CurrentCentenePolicy N/A

Clinical Policy: Glycerol Phenylbutyrate (Ravicti)

Defines medical necessity criteria, prior authorization and continuation criteria for glycerol phenylbutyrate (Ravicti) for treatment of urea cycle disorders (UCDs) for Centene lines of business including Commercial, HIM, and Medicaid.

Policy Summary

PayerCentene

PolicyGlycerol Phenylbutyrate (Ravicti) coverage criteria

Policy CodePolicy N/A

Change TypeNo material changes

Effective Date05.01.16

Next Review DateN/A

Key ActionSubmit prior authorization with documentation demonstrating diagnosis confirmation, prescriber qualifications, step therapy attempts, and that requested dose does not exceed 17.5 mL/day.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

17.5 mL/daymaximum Ravicti dose

12 moinitial approval duration (Commercial)

6 moinitial approval duration (Medicaid/HIM)

1.1 g/mLproduct availability

Health Planpolicy owner

Coverage and Medical Necessity Criteria

Policy Summary

PayerCentene

PolicyGlycerol Phenylbutyrate (Ravicti) coverage criteria

Policy CodePolicy N/A

Change TypeNo material changes

Effective Date05.01.16

Next Review DateN/A

Key ActionSubmit prior authorization with documentation demonstrating diagnosis confirmation, prescriber qualifications, step therapy attempts, and that requested dose does not exceed 17.5 mL/day.

SourceLink

On This Page

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Initial Approval Criteria

Covered when ALL of the following are met:

Initial criteria

Diagnosis of a urea cycle disorder (UCD) caused by one or more of the following confirmed by enzymatic, biochemical, or genetic analysis: carbamyl phosphate synthetase 1 (CPS1) deficiency; ornithine transcarbamylase (OTC) deficiency; argininosuccinate synthetase (AS) deficiency (classic citrullinemia, type I); argininosuccinate lyase (ASL) deficiency (argininosuccinic aciduria); or arginase deficiency.
Prescribed by or in consultation with a physician experienced in treating metabolic disorders.
Step therapy for CPS1, OTC, or AS deficiencies
- Member must use generic sodium phenylbutyrate unless contraindicated or clinically significant adverse events are experienced.
- If member has intolerance or contraindication to generic sodium phenylbutyrate, member must use Pheburane unless contraindicated or clinically significant adverse effects are experienced.
Dose does not exceed 17.5 mL (19 g) per day.max dose 17.5 mL/day

Continued Therapy Criteria

Covered when ALL of the following are met:

Continuation

Member is currently receiving Ravicti via the Centene benefit or previously met initial approval criteria.
Step therapy for CPS1, OTC, or AS deficiencies
- Member must use generic sodium phenylbutyrate unless contraindicated or clinically significant adverse events are experienced.
- If member has intolerance or contraindication to generic sodium phenylbutyrate, member must use Pheburane unless contraindicated or clinically significant adverse effects are experienced.
Member is responding positively to therapy.

Requests for uses of glycerol phenylbutyrate (Ravicti) that are for non‑FDA approved indications and are not specifically addressed in this policy will not be authorized unless the provider supplies adequate documentation of safety and efficacy consistent with applicable off‑label use policies. Relevant off‑label policies include CP.CPA.09 for commercial lines, HIM.PA.154 for health insurance marketplace, and CP.PMN.53 for Medicaid, or the request is supported by the member’s evidence of coverage document.

Coverage and payment for Ravicti are subject to the terms, conditions, exclusions, and limitations set forth in the member’s coverage documents (for example, evidence of coverage, certificate of coverage, policy, or contract of insurance), as well as applicable state and federal requirements and Health Plan‑level administrative policies and procedures. This clinical policy is a guidance tool to assist in coverage determinations and does not guarantee payment or constitute a contract.

Prior Authorization, Documentation, and Step Therapy

Prior Authorization

Prior Authorization Required

Prior Authorization Required — Prior authorization (PA) is required for Ravicti and related therapies. Provider must submit a PA request following the Health Plan's PA procedures. Approval is contingent upon meeting the clinical criteria in this policy and any Health Plan‑level PA requirements.

