Antihemophilic Factor (recombinant), Fc Fusion Protein

Hemophilia A

On-demand treatment and control of bleeding episodes in patients with hemophilia A (congenital factor VIII deficiency; classic hemophilia).1 4

Maintenance of hemostasis in patients with hemophilia A undergoing surgery (i.e., perioperative management of bleeding).1 4

Routine prophylaxis (i.e., administration at regular intervals) to reduce frequency of bleeding events.1 4 Such prophylaxis considered the current standard of care for patients with hemophilia A.17 215 218 Decreases frequency of spontaneous musculoskeletal bleeding, preserves joint function, and improves quality of life.215 224 231

Designated an orphan drug by FDA for treatment of hemophilia A.3

Half-life of antihemophilic factor (recombinant), Fc fusion protein is longer than that of conventional preparations of recombinant antihemophilic factor; may allow for less frequent dosing and possibly improve patient compliance with prophylactic regimens.1 4 5 8 10 16 17

Several antihemophilic factor concentrates are currently available in the US, including a variety of recombinant and plasma-derived preparations; the Medical and Scientific Advisory Council (MASAC) of the National Hemophilia Foundation recommends preferential use of recombinant antihemophilic factor preparations because of their potentially superior safety profile with respect to pathogen transmission.218 246 247 Other experts (e.g., World Federation of Hemophilia) state that choice of preparation should be determined by local criteria.215 When selecting an appropriate antihemophilic factor preparation, consider characteristics of each clotting factor concentrate, individual patient variables, patient/provider preference, and emerging data.215 243 246 247

Not indicated for the treatment of von Willebrand disease.1

Absorption

Plasma Concentrations

In adults, mean recovery of factor VIII is approximately 2.26 units/dL for each 1 unit/kg of antihemophilic factor (recombinant), Fc fusion protein administered.1 Mean incremental recoveries in pediatric patients 1–5, 6–11, and 12–17 years of age are 1.92, 2.44, and 1.85 units/dL, respectively.1

Duration

Compared with conventional preparations of recombinant antihemophilic factor, exhibits increased time after dosing to 1% factor VIII activity suggesting potentially longer therapeutic duration.5

Distribution

Extent

Not known whether antihemophilic factor (recombinant), Fc fusion protein is distributed into milk.1

Elimination

Half-life

Following administration of a single 50-unit/kg dose given as a 10-minute IV injection in adults, terminal half-life was approximately 19.7 hours and time to reach 1% factor VIII activity was 5.1 days.1

Compared with adults, pediatric patients appear to have a shorter half-life; in one study, half-life was approximately 12.7, 14.9, or 16.4 hours in pediatric patients 1–5, 6–11, or 12–17 years of age, respectively.1

In pediatric patients 1–5 years of age, clearance is substantially increased compared with older pediatric patients and adults.1 256

Compared with conventional preparations of recombinant antihemophilic factor, half-life of antihemophilic factor (recombinant), Fc fusion protein is prolonged and clearance reduced.5 8

Storage

Parenteral

2–8°C in original package; do not freeze or expose to direct sunlight.1 Freezing may damage prefilled diluent syringe.1

May store at room temperature (≤30°C) for a single period of up to 6 months or until expiration date on label.1 Do not place back into refrigerator after storage at room temperature.1

May store at room temperature (≤30°C); protect from direct sunlight.1 Use solution within 3 hours of reconstitution.1

Excipients in commercially available drug preparations may have clinically important effects in some individuals; consult specific product labeling for details.

Please refer to the ASHP Drug Shortages Resource Center for information on shortages of one or more of these preparations.