Subject: Factor VIII 1937-A, 1938-A, 1946-A, 1939-A, 1945-A, 2688-A SGM P2024

Policy:

      I.  INDICATIONS
 
          The indications below including FDA-approved indications and compendial uses are
          considered a covered benefit provided that all the approval criteria are met and the member
          has no exclusions to the prescribed therapy.

                                                             

  Table: Factor VIII Concentrates and Covered Uses

Brand	Generic	FDA-Approved Indication(s)	Compendial Indication(s)
Recombinant Factor VIII Concentrates
Advate	antihemophilic factor [recombinant]	Hemophilia A	Acquired Hemophilia A
Afstyla	antihemophilic factor [recombinant], single chain	Hemophilia A
Kogenate FS	antihemophilic factor [recombinant]	Hemophilia A	Acquired Hemophilia A
Kovaltry	antihemophilic factor [recombinant]	Hemophilia A
Novoeight	antihemophilic factor [recombinant]	Hemophilia A	Acquired Hemophilia A
Nuwiq	antihemophilic factor [recombinant]	Hemophilia A
Recombinate	antihemophilic factor [recombinant]	Hemophilia A	Acquired Hemophilia A
Xyntha	antihemophilic factor [recombinant]	Hemophilia A	Acquired Hemophilia A
Extended Half-life Recombinant Factor VIII Concentrates
Adynovate	antihemophilic factor [recombinant], PEGylated	Hemophilia A
Altuviiio	antihemophilic factor [recombinant], Fc-VWF-XTEN    fusion protein-ehtl	Hemophilia A
Eloctate	antihemophilic factor [recombinant], Fc fusion protein	Hemophilia A
Jivi	antihemophilic factor [recombinant], PEGylated-aucl	Hemophilia A
Esperoct	antihemophilic factor [recombinant],   Glycopegylated-exei	Hemophilia A
Human Plasma-Derived Factor VIII Concentrate
Hemofil M	antihemophilic factor [human] monoclonal antibody purified	Hemophilia A	Acquired Hemophilia A
Human Plasma-Derived Factor VIII Concentrates That Contain Von Willebrand Factor
Alphanate      Humate-P	antihemophilic factor/von Willebrand factor complex    [human]	Hemophilia A, von Willebrand    Disease	Acquired Hemophilia A, Acquired von   Willebrand Syndrome
Koate	antihemophilic factor [human]	Hemophilia A	Acquired Hemophilia A, von Willebrand   Disease

  All other indications are considered experimental/investigational and not medically necessary.


     II.  PRESCRIBER SPECIALTIES
          Must be prescribed by or in consultation with a hematologist.


   III.  CRITERIA FOR INITIAL APPROVAL

          A.  Hemophilia A
               Authorization of 12 months of Advate, Adynovate, Afstyla, Alphanate, Altuviiio, Eloctate,
               Esperoct, Hemofil M, Humate-P, Koate, Kogenate FS, Kovaltry, Novoeight, Nuwiq,
               Recombinate, or Xyntha may be granted for treatment of hemophilia A when either of the
               following criteria is met:
               1.  Member has mild disease (see Appendix A) and has had an insufficient response to
                    desmopressin or a documented clinical reason for not using desmopressin (see
                    Appendix B).
               2.  Member has moderate or severe disease (see Appendix A).

               Authorization of 12 months of Jivi may be granted for treatment of hemophilia A when BOTH
               of the following criteria are met:
               1.  Member has previously received treatment for hemophilia A with a factor VIII product.
               2.  Member is ≥ 12 years of age.

          B.  Von Willebrand Disease (VWD)
               Authorization of 12 months of Alphanate, Humate-P, or Koate may be granted for treatment
               of VWD when any of the following criteria is met:
               1.  Member has type 1, 2A, 2M, or 2N VWD and has had an insufficient response to
                    desmopressin or a documented clinical reason for not using desmopressin (see
                    Appendix B).
               2.  Member has type 2B or type 3 VWD.

          C.  Acquired Hemophilia A
               Authorization of 12 months of Advate, Alphanate, Hemofil M, Humate-P, Koate, Kogenate
               FS, Novoeight, Recombinate, or Xyntha may be granted for treatment of acquired
               hemophilia A.

          D.  Acquired von Willebrand Syndrome
               Authorization of 12 months of Alphanate or Humate-P may be granted for treatment of
               acquired von Willebrand syndrome.


   IV.  CONTINUATION OF THERAPY

          Authorization of 12 months may be granted for continued treatment in members requesting
          reauthorization for an indication listed in Section III when the member is experiencing benefit
          from therapy (e.g., reduced frequency or severity of bleeds).


     V.  APPENDICES

          Appendix A: Classification of Hemophilia by Clotting Factor Level (% Activity) and Bleeding Episodes

Severity	Clotting Factor Level % activity*	Bleeding Episodes
Severe	<1%	Spontaneous bleeding episodes, predominantly into joints and muscles    Severe bleeding with trauma, injury or surgery
Moderate	1% to 5%	Occasional spontaneous bleeding episodes    Severe bleeding with trauma, injury or surgery
Mild	6% to 40%	Severe bleeding with serious injury, trauma or surgery

*Factor assay levels are required to determine the diagnosis and are of value in monitoring treatment response.

 

Appendix B: Clinical Reasons For Not Utilizing Desmopressin in Patients with Hemophilia A and Type 1, 2A, 2M and 2N VWD

1. Age < 2 years
2. Pregnancy
3. Fluid/electrolyte imbalance
4. High risk for cardiovascular or cerebrovascular disease (especially the elderly)
5. Predisposition to thrombus formation
6. Trauma requiring surgery
7. Life-threatening bleed
8. Contraindication or intolerance to desmopressin
9. Severe type 1 von Willebrand disease
10. Stimate Nasal Spray is unavailable due to backorder/shortage issues (where applicable)

 

Place of Service:

Outpatient

The above policy is based on the following references:

1. Advate [package insert]. Lexington, MA: Takeda Pharmaceuticals U.S.A., Inc.; March 2023.
2. Jivi [package insert]. Whippany, NJ: Bayer HealthCare LLC; August 2018.
3. Kogenate FS [package insert]. Whippany, NJ: Bayer HealthCare LLC; May 2016.
4. Kogenate FS with BIO-SET [package insert]. Whippany, NJ: Bayer HealthCare LLC; May 2016.
5. Kogenate FS with Vial Adapter [package insert]. Whippany, NJ: Bayer HealthCare LLC; December 2019.
6. Kovaltry [package insert]. Whippany, NJ: Bayer Healthcare LLC; December 2022.
7. Novoeight [package insert]. Plainsboro, NJ: Novo Nordisk Inc., July 2020.
8. Nuwiq [package insert]. Paramus, NJ: Octapharma USA, Inc., June 2021.

Copyright Aetna Inc. All rights reserved. Pharmacy Clinical Policy Bulletins are developed by Aetna to assist in administering plan benefits and constitute neither offers of coverage nor medical advice. This Clinical Policy Bulletin contains only a partial, general description of plan or program benefits and does not constitute a contract. Aetna does not provide health care services and, therefore, cannot guarantee any results or outcomes. Participating providers are independent contractors in private practice and are neither employees nor agents of Aetna or its affiliates. Treating providers are solely responsible for medical advice and treatment of members. This Clinical Policy Bulletin may be updated and therefore is subject to change.

April 17, 2024