Submit PA requests per Health Plan instructions and portals
Approval subject to member benefit terms, state/federal requirements, and Health Plan administrative policies

Step Therapy

Step Therapy: Generic Sodium Phenylbutyrate then Pheburane

Step therapy is implemented as a stepwise redirection: members must generally try generic sodium phenylbutyrate first. If the member has an intolerance or contraindication to generic sodium phenylbutyrate, the member must use Pheburane unless contraindicated or clinically significant adverse effects occur. Specific step therapy operational details (forms, exceptions, appeals) are governed by the Health Plan.

Initial and continuation requests should demonstrate prior use of generic sodium phenylbutyrate when required
If intolerance or contraindication to generic sodium phenylbutyrate is documented, Pheburane is the next step unless also contraindicated

Documentation Required

Required Documentation

Required Documentation — Providers must supply clinical documentation to support that the member meets all approval criteria. Acceptable documentation includes chart notes, laboratory results, genetic/enzyme testing reports, consultation notes from a physician experienced in metabolic disorders, and any documentation of intolerance or contraindication to alternative therapies.

Diagnostic confirmation by enzymatic, biochemical, or genetic analysis when applicable
Documentation of prior trials, intolerance, or contraindications to generic sodium phenylbutyrate when relevant
For off‑label indications, include evidence of efficacy and safety per applicable off‑label use policies

Note

Administrative and Coverage Governance

Administrative Compliance Reminder — Coverage decisions and the administration of benefits are governed by the member's coverage documents and Health Plan administrative policies. Providers must follow Health Plan administrative procedures (PA submission, prior authorization forms, appeals processes, and state‑specific continuity of care requirements) when requesting and administering therapy.

Coverage subject to evidence of coverage, certificate of coverage, policy, contract of insurance, and applicable state/federal laws
Providers remain responsible for exercising professional medical judgment; this policy does not replace contractual or regulatory requirements

Clinical Background

Urea cycle disorders (UCDs) are inherited enzyme deficiencies that impair the conversion of nitrogen to urea and can lead to hyperammonemia. Ravicti (glycerol phenylbutyrate) is a nitrogen‑scavenging agent indicated for the chronic management of patients with UCDs who cannot be managed by dietary protein restriction and, when appropriate, amino acid supplementation. Ravicti is intended as a maintenance therapy and is not indicated for the treatment of acute hyperammonemia; additionally, safety and efficacy have not been established for certain rare defects such as N‑acetylglutamate synthase (NAGS) deficiency.

Key Definitions

inv-15: UCD — list of enzymes/conditions that define urea cycle disorders

Enzymes/conditions defining UCDsCarbamyl phosphate synthetase 1 (CPS1) deficiency; Ornithine transcarbamylase (OTC) deficiency; Argininosuccinate lyase (ASL) deficiency; Argininosuccinate synthetase (AS) deficiency; N-acetylglutamate synthase (NAGS) deficiency; Arginase deficiency

Pathophysiology summaryUCDs are caused by deficiency of enzymes in the urea cycle that transform nitrogen to urea

Management contextRavicti used as adjunct to dietary protein restriction and, when appropriate, amino acid supplementation

inv-16: Ravicti indication/limitations — labeled indication and limitation details

Labeled indicationIndicated for chronic management of patients with urea cycle disorders (UCDs) who cannot be managed by dietary protein restriction and/or amino acid supplementation alone; must be used with dietary protein restriction and, when appropriate, amino acid supplementation

Not for acute hyperammonemiaRavicti is not indicated for the treatment of acute hyperammonemia; more rapidly acting interventions are required

NAGS limitationSafety and efficacy of Ravicti for treatment of N-acetylglutamate synthase (NAGS) deficiency has not been established

OpenPayer

Clinical Policy: Glycerol Phenylbutyrate (Ravicti)

Coverage and Medical Necessity Criteria

Dosing Limits and Product Details

Prior Authorization, Documentation, and Step Therapy

Clinical Background

Key Definitions