NOVOSEVEN RT, Coagulation Factor VIIa (Recombinant) Form

Factor VIIa, recombinant (NovoSeven® RT) and coagulation factor VIIa (recombinant)-jncw (SevenFact®) are coagulation factors. FDA Approved Indication(s) NovoSeven RT is indicated for treatment of bleeding episodes and perioperative management in: • Adults and children with hemophilia A or B with inhibitors, congenital factor VII (FVII) deficiency, and Glanzmann’s thrombasthenia with refractoriness to platelet transfusions, with or without antibodies to platelets • Adults with acquired hemophilia SevenFact is indicated for the treatment and control of bleeding episodes occurring in adults and adolescents (12 years of age and older) with hemophilia A or B with inhibitors. Limitation(s) of use: SevenFact is not indicated for treatment of congenital factor VII deficiency. Policy/Criteria Provider must submit documentation (such as office chart notes, lab results or other clinical information) supporting that member has met all approval criteria.
It is the policy of health plans affiliated with Centene Corporation® that NovoSeven RT and SevenFact are medically necessary when the following criteria are met:
I. Initial Approval Criteria
A. Hemophilia A or B with Inhibitors, Congenital Factor VII Deficiency (must meet all):

1. Diagnosis of one of the following (a or b): a. Congenital or acquired hemophilia A or B with inhibitors; b. Congenital factor VII deficiency (NovoSeven RT requests only);
   2. Prescribed by or in consultation with a hematologist;
2. For SevenFact requests only: Age ≥ 12 years;

3. Request is for one of the following uses (a or b): a. Control and prevention of bleeding episodes; b. Perioperative management (NovoSeven RT requests only);

   5. Documentation of member’s current body weight (in kg); Page 1 of 8

   CLINICAL POLICY Factor VIIa, Recombinant

   6. Dose does not exceed one of the following (a or b): a. For NovoSeven requests (i or ii): i. Hemophilia: 90 mcg/kg every 2 hours; ii. Congenital factor VII deficiency: 30 mcg/kg every 4 hours; b. For SevenFact requests: 75 mcg/kg every 2 hours. Approval duration: 3 months
      B. Glanzmann’s Thrombasthenia (must meet all):
4. Diagnosis of Glanzmann’s thrombasthenia;
5. Request is for NovoSeven RT;
6. Prescribed by or in consultation with a hematologist;
7. Condition is refractory to platelet transfusions;
8. Request is for one of the following uses (a or b): a. Control and prevention of bleeding episodes; b. Perioperative management;
   6. Documentation of member’s current body weight (in kg);

9. Dose does not exceed 90 mcg/kg every two hours. Approval duration: 3 months
   C. Other diagnoses/indications (must meet 1 or 2):

   1. If this drug has recently (within the last 6 months) undergone a label change (e.g., newly approved indication, age expansion, new dosing regimen) that is not yet reflected in this policy, refer to one of the following policies (a or b): a. For drugs on the formulary (commercial, health insurance marketplace) or PDL (Medicaid), the no coverage criteria policy for the relevant line of business: CP.CPA.190 for commercial, HIM.PA.33 for health insurance marketplace, and CP.PMN.255 for Medicaid; or b. For drugs NOT on the formulary (commercial, health insurance marketplace) or PDL (Medicaid), the non-formulary policy for the relevant line of business: CP.CPA.190 for commercial, HIM.PA.103 for health insurance marketplace, and CP.PMN.16 for Medicaid; or
   2. If the requested use (e.g., diagnosis, age, dosing regimen) is NOT specifically listed under section III (Diagnoses/Indications for which coverage is NOT authorized) AND criterion 1 above does not apply, refer to the off-label use policy for the relevant line of business: CP.CPA.09 for commercial, HIM.PA.154 for health insurance marketplace, and CP.PMN.53 for Medicaid.
      II. Continued Therapy A. All Indications in Section I (must meet all):
   3. Member meets one of the following (a or b): a. Currently receiving medication via Centene benefit or member has previously met initial approval criteria; b. Member is currently receiving medication and is enrolled in a state and product with continuity of care regulations (refer to state specific addendums for CC.PHARM.03A and CC.PHARM.03B); Page 2 of 8

   CLINICAL POLICY Factor VIIa, Recombinant

   2. Member is responding positively to therapy;
10. Documentation of member’s current body weight (in kg);

11. If request is for a dose increase, new dose does not exceed one of the following (a or b): a. For NovoSeven requests (i or ii): i. Hemophilia: 90 mcg/kg every 2 hours; ii. Congenital factor VII deficiency: 30 mcg/kg every 4 hours; b. For SevenFact requests: 75 mcg/kg every 2 hours. Approval duration: 3 months
    B. Other diagnoses/indications (must meet 1 or 2):

