Ravicti (glycerol phenylbutyrate) | Ravicti cost | Ravicti price 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 (glycerol phenylbutyrate) | Ravicti cost | Ravicti price 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 (glycerol phenylbutyrate) | Ravicti cost | Ravicti price.
• 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 (glycerol phenylbutyrate) | Ravicti cost | Ravicti price.
• 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 (glycerol phenylbutyrate) | Ravicti cost | Ravicti price.
• 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 (glycerol phenylbutyrate) | Ravicti cost | Ravicti price.

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 (glycerol phenylbutyrate) | Ravicti cost | Ravicti price, including phenylacetylglutamine (PAGN) and PAA.
• CYP3A4 substrates with narrow therapeutic index (eg, alfentanil, quinidine, cyclosporine): Ravicti (glycerol phenylbutyrate) | Ravicti cost | Ravicti price may decrease exposure to the concomitant drug.
• Midazolam: Use of Ravicti (glycerol phenylbutyrate) | Ravicti cost | Ravicti price decreased exposure of midazolam with concomitant use.

Use and Important Safety Information

What is the most important safety information I should know about RAVICTI?
RAVICTI may cause serious side effects, including:
Nervous system side effects (Neurotoxicity) Phenylacetate (PAA), a breakdown product of RAVICTI, may cause nervous system side effects. Call your doctor or get medical help right away if you have any of these symptoms while taking RAVICTI:

Your doctor may do blood tests to measure the amount of PAA in your blood during your treatment with RAVICTI.

What is RAVICTI?

Do not take RAVICTI if you are allergic to phenylbutyrate. Call your doctor or go to the nearest hospital emergency room if you have wheezing, shortness of breath, cough, low blood pressure, flushing, nausea or a rash while taking Ravicti (glycerol phenylbutyrate) | Ravicti cost | Ravicti price.

Before taking RAVICTI, tell your doctor about all of your medical conditions, including if you:

• have liver or kidney problems.
• have pancreas or bowel (intestine) problems.
• are pregnant or plan to become pregnant. It is not known if Ravicti (glycerol phenylbutyrate) | Ravicti cost | Ravicti price will harm your unborn baby. If you become pregnant during treatment with RAVICTI, call Horizon at 1‐866‐479‐6742 to report the pregnancy.
• are breastfeeding or plan to breastfeed. It is not known if Ravicti (glycerol phenylbutyrate) | Ravicti cost | Ravicti price passes into your breast milk. Breastfeeding is not recommended during treatment with RAVICTI. Talk to your doctor about the best way to feed your baby if you take Ravicti (glycerol phenylbutyrate) | Ravicti cost | Ravicti price.

What are possible side effects of Ravicti (glycerol phenylbutyrate) | Ravicti cost | Ravicti price?

Ravicti (glycerol phenylbutyrate) | Ravicti cost | Ravicti price may cause serious side effects, including:

• See “What is the most important information I should know about Ravicti (glycerol phenylbutyrate) | Ravicti cost | Ravicti price?”

The most common side effects of Ravicti (glycerol phenylbutyrate) | Ravicti cost | Ravicti price in adults include:

• diarrhea
• gas
• headache
• abdomen (stomach) pain

• vomiting
• tiredness
• decreased appetite
• indigestion or heartburn

The most common side effects of Ravicti (glycerol phenylbutyrate) | Ravicti cost | Ravicti price in children 2 years to 17 years of age include:

• upper abdomen (stomach) pain
• rash
• nausea
• vomiting

• diarrhea
• decreased appetite
• headache

The most common side effects of RAVICTI in children 2 months to less than 2 years of age include:

• low white blood cell count (neutropenia)
• vomiting
• constipation
• diarrhea
• fever

• reduced food intake
• cough
• stuffy nose
• runny nose
• skin rash
• small round bumps on the skin

The most common side effects of RAVICTI in children less than 2 months of age include:

• vomiting
• rash
• gastroesophageal reflux
• increased levels of liver enzymes in the blood
• decreased appetite and reduced food intake
• low red blood cell count (anemia)
• cough
• loss of too much body fluid (dehydration)
• too much acid in the blood (acidosis)
• high blood platelet count (thrombocytosis)

• low blood platelet count (thrombocytopenia)
• low blood neutrophil count (type of white blood cell) (neutropenia)
• high white blood cell count (lymphocytosis)
• diarrhea
• gas
• constipation
• fever
• drowsiness (lethargy)
• irritability
• agitation