Last Review Date: 07/02/2024

Date of Origin: 12/16/2014

Dates Reviewed: 12/2014, 04/2015, 05/2015, 09/2015, 12/2015, 03/2016, 06/2016, 12/2016, 06/2017, 09/2017, 11/2017, 11/2018, 03/2019, 02/2020, 05/2020, 01/2021, 06/2021, 06/2022, 06/2023, 06/2024, 07/2024

1. Length of Authorization

Coverage is provided for 3 months and may be renewed thereafter, unless otherwise specified*.

Note: The cumulative amount of medication the patient has on-hand will be taken into account for authorizations. Up to 5 ‘on-hand’ doses for the treatment of acute bleeding episodes will be permitted at the time of the authorization request.

* Initial and renewal authorization periods may vary by specific covered indication

2. Dosing Limits

1. Quantity Limit (max daily dose) [NDC Unit]:

• NovoSeven RT 1000 mcg vial = 12 vials per 30 days
• NovoSeven RT 2000 mcg vial = 12 vials per 30 days
• NovoSeven RT 5000 mcg vial = 24 vials per 30 days
• NovoSeven RT 8000 mcg vial = 15 vials per 30 days
• Sevenfact 1 mg vial = 48 vials per 30 days
• Sevenfact 2 mg vial = 12 vials per 30 days
• Sevenfact 5 mg vial = 24 vials per 30 days

2. Max Units (per dose and over time) [HCPCS Unit]:

• 120,000 billable units per 30-day supply

3. Initial Approval Criteria ^1-4,9,10

Coverage is provided in the following conditions:

NovoSeven RT ONLY ¹

Hemophilia A (congenital factor VIII deficiency) † Ф

• Diagnosis of congenital factor VIII deficiency has been confirmed by blood coagulation testing; AND
• Patient has inhibitors to Factor VIII with a current or historical titer of ≥ 5 Bethesda Units (BU)**; AND
• Used as treatment in at least one of the following:

• Treatment and control of acute bleeding episodes (episodic treatment of acute hemorrhage); OR
• Perioperative management (*Authorizations valid for 1 month) ; OR
• Routine prophylaxis to prevent or reduce the frequency of bleeding episodes when the following criteria are also met:
  • Used as primary prophylaxis in patients with severe factor VIII deficiency (factor VIII level of <1%); OR

• Used as secondary prophylaxis in patients with at least TWO documented episodes of spontaneous bleeding into joints; OR
• Patient has documented trial and failure of Immune Tolerance Induction (ITI); AND
  • Patient has documented trial and failure or contraindication to Hemlibra

Acquired Hemophilia †

• Diagnosis of acquired hemophilia has been confirmed by blood coagulation testing; AND
• Used as treatment for one of the following:
  • Treatment and control of acute bleeding episodes (episodic treatment of acute hemorrhage); OR
  • Perioperative management (*Authorizations valid for 1 month) 

Hemophilia B (congenital factor IX deficiency aka Christmas disease) † Ф

• Diagnosis of congenital factor IX deficiency has been confirmed by blood coagulation testing; AND
• Patient has inhibitors to Factor IX with a current or historical titer of ≥ 5 Bethesda Units (BU)**; AND
• Used as treatment for one of the following:

• Treatment and control of acute bleeding episodes (episodic treatment of acute hemorrhage); OR
• Perioperative management (*Authorizations valid for 1 month); OR
• Routine prophylaxis to prevent or reduce the frequency of bleeding episodes when the following criteria are also met:
  • Used as primary prophylaxis in patients with severe factor IX deficiency (factor IX level of <1%); OR
  • Used as secondary prophylaxis in patients with at least TWO documented episodes of spontaneous bleeding into joints; OR
  • Patient has documented trial and failure of Immune Tolerance Induction (ITI)

Congenital Factor VII Deficiency † Ф

• Diagnosis of congenital factor VII deficiency has been confirmed by blood coagulation testing; AND
• Used as treatment for one of the following:

• Treatment and control of acute bleeding episodes (episodic treatment of acute hemorrhage); OR
• Perioperative management(*Authorizations valid for 1 month)

