Adult Dosing
Hemophilia B
- Initial dose = body weight (kg) x desired factor IX increase (% or IU/dL) x reciprocal of observed recovery (IU/dL per IU/kg)
BeneFIX
- Minor bleeding/perioperative management
- Individualize dose IV q12-24 hrs x 1-2 days
- Desired factor IX level = 20-30%
- Moderate bleeding/perioperative management
- Individualize dose IV q12-24 hrs; treat until bleeding stops and healing begins (2-7 days)
- Desired factor IX level = 25-50%
- Major bleeding/perioperative management
- Individualize dose IV q12-24 hrs x 7-10 days
- Desired factor IX level = 50-100%
- For incremental recovery of 0.8 IU/dL, calculate the dose as (IU) = [wt (kg) x desired factor IX increase (% or IU/dL) x 1.3 dL/kg]
Rixubis
- Minor bleeding
- Individualize dose IV q12-24 hrs atleast 1 day, until healing is achieved
- Desired factor IX level .= 20-30%
- Moderate bleeding
- Individualize dose IV q12-24 hrs; treat until bleeding stops and healing begins (2-7 days)
- Desired factor IX level = 25-50%
- Major bleeding
- Individualize dose IV q12-24 hrs x 7-10 days
- Desired factor IX level = 50-100%
- For incremental recovery of 0.9 IU/dL, calculate the dose as (IU) = [wt (kg) x desired factor IX increase (% or IU/dL) x 1.1 dL/kg]
Alprolix
- Minor and Moderate bleeding
- Circulating Factor IX level required 30-60 IU/dL. Repeat every 48 hours if there is further evidence of bleeding
- Major bleeding
- Circulating Factor IX level required 80-100 IU/dL. Consider a repeat dose after 6-10 hours and then every 24 hours for the first 3 days
- Due to the long half-life of therapy, the dose may be reduced and frequency of dosing may be extended after day 3 to every 48 hours or longer until bleeding stops and healing is achieved
Notes:- Dosage and duration of treatment for all factor IX products depend on the severity of factor IX deficiency, the location and extent of bleeding, and the patient’s clinical condition, age and recovery of factor IX
- Refer packahe insert for preparation and reconstitution procedure
Perioperative management
Rixubis
- Minor surgery
- Individualize dose IV q12-24 hrs atleast 1 day, until healing is achieved
- Desired factor IX level = 30-60%
- Major surgery
- Individualize dose IV q8-24 hrs x 7-10 days
- Desired factor IX level = 80-100%
Hemophilia B, Routine Prophylaxis
Rixubis
- 40-60 IU/kg IV bolus; not to exceed infusion rate of 10 mL/min
Alprolix
- Minor surgery
- Circulating Factor IX level required 50-80 IU/dL. Repeat as needed after 24-48 hours until bleeding stops and healing is achieved
- Major surgery
- Circulating Factor IX level required 60-100 IU/dL. Consider a repeat dose after 6-10 hours and then every 24 hours for the first 3 days
Pediatric Dosing
Hemophilia B
- Initial dose = body weight (kg) x desired factor IX increase (% or IU/dL) x reciprocal of observed recovery (IU/dL per IU/kg)
BeneFIX
- Children <15 yrs: Estimated dose (units) = [wt (kg) x desired factor IX level (% or IU/dL) x 1.4 (IU/kg per IU/dL)]
- Children >15 yrs: Estimated dose (units) = [wt (kg) x desired factor IX level (% or IU/dL) x 1.3 (IU/kg per IU/dL)]
- Minor bleeding/perioperative management
- Individualize dose IV q12-24 hrs x 1-2 days
- Desired factor IX level = 20-30%
- Moderate bleeding/perioperative management
- Individualize dose IV q12-24 hrs; treat until bleeding stops and healing begins (2-7 days)
- Desired factor IX level = 25-50%
- Major bleeding/perioperative management
- Individualize dose IV q12-24 hrs x 7-10 days
- Desired factor IX level = 50-100%
- For incremental recovery of 0.7 IU/dL, the dose is calculated as: Dose (IU) = body weight (kg) x desired factor IX increase (% or IU/dL) x 1.4 dL/kg
Notes:- In pediatric patients <15 yrs of age, 1 IU/kg will increase the circulating level of factor IX by approx 0.7% [IU/dL] of normal
- In pediatric patients >15 yrs of age, 1 IU/kg will increase the circulating level of factor IX by approx 0.8% [IU/dL] of normal
