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Hemophilia Products – von Willebrand Factor: Vonvendi®
Policy Number: PH-0344
Intravenous
Last Review Date: 06/04/2024
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, 10/2019, 02/2020, 06/2021, 03/2022, 06/2022, 06/2023, 06/2024
- 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
- Dosing Limits
A. Quantity Limit (max daily dose) [NDC Unit]:
- Vonvendi 450-850 units: 82 vials per 90-day supply
- Vonvendi 900-1700 units: 41 vials per 90-day supply
B. Max Units (per dose and over time) [HCPCS Unit]:
- 36,800 billable units per 90 day supply
- Initial Approval Criteria 1,2,8,10-12
Hemophilia Management Program |
Requirements for inhibitor tests 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. |
Coverage is provided in the following conditions:
- Patient is at least 18 years of age; AND
Von Willebrand Disease (vWD) † Ф
- Diagnosis of von Willebrand disease has been confirmed by blood coagulation and von Willebrand factor testing; AND
- Used as treatment in at least one of the following:
-
-
-
- On-demand treatment and control of bleeding episodes; AND
-
-
- Patient has severe vWD; OR
- Patient has mild or moderate vWD and the use of desmopressin is known or suspected to be ineffective or contraindicated; OR
-
- Perioperative management of bleeding (Note: Authorizations valid for 1 month); OR
- Routine prophylaxis to reduce the frequency of bleeding episodes; AND
-
- Patient has severe Type 3 vWD and is receiving on-demand therapy
Hemophilia Management Program |
For minimally treated patients (< 50 exposure days to factor products) previously receiving a different factor product, inhibitor testing is required at baseline, then at every comprehensive care visit (yearly for the mild and moderate patients, semi-annually for the severe patients) |
† FDA Approved Indication(s); ‡ Compendia Recommended Indication(s); Ф Orphan Drug
- 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.
- Renewal Criteria 1,2,8,10
Coverage may be renewed based upon the following criteria:
- Patient continues to meet the indication-specific relevant criteria identified in section III; AND
- Absence of unacceptable toxicity from the drug. Examples of unacceptable toxicity include: hypersensitivity reactions (including anaphylactic shock, generalized urticaria, angioedema, chest tightness, hypotension, shock, lethargy, nausea, vomiting, paresthesia, pruritus, restlessness, blurred vision, wheezing and/or acute respiratory distress), thromboembolic reactions (including disseminated intravascular coagulation [DIC], thromboembolism, venous thrombosis, pulmonary embolism, myocardial infarction, and stroke), 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
On-demand treatment and control of bleeding episodes
- Renewals will be approved for a 6 month authorization period
Perioperative management of surgical bleeding
- Coverage may NOT be renewed
Routine prophylaxis to reduce the frequency of bleeding episodes
- 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)
- Dosage/Administration 1
Indication |
Dose |
On-demand treatment and control of bleeding episodes VWD |
Minor (e.g., readily managed epistaxis, oral bleeding, menorrhagia):
Major (e.g., severe or refractory epistaxis, menorrhagia, GI bleeding, CNS trauma, hemarthrosis, or traumatic hemorrhage):
|
Perioperative management of bleeding VWD |
Elective Surgical Procedure A preoperative dose may be administered 12-24 hours prior to surgery to allow the endogenous factor VIII levels to increase to at least 30 IU/dL (minor surgery) or 60 IU/dL (major surgery) before the loading dose (1 hour preoperative dose) of rVWF, with or without rFVIII, is administered.
Emergency Surgery
Note: refer to the package insert for recommended VWF:RCo and FVIII:C target peak plasma levels and dosing guidelines for perioperative management of bleeding. |
Routine prophylaxis to reduce the frequency of bleeding episodes in patients with severe Type 3 VWD |
*Treat breakthrough bleeding as per the dosing guidelines for minor and major bleeding. |
- Billing Code/Availability Information
Hemophilia products are covered under the prescription drug benefits of a member’s plan. Claims for hemophilia products submitted for payment under any benefit section of the member’s plan (other than prescription drug benefits) will be denied as non-covered benefits. The only exceptions to this are claims for hemophilia products used in an inpatient facility or for emergency use, accidents or surgery (Type Services A, S, or 2) in the following settings:
If home health nursing assistance is needed for drug administration, the hemophilia product should be accessed and paid through the member’s prescription benefit coverage. Nursing services should be billed only for the administration of the hemophilia product under the member’s home health benefits. |
HCPCS Code & NDC(s):
Drug |
Manufacturer |
HCPCSCode |
1 Billable Unit Equiv. |
Vial Size |
NDC |
Vonvendi |
Takeda Pharmaceuticals USA, Inc. |
J7179 |
1 IU |
450-850 IU |
00944-7551-xx |
900-1700 IU |
00944-7553-xx |
- References
- Vonvendi [package insert]. Lexington, MA; Takeda Pharmaceuticals USA, Inc.; March 2023. Accessed May 2024.
- Guidelines for the Management of Hemophilia. 3rd Edition. World Federation of Hemophilia 2020. Available at: https://www1.wfh.org/publications/files/pdf-1863.pdf. Accessed May 2024.
- Graham A1, Jaworski K. Pharmacokinetic analysis of anti-hemophilic factor in the obese patient. Haemophilia. 2014 Mar;20(2):226-9.
