CurrentCareSource Georgia MedicaidPolicy GA-MED-P-366579

Ravicti (glycerol phenylbutyrate) for Urea Cycle Disorders

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CareSource Georgia Medicaid coverage and prior authorization requirements for Ravicti as adjunctive treatment for chronic urea cycle disorders when dietary management alone is insufficient.

Policy Summary

PayerCareSource Georgia Medicaid

PolicyRavicti (glycerol phenylbutyrate) for Urea Cycle Disorders

Policy CodePolicy GA-MED-P-366579

Change TypeMaterial revision

Effective DateFeb 1, 2026

Next Review DateN/A

Key ActionSubmit pharmacy prior authorization with confirmatory UCD diagnostic results and documentation of all initial coverage requirements.

SourceLink

POLICY UPDATE CHANGES

Confirmatory genetic testing or enzyme activity assay results were added as a diagnostic requirement for UCD coverage.

The sodium phenylbutyrate trial requirement was revised and condensed to intolerance, contraindication, or failure to normalize ammonia despite optimized dosing.

A restriction was added against concomitant use of Ravicti with any formulation of sodium phenylbutyrate.

Maximum dosing and a quantity limit were added to the policy.

The initial authorization duration was changed from 12 months to 6 months and renewal criteria were revised.

6 monthsInitial authorization duration

12 months

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CurrentCareSource Georgia MedicaidPolicy GA-MED-P-366579

Ravicti (glycerol phenylbutyrate) for Urea Cycle Disorders

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Sign up for caresourcegeorgia Policy GA-MED-P-366579 alerts

Get alerted when Policy GA-MED-P-366579 changes without checking for updates manually.

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CareSource Georgia Medicaid coverage and prior authorization requirements for Ravicti as adjunctive treatment for chronic urea cycle disorders when dietary management alone is insufficient.

Policy Summary

PayerCareSource Georgia Medicaid

PolicyRavicti (glycerol phenylbutyrate) for Urea Cycle Disorders

Policy CodePolicy GA-MED-P-366579

Change TypeMaterial revision

Effective DateFeb 1, 2026

Next Review DateN/A

Key ActionSubmit pharmacy prior authorization with confirmatory UCD diagnostic results and documentation of all initial coverage requirements.

SourceLink

POLICY UPDATE CHANGES

Confirmatory genetic testing or enzyme activity assay results were added as a diagnostic requirement for UCD coverage.

The sodium phenylbutyrate trial requirement was revised and condensed to intolerance, contraindication, or failure to normalize ammonia despite optimized dosing.

A restriction was added against concomitant use of Ravicti with any formulation of sodium phenylbutyrate.

Maximum dosing and a quantity limit were added to the policy.

The initial authorization duration was changed from 12 months to 6 months and renewal criteria were revised.

6 monthsInitial authorization duration

12 months

Reauthorization duration

525 mL/30 daysQuantity limit

17.5 mL/dayMaximum daily dose

150 or >100 µmol/LHyperammonemia threshold

Coverage Criteria

Initial Therapy

Covered for initial authorization when ALL of the following criteria are met:

ALL of the following

Medication is prescribed by, or in consultation with, a metabolic or genetic specialist.
Member has a documented diagnosis of a urea cycle disorder (UCD), such as a deficiency involving carbamoyl phosphate synthetase (CPS), ornithine transcarbamylase (OTC), or argininosuccinic acid synthetase (AS).
The UCD diagnosis is confirmed by genetic testing or enzyme activity assay results.
Member has a history of hyperammonemia: 150 µmol/L (>260 µg/dL) or higher in neonates, or >100 µmol/L (175 µg/dL) in older children and adults.
Ravicti is prescribed as adjunctive therapy to dietary protein restriction.
ONE of the following documented sodium phenylbutyrate trial outcomes
- Intolerance due to taste disturbance severe enough to induce vomiting or interfere with adherence.
- Contraindication, such as congestive heart failure, severe renal insufficiency, or a clinical state in which sodium retention with edema occurs.
- Failure to normalize ammonia levels despite optimized dosing.
Member does not have N-acetylglutamate synthase (NAGS) deficiency.
Ravicti is not being used to treat acute hyperammonemia.
Ravicti is not being used concomitantly with any formulation of sodium phenylbutyrate.
Prescribed dosage is within the policy limits: 4.5 to 11.2 mL/m²/day (5 to 12.4 g/m²/day), administered in 3 equally divided doses, with a maximum total daily dosage of 17.5 mL (19 g).
Quantity limit: 525 mL per 30 days. If switching from sodium phenylbutyrate, refer to prescribing information.

Continuation Therapy

Covered for reauthorization when ALL of the following criteria are met:

ALL of the following

Documentation of ongoing dietary management.
Chart notes document a positive response to therapy, such as normalized ammonia levels or a reduced number of hyperammonemic crises.

