Indications for BENEFIX:
On-demand treatment and control of bleeding in hemophilia B. Perioperative management in patients with hemophilia B. Routine prophylaxis to reduce the frequency of bleeding episodes in patients aged ≥16yrs with hemophilia B.
Limitations of Use:
Not for induction of immune tolerance in patients with hemophilia B.
Adults and Children:
See full labeling. Initial Dose Required (IU) = Body Weight (kg) x Desired FIX increase x Reciprocal of observed recovery (IU/kg per IU/dL). Average recovery (≥12yrs): Dose Required (IU) = Body Weight (kg) x Desired FIX increase x 1.3 (IU/kg per IU/dL); (<12yrs): Dose Required (IU) = Body Weight (kg) x Desired FIX increase x 1.4 (IU/kg per IU/dL). Give by IV infusion over several minutes. If inhibitor present or low factor IX recovery, may need higher doses. Hemorrhage (Minor): 20–30% increase every 12–24hrs for 1–2 days; (Moderate): 25–50% increase every 12–24hrs for 2–7 days until resolved; (Major): 50–100% increase every 12–24hrs for 7–10 days. Routine prophylaxis (≥16yrs): 100 IU/kg once weekly; adjust dose or frequency based on clinical response.
Hamster protein hypersensitivity.
Safety and efficacy as continuous infusion: not established. Perform initial (approx. 10–20) administrations under medical supervision. Monitor closely for acute hypersensitivity reactions (esp. during the early phases); discontinue immediately if occurs and treat appropriately. Monitor for development of factor IX inhibitors; increased risk of anaphylaxis. Pregnancy. Nursing mothers.
Fever, cough, nausea, inj site reaction/pain, headache, dizziness, rash; hypersensitivity reactions, inhibitor development, thromboembolic complications, nephrotic syndrome.
Generic Drug Availability:
Kit—1 (vial + diluent, supplies)