State and School Employees' Health Insurance Plan - Hemophilia Therapies

POLICY NUMBER

S.5.01.433

Advate (antihemophilic factor-recombinant kit)

Adynovate (antihemophilic factor-recombinant pegylated kit)

Afstyla (antihemophilic factor-recombinant, single chain)

Alphanate (antihemophilic factor/von Willebrand factor complex-human kit)

Alphanine SD (antihemophilc factor (Human)

Alprolix [antihemophilic factor (recombinant), Fc fusion protein]

BeneFIX [antihemophilic factor (recombinant)]

Coagadex [coagulation factor X (human)]

Eloctate [antihemophilic factor (Recombinant), Fc Fusion Protein]

Esperoct [antihemophilic factor (Recombinant), glycopegylated-exei]

Feiba [anti-inhibitor]

Hemofil M [antihemophilic factor (Human), Method M, monoclonal purifieds]

Hympavzi (marstacimab-hncq)

Humate-P [antihemophilic factor / von Willebrand factor complex (human)]

Idelvion [antihemophilic factor (recombinant), albumin fusion protein]

Ixinity [antihemophilic factor (recombinant)]

Jivi [antihemophilic factor (recombinant)]

Koate [antihemophilic factor (Human)]

Kogenate FS [antihemophilic factor (Recombinant)]

Kovaltry [antihemophilic factor (Recombinant)]

NovoEight [antihemophilic factor (Recombinant)]

Nuwiq [antihemophilic factor (Recombinant)]

Obizur [antihemophilic factor (Recombinant)]

Profilnine SD [antihemophilic complex (human)]

Qfitlia (fitusiran)

Rebinyn [antihemophilic factor (recombinant), GlycoPEGylated]

Recombinate [antihemophilic factor (Recombinant)

Rixubis [antihemophilic factor (recombinant)]

Roctavian (valoctocogene roxaparvovec-rvox)

Sevenfact [antihemophilic factor (recombinant)]

Vonvendi [von Willebrand factor complex (recombinant)]

Wilate [antihemophilic complex (human)]

Xyntha [antihemophilic factor (Recombinant)]

Please perform a search of the State Health Plan Medical Drug Formulary for drugs administered and billed through the medical setting.

DESCRIPTION

Hemophilia is a rare bleeding disorder in which the blood does not clot normally. Hemophilia is usually inherited and caused by a defect in one of the genes that determine how the body makes blood clotting factor VIII or IX. Rarely, hemophilia can be acquired. This can happen if the body forms antibodies that attack the clotting factors in the bloodstream, preventing the clotting factors from working. People born with hemophilia have little or no clotting factor, which is a protein needed for normal blood clotting. These proteins work with platelets, which are small blood cell fragments that form in the bone marrow, to help the blood clot. When blood vessels are injured, clotting factors help platelets stick together to plug cuts and breaks on the vessels and stop bleeding.

The two main types of hemophilia are A and B. People with hemophilia A, also called Classic hemophilia or Factor VIII deficiency, are missing or have low levels of clotting factor VIII. About 8 out of 10 people with hemophilia have type A. People with hemophilia B, also called Christmas disease or Factor IX deficiency, are missing or have low levels of clotting factor IX.

Factor X (ten) deficiency, also known as Stuart-Prower Factor Deficiency, is a disorder caused by a lack of protein called factor X in the blood. The factor X protein plays an important role in activating the enzymes that help to form a clot. Deficiency of this protein leads to problems with coagulation. The incidence of Factor X deficiency is estimated at 1 in 500,000 to 1 in a million.

Hemophilia can be mild, moderate, or severe, depending on how much clotting factor is in the blood. About 7 out of 10 people who have hemophilia A have the severe form of the disorder. People who do not have hemophilia have a factor VIII activity of 100 percent. People who have severe hemophilia A have a factor VIII activity of less than 1 percent. Hemophilia usually occurs in males. About 1 in 5,000 males are born with hemophilia each year.

The main treatment for hemophilia is called replacement therapy. Concentrates of clotting factor are slowly dripped or injected into a vein. Clotting factor concentrates can be made from human blood or recombinant clotting factors. Replacement therapy can be preventive/prophylactic or used on an as-needed basis called demand therapy.

POLICY

The use of samples by an individual will not be considered current or stable therapy for purposes of Medical Policy review.

