CurrentBlue Cross Blue Shield - IowaPolicy 05.04.42

Bylvay (odevixibat) — Coverage Criteria for PFIC and Alagille Syndrome

Defines coverage criteria, prior authorization, dosing limits, and documentation requirements for Bylvay (odevixibat) to treat pruritus in patients with progressive familial intrahepatic cholestasis (PFIC) and Alagille syndrome (ALGS) for Wellmark/Blue Cross Blue Shield members.

Policy Summary

PayerBlue Cross Blue Shield - Iowa

PolicyBylvay (odevixibat) — Coverage Criteria for PFIC and Alagille Syndrome

Policy CodePolicy 05.04.42

Change TypeNo material change

Effective DateDec 4, 2024

Next Review DateOct 1, 2025

Key ActionSubmit prior authorization with genetic testing results for initial requests and chart documentation of benefit for continuation.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

2FDA-approved indications

6Initial auth (months)

12Continuation (months)

6 mgMax daily dose

genetic testInitial docs

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Coverage and Medical Necessity Criteria

PFIC Initial Therapy

Covered when ALL of the following are met for PFIC (authorization of 6 months):

PFIC initial authorization: 1. Confirmed molecular diagnosis of PFIC (e.g., ATP8B1, ABCB11, ABCB4, TJP2, or MYO5B gene variants).

genetic testing required for initial requests

PFIC cholestasis evidence: 2. Evidence of cholestasis (e.g., elevated serum bile acid level).

No other liver disease: 3. No other concomitant liver disease (e.g., biliary atresia, liver cancer, alternate non-PFIC related etiology of cholestasis).

No prior transplant: 4. Member has not received a liver transplant.

Age minimum: 5. Member is 3 months of age or older.>= 3 months

Dosing limits: Dosing per FDA labeling: start 40 mcg/kg once daily; if no improvement after 3 months may increase in 40 mcg/kg increments up to 120 mcg/kg once daily; total daily dose not to exceed 6 mg.<= 6 mg/day

Quantity limits specified by formulation

ALGS Initial Therapy

Covered when ALL of the following are met for ALGS (authorization of 6 months):

ALGS diagnosis: 1. Diagnosis of ALGS established by one of: i) genetic testing (e.g., JAG1 or NOTCH2 gene variants), ii) family history of ALGS plus one or more major clinical features, iii) bile duct paucity plus three or more major clinical features, or iv) four or more major clinical features.

Appendix lists major clinical features of ALGS

ALGS cholestasis evidence: 2. Evidence of cholestasis (e.g., elevated serum bile acid level).

No other liver disease: 3. No history or presence of other concomitant liver disease (e.g., biliary atresia, PFIC, liver cancer).

No prior transplant: 4. Member has not received a liver transplant.

Continuation of Therapy

Covered when ALL of the following are met for continuation (authorization of 12 months):

Documented benefit: Member is experiencing benefit from therapy (e.g., improvement in pruritus) documented in chart notes or medical record.

Applicable to all members including new members

Coverage will not be provided for members who have PFIC type 2 with variants in the ABCB11 gene that result in a non-functional or complete absence of the bile salt export pump (BSEP) protein.

Bylvay is considered not medically necessary for members who do not meet the criteria set forth in this policy.

Procedures, Dosing Limits, and Codes

Procedures and Billing Codesmixed

No codes listed

inv-07: Maximum total daily dose — Not to exceed 6 mg total daily dose.

Maximum total daily doseNot to exceed 6 mg total daily dose (per FDA-recommended dosing across formulations).

PFIC dosing pathwayStart 40 mcg/kg once daily; if no improvement after 3 months, may increase in 40 mcg/kg increments up to 120 mcg/kg once daily (subject to 6 mg/day maximum).

ALGS dosing noteTypical ALGS dosing is 120 mcg/kg once daily with option to reduce to 40 mcg/kg/day for tolerability and later re-escalate (total daily dose still must not exceed 6 mg).

Authorization, Documentation, and Prescriber Requirements

Prior Authorization

Prior Authorization Required

Prior authorization required — initial approvals of 6 months for PFIC (age ≥3 months) and ALGS (age ≥12 months) when indication-specific criteria are met.

