CurrentCignaPolicy IP0648

Beqvez (fidanacogene elaparvovec-dzkt) gene therapy for hemophilia B

Defines Cigna coverage, prior authorization, and medical necessity criteria for one-time intravenous Beqvez gene therapy in adults with moderate to severe hemophilia B, including required testing and prescriber qualifications.

Policy Summary

PayerCigna

PolicyBeqvez (fidanacogene elaparvovec-dzkt) gene therapy for hemophilia B

Policy CodePolicy IP0648

Change TypeCoding and criteria revisions

Effective Date

Next Review DateOct 17, 2024

Key ActionObtain prior authorization and document baseline Factor IX level, AAVRh74var antibody status, and required labs to support a one-time dose approval.

SourceLink

POLICY UPDATE CHANGES

Added HCPCS/CPT/HCPCS-like codes C9172 and J1414 and updated descriptions/effective dates for C9399, J3490, and J3590.

Requirement that patients do not have a history of Factor IX inhibitors was moved from an approval criterion to a 'Conditions Not Recommended for Approval' (i.e., added to exclusions).

Removed requirement that prophylactic Factor IX therapy will not be given after Beqvez administration once adequate Factor IX levels have been achieved.

one-timedose per lifetime (single infusion)

5 x 10^11 vg/kgrecommended dose

≤ 2%Factor IX threshold

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Coverage and Medical Necessity Criteria

FDA-Approved Indication

Approve a one-time (per lifetime) single dose if the patient meets ALL of the following:

Eligibility basic

Demographics and prior therapy: A) Patient is male; AND B) Patient is ≥ 18 years of age; AND C) Patient has not received a gene therapy for hemophilia B in the past (verification in claims history required); AND D) Patient has moderately severe or severe hemophilia B as evidenced by baseline Factor IX level ≤ 2% of normal (documentation required); AND N) The medication is prescribed by a hemophilia specialist physician.
Clinical need: E) Patient meets ONE of the following: (i) history of use of Factor IX therapy for ≥ 150 exposure days; OR (ii) history of life‑threatening hemorrhage requiring on‑demand Factor IX; OR (iii) history of repeated, serious spontaneous bleeding episodes requiring on‑demand Factor IX.
Immunologic and infection criteria: F) Negative for neutralizing antibodies to AAVRh74var by an approved test; AND G) Factor IX inhibitor titer testing performed within 30 days and negative; AND H) No active hepatitis B or C infection and not currently receiving antiviral therapy for prior exposure; AND I) No uncontrolled HIV infection.
Laboratory and organ function: J) Within 30 days: ALT ≤ 2x ULN; AST ≤ 2x ULN; total bilirubin ≤ 1.5x ULN; alkaline phosphatase ≤ 2x ULN; AND K) No evidence of advanced liver impairment or advanced fibrosis; AND L) Platelet count ≥ 100 x 10^9/L within 30 days; AND M) Creatinine ≤ 2.0 mg/dL within 30 days; AND O) Current body weight obtained within 30 days.
Dosing: P) If criteria A through P are met, approve one dose (vials in a kit) to provide a one‑time single dose of 5 x 10^11 vector genomes/kg by IV infusion; dose weight calculation uses adjusted body weight for BMI > 30 kg/m^2.

Patient is male; ≥ 18 years old; has not received a gene therapy for hemophilia B; baseline (without Factor IX replacement) Factor IX level ≤ 2% of normal; and medication prescribed by a hemophilia specialist.

Beqvez (fidanacogene elaparvovec-dzkt) is covered only when all policy criteria are met for the FDA‑approved indication (one-time, per‑lifetime single IV dose). Beqvez is considered not medically necessary for any use that does not meet the criteria in this policy. Explicit exclusions called out in the policy include: prior receipt of gene therapy for hemophilia B and patients with a history of Factor IX inhibitors. These exclusions reflect reasons for patient exclusion in the pivotal clinical study and are listed as not medically necessary for coverage under this policy.