InitialEvaluation

Coverage for the requested medication(s) for the immediate treatment of Hemophilia may be considered medically necessary when ALL of the following criteria are met::

1. ALL of the following:

2. 1. The individual has a diagnosis of one of the following:

   2. 1. Hemophilia A;

      2. Hemophilia B/Factor IX deficiency;

      3. Factor X deficiency/Stuart-Prower Factor deficiency;

      4. Von Willebrand disease;

   3. The individual is currently bleeding;

   4. The individual is out of medication (need immediate use);

3. The individual does not have any FDA labeled contraindication(s) to therapy with the requested agent;

4. The prescriber must provide the actual prescribed dose with ALL of the following supporting documentation:

5. 1. Individual’s age;

   2. Individual’s weight;

   3. Severity of the factor deficiency (i.e., severe= <1% factor activity, moderate=≥1 to ≤5% factor activity, mild= >5 to 40% factor activity);

   4. Activity level;

   5. Bleed history;

   6. Current and desired trough level;

   7. Inhibitor status;

6. The quantity (number of doses) requested is appropriate based on submitted supportive documentation and ONE of the following:

7. 1. The dose is within the FDA labeled dosing;

   2. The prescriber has provided clinical reasoning with supportive documentation for the higher dosing.

Length of Approval:

• Immediate Use: Based upon request

Esperoct, Hympavzi, Jivi, Qfitlia, and Roctavian are considered not medically necessary as there are other alternatives covered by the plan for the treatment of Hemophilia A and Hemophilia B.

Services related to delivery and/or administration of a medication determined to be not medically necessary will also be considered not medically necessary.

POLICY EXCEPTIONS

State Health Plan (State and School Employees): Self-administered drugs may be covered under a prescription drug benefit plan administered by the State Health Plan’s Pharmacy Benefit Manager. Please perform a formulary drug search at https://www.dfa.ms.gov/cvs-caremark and submit any required Prior Authorization Requests for coverage determination to the Plan’s Pharmacy Benefit Manager. Services related to delivery and/or administration of a self-administered drug are not covered under the medical benefit.

POLICY GUIDELINES

Medical Policy Manual coverage guidelines should not be used in lieu of the Participant's specific benefit plan language outlined in the Mississippi's State and School Employees’ Life and Health Insurance Plan.

Medically Necessary is defined as those services, treatments, procedures, equipment, drugs, devices, items or supplies furnished by a covered Provider that are required to identify or treat a Participant's illness, injury or Mental Health Disorders, and which Company determines are covered under this Benefit Plan based on the criteria as follows in A through D:

A. consistent with the symptoms or diagnosis and treatment of the Participant's condition, illness, or injury; and

B. appropriate with regard to standards of good medical practice; and

C. not solely for the convenience of the Participant, his or her Provider; and

D. the most appropriate supply or level of care which can safely be provided to the Participant. When applied to the care of an Inpatient, it further means that services for the Participant's medical symptoms or conditions require that the services cannot be safely provided to the Participant as an Outpatient.

For the definition of medical necessity, “standards of good medical practice” means standards that are based on credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, and physician specialty society recommendations, and the views of medical practitioners practicing in relevant clinical areas and any other relevant factors. BCBSMS makes no payment for services, treatments, procedures, equipment, drugs, devices, items or supplies which are not documented to be Medically Necessary. The fact that a Physician or other Provider has prescribed, ordered, recommended, or approved a service or supply does not in itself, make it Medically Necessary.

Investigative is defined as the use of any treatment procedure, facility, equipment, drug, device, or supply not yet recognized as a generally accepted standard of good medical practice for the treatment of the condition being treated and; therefore, is not considered medically necessary. For the definition of Investigative, “generally accepted standards of medical practice” means standards that are based on credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, and physician specialty society recommendations, and the views of medical practitioners practicing in relevant clinical areas and any other relevant factors. In order for equipment, devices, drugs or supplies [i.e, technologies], to be considered not investigative, the technology must have final approval from the appropriate governmental bodies, and scientific evidence must permit conclusions concerning the effect of the technology on health outcomes, and the technology must improve the net health outcome, and the technology must be as beneficial as any established alternative and the improvement must be attainable outside the testing/investigational setting.

POLICY HISTORY

07/01/2023: New policy added.

07/06/2023: Code Reference section updated to add new HCPCS code J7213, effective 07/01/2023.

04/01/2024: Policy updated to state that Roctavian is considered not medically necessary as there are other alternatives covered by the plan for the treatment of Hemophilia A. Sources updated. Code Reference section updated to add HCPCS code J1412 as not medically necessary.

06/27/2024: Policy revised to update Length of Approval.

06/03/2025: Policy reviewed by Pharmacy & Therapeutics (P&T) Committee; no changes.

09/15/2025: Policy updated to add Hympavzi as not medically necessary as there are other alternatives covered by the plan for the treatment of Hemophilia A and Hemophilia B. Sources updated. Code Reference section updated to add HCPCS code J7172 to the Not Medically Necessary Codes table.

