Prior Auth Protocol

FDA Approved Indications:

For use as a nitrogen-binding agent for chronic management of adult and pediatric patients ≥2 years of age with urea cycle disorders (UCDs) who cannot be managed by dietary protein restriction and/or amino acid supplementation alone. Ravicti must be used with dietary protein restriction and, in some cases, dietary supplements (i.e., essential amino acids, arginine, citrulline, protein-free calorie supplements).
- Limitations of Use: Ravicti is not indicated for treatment of acute hyperammonemia in patients with UCDs. Safety and efficacy for treatment of N-acetylglutamate synthase (NAGS) deficiency has not been established. The use of Ravicti in patients <2 months of age is contraindicated.

Health Net Approved Indications and Usage Guidelines:

Confirmed diagnosis of a urea cycle disorder (UCD) that cannot be managed by dietary protein restriction alone

Coverage is Not Authorized For:

Patients <2 months of age
Non-FDA approved indications, which are not listed in the Health Net Approved Indications and Usage Guidelines section, unless there is sufficient documentation of efficacy and safety in the published literature.

General Information:

Children <2 months of age may have immature pancreatic exocrine function, which could impair hydrolysis of Ravicti, leading to impaired absorption of Phenylbutyrate and hyperammonemia.

Therapeutic Alternatives:

Drug	Dosing Regimen	Dose Limit/ Maximum Dose
Buphenyl^R (sodium phenylbutyrate ) 500mg tablets Sodium Phenylbutyrate (Buphenyl^R) 3gm/dose powder	Children weighing less than 20 kg: 450-600 mg/kg/day PO Children weighing more than 20 kg and adults: 9.9-13 g/m²/day PO The tablets and powder are to be taken in equally divided amounts with each meal or feeding (i.e., three to six times per day).	20 grams (40 tablets) per day

* Requires Prior Authorization

Recommended Dosing Regimen and Authorization Limit:

Drug	Dosing Regimen	Authorization Limit
Ravicti	Initial Dosage in Phenylbutyrate-Naive Patients Recommended dosage range is 4.5 to 11.2 mL/m² PO QD (5 to 12.4 g/m²/day). For patients with some residual enzyme activity who are not adequately controlled with dietary restriction, starting dose is 4.5 mL/m²/day. Switching From Sodium Phenylbutyrate to Ravicti Daily dosage of Ravicti (mL) = daily dosage of sodium phenylbutyrate (g) x 0.86. Take with food and administer directly into mouth via oral syringe or dosing cup. Total daily dosage is given in 3 equally divided dosages, rounded up to nearest 0.5 mL. Maximum daily dosage is 17.5 mL (19 g). Must be used with dietary protein restriction.	Length of Benefit

Product Availability:

Oral liquid: 1.1 g/mL of glycerol phenylbutyrate (delivers 1.02 g/mL of phenylbutyrate) 25 ml glass bottles

References:

Ravicti [package insert] South San Francisco, CA: Hyperion Therapeutics; June, 2014.
Micromedex^R Healthcre Series [Internet database]. Greenwood Village, CO: Thomson Healthcare. Updated periodically. Accessed January 5, 2016.