Additional exclusions highlighted in the policy and placed in the Conditions Not Recommended for Approval include: prior receipt of any gene therapy for hemophilia B and a history of Factor IX inhibitors. The revision history documents that the Factor IX inhibitor criterion was moved from an approval criterion into the not‑recommended/exclusions section, and prior gene therapy remains an explicit exclusion consistent with the pivotal trial exclusion criteria.

Billing Codes, Dose Calculation, and Dosing

Considered Medically Necessary / Billing codesmixedCovered

C9172	Injection, fidanacogene elaparvovec-dzkt, per therapeutic dose (Code effective dates 10/1/2024-12/31/2024)
C9399	Unclassified drugs or biologicals (Code effective until 12/31/2024)
J1414	Injection, fidanacogene elaparvovec-dzkt, per therapeutic dose (Code effective 1/1/2025)
J3490	Unclassified drugs (Code effective until 12/31/2024)
J3590	Unclassified biologics (Code effective until 12/31/2024)

Dose weight calculation

Dose weight calculation (adjusted body weight)For BMI > 30 kg/m2 use: Dose Weight (kg) = 30 kg/m2 × [Height (m)]^2

When to apply adjusted weightApply only when patient body mass index (BMI) is greater than 30 kg/m2 (documentation required)

Measurement timingUse height measured in meters from the current visit or chart to compute dose weight; current body weight must be obtained within 30 days

Recommended dose

Recommended single dose5 × 10^11 vector genomes per kg of body weight given as a one-time (per lifetime) single IV infusion

RouteIntravenous (IV) infusion

Prior Authorization, Documentation, and Claims Guidance

Prior Authorization

Prior Authorization Recommended

Prior Authorization is recommended. Approval is provided for a one‑time (per lifetime) single dose when the patient meets the policy clinical and dosing criteria and dosing verification is completed by a Medical Director. Because evaluation and long‑term monitoring require specialized skills, Beqvez must be prescribed by a hemophilia specialist physician. All approvals will be forwarded to the Medical Director for review.

Approval limited to one-time (per lifetime) single dose
Prescriber must be a hemophilia specialist physician
Medical Director verification required for weight‑based dosing and claims history

Prior Authorization

Therapy History Requirement

Eligibility requires documentation of current or prior use of Factor IX prophylaxis OR a history of life‑threatening hemorrhage or repeated, serious spontaneous bleeding episodes with on‑demand Factor IX use as specified in the clinical criteria. If claims history is available, prior receipt of Beqvez or Hemgenix must be verified in claims; if claims are not available, the prescribing physician must attest the patient has not previously received these gene therapies.

Clinical Background

Hemophilia B is an X‑linked genetic bleeding disorder caused by deficient or absent blood Factor IX. Disease severity correlates with endogenous Factor IX activity: severe disease is typically <1% activity, moderate 1–5%, and mild 6–49%. The policy targets adults with moderately severe or severe hemophilia B (baseline Factor IX activity ≤ 2% of normal) and aims to raise Factor IX levels with a one‑time AAV‑based gene therapy infusion. Key clinical considerations for eligibility include absence of neutralizing antibodies to the AAVRh74var capsid and appropriate liver, platelet, and renal laboratory parameters prior to treatment.

Key Definitions and Thresholds

Moderately severe or severe hemophilia B

Definition (policy threshold)Baseline (without Factor IX replacement) Factor IX activity ≤ 2% of normal is required by the policy

Severity classification contextModerately severe hemophilia B corresponds to Factor IX levels ~1%–5% and severe <1% (policy uses ≤2% as eligibility cutoff)

Documentation requiredBaseline Factor IX level must be documented (measurement without recent Factor IX replacement) as part of eligibility

AAVRh74var neutralizing antibodies

Required immunologic statusNegative for neutralizing antibodies to AAVRh74var as detected by an FDA‑approved test (documentation required)

Test typeDetection must be by an FDA‑approved neutralizing antibody test for AAVRh74var capsid