Usual Adult Dose for Hemophilia A

CONGENITAL HEMOPHILIA A OR B WITH INHIBITORS:
Hemostatic:
90 mcg/kg IV bolus injection once every 2 hours (adjustable based on bleeding severity) until hemostasis is achieved or treatment is deemed inadequate

Post-hemostatic:
90 mcg/kg IV bolus injection once every 3 to 6 hours for severe bleeds

Perioperative Management:
Initial: 90 mcg/kg IV bolus injection once immediately before surgery; repeat every 2 hours for surgery duration (additional bolus doses may be required with major surgery).
Post-surgical:

• Minor surgery: 90 mcg/kg IV bolus injection once every 2 hours for 48 hours, then every 2 to 6 hours until healing occurs.
• Major surgery: 90 mcg/kg IV bolus injection once every 2 hours for 5 days, then every 4 hours until healing occurs.

ACQUIRED HEMOPHILIA:
Non-perioperative Management:
70 to 90 mcg/kg IV bolus injection once every 2 to 3 hours until hemostasis is achieved

Perioperative Management:
70 to 90 mcg/kg IV bolus injection once immediately before surgery; repeat every 2 to 3 hours for surgery duration and until hemostasis is achieved.

Usual Adult Dose for Hemophilia B

CONGENITAL HEMOPHILIA A OR B WITH INHIBITORS:
Hemostatic:
90 mcg/kg IV bolus injection once every 2 hours (adjustable based on bleeding severity) until hemostasis is achieved or treatment is deemed inadequate

Post-hemostatic:
90 mcg/kg IV bolus injection once every 3 to 6 hours for severe bleeds

Perioperative Management:
Initial: 90 mcg/kg IV bolus injection once immediately before surgery; repeat every 2 hours for surgery duration (additional bolus doses may be required with major surgery).
Post-surgical:

• Minor surgery: 90 mcg/kg IV bolus injection once every 2 hours for 48 hours, then every 2 to 6 hours until healing occurs.
• Major surgery: 90 mcg/kg IV bolus injection once every 2 hours for 5 days, then every 4 hours until healing occurs.

ACQUIRED HEMOPHILIA:
Non-perioperative Management:
70 to 90 mcg/kg IV bolus injection once every 2 to 3 hours until hemostasis is achieved

Perioperative Management:
70 to 90 mcg/kg IV bolus injection once immediately before surgery; repeat every 2 to 3 hours for surgery duration and until hemostasis is achieved.

Usual Adult Dose for Factor VII Deficiency

15 to 30 mcg/kg IV bolus injection once every 4 to 6 hours until hemostasis is achieved

Comments: Doses as low as 10 mcg/kg have been effective.

Usual Adult Dose for Glanzmann's Thrombasthenia

Non-perioperative Management:
90 mcg/kg IV bolus injection once every 2 to 6 hours until hemostasis is achieved in severe bleeding episodes requiring systemic hemostatic therapy

Perioperative Management:
Initial: 90 mcg/kg IV bolus injection immediately before surgery; repeat every 2 hours for procedure duration.
Post-surgical: 90 mcg/kg IV bolus injection once every 2 to 6 hours

Usual Pediatric Dose for Hemophilia A

CONGENITAL HEMOPHILIA A OR B WITH INHIBITORS:
Hemostatic:
90 mcg/kg IV bolus injection once every 2 hours (adjustable based on bleeding severity) until hemostasis is achieved or treatment is deemed inadequate

Post-hemostatic:
90 mcg/kg IV bolus injection once every 3 to 6 hours for severe bleeds

Perioperative Management:
Initial: 90 mcg/kg IV bolus injection once immediately before surgery; repeat every 2 hours for surgery duration (additional bolus doses may be required with major surgery).
Post-surgical:

• Minor surgery: 90 mcg/kg IV bolus injection once every 2 hours for 48 hours, then every 2 to 6 hours until healing occurs.
• Major surgery: 90 mcg/kg IV bolus injection once every 2 hours for 5 days, then every 4 hours until healing occurs.

ACQUIRED HEMOPHILIA:
Non-perioperative Management:
70 to 90 mcg/kg IV bolus injection once every 2 to 3 hours until hemostasis is achieved

Perioperative Management:
70 to 90 mcg/kg IV bolus injection once immediately before surgery; repeat every 2 to 3 hours for surgery duration and until hemostasis is achieved.

