Product Details

Ravicti

Glycerol Phenylbutyrate
1.1 mg/mL
Oral Liquid
25-mL Glass Bottle

DIN/PIN/NPN

02453304

Manufacturer

Horizon Therapeutics Ireland DAC

Formulary Listing Date

2018-04-09  

Unit Price

48.0000

Amount MOH Pays

48.0000

Coverage Status

Exceptional Access Program Product

ODB Formulary Therapeutic Classification

Therapeutic Note

NO

ATC Code

A16AX09

Interchangeable Products

NO  

LU Clinical Criteria

NO  

EAP Criteria

Therapeutic Class Reimbursement Criteria
Metabolic/Genetic Modifiers

Glycerol phenylbutyrate

  • Brand(s): Ravicti
  • Dosage Form/Strength: 1.1g/mL-25mL bottle

For the management of patients with chronic urea cycle disorders (UCD) who meet all the following criteria: 

  1. Glycerol phenylbutyrate is being used as a nitrogen binding agent; AND 

  2. Patient has demonstrated that they cannot be managed by dietary protein restriction and/or amino acid supplementation alone1; AND 

  3. Patient is under the care of a physician with expertise in the treatment of patients with UCD or in consultation with a physician with this expertise. 

1The initial request should include levels for blood ammonia and glutamine levels demonstrating inadequate effects of protein restriction or amino acid supplementation. 

Exclusion Criteria: 

  • Is not used in combination with other forms of phenylbutyrate 

  • Will not be funded for patients who are not using a low protein diet while on treatment 

  • Not funded for the management of acute hyperammonemia 

  • Not funded for patients under 2 months of age

Recommended dose: 5 g/m2 to 12.4 g/m2 per day

Approval duration: 1 year 

Renewal Criteria: 

Renewals will be considered in patients who demonstrate benefit from treatment2 and who have not developed unacceptable toxicities requiring discontinuation. 

2At the time of renewal, please provide recent (within 3 months) blood ammonia and glutamine levels while on treatment and address the number and severity of hyperammonemic events experienced while on treatment in the previous 12 months and any treatment emergent events requiring urgent care or hospitalization. 

First renewal: 1 year

Subsequent renewals: 2 years

EAP Drug Request Form:

Standard Form for EAP Drug Requests

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