Kalydeco

Ivacaftor, the active ingredient in Kalydeco, is a CFTR potentiator presented as a tablet for oral use. It enhances chloride transport in cells of patients with cystic fibrosis who carry specific gating mutations such as G551D, improving lung function and reducing disease severity.

GNH USA supplies Kalydeco as a licensed, GDP‑compliant international pharmaceutical supplier serving hospitals, pharmacies and clinics worldwide, providing reliable sourcing, regulatory support and consistent product quality across multiple markets. The company adheres to stringent quality‑assurance protocols and offers flexible shipment options to meet diverse regional requirements.

Kalydeco acts on the cystic fibrosis transmembrane conductance regulator (CFTR) pathway to treat patients with specific genetic variants.

- Cystic fibrosis with G551D mutation: improves lung function and reduces pulmonary exacerbations. - Cystic fibrosis with R117H mutation: enhances chloride transport and stabilises disease progression. - Cystic fibrosis with other responsive gating mutations: provides measurable clinical benefit in lung function and symptom control.

Adverse reactions have been reported with ivacaftor therapy.

Common Side Effects: - Headache: mild to moderate intensity. - Diarrhoea: usually transient. - Upper respiratory tract infection: nasopharyngitis or sinusitis. - Nausea: occasional, may lessen over time. - Abdominal pain: mild discomfort. - Rash: skin irritation or erythema.

Serious or Rare Side Effects: - Elevated liver enzymes: monitor hepatic function. - Cataracts: periodic ophthalmologic assessment recommended. - Severe allergic reaction: anaphylaxis or angioedema. - Cardiac arrhythmia: QT prolongation in susceptible individuals. - Pulmonary haemorrhage: rare but potentially life‑threatening.

Patients should be evaluated for safety considerations before initiating therapy.

- Liver function monitoring: assess baseline transaminases and repeat periodically. - Vision assessment: schedule regular eye examinations for cataract detection. - Cardiac evaluation: review QT interval and electrolyte status in patients with arrhythmia risk. - Drug interaction review: check concomitant medications that affect CYP3A metabolism. - Hypersensitivity: discontinue if signs of severe allergic reaction appear. - Store at ambient temperature: keep tablets in the original container, protect from moisture and heat.

Ivacaftor is metabolised primarily by CYP3A enzymes, making it susceptible to pharmacokinetic interactions.

Major Interactions (Avoid): - Strong CYP3A inducers (e.g., rifampin, carbamazepine): may markedly reduce ivacaftor exposure. - Strong CYP3A inhibitors (e.g., ketoconazole, itraconazole): can increase ivacaftor plasma levels and risk toxicity. - Antacids containing aluminium or magnesium: may decrease absorption if taken simultaneously.

Moderate Interactions (Monitor Closely): - Azole antifungals (e.g., fluconazole): may modestly raise ivacaftor concentrations. - Macrolide antibiotics (e.g., erythromycin): require periodic liver function testing. - Warfarin: monitor INR as ivacaftor may affect anticoagulant effect.