11/01/2025: Policy statement updated to add Qfitlia as not medically necessary as there are other alternatives covered by the plan for the treatment of Hemophilia A and Hemophilia B. Sources updated. Code Reference section updated to add new HCPCS code J7174 to the Not Medically Necessary Codes table.

SOURCE(S)

1. Advate prescribing information. Takeda Pharmaceuticals America, Inc. April 2023. Last accessed June 2023.

2. Adynovate prescribing information. Takeda Pharmaceuticals America, Inc. April 2023. Last accessed June 2023.

3. Afstyla prescribing information. CSL Behring Lengnau AG. June 2021. Last accessed June 2023.

4. Alphanate prescribing information. Grifols USA, LLC. March 2023. Last accessed June 2023.

5. AlphaNine SD prescribing information. Grifols USA, LLC. December 2021. Last accessed June 2023.

6. Alprolix prescribing information. Bioverativ Therapeutics, Inc. May 2023. Last accessed June 2023.

7. BeneFix prescribing information. Wyeth BioPharma Division of Wyeth Pharmaceuticals, LLC. March 2023. Last accessed June 2023.

8. Coagadex prescribing information. Bio Products Laboratory Limited. April 2023. Last accessed June 2023.

9. Eloctate prescribing information. Bioverativ Therapeutics, Inc. May 2023. Last accessed June 2023.

10. Esperoct prescribing information. Novo Nordisk. August 2022. Last accessed June 2023.

11. Feiba prescribing information. Takeda Pharmaceuticals America, Inc. May 2023. June 2023.

12. Hemofil M prescribing information. Takeda Pharmaceuticals America, Inc. March 2023. Last accessed June 2023.

13. Hympavzi prescribing information. Pfizer Laboratories Div Pfizer Inc. February 2025. Last accessed September 2025.

14. Humate P prescribing information. CSL Behring GmbH. August 2020. Last accessed June 2023.

15. Idelvion prescribing information. CSL Behring Lengnau AG. July 2021. Last accessed June 2023.

16. Ixinity prescribing information. Medexus Pharma, Inc. December 2022. Last accessed June 2023.

17. Jivi prescribing information. Bayer HealthCare LLC. August 2018. Last accessed June 2023.

18. Koate prescribing information. Kedrion Biopharma, Inc. April 2022. Last accessed June 2023.

19. Kogenate FS prescribing information. Bayer HealthCare, LLC. December 2019. Last accessed June 2023.

20. Kovaltry prescribing information. Bayer HealthCare, LLC. December 2021. Last accessed June 2023.

21. National Heart, Lung, and Blood Institute

    http://www.nhlbi.nih.gov/health/health-topics/topics/hemophilia/

22. Novoeight prescribing information. Novo Nordisk. July 2020. Last accessed June 2023.

23. Nuwiq prescribing information. Octapharma USA, Inc. June 2021. Last accessed June 2023.

24. Obizur prescribing information. Takeda Pharmaceuticals America, Inc. March 2023. Last accessed June 2023.

25. Profilnine prescribing information. Grifols USA, LLC. December 2021. Last accessed June 2023.

26. Qfitlia prescribing information. Genzyme Corporation. September 2025. Last accessed October 2025.

27. Rebinyn prescribing information. Novo Nordisk. August 2022. Last accessed June 2023.

28. Recombinate prescribing information. Takeda Pharmaceuticals America, Inc. March 2023. Last accessed June 2023.

29. Rixubis prescribing information. Takeda Pharmaceuticals America, Inc. March 2023. Last accessed June 2023.

30. Roctavian prescribing information. BioMarin Pharmaceutical Inc. March 2024. Last accessed March 2024.

31. Sevenfact prescribing information. Laboratoire Français du Fractionnement et des Biotechnologies Société Anonyme (LFB S.A.). November 2022. Last accessed June 2023.

32. Vonvendi prescribing information. Takeda Pharmaceuticals America, Inc. April 2023. Last accessed June 2023.

33. Wilate prescribing information. Octapharma USA, Inc. November 2019. Last accessed June 2023.

34. Xyntha prescribing information. Wyeth BioPharma Division of Wyeth Pharmaceuticals, LLC. March 2023. Last accessed June 2023.

CODE REFERENCE

This may not be a comprehensive list of procedure codes applicable to this policy.

The code(s) listed below are ONLY medically necessary if the procedure is performed according to the "Policy" section of this document.

Covered Codes for Factor VIII        

Covered Codes for Factor IX

Covered Codes for Factor X

 Not Medically Necessary Codes

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