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Physician Resources
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Patient Resources - Savings & Support
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Savings & Support - Indications & Usage
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INDICATIONS AND USAGE
RAVICTI is a nitrogen-binding agent indicated for chronic management of patients 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.
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.
Please see Full Prescribing Information for complete details.
- Dosage & Administration
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DOSAGE AND ADMINISTRATION
- RAVICTI should be prescribed by a physician experienced in management of UCDs. For administration and preparation, see Full Prescribing Information for more details.
Switching From Sodium Phenylbutyrate Tablets or Powder to RAVICTI:
- Patients should receive the dosage of RAVICTI that contains the same amount of phenylbutyric acid, see Full Prescribing Information for conversion.
Initial Dosage in Phenylbutyrate-Naïve Patients:
- Recommended dosage range is 4.5 to 11.2 mL/m2 /day (5 to 12.4 g/m2 /day).
- For patients with some residual enzyme activity not adequately controlled with dietary restriction, the recommended starting dose is 4.5 mL/m2 /day.
- Take into account patient's estimated urea synthetic capacity, dietary protein intake, and diet adherence, see Full Prescribing Information for more details.
Dosage Adjustment and Monitoring:
- Follow plasma ammonia levels to determine the need for dosage titration, see Full Prescribing Information for more details.
Dosage Modifications in Patients with Hepatic Impairment:
- For patients with moderate to severe hepatic impairment, the recommended starting dosage is at the lower end of the recommended dosing range (4.5 mL/m2/day) and the dosage should be kept at the lowest necessary to control the patient’s plasma ammonia. See Full Prescribing Information for more details.
DOSAGE FORMS AND STRENGTHS
Oral liquid: 1.1 g/mL of glycerol phenylbutyrate (delivers 1.02 g/mL of phenylbutyrate). See Full Prescribing Information for more details.
Please see Full Prescribing Information for complete details.
- Contraindications
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CONTRAINDICATIONS
Known hypersensitivity to phenylbutyrate.
Please see Full Prescribing Information for complete details.
- Warnings & Precautions
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WARNINGS AND PRECAUTIONS
- Neurotoxicity: Phenylacetate (PAA), the active moiety of RAVICTI, may be neurotoxic. If symptoms of vomiting, nausea, headache, somnolence or confusion are present in the absence of high ammonia or other intercurrent illness which explains these symptoms, consider the potential for PAA neurotoxicity which may need reduction in the RAVICTI dosage.
- Pancreatic Insufficiency or Intestinal Malabsorption: Low or absent pancreatic enzymes or intestinal disease resulting in fat malabsorption may result in reduced or absent digestion of RAVICTI and/or absorption of phenylbutyrate and reduced control of plasma ammonia. Monitor ammonia levels closely in patients with pancreatic insufficiency or intestinal malabsorption.
Please see Full Prescribing Information for complete details.
- Adverse Reactions
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ADVERSE REACTIONS
Most common adverse reactions (≥10%) in adults are: diarrhea, flatulence, and headache.
To report SUSPECTED ADVERSE REACTIONS, contact Horizon at 1- 866-479-6742 or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.
Please see Full Prescribing Information for complete details.
- Drug Interactions
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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 phenylacetylglutamine (PAGN) and Phenylacetate (PAA).
- CYP3A4 Substrates with narrow therapeutic index (e.g., alfentanil, quinidine, cyclosporine): RAVICTI may decrease exposure of CYP3A4 substrates; monitor for decreased efficacy of the narrow therapeutic index drug.
- Midazolam: Decreased exposure of midazolam; monitor for suboptimal effect of midazolam.
Please see Full Prescribing Information for complete details.
- Use in Specific Populations
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USE IN SPECIFIC POPULATIONS
Lactation: Breastfeeding is not recommended due to lack of data.
Please see Full Prescribing Information for complete details.
The information provided in this site is intended only for US healthcare professionals.
RAVICTI and the HORIZON logo are trademarks owned by or licensed to Horizon. All other trademarks are the property of their respective owners.
© 2023 Horizon Therapeutics plc P-RVT-US-00215 09/23
Please see Full Prescribing Information.
INDICATION AND IMPORTANT SAFETY INFORMATION
INDICATION
RAVICTI (glycerol phenylbutyrate) Oral Liquid is indicated for use as a nitrogen-binding agent for chronic management of patients with urea cycle disorders (UCDs) who cannot be managed by dietary protein restriction and/or supplementation alone. RAVICTI must be used with dietary protein restriction and, in some cases, dietary supplements (e.g. 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.
IMPORTANT SAFETY INFORMATION
CONTRAINDICATIONS
- Patients with known hypersensitivity to phenylbutyrate: Reactions include wheezing, dyspnea, coughing, hypotension, flushing, nausea, and rash.
WARNINGS AND PRECAUTIONS
- Neurotoxicity: Phenylacetate (PAA), the major metabolite of RAVICTI, may be toxic at levels of 500 micrograms/mL or greater. If symptoms of vomiting, nausea, headache, somnolence, or confusion, are present in the absence of high ammonia or other intercurrent illness which explains these symptoms, consider the potential for PAA neurotoxicity which may need reduction in the RAVICTI dosage.
- Pancreatic Insufficiency or Intestinal Malabsorption: Low or absent pancreatic enzymes or intestinal disease resulting in fat malabsorption may result in reduced or absent digestion of RAVICTI and/or absorption of phenylbutyrate and reduced control of plasma ammonia. Monitor ammonia levels closely.
ADVERSE REACTIONS
The most common adverse reactions reported in clinical trials (at least 10% of patients) were:
- Adult patients: diarrhea, flatulence, and headache occurred during 4-week treatment (n=45) with RAVICTI; nausea, vomiting, diarrhea, decreased appetite, dizziness, headache, and fatigue occurred during 12-month treatment (n=51) with RAVICTI.
- Pediatric patients ages 2 to 17 years: upper abdominal pain, rash, nausea, vomiting, diarrhea, decreased appetite, and headache occurred during 12-month treatment (n=26) with RAVICTI.
- Pediatric patients ages 2 months to less than 2 years: neutropenia, vomiting, constipation, diarrhea, pyrexia, hypophagia, cough, nasal congestion, rhinorrhea, rash, and papule occurred during 12-month treatment (n=17) with RAVICTI.
- Pediatric patients less than 2 months of age: vomiting, rash, gastroesophageal reflux, increased hepatic enzymes, feeding disorder (decreased appetite, hypophagia), anemia, cough, dehydration, metabolic acidosis, thrombocytosis, thrombocytopenia, neutropenia, lymphocytosis, diarrhea, flatulence, constipation, pyrexia, lethargy, and irritability/agitation occurred during 24-month treatment (n=16) with RAVICTI.
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 phenylacetylglutamine (PAGN) and PAA.
- CYP3A4 substrates with narrow therapeutic index (eg, alfentanil, quinidine, cyclosporine): RAVICTI may decrease exposure to the concomitant drug.
- Midazolam: Use of RAVICTI decreased exposure of midazolam with concomitant use.
USE IN SPECIFIC POPULATIONS
- Pregnancy: RAVICTI should be used with caution in patients who are pregnant or planning to become pregnant. Based on animal data, RAVICTI may cause fetal harm. Report pregnancies to Horizon at 1‐866‐479‐6742.
- Lactation: breastfeeding is not recommended during treatment with RAVICTI. There are no data on the presence of RAVICTI in human milk, the effects on the breastfed infant, nor the effects on milk production.
Please see Full Prescribing Information.