AFSTYLA 1000 IU Powder and Solvent for Solution for Injection

What is AFSTYLA 1000 IU

AFSTYLA 1000 IU is a recombinant, single-chain human coagulation factor VIII (FVIII), indicated for the treatment and prophylaxis of bleeding episodes in patients with Hemophilia A (congenital factor VIII deficiency). The active ingredient, Lonoctocog alfa, is a long-acting factor VIII replacement therapy designed for intravenous administration.

• Form: Powder and solvent for solution for injection.
• Packaging: Each kit includes one glass vial of powder, one glass vial containing 2.5 mL of Water for Injection (WFI), a filter transfer device (20/20), and an administration set (including syringe, venipuncture set, alcohol swabs, and plaster).
• Indication: Used for both on-demand treatment of bleeding episodes and routine prophylaxis to reduce bleeding frequency in Hemophilia A patients.
• Patient Group: Suitable for both pediatric and adult patients.
• Mechanism: Replaces the missing or deficient FVIII, promoting blood clotting and hemostasis.

AFSTYLA is not indicated for the treatment of von Willebrand disease. It is a high-purity product that exhibits improved stability due to its single-chain structure, contributing to a longer half-life and extended activity in the bloodstream.

How to use AFSTYLA 1000 IU

AFSTYLA 1000 IU should be used under the supervision of a healthcare professional experienced in treating Hemophilia A. The reconstitution and administration steps must be followed carefully to ensure safety and effectiveness.

• Preparation:
  • Wash hands thoroughly before handling the product.
  • Bring the vials of powder and solvent (WFI) to room temperature.
  • Use the provided filter transfer device to mix the solvent into the powder vial aseptically.
  • Gently swirl (do not shake) until the powder dissolves completely, forming a clear solution.
• Administration:
  • Draw the reconstituted solution into the provided disposable syringe using the administration set.
  • Use the venipuncture set to inject the solution intravenously.
  • Administer slowly over several minutes (rate depends on patient comfort and physician's advice).
• Disposal: Dispose of all used items (syringe, swabs, etc.) in accordance with local biohazard waste disposal regulations.

Always use AFSTYLA exactly as prescribed. Patients or caregivers can be trained for home use, but regular follow-up with a hemophilia treatment center is advised.

Mode of Action AFSTYLA 1000 IU

Lonoctocog alfa, the active substance in AFSTYLA, is a recombinant fusion protein designed to mimic the natural coagulation Factor VIII function, with enhanced molecular stability.

• Recombinant Origin: Produced using genetically modified CHO (Chinese Hamster Ovary) cells, free from human or animal components.
• Single-Chain Design: The molecule is designed with a covalently bound heavy and light chain, offering enhanced stability and higher binding affinity to von Willebrand factor (vWF), which protects FVIII in circulation.
• Activation Mechanism: After intravenous administration, Lonoctocog alfa is activated by thrombin, releasing the active FVIII to participate in the coagulation cascade.
• Coagulation Cascade Role: Factor VIII acts as a cofactor to Factor IXa in the activation of Factor X, leading to thrombin generation and the formation of a stable blood clot.
• Extended Half-life: The single-chain design results in reduced degradation and prolonged circulation time compared to traditional FVIII products.

AFSTYLA restores clotting ability in Hemophilia A patients, reducing the frequency and severity of bleeding episodes and allowing for improved prophylactic management.

AFSTYLA 1000 IU Interactions AFSTYLA 1000 IU

AFSTYLA generally has a low potential for drug interactions due to its protein nature and specific action on the coagulation pathway. However, certain considerations should be noted:

• Antifibrinolytics: Drugs like tranexamic acid or aminocaproic acid may be used adjunctively with AFSTYLA in surgery or dental procedures but should be managed carefully to avoid thrombosis risk.
• Immune Response: Immune-modulating drugs (e.g., corticosteroids, immunosuppressants) may influence the development or suppression of neutralizing antibodies (inhibitors) against FVIII.
• Other Clotting Factor Products: Concomitant use of different FVIII products should be avoided as it may complicate efficacy monitoring or lead to immunological responses.
• Laboratory Interference: AFSTYLA may affect clotting assays (e.g., APTT) used in diagnosing other conditions. Ensure laboratory staff are aware the patient is on recombinant FVIII.
• Vaccines: No known interactions, but as with any injectable therapy, avoid simultaneous intramuscular vaccination during acute bleeding or if on high FVIII doses.

