CurrentNeighborhood Health Plan of Rhode IslandPolicy N/A

Vonvendi (intravenous von Willebrand factor) coverage

Policy governs coverage, authorization, dispensing, dosing, and renewal criteria for Vonvendi (intravenous recombinant von Willebrand factor) for members of Medicaid, Commercial, and Medicare-Medicaid Plan populations.

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyVonvendi (intravenous von Willebrand factor) coverage

Policy CodePolicy N/A

Change TypeNo material changes

Effective Date01/01/2020

Next Review DateN/A

Key ActionObtain prior authorization (initial auth typically for 3 months) and confirm ICD-10 diagnosis and indication per policy.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

3 monthstypical initial authorization period

82pharmacy qty limit (450-850 U)

41pharmacy qty limit (900-1700 U)

36,800medical-benefit max units /90d

J7179HCPCS J-code

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ICD-10 covered codes

Coverage & Medical Necessity Criteria

Initial Therapy — Covered when ALL of the following are met

Covered when ALL of the following are met

vWD coverage general criteria

Indications: On-demand treatment and control of bleeding episodes; OR Perioperative management of bleeding; OR Routine prophylaxis to reduce the frequency of bleeding episodes.
Severity / prior therapy requirements: Patient has severe vWD; OR patient has mild or moderate vWD and desmopressin is known or suspected to be ineffective or contraindicated; OR routine prophylaxis limited to severe Type 3 vWD receiving on-demand therapy.

Renewal Criteria

Renewal criteria depending on indication

Renewal - on-demand: Patient continues to meet the indication-specific relevant criteria identified in initial coverage; AND absence of unacceptable toxicity (examples include serious hypersensitivity, thromboembolic events, development of neutralizing antibodies); AND cumulative on-hand doses accounted for (authorization may allow up to 5 doses on-hand).

Renewals for on-demand treatment will be approved for a 6-month authorization period.

Renewal - perioperative: Perioperative management coverage may NOT be renewed.

Renewal - prophylaxis: Renewals for routine prophylaxis will be approved for a 6-month authorization period; AND patient has demonstrated beneficial response to therapy (for example, decreased frequency of bleeding episodes from pre-treatment baseline).

Dosing and Administration

Dosing and administration criteria

On-demand dosing: Administer Vonvendi for each bleeding episode. If baseline FVIII:C is <40% or unknown, administer an approved recombinant (non-vWF) factor VIII (rFVIII) with Vonvendi; administer the complete dose of Vonvendi followed by rFVIII within 10 minutes. Dose calculations may use an approximate mean recovery of 2 (IU/dL)/(IU/kg).

Loading and maintenance dosing: Typical loading dose: 40-50 IU/kg; maintenance: 40-50 IU/kg every 8-24 hours as clinically required for minor bleeding. For major or severe bleeding: loading dose 50-80 IU/kg; maintenance 40-60 IU/kg every 8-24 hours as clinically required.

Refer to the package insert for complete guidance.

Perioperative dosing: For elective surgery a preoperative dose may be administered 12-24 hours before surgery to allow endogenous FVIII to rise to target levels; assess baseline FVIII:C and VWF:RCo and measure levels within 3 hours before procedure. If baseline FVIII:C meets target (>=30 IU/dL minor; >=60 IU/dL major), a dose of Vonvendi alone may be given within 1 hour of the procedure; if below target, administer Vonvendi followed by rFVIII within 10 minutes. If preoperative dosing is not feasible, use emergency dosing guidance and calculate loading dose using incremental recovery (IR), assuming IR ~2.0 IU/dL per IU/kg if IR is unknown.

Coverage Criteria

Covered when the member has one of the listed von Willebrand disease diagnoses and billing follows payer/CMS coding guidance referenced in the policy references.

Primary coverage condition: Member has an ICD-10 diagnosis code for von Willebrand disease listed in Appendix 1 (D68.01, D68.020, D68.021, D68.022, D68.023, D68.03, D68.04, D68.09).

Providers should ensure appropriate diagnosis coding per payer and follow referenced CMS local coverage/billing guidance.

Perioperative management of surgical bleeding is a covered indication for Vonvendi, but coverage for perioperative management may NOT be renewed. Providers should plan authorizations accordingly and obtain a new authorization for separate perioperative events as needed.

No explicit policy exclusions are listed in the provided excerpts. Refer to the full policy and referenced guidance documents for any exclusions not captured here.

If a patient experiences unacceptable toxicitynot medically necessary. Such events should prompt treatment reassessment and documentation to support any change in therapy or discontinuation.

Within the provided excerpts there are no additional explicit conditions described that would be categorized as 'not medically necessary.' Providers should follow the policy's stated criteria and the cited clinical guidance for circumstances requiring treatment discontinuation or denial.

Billing, Codes & Target Levels

HCPCS & NDCmixed

J7179	Vonvendi, billable unit = 1 IU
NDC 00944-7553	Vonvendi NDC

Vial sizes (informational)neutral

450-850 units	Vonvendi vial size
900-1700 units	Vonvendi vial size

Covered ICD-10 Diagnosis CodesICD-10Covered

D68.01	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	Willebrand disease, type 2N
D68.03	Von Willebrand disease, type 3
D68.04	Acquired von Willebrand disease
D68.09	Other von Willebrand disease

Target FVIII:C levels for surgery

Minor surgery target FVIII:C≥30 IU/dL

Major surgery target FVIII:C≥60 IU/dL

Perioperative measurement timingAssess FVIII:C within 3 hours before surgery and consider a 12–24 hour preoperative Vonvendi dose if needed

Authorization, Documentation & Billing Actions for Providers

Prior Authorization

Prior Authorization Required

Prior authorization is required for Vonvendi therapy. Initial authorizations are generally provided for 3 months (may vary by indication). Coverage requires the member be ≥18 years of age with confirmed von Willebrand disease and meeting indication‑specific criteria. Authorizations will consider the cumulative on‑hand medication (up to 5 doses for acute episodes may be permitted).