Ravicti is excluded when used for acute hyperammonemia, in members with N-acetylglutamate synthase (NAGS) deficiency, or concomitantly with any formulation of sodium phenylbutyrate. Dosage is limited to 4.5 to 11.2 mL/m²/day (5 to 12.4 g/m²/day) in 3 equally divided doses, up to a maximum total daily dosage of 17.5 mL (19 g), with a quantity limit of 525 mL per 30 days. If switching from sodium phenylbutyrate, refer to prescribing information.

If all initial authorization requirements are met, coverage is approved for 6 months. If all reauthorization requirements are met, coverage is approved for an additional 12 months.

Ravicti (glycerol phenylbutyrate) is not medically necessary for the treatment of conditions not listed in this policy. For any other indication, refer to the applicable Off-Label policy.

Clinical Thresholds and Dosage Parameters

Hyperammonemia threshold

PopulationNeonates

Threshold150 µmol/L or higher

Converted threshold>260 µg/dL

Hyperammonemia threshold

PopulationOlder children and adults

Threshold>100 µmol/L

Converted threshold175 µg/dL

Authorization and Documentation Requirements

Prior Authorization

Initial Ravicti Prior Authorization Required

Pharmacy prior authorization is required. Ravicti (glycerol phenylbutyrate) is covered for chronic management of qualifying urea cycle disorders when all initial clinical and documentation requirements are met. Approval is for 6 months when criteria are satisfied.

Prescribed by or in consultation with a metabolic or genetic specialist.
Documented UCD with confirmatory genetic testing or enzyme activity assay results.
History of hyperammonemia: ≥150 µmol/L (>260 µg/dL) in neonates or >100 µmol/L (175 µg/dL) in older children and adults.
Used as adjunctive therapy to dietary protein restriction, with dietary supplements when clinically appropriate.
Qualifying sodium phenylbutyrate trial, no N-acetylglutamate synthase (NAGS) deficiency, no acute hyperammonemia treatment, and no concomitant sodium phenylbutyrate.
Dose: 4.5–11.2 mL/m²/day (5–12.4 g/m²/day) in 3 equally divided doses; maximum 17.5 mL (19 g) per day.
Quantity limit: 525 mL per 30 days. If switching from sodium phenylbutyrate, follow prescribing information.

Clinical Background

Urea cycle disorders (UCDs) are rare inherited deficiencies of enzymes involved in converting nitrogen waste to urea. When ureagenesis is impaired, nitrogen accumulates as ammonia, causing hyperammonemia, which is associated with neurologic manifestations and risk of brain damage. Ravicti is a phenylbutyrate-derived nitrogen-binding agent used for chronic UCD management when dietary protein restriction and, when needed, dietary supplements are insufficient.

Key Definitions

Urea cycle disorders

ConditionUrea cycle disorders are rare inherited deficiencies in any of the enzymes involved in the urea cycle.

Impaired processThe enzyme deficiency makes ureagenesis defective.

ResultAmmonia accumulates because nitrogen waste is not adequately cleared as urea.

Hyperammonemia

DefinitionHyperammonemia is a marker of inadequate nitrogen detoxification.

Clinical significanceIts severity strongly correlates with brain damage.

Common presentation

Initial Ravicti Therapy

Initial Ravicti Therapy Criteria

Covered for initial authorization when ALL of the following are met:

Initial Ravicti authorization

Specialist, diagnosis, and clinical need
- Ravicti is prescribed by, or in consultation with, a metabolic or genetic specialist; the member has a documented urea cycle disorder, such as a deficiency involving carbamylphosphate synthetase (CPS), ornithine transcarbamylase (OTC), or argininosuccinic acid synthetase (AS); and the diagnosis is confirmed by genetic testing or enzyme activity assay results.
- The member has a history of hyperammonemia meeting the applicable age-based threshold, and Ravicti is prescribed as adjunctive therapy to dietary protein restriction.150 µmol/L (>260 µg/dl) or higher in neonates; >100 µmol/L (175 µg/dl) in older children and adults
Required sodium phenylbutyrate trial

Ravicti Reauthorization

Ravicti Reauthorization Criteria

Covered for reauthorization when ALL of the following are met:

Continuation therapy

Documentation shows ongoing dietary management.
Chart notes document a positive response to therapy, such as normalized ammonia levels or a reduced number of hyperammonemic crises.

Required Prior Therapy

Step	Medication required before Ravicti	Qualifying outcome
1	Sodium phenylbutyrate trial	At least one of the following: severe taste-related intolerance causing vomiting or interfering with adherence; a qualifying contraindication, such as congestive heart failure, severe renal insufficiency, or sodium-retaining edema; or failure to normalize ammonia levels despite optimized dosing.

Ravicti Dose and Quantity Limits

Quantity limits

MedicationRavicti

Quantity limit525 mL

Limit periodPer 30 days

Standard dosage

Standard dosage range4.5 to 11.2 mL/m²/day

Equivalent range5 to 12.4 g/m²/day

Administration3 equally divided doses

Policy Summary

PayerCareSource Georgia Medicaid

PolicyRavicti (glycerol phenylbutyrate) for Urea Cycle Disorders

Policy CodePolicy GA-MED-P-366579

Change TypeMaterial revision

Effective DateFeb 1, 2026

Next Review DateN/A

Key ActionSubmit pharmacy prior authorization with confirmatory UCD diagnostic results and documentation of all initial coverage requirements.