Always inform the healthcare provider of all medications, supplements, or herbal remedies being taken. While interactions are rare, a comprehensive review helps ensure optimal therapy and safety.

Dosage of AFSTYLA 1000 IU

The dosage of AFSTYLA 1000 IU depends on the severity of Factor VIII deficiency, the location and severity of bleeding, individual pharmacokinetics, and whether the therapy is prophylactic or on-demand.

• General Dosing Formula:
  Required IU = body weight (kg) × desired FVIII rise (IU/dL or % of normal) × 0.5
• On-Demand Treatment:
  • Mild bleeding (e.g., early hemarthrosis): 20–40 IU/kg
  • Moderate bleeding (e.g., muscle bleeding): 30–60 IU/kg
  • Severe bleeding (e.g., intracranial, gastrointestinal): 60–80 IU/kg initially, then maintenance doses as needed
• Prophylaxis:
  • Recommended: 20–50 IU/kg two to three times per week
  • Can be adjusted based on bleeding phenotype and pharmacokinetic response
• Surgical Prophylaxis:
  • Pre-operative: 30–60 IU/kg to ensure adequate FVIII level before surgery
  • Post-operative: Maintenance dosing every 8–24 hours, tailored to procedure type and bleeding risk

Important: Monitor FVIII levels when possible to tailor individual therapy. Patients may develop inhibitors that require dose adjustment or alternative therapy.

Possible side effects of AFSTYLA 1000 IU

While AFSTYLA is generally well-tolerated, some patients may experience side effects, ranging from mild to serious. Monitoring and prompt reporting of any adverse reactions are essential.

• Common Side Effects:
  • Headache
  • Injection site reactions (e.g., redness, pain)
  • Nausea
• Less Common but Serious:
  • Development of Factor VIII inhibitors (neutralizing antibodies)
  • Hypersensitivity reactions (rash, urticaria)
  • Anaphylaxis (rare but serious)
• Immune Response:
  • Formation of antibodies may reduce efficacy.
  • Routine inhibitor testing is recommended, especially in previously untreated patients (PUPs).
• Other Possible Symptoms:
  • Dizziness
  • Fatigue
  • Fever

Seek medical attention immediately in case of signs of allergic reaction or loss of effectiveness during treatment. A detailed adverse effect monitoring plan should be established for long-term users.

AFSTYLA 1000 IU Contraindications AFSTYLA 1000 IU

AFSTYLA is contraindicated in patients with the following conditions:

• Known Hypersensitivity:
  • Allergy to Lonoctocog alfa or any excipients (e.g., polysorbate 80, mannitol, sodium chloride).
• History of Anaphylaxis:
  • Patients who previously experienced life-threatening reactions to Factor VIII products.
• Presence of Inhibitors:
  • High-titer inhibitors against FVIII render AFSTYLA ineffective and may require bypassing agents.

AFSTYLA is not intended for the treatment of von Willebrand disease. Caution is advised in patients with a history of allergic disorders. A thorough clinical history should be evaluated prior to initiating treatment.

Storage of AFSTYLA 1000 IU

Proper storage of AFSTYLA is essential to preserve its stability and efficacy:

• Unopened Vial Storage:
  • Store in a refrigerator at 2°C to 8°C (36°F to 46°F).
  • May be stored at room temperature (up to 25°C) for a single period of up to 6 months.
  • Do not return to refrigerator once stored at room temperature.
• Protection from Light:
  • Keep the vials in the original package to protect from light exposure.
• After Reconstitution:
  • Use the solution within 4 hours.
  • Do not refrigerate or freeze after reconstitution.
  • Do not use if the solution is cloudy or contains visible particles.
• Disposal:
  • Dispose of unused product and waste material according to local regulations.

Always keep AFSTYLA out of reach of children. Do not use after the expiration date stated on the label and outer carton.