Usual Pediatric Dose for Hemophilia B

CONGENITAL HEMOPHILIA A OR B WITH INHIBITORS:
Hemostatic:
90 mcg/kg IV bolus injection once every 2 hours (adjustable based on bleeding severity) until hemostasis is achieved or treatment is deemed inadequate

Post-hemostatic:
90 mcg/kg IV bolus injection once every 3 to 6 hours for severe bleeds

Perioperative Management:
Initial: 90 mcg/kg IV bolus injection once immediately before surgery; repeat every 2 hours for surgery duration (additional bolus doses may be required with major surgery).
Post-surgical:

• Minor surgery: 90 mcg/kg IV bolus injection once every 2 hours for 48 hours, then every 2 to 6 hours until healing occurs.
• Major surgery: 90 mcg/kg IV bolus injection once every 2 hours for 5 days, then every 4 hours until healing occurs.

ACQUIRED HEMOPHILIA:
Non-perioperative Management:
70 to 90 mcg/kg IV bolus injection once every 2 to 3 hours until hemostasis is achieved

Perioperative Management:
70 to 90 mcg/kg IV bolus injection once immediately before surgery; repeat every 2 to 3 hours for surgery duration and until hemostasis is achieved.

Usual Pediatric Dose for Factor VII Deficiency

15 to 30 mcg/kg IV bolus injection once every 4 to 6 hours until hemostasis is achieved

Comments: Doses as low as 10 mcg/kg have been effective.

Usual Pediatric Dose for Glanzmann's Thrombasthenia

Non-perioperative Management:
90 mcg/kg IV bolus injection once every 2 to 6 hours until hemostasis is achieved in severe bleeding episodes requiring systemic hemostatic therapy

Perioperative Management:
Initial: 90 mcg/kg IV bolus injection immediately before surgery; repeat every 2 hours for procedure duration.
Post-surgical: 90 mcg/kg IV bolus injection once every 2 to 6 hours

Renal Dose Adjustments

Data not available

Liver Dose Adjustments

Data not available

Precautions

US BOXED WARNING:

• THROMBOSIS: Serious arterial and venous thrombotic events have been reported following administration of this drug. Discuss the risks and explain the signs and symptoms of thrombotic and thromboembolic events to patients who will receive this drug. Monitor patients for signs or symptoms of activation of the coagulation system and for thrombosis.

Safety and efficacy have not been established to determine differences among age groups (0 to 16 years); clinical trials were conducted with dosing based on body weight, not age.

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration advice:

• For IV bolus injection only.
• Administer as soon as possible following bleeding onset or immediately before scheduled procedure.
• The manufacturer product information should be consulted.

Storage requirements:

• Prior to reconstitution: Store powder and histidine diluent between 2 and 25C (36 to 77F); do not freeze; protect from light.
• After reconstitution: Store at room temperature or refrigerated up to 3 hours; do not freeze or store in syringes.

Reconstitution/preparation techniques: The manufacturer product information should be consulted.

IV compatibility: Do not mix with other infusion solutions.

General:

• Coagulation parameters do not necessarily correlate with or predict effectiveness of this drug; use hemostasis evaluation to determine effectiveness.
• Congenital hemophilia A or B with inhibitors: Outcome decision was made for a majority of patients treated for joint or muscle bleeds within 8 doses; more doses were required for severe bleeds. A majority of patients who reported adverse experiences received more than 12 doses.

Monitoring:

• Monitor for signs or symptoms of activation of the coagulation system or thrombosis.
• Monitor and minimize the duration of any post-hemostatic dosing.
• Monitor prothrombin time (PT) and factor VII coagulant activity in factor VII deficient patients before and after administering this drug.
• Monitor for factor VII antibodies in factor VII deficient patients treated with this drug.

Patient advice:

• Inform patients to seek immediate medical attention if early signs of hypersensitivity occur (e.g., hives, urticaria, chest tightness, wheezing, hypotension, and anaphylaxis).
• Advise patients to seek immediate medical attention if signs of thrombosis occur (e.g., new onset swelling and pain in the limbs or abdomen, new onset chest pain, shortness of breath, loss of sensation or motor power, or altered consciousness or speech).