Initial auth generally provided for 3 months (may vary by indication)
Member must be ≥18 years of age
Confirmed diagnosis of von Willebrand disease required
Authorization considers on‑hand supply (up to 5 acute doses allowed)

Documentation Required

Diagnosis Coding Requirement

Coverage is tied to specific ICD‑10 diagnosis codes for von Willebrand disease. Ensure the patient’s claim uses an appropriate covered diagnosis code from the policy appendix.

Key Definitions and Clinical Terms

Severe Type 3 vWD

DefinitionSevere Type 3 vWD: a form of von Willebrand disease characterized by very low or absent VWF; identified in the policy as qualifying for routine prophylaxis and specific on‑demand therapy dosing considerations.

Prophylaxis eligibilityRoutine prophylaxis initiation specified for severe Type 3 VWD: 40–60 IU/kg twice weekly, may increase to 60 IU/kg twice weekly for breakthrough bleeding.

On‑demand treatment notePatients with severe Type 3 vWD receiving on‑demand therapy are included in coverage criteria and dosing guidance; may require concurrent rFVIII if baseline FVIII <40% or unknown.

Von Willebrand disease (VWD)

DefinitionVon Willebrand disease (VWD): a group of inherited or acquired bleeding disorders characterized by deficient or defective von Willebrand factor.

Covered ICD-10 subtypes (listed)

Clinical Background

Vonvendi is a recombinant von Willebrand factor product indicated to treat and prevent bleeding in patients with von Willebrand disease. Indications in the policy include on-demand treatment of bleeding episodes, perioperative management of surgical bleeding, and routine prophylaxis (notably for severe Type 3 vWD). Dosing and monitoring recommendations reference baseline VWF and FVIII measurements and may require concurrent recombinant factor VIII when FVIII is low; consult the full prescribing information and referenced hematology guidelines for detailed administration and monitoring guidance.

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyVonvendi (intravenous von Willebrand factor) coverage

Policy CodePolicy N/A

Change TypeNo material changes

Effective Date01/01/2020

Next Review DateN/A

Key ActionObtain prior authorization (initial auth typically for 3 months) and confirm ICD-10 diagnosis and indication per policy.

SourceLink

On This Page

Target FVIII:C: >=30 IU/dL for minor surgery; >=60 IU/dL for major surgery.

Prophylaxis dosing: For initiation of routine prophylaxis in severe Type 3 VWD: administer 40-60 IU/kg twice weekly; increase to 60 IU/kg twice weekly may be considered for breakthrough bleeding.

Covered diagnosis codes: D68.01, D68.020, D68.021, D68.022, D68.023, D68.03, D68.04, D68.09

Documentation Required

Desmopressin Consideration

For patients with mild or moderate vWD, the policy requires prior consideration of desmopressin. Vonvendi (or other factor product) is covered when desmopressin is known or suspected to be ineffective or is contraindicated.

Desmopressin must be known or suspected ineffective OR contraindicated for mild/moderate vWD coverage

Documentation Required

Dispensing Authorization Documentation

Monthly dispensing authorization by the rendering provider must include original prescription information, the requested amount to be dispensed, available vial sizes from the manufacturer, current patient product inventory and bleed history, and clinical rationale. The cumulative amount on‑hand will be considered and patients should not have more than 5 extra doses for acute episodes. Factor dosing and vial limits (maximum three vials per dose) must follow the hemophilia management program guidance.

Submit original prescription, requested amount, and vial sizes available from manufacturer
Provide clinical history including patient product inventory and bleed history
Do not dispense >3 vials per dose; consider +/‑1% dosing guidance
Patients should not have >5 extra on‑hand doses for acute bleeding

Billing Rule

Billing and Coding Documentation

Follow applicable CMS local coverage and coding guidance when billing. Noncompliance with referenced billing/coding articles may result in claim denials. Providers should consult Palmetto GBA, Novitas Solutions, and First Coast local coverage articles for Hemophilia Factor Products and Anti‑Inhibitor Coagulant Complex billing rules.

Refer to Palmetto GBA Local Coverage Article (AICC) guidance
Refer to Novitas Solutions Local Coverage Article for Hemophilia Factor Products
Refer to First Coast Service Options Local Coverage Article for Hemophilia Factor Products

Denial Risk

Triggers for Denial & Billing/Coding Denial Risk

Denial risk exists if the patient does not meet age, confirmed diagnostic, or indication‑specific criteria; if desmopressin consideration is not documented when required; or if there is evidence of unacceptable toxicity (for example, hypersensitivity, thromboembolic events, or development of inhibitors). Billing/coding denials may also occur if provider submission does not follow the referenced CMS local coverage and coding guidance.

Denial triggers: not ≥18 years, no confirmed vWD testing, indication criteria not met, desmopressin consideration absent when required
Unacceptable toxicity examples: hypersensitivity/anaphylaxis, thromboembolic events, neutralizing antibodies (inhibitors)
Billing/coding denial risk if submission does not follow Palmetto/Novitas/First Coast guidance

D68.01 (type 1); D68.020 (type 2A); D68.021 (type 2B); D68.022 (type 2M); D68.023 (type 2N); D68.03 (type 3); D68.04 (acquired VWD); D68.09 (other VWD).

Diagnostic confirmationPolicy requires diagnosis confirmed by blood coagulation and von Willebrand factor testing for coverage eligibility.