Initial authorization: up to 6 months for PFIC-related pruritus when all PFIC criteria are met.
Initial authorization: up to 6 months for cholestatic pruritus in ALGS when all ALGS criteria are met.
Continuation authorization: up to 12 months when member demonstrates benefit (e.g., improvement in pruritus).

Note

Concomitant Therapy Restriction

Member cannot use the requested medication concomitantly with any other ileal bile acid transporter (IBAT) inhibitor.

Examples of other IBAT inhibitors: Livmarli (obeticholic acid listed as an example in source).

Clinical Background

Bylvay (odevixibat) is an ileal bile acid transporter (IBAT) inhibitor indicated for treatment of cholestatic pruritus in patients with progressive familial intrahepatic cholestasis (PFIC) and Alagille syndrome (ALGS). Prescribing should follow labeled dosing and monitoring for clinical benefit; efficacy may be limited in a subgroup of PFIC type 2 patients with specific ABCB11 variants that abolish BSEP function. Members must not receive Bylvay concomitantly with another IBAT inhibitor.

Key Condition Definitions

inv-13: PFIC (progressive familial intrahepatic cholestasis)

DefinitionProgressive familial intrahepatic cholestasis (PFIC) is a genetic cholestatic liver disease associated with pruritus and elevated serum bile acids.

Age indicationBylvay is indicated for treatment of pruritus in patients 3 months of age and older with PFIC.

Effectiveness limitationBylvay may be ineffective in a subgroup of PFIC type 2 patients with specific ABCB11 variants resulting in non-functional or absent BSEP protein.

inv-14: Alagille syndrome (ALGS) — definition and age-based indication for Bylvay.

DefinitionAlagille syndrome (ALGS) is a multisystem genetic disorder characterized by cholestasis and other major clinical features (hepatic, cardiac, skeletal, ophthalmologic, characteristic facial features, vascular, renal).

Age indication for Bylvay

Policy Summary

PayerBlue Cross Blue Shield - Iowa

PolicyBylvay (odevixibat) — Coverage Criteria for PFIC and Alagille Syndrome

Policy CodePolicy 05.04.42

Change TypeNo material change

Effective DateDec 4, 2024

Next Review DateOct 1, 2025

Key ActionSubmit prior authorization with genetic testing results for initial requests and chart documentation of benefit for continuation.

SourceLink

On This Page

Age minimum: 5. Member is 12 months of age or older.>= 12 months

Dosing limits ALGS: Dosing per FDA labeling: typically 120 mcg/kg once daily; dose reduction to 40 mcg/kg/day may be considered for tolerability with later re-escalation; follow quantity limits and do not exceed total daily dose of 6 mg.<= 6 mg/day

Quantity limits specified by formulation

Documentation Required

Required Documentation

Submission of the following information is necessary to initiate the prior authorization review: Initial requests must include genetic testing results confirming a diagnosis of progressive familial intrahepatic cholestasis (PFIC) or Alagille syndrome (ALGS), if applicable. Continuation requests must include chart notes or medical record documentation showing a benefit from therapy (e.g., improvement in pruritus).

Initial request: genetic testing results confirming molecular diagnosis (e.g., PFIC genes such as ATP8B1, ABCB11, ABCB4, TJP2, MYO5B; or ALGS genes such as JAG1 or NOTCH2) where applicable.
Continuation request: recent chart notes or medical records documenting clinical benefit (for example, reduction in pruritus).
Authorization length: initial approvals up to 6 months; continuation approvals up to 12 months when benefit documented.

Denial Risk

Denial Conditions

Coverage will not be provided for members who have PFIC type 2 with variants in the ABCB11 gene resulting in non-functional or complete absence of the bile salt export pump (BSEP) protein.

Other standard denial conditions apply if member does not meet the indication-specific criteria for PFIC or ALGS.
Bylvay is considered not medically necessary for members who do not meet the criteria set forth in this policy.

Bylvay is indicated for treatment of cholestatic pruritus in patients 12 months of age and older with ALGS.

Diagnostic approachesDiagnosis can be established by genetic testing (e.g., JAG1 or NOTCH2 variants), family history plus ≥1 major clinical feature, bile duct paucity plus ≥3 major clinical features, or ≥4 major clinical features.