azole antifungals (e.g., fluconazole): may raise ivacaftor concentrations; monitor clinical response.
• Macrolide antibiotics (e.g., clarithromycin): can increase exposure; consider dose adjustment.
• Oral contraceptives: ivacaftor may reduce efficacy; advise alternative or additional contraception methods.