HEMLIBRA 30 mg/ml

Indications

• Prophylaxis of bleeding episodes in patients with hemophilia A
• Management of hemophilia A in patients with factor VIII inhibitors

Dosage

Children: For children aged 0 to 17 years, the recommended dose is 3 mg/kg once weekly for the first four weeks, followed by a maintenance dose of 1.5 mg/kg once weekly. Alternatively, a dose of 6 mg/kg once every two weeks may be used. Always refer to the B

Adults: The recommended dosage for adults is 3 mg/kg once weekly for the first four weeks, followed by a maintenance dose of 1.5 mg/kg once weekly. Alternatively, a dose of 6 mg/kg once every two weeks may be used.

Mechanism of action

Emicizumab acts by binding to both activated factor IX and factor X, which mimics the action of factor VIII, thus promoting the conversion of factor X to activated factor X (Xa) in the coagulation cascade. This restores the blood clotting mechanism in hemophilia A patients who are deficient in factor VIII.

Pharmacodynamics

Emicizumab enhances thrombin generation and supports hemostasis by facilitating the interaction between factor IXa and factor X. Its unique mechanism allows it to bypass the need for activated factor VIII, making it effective even in patients with inhibitors against factor VIII. The pharmacodynamic effects include a reduction in bleeding episodes and improved control of coagulation in patients with hemophilia A.

Pharmacokinetics

After subcutaneous administration, emicizumab demonstrates linear pharmacokinetics with a mean half-life of approximately 30 days, allowing for once-weekly or biweekly dosing. It is primarily eliminated through the reticuloendothelial system and does not undergo significant metabolism. The drug shows a steady-state concentration achieved within 3–6 weeks after initiation of therapy.

Contra-indications

• Fibrinolytic conditions
• History of convulsions
• Thromboembolic disease

Adverse effects

• Diarrhoea
• Nausea
• Vomiting
• Allergic dermatitis
• Colour vision change
• Seizure (more common at high doses)
• Visual impairment
• Hypotension
• Malaise

Precautions

• Monitor liver function during long-term treatment
• Establish cause of irregular menstrual bleeding before initiation

Pregnancy

No evidence of teratogenicity in animal studies; manufacturer advises use only if potential benefit outweighs risk-crosses the placenta.

Breast-feeding

Use with caution as it is not known if it is excreted in human milk.

Storage

Store at 2-8 degrees Celsius. Protect from light.

Formulations

• Subcutaneous injection
• Solution for injection