- Croteau SE1, Neufeld EJ. Transition considerations for extended half-life factor products. Haemophilia. 2015 May;21(3):285-8.
- 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).
- MASAC Recommendation Concerning Prophylaxis for Hemophilia A and B with and without Inhibitors. MASAC Document #267; April 2022. Available at: https://www.bleeding.org/healthcare-professionals/guidelines-on-care/masac-documents/masac-document-267-masac-recommendation-concerning-prophylaxis-for-hemophilia-a-and-b-with-and-without-inhibitors. Accessed May 2024.
- MASAC Recommendations Regarding the Treatment of von Willebrand Disease. MASAC Document #266; March 2021. Available at: https://www.bleeding.org/healthcare-professionals/guidelines-on-care/masac-documents/masac-document-266-masac-recommendations-regarding-the-treatment-of-von-willebrand-disease. Accessed May 2024.
- Connell NT, Flood VH, Brignardello-Petersen R, et al. ASH ISTH NHF WFH 2021 guidelines on the management of von Willebrand disease. Blood Advances, Volume 5, Issue 1, 2021, Pages 301-325, https://doi.org/10.1182/bloodadvances.2020003264.
- Franchini M, Seidizadeh, Mannucci P, et al. Prophylactic management of patients with von Willebrand disease. Ther Adv Hematol. 2021; 12: 20406207211064064. Published online 2021 Dec 22. doi: 10.1177/20406207211064064.
- MASAC Recommendations Concerning Products Licensed For The Treatment Of Hemophilia And Selected Disorders of the Coagulation System. MASAC Document #284 (Replaces Document #280); April 2024. Available at https://www.bleeding.org/healthcare-professionals/guidelines-on-care/masac-documents/masac-document-284-masac-recommendations-concerning-products-licensed-for-the-treatment-of-hemophilia-and-selected-disorders-of-the-coagulation-system. Accessed May 2024.
- James PD, Connell NT, Ameer B, et al. ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease. Blood Adv. 2021 Jan 12;5(1):280-300. doi: 10.1182/bloodadvances.2020003265. PMID: 33570651; PMCID: PMC7805340.
- James P. (2023). von Willebrand disease (VWD): Treatment of minor bleeding, use of DDAVP, and routine preventive care. In Leung LLK, Tirnauer JS (Eds.), UptoDate. Last updated: Sept 11, 2023. Accessed on: May 8, 2024. Available from https://www.uptodate.com/contents/von-willebrand-disease-vwd-treatment-of-minor-bleeding-use-of-ddavp-and-routine-preventive-care?search=von%20willebrand%20disease&source=search_result&selectedTitle=4%7E150&usage_type=default&display_rank=4.
- First Coast Service Options, Inc. Local Coverage Article: Billing and Coding: Hemophilia Factor Products (A56482). Centers for Medicare & Medicaid Services Inc. Updated on 09/29/2023 with effective date 10/01/2023. Accessed May 2024.
- 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.
- 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
ICD-10 |
ICD-10 Description |
D68.01 |
Von Willebrand disease, type 1 |
D68.020 |
Von Willebrand disease, type 2A |
D68.021 |
Von Willebrand disease, type 2B |
D68.022 |
Von Willebrand disease, type 2M |
D68.023 |
Von Willebrand disease, type 2N |
D68.03 |
Von Willebrand disease, type 3 |
D68.04 |
Acquired von Willebrand disease |
D68.09 |
Other von Willebrand disease |
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.
Medicare Part B Covered Diagnosis Codes |
||
Jurisdiction |
NCD/LCA/LCD Document (s) |
Contractor |
H,L |
A56433 |
Novitas Solutions, Inc. |
J,M |
A56065 |
Palmetto GBA |
N |
A56482 |
First Coast Service Options, Inc. Insurance Corp (WPS) |
Medicare Part B Administrative Contractor (MAC) Jurisdictions |
||
Jurisdiction |
Applicable State/US Territory |
Contractor |
E (1) |
CA, HI, NV, AS, GU, CNMI |
Noridian Healthcare Solutions, LLC |
F (2 & 3) |
AK, WA, OR, ID, ND, SD, MT, WY, UT, AZ |
Noridian Healthcare Solutions, LLC |
5 |
KS, NE, IA, MO |
Wisconsin Physicians Service Insurance Corp (WPS) |
6 |
MN, WI, IL |
National Government Services, Inc. (NGS) |
H (4 & 7) |
LA, AR, MS, TX, OK, CO, NM |
Novitas Solutions, Inc. |
8 |
MI, IN |
Wisconsin Physicians Service Insurance Corp (WPS) |
N (9) |
FL, PR, VI |
First Coast Service Options, Inc. |
J (10) |
TN, GA, AL |
Palmetto GBA |
M (11) |
NC, SC, WV, VA (excluding below) |
Palmetto GBA |
L (12) |
DE, MD, PA, NJ, DC (includes Arlington & Fairfax counties and the city of Alexandria in VA) |
Novitas Solutions, Inc. |
K (13 & 14) |
NY, CT, MA, RI, VT, ME, NH |
National Government Services, Inc. (NGS) |
15 |
KY, OH
|
CGS Administrators, LLC |
VWF_HEMOPHILIA PRODUCTS - Prior Auth Criteria |
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