CurrentCignaPolicy N/A

Metabolic Disorders - Phenylbutyrate Products Preferred Specialty Management Policy

Defines Cigna's preferred specialty management approach for sodium and glycerol phenylbutyrate products used as chronic adjunctive therapy in urea cycle disorders (UCDs), including prior authorization and exception criteria for preferred vs non-preferred products.

Policy Summary

PayerCigna

PolicyMetabolic Disorders - Phenylbutyrate Products Preferred Specialty Management Policy

Policy CodePolicy N/A

Change TypeDocumentation requirement added

Effective DateJan 1, 2024

Next Review DateOct 23, 2024

Key ActionObtain and submit documentation (e.g., chart notes or prescription records) when requesting Ravicti based on a sodium-restricted diet or contraindication to a high-sodium diet.

SourceLink

POLICY UPDATE CHANGES

Requirement for documentation of a sodium-restricted diet or contraindication to a high sodium diet was added for Ravicti exceptions.

4products listed

1 yearapproval duration

Preferred→Non-Preferredstep requirement

NCapplicable state

Coverage Criteria

Non-Preferred Product Exception Criteria

Non-preferred products are covered as medically necessary when the specified exception criteria are met.

Buphenyl/Olpruva exception

A) Patient meets the standard Metabolic Disorders - Phenylbutyrate Products Prior Authorization Policy criteria.
B) Patient meets ONE of the following
- i. Patient has tried generic sodium phenylbutyrate.
- ii. Patient has tried Pheburane.

Ravicti exception

Policy Summary

PayerCigna

PolicyMetabolic Disorders - Phenylbutyrate Products Preferred Specialty Management Policy

Policy CodePolicy N/A

Change TypeDocumentation requirement added

Effective DateJan 1, 2024

Next Review DateOct 23, 2024

Key ActionObtain and submit documentation (e.g., chart notes or prescription records) when requesting Ravicti based on a sodium-restricted diet or contraindication to a high-sodium diet.

SourceLink

On This Page

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This policy addresses chronic adjunctive therapy with phenylbutyrate products for urea cycle disorders (UCDs). Phenylbutyrate agents are indicated in combination with dietary management for UCDs that involve deficiencies such as carbamylphosphate synthetase (CPS), ornithine transcarbamylase (OTC), or argininosuccinic acid synthetase (AS). Of the available agents, Ravicti (glycerol phenylbutyrate) is the only product that does not contain sodium. (See Preferred vs Non-Preferred product lists.)

Sodium phenylbutyrate products and Ravicti are intended for chronic management and are not indicated for treatment of acute hyperammonemia, which requires rapid-acting interventions. Ravicti is indicated when dietary protein restriction and/or amino acid supplementation alone are insufficient; its safety and efficacy have not been established for N-acetylglutamate synthetase deficiency. Specific product use considerations (for example, administration options and weight/BSA limitations) are noted in the overview section of the clinical policy.

Provider Actions and Requirements

Prior Authorization

Prior Authorization Required

All medications (Preferred and Non-Preferred) require standard prior authorization. Providers must submit a prior authorization request and meet the Metabolic Disorders - Phenylbutyrate Products prior authorization criteria for approval. Approvals are provided for the duration noted in the authorization.

Applies to generic sodium phenylbutyrate (tablets or powder), Pheburane, Buphenyl, Olpruva, Ravicti
If member meets standard prior authorization criteria but has not tried a Preferred Product, offer review for a Preferred Product per the standard prior authorization policy

Step Therapy

Step Therapy: Try Preferred First

A step requirement directs patients to try a Preferred Product before approval of a Non-Preferred Product. If the patient has met the standard prior authorization criteria but has not tried a Preferred Product, the prescriber will be asked to consider a Preferred Product and a review will be offered for the Preferred Product using the standard prior authorization policy.

Preferred Products: generic sodium phenylbutyrate (tablets or powder), Pheburane
Non-Preferred Products subject to step requirement: Buphenyl, Olpruva, Ravicti

Documentation Required

Documentation Requirements

Documentation is required where indicated in the criteria. Acceptable documentation includes chart notes, prescription claims records, prescription receipts, and any additional records necessary to support exception criteria (for example, documentation of a sodium-restricted diet when requesting Ravicti).

Documentation may include: chart notes, prescription claims records, prescription receipts
Ravicti-specific documentation: documentation that the patient is on a sodium-restricted diet or that a high sodium diet is contraindicated (when claiming this exception)

Denial Risk

Non-Preferred Product Denial Risk

Requests for Non-Preferred Products that do not meet the specified Non-Preferred Product exception criteria are considered not medically necessary and may be denied. Providers should ensure all required criteria and documentation are submitted to avoid denial.

Non-Preferred exception criteria must be met for coverage; otherwise request may be denied as not medically necessary
Buphenyl/Olpruva: require meeting standard prior authorization criteria AND trial of generic sodium phenylbutyrate or Pheburane (approve for 1 year if met)
Ravicti: require meeting standard prior authorization criteria AND one of: trial of generic sodium phenylbutyrate, trial of Pheburane, documentation of sodium-restricted diet or contraindication to high sodium, or inability to eat soft food without a feeding tube (approve for 1 year if met)
If 1A is met but 1B is not, offer review for a Preferred Product using the standard prior authorization policy

Background

Phenylbutyrate products act as ammonia-scavenging agents used as adjunctive chronic therapy with dietary management in patients with UCDs. Sodium-containing formulations (sodium phenylbutyrate products) are indicated for chronic management of adult and pediatric patients with UCDs due to CPS, OTC, or AS deficiencies. Buphenyl (sodium phenylbutyrate) and Pheburane can be given orally to pediatric patients weighing <20 kg; Buphenyl powder is compatible with feeding tube administration. Olpruva has labeled use in patients >20 kg and with body surface area >1.2 m2. Ravicti (glycerol phenylbutyrate) contains no sodium and is indicated for chronic management when dietary measures alone are insufficient; like the sodium products, it is not indicated for acute hyperammonemia.

Definitions

inv-09: Urea cycle disorders (UCDs) — definition and examples of enzyme deficiencies

DefinitionInherited metabolic disorders resulting in deficiencies of urea cycle enzymes (e.g., carbamylphosphate synthetase [CPS], ornithine transcarbamylase [OTC], argininosuccinic acid synthetase [AS]) that impair ammonia detoxification and require dietary management with adjunctive ammonia‑scavenging therapy.

Typical chronic managementPhenylbutyrate products are indicated in combination with dietary management as adjunctive chronic therapy for UCDs when dietary protein restriction and/or amino acid supplementation alone are insufficient.

Agents and sodium contentOf available phenylbutyrate agents, Ravicti (glycerol phenylbutyrate) does not contain sodium; other sodium phenylbutyrate products do contain sodium.

Pediatric dosing notesBuphenyl and Pheburane can be administered orally in pediatric patients weighing < 20 kg; Buphenyl powder is compatible with feeding tube administration.

Indication limitsPhenylbutyrate products (including Ravicti) are not indicated for treatment of acute hyperammonemia, which requires rapid‑acting interventions.

Ravicti-specific noteRavicti is indicated for chronic management of UCDs that cannot be managed by dietary measures alone; safety and efficacy for N‑acetylglutamate synthetase deficiency have not been established.