BEBULIN, Factor IX Complex PROFILNINE, Factor IX Complex Form

Factor IX complex (human) (Profilnine®) contains factor IX, II, X, and low levels of factor VII. FDA Approved Indication(s) Profilnine is indicated for the prevention and control of bleeding episodes in patients with factor IX deficiency (hemophilia B). Limitation(s) of use: Profilnine contains non-therapeutic levels of factor VII and is not indicated for use in the treatment of 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 Profilnine is medically necessary when the following criteria are met:
I. Initial Approval Criteria
A. Congenital Hemophilia B (must meet all):

1. Diagnosis of congenital hemophilia B (factor IX deficiency);
   2. Prescribed by or in consultation with a hematologist;
   3. Age ≥ 18 years;
   4. Request is for prevention and control of bleeding episodes;
   5. Documentation of member’s current body weight (in kg);
   6. Dose does not exceed the FDA approved maximum recommended dose for the relevant indication. Approval duration: 3 months
      B. Other diagnoses/indications (must meet 1 or 2):

2. 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 Page 1 of 5

   CLINICAL POLICY Factor IX Complex, Human 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

3. 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. Congenital Hemophilia B (must meet all):
4. 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);
   2. Member is responding positively to therapy;
   3. Documentation of member’s current body weight (in kg);
   4. If request is for a dose increase, new dose does not exceed the FDA approved maximum recommended dose for the relevant indication. Approval duration: 3 months B. Other diagnoses/indications (must meet 1 or 2):
5. 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

6. 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 – Page 2 of 5

   CLINICAL POLICY Factor IX Complex, Human 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 Appendix C: Contraindications/Boxed Warnings None reported V. Dosage and Administration
   Indication Dosing Regimen Hemophilia B Minor to moderate bleeding episodes: 20-30 IU/kg IV every 16-24 hours Major bleeding episodes: 30-50 IU/kg IV followed by 20 IU/kg IV every 16-24 hours Surgery: 30-50 IU/kg IV prior to surgery, followed by the same dose every 16-24 hours thereafter VI. Product Availability
   Vials: 500, 1,000, 1,500 IU Maximum Dose 50 IU/kg VII.