Glanzmann’s Thrombasthenia † Ф

• Diagnosis of Glanzmann Thrombasthenia has been confirmed by blood coagulation testing; AND
• Used as treatment for one of the following:

• Treatment and control of acute bleeding episodes (episodic treatment of acute hemorrhage); OR
• Perioperative management (*Authorizations valid for 1 month); AND

• The use of platelet transfusions is known or suspected to be ineffective or contraindicated

Sevenfact ONLY ²

Hemophilia A (Congenital Factor VIII Deficiency)/Hemophilia B (Congenital Factor IX Deficiency) †

• Patient is at least 12 years of age; AND
• Diagnosis of congenital factor VIII or IX deficiency has been confirmed by blood coagulation testing; AND
• Patient has Hemophilia A (Factor VIII) inhibitors or Hemophilia B (Factor IX) inhibitors with a current or historical titer of ≥ 5 Bethesda Units (BU)**; AND
• Used as treatment and control of acute bleeding episodes (episodic treatment of acute hemorrhage); AND

• Will not be used for the treatment of Congenital Factor VII Deficiency

**Note: Patients with inhibitor titer levels >0.6 BU to <5 BU who are not responding to or are not a candidate for standard factor replacement, will be evaluated on a case-by-case basis.

† FDA Approved Indication(s); ‡ Compendia Recommended Indication(s); Ф Orphan Drug

4. Dispensing Requirements for Rendering Providers (Hemophilia Management Program)

•  Prescriptions cannot be filled without an expressed need from the patient, caregiver or prescribing practitioner. Auto-filling is not allowed.
• Monthly, rendering provider must submit for authorization of dispensing quantity before delivering factor product. Information submitted must include:
  • Original prescription information, requested amount to be dispensed, vial sizes available to be ordered from the manufacturer, and patient clinical history (including patient product inventory and bleed history)
  • Factor dose should not exceed +1% of the prescribed dose and a maximum of three vials may be dispensed per dose. If unable to provide factor dosing within the required threshold, below the required threshold, the lowest possible dose able to be achieved above +1% should be dispensed. Prescribed dose should not be increased to meet assay management requirements.
• The cumulative amount of medication(s) the patient has on-hand should be taken into account when dispensing factor product. Patients should not have more than 5 extra doses on-hand for the treatment of acute bleeding episodes.
• Dispensing requirements for renderings providers are a part of the hemophilia management program. This information is not meant to replace clinical decision making when initiating or modifying medication therapy and should only be used as a guide.

5. Renewal Criteria ^1-4,9-10

Coverage can be renewed based upon the following criteria:

• Patient continues to meet the indication-specific relevant criteria such as concomitant therapy requirements (not including prerequisite therapy), performance status, etc. identified in section III; AND
• Absence of unacceptable toxicity from the drug. Examples of unacceptable toxicity include:  hypersensitivity reactions including anaphylaxis (e.g., hives, itching, rash, difficulty breathing, swelling around the mouth/throat, chest tightness, wheezing, dizziness/fainting, low blood pressure, etc.), serious arterial and venous thrombotic events, development of neutralizing antibodies (inhibitors), etc.; AND
• Any increases in dose must be supported by an acceptable clinical rationale (i.e., weight gain, half-life study results, increase in breakthrough bleeding when patient is fully adherent to therapy, etc.); AND
• The cumulative amount of medication(s) the patient has on-hand will be taken into account when authorizing. The authorization will allow up to 5 doses on-hand for the treatment of acute bleeding episodes as needed for the duration of the authorization; AND

Treatment and control of acute bleeding episodes (NovoSeven RT/Sevenfact)

• Renewals will be approved for a 6-month authorization period

Perioperative management of bleeding (NovoSeven RT Only)

• Coverage may NOT be renewed

Routine prophylaxis to prevent or reduce the frequency of bleeding episodes (NovoSeven RT Only)

• Renewals will be approved for a 12-month authorization period; AND
• Patient has demonstrated a beneficial response to therapy (i.e., the frequency of bleeding episodes has decreased from pre-treatment baseline)

6. Dosage/Administration ^1-4

7. Billing Code/Availability Information

HCPCS Code(s) & NDC(s):

