Prescribing Information (PDF) 
Medication Guide (PDF)

Indication and Usage

RAVICTI is indicated 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 (eg, essential amino acids, arginine, citrulline, protein-free calorie supplements).

LIMITATIONS OF USE:

• RAVICTI is not indicated for the treatment of acute hyperammonemia in patients with UCDs because more rapidly acting interventions are essential to reduce plasma ammonia levels.
• The safety and efficacy of RAVICTI for the treatment of N-acetylglutamate synthase (NAGS) deficiency has not been established.
• The use of RAVICTI in patients <2 months of age is contraindicated.

Important Safety Information

WARNINGS AND PRECAUTIONS:

• Neurotoxicity: Phenylacetate (PAA), a major metabolite of RAVICTI, may be toxic at levels ≥500 μg/mL. Reduce RAVICTI dosage if symptoms of neurotoxicity, including vomiting, nausea, headache, somnolence, confusion, or sleepiness, are present in the absence of high ammonia or other intercurrent illnesses.
• Reduced Phenylbutyrate Absorption in Pancreatic Insufficiency or Intestinal Malabsorption: Monitor ammonia levels closely.

ADVERSE EVENTS:

• The most common adverse reactions (occurring in ≥10% of adult patients) reported during short-term and long-term treatment with RAVICTI were diarrhea, flatulence, and headache; and nausea, vomiting, diarrhea, decreased appetite, hyperammonemia, dizziness, headache and fatigue, respectively.
• Adverse events occurring in ≥10% of pediatric patients during long-term treatment with RAVICTI were upper abdominal pain, rash, nausea, vomiting, diarrhea, decreased appetite, hyperammonemia, and headache.

DRUG INTERACTIONS:

• Corticosteroids, valproic acid, or haloperidol: May increase plasma ammonia level. Monitor ammonia levels closely.
• Probenecid: May affect renal excretion of metabolites of RAVICTI, including PAGN and PAA.

SPECIAL POPULATION:

• Pregnancy: Based on animal data, may cause fetal harm.
• Nursing Mothers: Discontinue nursing or discontinue the drug.

To learn more about RAVICTI, please see the full Prescribing Information (PDF), Medication Guide (PDF), press release (PDF), and visit the website.

RAVICTI is a trademark of Hyperion Therapeutics, Inc.