Usual Adult Dose for Hemophilia A with Inhibitors

Loading dose: 3 mg/kg subcutaneously once a week for the first 4 weeks

Maintenance dose: 1.5 mg/kg subcutaneously once a week, or 3 mg/kg subcutaneously once every two weeks, or 6 mg/kg subcutaneously once every four weeks

Comments:

• Discontinue prophylactic use of bypassing agents the day before starting this drug; prophylactic use of factor VIII (FVIII) products may be continued during the first week of prophylaxis with this drug.
• Maintenance dose selection should be based on provider preference with consideration given to patient adherence.

Use: Routine prophylaxis to prevent or reduce the frequency of bleeding episodes in patients with hemophilia A (congenital factor VIII deficiency) with factor VIII inhibitors.

Usual Pediatric Dose for Hemophilia A with Inhibitors

Newborn or Older:
Loading dose: 3 mg/kg subcutaneously once a week for the first 4 weeks

Maintenance dose: 1.5 mg/kg subcutaneously once a week, or 3 mg/kg subcutaneously once every two weeks, or 6 mg/kg subcutaneously once every four weeks

Comments:

• Discontinue prophylactic use of bypassing agents the day before starting this drug; prophylactic use of factor VIII (FVIII) products may be continued during the first week of prophylaxis with this drug.
• Maintenance dose selection should be based on provider preference with consideration given to patient adherence.

Use: Routine prophylaxis to prevent or reduce the frequency of bleeding episodes in newborn or older pediatric patients with hemophilia A (congenital factor VIII deficiency) with factor VIII inhibitors.

Renal Dose Adjustments

Data not available

Liver Dose Adjustments

Data not available

Precautions

US BOXED WARNING:

• THROMBOTIC MICROANGIOPATHY AND THROMBOEMBOLISM: Cases of thrombotic microangiopathy and thrombotic events were reported when on average a cumulative amount of greater than 100 units/kg/24 hours of activated prothrombin complex concentrate (aPCC) was administered for 24 hours or more to patients receiving this drug for prophylaxis. Monitor for the development of thrombotic microangiopathy and thrombotic events if aPCC is administered. Discontinue aPCC and suspend dosing of this drug if symptoms occur.

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration advice:

• This drug is intended for use under the guidance of a healthcare provider. After proper training, a patient or caregiver may administer this drug if deemed appropriate by a healthcare provider. Self-administration is not recommended for children less than 7 years old.
• Administer each injection at a different anatomic location (upper outer arms, thighs, or any quadrant of the abdomen) than the previous injection. Administration in the upper outer arm should only be performed by a healthcare provider or caregiver. Do not inject into moles, scars, or areas where the skin is tender, bruised, red, hard, or not intact.
• If a dose is missed, administer as soon as possible and then resume usual dosing schedule. Do not administer two doses on the same day.

Storage requirements:

• Refrigerate at 2C to 8C (36F to 46F) in the original carton to protect from light. Unopened vials may be stored out of refrigeration, but the temperature and total combined time out of refrigeration should not exceed 30C (86F) and 7 days (at a temperature below 30C [86F]), respectively. Do not freeze.
• Do not shake.
• Discard any unused drug or any drug removed from the vial and not used immediately.

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

Monitoring:

• Cardiovascular: Monitor for the development of thrombotic microangiopathy and thrombotic events if activated prothrombin complex concentrate (aPCC) is administered.

Patient advice:

• Advise the patient and/or caregiver to seek immediate medical attention if any signs or symptoms of thrombotic microangiopathy or thromboembolism occur.
• Instruct the patient and/or caregiver to notify their healthcare provider that this drug may interfere with laboratory coagulation tests prior to any blood tests or medical procedures.
• Advise the patient and/or caregiver to discontinue prophylactic use of bypassing agents the day before starting this drug; prophylactic use of factor VIII (FVIII) may be continued for the first week of prophylaxis with this drug.
• Discuss the appropriate dosing of concomitant agents such as bypassing agents or FVIII with the patient and/or caregiver prior to starting this drug.