CurrentBlue Cross Blue Shield - IowaPolicy 05.04.42

Bylvay (odevixibat) drug coverage

Defines medical necessity, prior authorization, dosing limits, and documentation requirements for Bylvay (odevixibat) for treatment of pruritus in PFIC and Alagille syndrome for Wellmark members.

Policy Summary

PayerBlue Cross Blue Shield - Iowa

PolicyBylvay (odevixibat) drug coverage

Policy CodePolicy 05.04.42

Change TypeNo material changes

Effective DateSep 26, 2024

Next Review Date

Key ActionSubmit prior authorization with genetic test results (if applicable) and documentation of benefit for continuation; medication must be prescribed by or in consultation with a hepatologist or gastroenterologist.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

6 moinitial authorization duration

12 mocontinuation authorization duration

6 mg/daymax total daily dose

360/30dstandard limit

PFIC ≥3m; ALGS ≥12mage & indication

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Coverage Criteria for Bylvay (odevixibat)

Initial Therapy — PFIC

Authorization of 6 months may be granted for treatment of pruritus in PFIC when ALL of the following are met:

PFIC molecular diagnosis: Member has a confirmed molecular diagnosis of PFIC (e.g., mutations in ATP8B1, ABCB11, ABCB4, TJP2, MYO5B).

genetic confirmation required

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

No other concomitant liver disease: Member does not have any other concomitant liver disease (e.g., biliary atresia, liver cancer, alternate non-PFIC related etiology of cholestasis).

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

Age requirement: Member is 3 months of age or older.

Initial Therapy — ALGS

Authorization of 6 months may be granted for treatment of cholestatic pruritus in Alagille syndrome when ALL of the following are met:

ALGS diagnostic criteria: Member has a diagnosis of Alagille syndrome established by one of the following: genetic testing (e.g., mutations in JAG1 or NOTCH2); family history in a first-degree relative plus two or more major clinical features; family history plus bile duct paucity and one or more major clinical features; bile duct paucity plus three or more major clinical features; or four or more major clinical features.

See Appendix for major clinical features of ALGS

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

No other concomitant liver disease: Member does not have a history or presence of other concomitant liver disease (e.g., biliary atresia, PFIC, liver cancer).

No liver transplant:

Continuation Therapy

Authorization of 12 months may be granted for continuation of therapy when ALL of the following are met:

Demonstrated clinical benefit: Member is experiencing benefit from therapy (e.g., improvement in pruritus).

Chart notes or medical records documenting benefit required for continuation

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

Bylvay (odevixibat) is considered not medically necessary for members who do not meet the specific coverage criteria set forth in this policy.

Procedures, Billing, and Dose Limits

Procedures and Billing Codesmixed

No codes listed

inv-07: Maximum total daily dose — 6 mg/day and related dosing limits

Maximum total daily doseDo not exceed a total daily dose of 6 mg per day (all formulations).

PFIC dosingStart 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, not to exceed 6 mg/day.

ALGS dosing120 mcg/kg once daily; dose reduction to 40 mcg/kg/day may be considered for tolerability, then titrate back to 120 mcg/kg/day as tolerated (not to exceed 6 mg/day).

Formulation limits (pellets/capsules)Quantity standard limits: 200 mcg pellets = 360 per 30 days; 600 mcg pellets = 120 per 30 days; 400 mcg capsule = 540 per 30 days; 1200 mcg capsule = 180 per 30 days.

Prior Authorization, Documentation, and Prescriber Requirements

Prior Authorization

Prior Authorization Required

Prior authorization is required. Initial authorizations may be granted for up to 6 months when criteria are met; continuation authorizations may be granted for up to 12 months for members demonstrating benefit from therapy (e.g., improvement in pruritus).

Initial prior authorization duration: up to 6 months
Continuation prior authorization duration: up to 12 months when documentation of benefit is provided

Documentation Required

Required Documentation

Submit the following documentation with prior authorization requests. For initial requests include genetic testing results confirming the diagnosis (e.g., PFIC with causative gene mutation or ALGS with JAG1/NOTCH2 variants) when applicable. For continuation requests include chart notes or medical records documenting clinical benefit from therapy (for example, documented improvement in pruritus).

Initial requests: genetic testing results confirming PFIC or ALGS when applicable
Continuation requests: clinical chart notes or medical records demonstrating benefit from therapy (e.g., reduced pruritus)

Note

Prescriber Specialty Requirement

Medication must be prescribed by or in consultation with a hepatologist or gastroenterologist.

Denial Risk

Exclusion — PFIC Type 2 (ABCB11)

Coverage will not be provided for members with PFIC type 2 who have ABCB11 variants resulting in a non-functional or absent bile salt export pump (BSEP). Such cases are considered not medically necessary for this therapy.

Documentation Required

Provider Action — Documentation Details

Provider note: ensure genetic testing reports clearly identify the causative gene/variant and that medical records for continuation requests document objective or patient-reported improvement (for example, decreased pruritus severity, reduced scratching, improved sleep). Include prior treatment history and transplant status where applicable.

Indication Age Definitions

inv-14: PFIC indication — Bylvay indicated for pruritus in PFIC for patients >= 3 months

Indication (FDA)Bylvay is indicated for treatment of pruritus in patients with progressive familial intrahepatic cholestasis (PFIC).

Minimum ageApproved for use in patients 3 months of age and older for PFIC-related pruritus.

PFIC dosing summaryPFIC dosing: start 40 mcg/kg once daily; may titrate up to 120 mcg/kg once daily if needed, not to exceed 6 mg/day.

inv-15: ALGS indication — Bylvay indicated for cholestatic pruritus in Alagille syndrome for patients >= 12 months

Indication (FDA)Bylvay is indicated for treatment of cholestatic pruritus in patients with Alagille syndrome (ALGS).

Minimum ageApproved for use in patients 12 months of age and older for ALGS-related cholestatic pruritus.

ALGS dosing summaryALGS dosing: 120 mcg/kg once daily; dose reduction to 40 mcg/kg/day may be considered for tolerability, but total daily dose must not exceed 6 mg.