- Dosage and duration of treatment for all factor IX products depend on the severity of factor IX deficiency, the location and extent of bleeding, and the patient’s clinical condition, age and recovery of factor IX
Alprolix
- Minor and Moderate bleeding for > 12 yrs
- Circulating Factor IX level required 30-60 IU/dL. Repeat every 48 hours if there is further evidence of bleeding
- Major bleeding for > 12 yrs
- Circulating Factor IX level required 80-100 IU/dL. Consider a repeat dose after 6-10 hours and then every 24 hours for the first 3 days
- Due to the long half-life of therapy, the dose may be reduced and frequency of dosing may be extended after day 3 to every 48 hours or longer until bleeding stops and healing is achieved
Perioperative management
Alprolix
- Minor surgery for > 12 yrs
- Circulating Factor IX level required 50-80 IU/dL. Repeat as needed after 24-48 hours until bleeding stops and healing is achieved
- Major surgery for > 12 yrs
- Circulating Factor IX level required 60-100 IU/dL. Consider a repeat dose after 6-10 hours and then every 24 hours for the first 3 days
- Coagulation Factor IX (Recombinant) is not indicated for the treatment of other factor deficiencies (e.g., factors II, VII, VIII, and X), hemophilia A with inhibitors to factor VIII, reversal of coumarin-induced anticoagulation, or for the treatment of bleeding due to low levels of liver-dependent coagulation factors
- If bleeding is not controlled with the recommended dose, determine the plasma level of factor IX and administer sufficient dose to achieve a satisfactory clinical response
- Allergic-type hypersensitivity reactions, including anaphylaxis, have been reported following administration of coagulation factor IX products. Inform patients of the early signs and symptoms of hypersensitivity reactions such as hives, pruritus, rash, facial swelling, dizziness, urticaria, chest tightness, nausea, cough, dyspnea, wheezing, flushing, hypotension, discomfort, and fatigue. Advise patients to discontinue the drug and immediately contact their clinician for appropriate emergency care, depending on the severity of reaction, if such symptoms occur
- The diluent vial of this product may contain dry natural rubber that can cause hypersensitivity reactions in some individuals with known or possible latex sensitivity
- Immune tolerance induction with factor IX products in hemophilia B patients with factor IX inhibitors and a history of allergic reactions to factor IX may cause nephrotic syndrome
- Thromboembolic complications have been associated with the use of factor IX Complex concentrates. Use of factor IX-containing products in patients with signs of fibrinolysis or disseminated intravascular coagulation (DIC) may be potentially hazardous
- Activity-neutralizing antibodies (inhibitors) have been observed in patients receiving factor IX products; hence, monitor all patients for the development of factor IX inhibitors using appropriate clinical observations and lab tests
- Patients with factor IX inhibitors may be at an increased risk of anaphylaxis following subsequent dose of factor IX. Evaluate patients experiencing allergic reactions for the presence of an inhibitor
- Due to the potential for allergic reactions with factor IX concentrates, the initial administrations should be performed under medical supervision where proper medical care for allergic reactions could be provided
- Relationship may exist between the presence of major deletion mutations in the factor IX gene and an increased risk of inhibitor formation and of acute hypersensitivity reactions. Closely monitor patients known to have major deletion mutations of the factor IX gene for signs and symptoms of acute hypersensitivity reactions, particularly during the early phases of initial exposure
Cautions: Use cautiously in
- Liver disease
- Post-operative patients
- Neonates
- Risk of thromboembolic phenomena
- DIC
- Fibrinolysis
- Hypersensitivity to latex
Pregnancy Category:C
Breastfeeding: Safety unknown. Manufacturer recommends caution.