8. References

1. NovoSeven RT [package insert]. Bagsvaerd, Denmark; Novo Nordisk; July 2020. Accessed May 2024.
2. Sevenfact [package insert]. Puteaux, France; LFB S.A., June 2024. Accessed June 2024.
3. MASAC Recommendations Concerning Products Licensed For The Treatment Of Hemophilia And Selected Disorders of the Coagulation System. Revised April 11, 2024. National Hemophilia Foundation. MASAC Document #284; April 2024. Available at: https://www.bleeding.org/. Accessed May 2024.
4. Guidelines for the Management of Hemophilia. 3^rd Edition. World Federation of Hemophilia. 2020. Available at: https://www1.wfh.org/publications/files/pdf-1863.pdf.  Accessed May 2024.
5. Annual Review of Factor Replacement Products. Oklahoma Health Care Authority Review Board. Updated April 2016. Accessed May 2024.
6. Graham A1, Jaworski K. Pharmacokinetic analysis of anti-hemophilic factor in the obese patient. Haemophilia. 2014 Mar;20(2):226-9.
7. Croteau SE1, Neufeld EJ. Transition considerations for extended half-life factor products. Haemophilia. 2015 May;21(3):285-8.
8. Mingot-Castellano, et al. Application of Pharmacokinetics Programs in Optimization of Haemostatic Treatment in Severe Hemophilia a Patients: Changes in Consumption, Clinical Outcomes and Quality of Life. Blood. 2014 December; 124 (21).
9. MASAC Recommendation Concerning Prophylaxis for Hemophilia A and B with and without Inhibitors. Revised April 27, 2022. National Hemophilia Foundation. MASAC Document #267; April 2022. Available at: http://www.bleeding.org/. Accessed May 2024.
10. Hoots, WK. (2024). Hemophilia A and B: Routine management including prophylaxis. In Leung LLK, Tirnauer JS (Eds.), UptoDate. Last updated: April 16, 2024. Accessed May 13, 2024. Available from https://www.uptodate.com/contents/hemophilia-a-and-b-routine-management-including-prophylaxis?search=hemophilia%20a&source=search_result&selectedTitle=2~150&usage_type=default&display_rank=2#H978189854
11. Rayment R, Chalmers E, Forsyth K, et al. Guidelines on the use of prophylactic factor replacement for children and adults with Haemophilia A and B. B J Haem:190;5, Sep2020. https://onlinelibrary.wiley.com/doi/10.1111/bjh.16704. Accessed May 2024.
12. First Coast Service Options, Inc. Local Coverage Article: Billing and Coding: Hemophilia Clotting Factors (A56482). Centers for Medicare & Medicaid Services Inc. Updated on 09/29/2023 with effective date 10/01/2023. Accessed May 2024.
13. Palmetto GBA. Local Coverage Article: Billing and Coding: Guidance for Anti-Inhibitor Coagulant Complex (AICC) National Coverage Determination (NCD) 110.3 (A56065). Centers for Medicare & Medicaid Services Inc. Updated on 11/14/2022 with effective date 11/24/2022. Accessed May 2024.
14. Novitas Solutions, Inc. Local Coverage Article: Billing and Coding: Hemophilia Factor Products (A56433). Centers for Medicare & Medicaid Services Inc. Updated on 09/29/2023 with effective date 10/01/2023. Accessed May 2024.

Appendix 1 – Covered Diagnosis Codes

Appendix 2 – Centers for Medicare and Medicaid Services (CMS)

The preceding information is intended for non-Medicare coverage determinations. Medicare coverage for outpatient (Part B) drugs is outlined in the Medicare Benefit Policy Manual (Pub. 100-2), Chapter 15, §50 Drugs and Biologicals. In addition, National Coverage Determinations (NCDs) and/or Local Coverage Determinations (LCDs) may exist and compliance with these policies is required where applicable. Local Coverage Articles (LCAs) may also exist for claims payment purposes or to clarify benefit eligibility under Part B for drugs which may be self-administered. The following link may be used to search for NCD, LCD, or LCA documents: https://www.cms.gov/medicare-coverage-database/search.aspx. Additional indications, including any preceding information, may be applied at the discretion of the health plan.