SourceLink

On This Page

Daily dosage range4.5 to 11.2 mL/m²/day

Equivalent dosage range5 to 12.4 g/m²/day

Administration3 equally divided doses

Maximum total daily dosage

Maximum volume17.5 mL per day

Maximum amount19 g per day

Applies toMaximum total daily dosage

Quantity limit

MedicationRavicti

Quantity limit525 mL

PeriodPer 30 days

Ravicti Reauthorization

For reauthorization, the provider must demonstrate that Ravicti remains clinically appropriate for chronic UCD management. When the requirements are met, reauthorization is granted for an additional 12 months.

Document ongoing dietary management, including dietary protein restriction as clinically indicated.
Submit chart documentation of a positive response, such as normalized ammonia levels or a reduced number of hyperammonemic crises.

Unsupported block type: step_therapy

Documentation Required

Initial Authorization Documentation

Initial authorization records must establish specialist involvement, the qualifying UCD, confirmatory diagnosis, hyperammonemia history, dietary treatment, and the required prior sodium phenylbutyrate evaluation.

Prescriber is a metabolic or genetic specialist, or the medication is prescribed in consultation with one.
Include the documented UCD diagnosis, such as CPS, OTC, or argininosuccinic acid synthetase deficiency.
Provide genetic testing or enzyme activity assay results confirming the diagnosis.
Provide records documenting qualifying hyperammonemia and the use of Ravicti as an adjunct to dietary protein restriction.
Include sodium phenylbutyrate trial history and supporting records for intolerance, contraindication, or inadequate ammonia response.

Documentation Required

Reauthorization Documentation

Renewal requests must show that dietary management continues and that the member is benefiting from Ravicti therapy.

Submit current documentation of ongoing dietary management.
Include chart notes showing a positive response, such as normalized ammonia levels or fewer hyperammonemic crises.
Approved reauthorization duration is 12 months when requirements are met.

Denial Risk

Insufficient Diagnosis or Clinical Evidence

Coverage may be denied when the diagnosis or clinical evidence does not establish medical necessity under the policy.

No documented qualifying UCD diagnosis.
Genetic testing or enzyme activity assay confirmation is missing.
Qualifying history of hyperammonemia is not documented.
Ravicti is not being used as an adjunct to dietary protein restriction.
Required clinical records are incomplete or do not support the requested therapy.

Denial Risk

No Qualifying Sodium Phenylbutyrate Trial

Initial coverage may be denied when the member has not completed an adequate sodium phenylbutyrate trial or when the trial record does not support a qualifying exception.

No documented trial of sodium phenylbutyrate.
Taste intolerance is not severe enough to cause vomiting or interfere with adherence.
No qualifying contraindication is documented.
Ammonia levels were not shown to remain elevated despite optimized sodium phenylbutyrate dosing.

Denial Risk

Excluded Use or Prohibited Combination

Ravicti is not covered for excluded uses or in prohibited combinations under this policy.

Do not use for N-acetylglutamate synthase (NAGS) deficiency.
Do not use to treat acute hyperammonemia.
Do not use concomitantly with any formulation of sodium phenylbutyrate.

Denial Risk

Unlisted Indication

CareSource considers Ravicti not medically necessary for conditions or indications not addressed in this policy. For other indications, refer to the Off-Label policy.

Coverage is limited to chronic management of qualifying urea cycle disorders that meet the policy criteria.

Signs and symptoms can present at any age and are mainly neurologic.

Nitrogen-binding agent

AgentA nitrogen-binding or nitrogen-scavenging agent

FunctionRemoves excess nitrogen and ammonia from the body.

MechanismActs through an alternate pathway.

At least one qualifying outcome

Taste disturbance is severe enough to induce vomiting or interfere with adherence.
A contraindication exists, such as congestive heart failure, severe renal insufficiency, or a clinical state in which sodium retention with edema occurs.
Ammonia levels failed to normalize despite optimized sodium phenylbutyrate dosing.

Excluded uses and dosing requirements

The member does not have N-acetylglutamate synthase (NAGS) deficiency; Ravicti is not being used to treat acute hyperammonemia; and Ravicti is not being used concomitantly with any formulation of sodium phenylbutyrate.
The prescribed dose is within the policy limits.4.5 to 11.2 mL/m²/day (5 to 12.4 g/m²/day) in 3 equally divided doses; maximum total daily dosage of 17.5 mL (19 g); quantity limit of 525 mL per 30 days
If switching from sodium phenylbutyrate, see prescribing information.

Maximum total daily dosage

Maximum total daily volume17.5 mL

Maximum total daily amount19 g

MedicationRavicti