    1. If this drug has recently (within the last 6 months) undergone a label change (e.g., newly approved indication, age expansion, new dosing regimen) that is not yet reflected in this policy, refer to one of the following policies (a or b): a. For drugs on the formulary (commercial, health insurance marketplace) or PDL (Medicaid), the no coverage criteria policy for the relevant line of business: CP.CPA.190 for commercial, HIM.PA.33 for health insurance marketplace, and CP.PMN.255 for Medicaid; or b. For drugs NOT on the formulary (commercial, health insurance marketplace) or PDL (Medicaid), the non-formulary policy for the relevant line of business: CP.CPA.190 for commercial, HIM.PA.103 for health insurance marketplace, and CP.PMN.16 for Medicaid; or
    2. If the requested use (e.g., diagnosis, age, dosing regimen) is NOT specifically listed under section III (Diagnoses/Indications for which coverage is NOT authorized) AND criterion 1 above does not apply, refer to the off-label use policy for the relevant line of business: CP.CPA.09 for commercial, HIM.PA.154 for health insurance marketplace, and CP.PMN.53 for Medicaid.
       III. Diagnoses/Indications for which coverage is NOT authorized:
       A. Non-FDA approved indications, which are not addressed in this policy, unless there is sufficient documentation of efficacy and safety according to the off label use policy – CP.CPA.09 for commercial, HIM.PA.154 for health insurance marketplace, and CP.PMN.53 for Medicaid, or evidence of coverage documents.
       IV. Appendices/General Information Appendix A: Abbreviation/Acronym Key FDA: Food and Drug Administration Appendix B: Therapeutic Alternatives Not applicable Page 3 of 8

    CLINICAL POLICY Factor VIIa, Recombinant Appendix C: Contraindications/Boxed Warnings • Contraindication(s):
    o NovoSeven RT: none reported o SevenFact: known allergy to rabbits or rabbit proteins; severe hypersensitivity reaction to SevenFact or any of its components • Boxed warning(s): thrombosis Appendix D: General Information • Congenital hemophilia A is a deficiency of factor VIII. • Congenital hemophilia B is a deficiency of factor IX. • Acquired hemophilia is evidenced by presence of coagulation factor inhibitors (autoantibodies). V. Dosage and Administration Indication Treatment of bleeding episodes Drug Name Factor VIIa, recombinant (NovoSeven RT)
    Dosing Regimen Congenital hemophilia A or B with inhibitors: • 90 mcg/kg IV every 2 hrs, adjustable based on severity of bleeding until hemostasis is achieved • 90 mcg/kg IV every 3-6 hrs after hemostasis is achieved for severe bleeds Maximum Dose Congenital factor VII deficiency: 30 mcg/kg every 4 hrs All other indications: 90 mcg/kg every 2 hrs Factor VIIa, recombinant (NovoSeven RT) Peri- operative management Congenital factor VII deficiency: 15-30 mcg/kg IV every 4-6 hrs until hemostasis is achieved Glanzmann’s thrombasthenia: 90 mcg/kg IV every 2-6 hrs until hemostasis is achieved Acquired hemophilia: 70-90 mcg/kg IV every 2-3 hrs until hemostasis is achieved Congenital hemophilia A or B with inhibitors: Minor surgery: • 90 mcg/kg IV immediately before surgery, repeat every 2 hrs during surgery • 90 mcg/kg IV every 2 hrs after surgery for 48 hours, then every Page 4 of 8 Congenital factor VII deficiency: 30 mcg/kg every 4 hrs Glanzmann’s thrombasthenia: 140 mcg/kg every 2 hrs

    CLINICAL POLICY Factor VIIa, Recombinant Drug Name Indication Dosing Regimen 2-6 hrs until healing has occurred Major surgery: • 90 mcg/kg IV immediately before surgery, repeat every 2 hrs during surgery • 90 mcg/kg IV every 2 hrs after surgery for 5 days, then every 4 hrs or by continuous infusion at 50 mcg/kg/hr until healing has occurred • Additional boluses can be given Congenital factor VII deficiency: 15-30 mcg/kg IV immediately before surgery and every 4-6 hours for the duration of surgery and until hemostasis is achieved Note: doses as low as 10 mcg/kg can be effective Glanzmann’s thrombasthenia: • 90 mcg/kg IV immediately before surgery and repeat every 2 hrs for the duration of the procedure • 90 mcg/kg IV every 2-6 hrs to prevent post-operative bleeding • Higher doses of 100-140 mcg/kg can be used for surgical patients who have clinical refractoriness with or without platelet-specific antibodies Acquired hemophilia: 70-90 mcg/kg immediately before surgery and every 2-3 hrs for the duration of surgery and until hemostasis is achieved For mild or moderate bleeds: 75 mcg/kg IV every 3 hrs until hemostasis is achieved
    OR
    Page 5 of 8 Coagulation factor VIIa (recombinant)- Treatment and control of bleeding episodes Maximum Dose All other indications: 90 mcg/kg every 2 hrs 75 mcg/kg every 3 hrs

    CLINICAL POLICY Factor VIIa, Recombinant Indication Drug Name jncw (SevenFact) Maximum Dose Dosing Regimen Initial dose of 225 mcg/kg; if hemostasis is not achieved within 9 hrs, additional 75 mcg/kg every 3 hrs as needed to achieve hemostasis For severe bleeds: 225 mcg/kg, followed if necessary 6 hrs later with 75 mcg/kg every 2 hrs VI. Product Availability
    Drug Name Factor VIIa, recombinant (NovoSeven RT) Coagulation factor VIIa (recombinant)-jncw (SevenFact) Availability Powder for reconstitution in single-use vials: 1 mg, 2 mg, 5 mg, 8 mg Lyophilized powder for reconstitution in single-use vials: 1 mg, 5 mg
    VII.