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Here are tools that may be shared with your patients to help them learn more about RAVICTI
HIPAA Form
Use this form for authorization to use or provide patient-protected health information.
HIPAA Form (Spanish)
A Spanish-language form for authorization to use or provide patient-protected information.
Administering RAVICTI by Mouth
Directions for taking RAVICTI by mouth.
Administering RAVICTI by MouthDirections for taking RAVICTI by mouth.
Directions for taking RAVICTI by mouth.
View video transcriptWelcome to the RAVICTI® (glycerol phenylbutyrate) Oral Liquid instructional dosing video.
In this video you will see step-by- step instructions for the oral administration of RAVICTI for you or your child.
Use for RAVICTI® (glycerol phenylbutyrate) Oral Liquid
RAVICTI is a prescription medicine used for long-term management of high blood levels of ammonia (hyperammonemia) caused by a condition called a urea cycle disorder (UCD). RAVICTI should be used if the UCD cannot be managed with a low-protein diet and dietary supplements alone. RAVICTI must be used along with a low-protein diet and in some cases, dietary supplements.
RAVICTI is not used to treat extremely high levels of ammonia in the blood (hyperammonemic crisis) in people with UCDs.
It is not known if RAVICTI is safe and effective for the treatment of N-acetylglutamate synthase (NAGS) deficiency.
Please listen to the RAVICTI Important Safety Information at the end of this video. For additional important safety information, see the Medication Guide at RAVICTI.com and discuss with your doctor.
This video is intended to complement the instructions you have already received from your doctor. Always take RAVICTI exactly as directed by your doctor.
Do not mix RAVICTI with formula or any other liquids.
Now, let’s get started.
Your RAVICTI shipment includes a 25-mL bottle of RAVICTI, a blue AdaptaCap® Bottle Adapter, and an oral dosing syringe.
When dosing RAVICTI, be sure to use the oral dosing syringe provided with the product.
First, remove the childproof cap on the RAVICTI bottle. To do that, push down on the cap while twisting it to the left.
Then, attach the blue AdaptaCap® Bottle Adapter onto the bottle. Put it on top of the bottle and twist it to the right.
Make sure it is secured tightly on the bottle.
While holding the RAVICTI bottle securely, place the tip of the oral dosing syringe into the AdaptaCap®Bottle Adapter.
Turn the bottle upside down with the oral dosing syringe still inserted.
Now you’re ready to fill the oral dosing syringe with RAVICTI.
Get the medicine into the oral dosing syringe by pulling the plunger back slowly. Pulling slowly will help avoid large air bubbles from forming in the medicine.
Pull the plunger back until the first line of the black rubber stopper is even with the dosing mark. The dosing marks are on the barrel of the oral dosing syringe. Match the dosing mark with the amount of medicine prescribed by your doctor.
Then, turn the bottle upright again.
If there are large air bubbles, push the medicine back into the bottle. Do this slowly.
Then draw up the medicine again.
Once you have your oral dosing syringe ready, place it into the mouth. Push the plunger to squirt the medicine directly into the mouth.
Make sure all of the medicine is pushed out of the oral dosing syringe and swallowed.
Put the cap on the oral dosing syringe.
Drink liquid or eat some food right after taking RAVICTI.
This helps ensure that all of the medicine is swallowed.
Close the tab on the AdaptaCap® Bottle Adapter.
Make sure it’s closed tightly.
You can use 1 oral dosing syringe and 1 blue AdaptaCap® Bottle Adapter for all doses each day. You can also choose to use a new oral dosing syringe with each dose. Do not rinse the AdaptaCap® Bottle Adapter or oral dosing syringe between doses.
After the last dose of the day, dispose of the oral dosing syringe.
If you take too much RAVICTI, call your doctor or go to the nearest hospital emergency room right away.
You should consult your physician if you have missed a dose of RAVICTI.
Do not mix RAVICTI with formula or any other liquids.
Administering RAVICTI by Nasogastric or G-Tube
Directions for taking RAVICTI by nasogastric or G-tube.
Administering RAVICTI by Nasogastric or G-TubeDirections for taking RAVICTI via nasogastric or gastronomy feeding tube
Directions for taking RAVICTI by nasogastric or G-tube.
View video transcriptWelcome to the RAVICTI® (glycerol phenylbutyrate) Oral Liquid instructional dosing video.
In this video, you will see step-by-step instructions for administration of RAVICTI if you or your child have a nasogastric or gastrostomy feeding tube.
If you can swallow liquids, it is recommended you take RAVICTI by mouth.
Use for RAVICTI® (glycerol phenylbutyrate) Oral Liquid
RAVICTI is a prescription medicine used for long-term management of high blood levels of ammonia (hyperammonemia) caused by a condition called a urea cycle disorder (UCD). RAVICTI should be used if the UCD cannot be managed with a low-protein diet and dietary supplements alone. RAVICTI must be used along with a low-protein diet and in some cases, dietary supplements.
RAVICTI is not used to treat extremely high levels of ammonia in the blood (hyperammonemic crisis) in people with UCDs
.
It is not known if RAVICTI is safe and effective for the treatment of N-acetylglutamate synthase (NAGS) deficiency.
Please listen to the RAVICTI Important Safety Information at the end of this video. For additional important safety information, see the Medication Guide at RAVICTI.com and discuss with your doctor.
This video is intended to complement the instructions you have already received from your doctor. Always take RAVICTI exactly as directed by your doctor.
Do not mix RAVICTI with formula or any other liquids.
Now, let’s get started.
Your RAVICTI shipment includes: a 25-mL bottle of RAVICTI, a blue AdaptaCap®Bottle Adapter, and an oral dosing syringe.
When dosing RAVICTI, be sure to use the oral dosing syringe provided with the product.
First, remove the childproof cap on the RAVICTI bottle. To do that, push down on the cap while twisting it to the left.
Then, attach the blue AdaptaCap® Bottle Adapter onto the bottle. Put it on top of the bottle and twist it to the right.
Make sure it is secured tightly on the bottle.
While holding the RAVICTI bottle securely, place the tip of the oral dosing syringe into the AdaptaCap®Bottle Adapter.
Turn the bottle upside down with the oral dosing syringe still inserted.
Now you’re ready to fill the oral dosing syringe with RAVICTI.
Get the medicine into the oral dosing syringe by pulling the plunger back slowly. Pulling slowly will help avoid large air bubbles from forming in the medicine.
Pull the plunger back until the first line of the black rubber stopper is even with the dosing mark. The dosing marks are on the barrel of the oral dosing syringe. Match the dosing mark with the amount of medicine prescribed by your doctor.
Then, turn the bottle upright again.
If there are large air bubbles, push the medicine back into the bottle. Do this slowly.
Then draw up the medicine again.
Next, place the tip of the oral dosing syringe into the smaller port of the feeding tube. The smaller port of the nasogastric or gastrostomy feeding tube is used for medicine. Ask your doctor if you are not sure which port to use.
Using the plunger of the oral dosing syringe, slowly and carefully push RAVICTI into the feeding tube. Make sure all of the medicine is pushed out of the oral dosing syringe.
After you have pushed all of the RAVICTI into the feeding tube, remove the oral dosing syringe, put the cap on, and set it aside.
Close the tab on the AdaptaCap® Bottle Adapter. Make sure it’s closed tightly.
Use a separate large syringe to flush water into the feeding tube. A large syringe can be found in the box containing your feeding tube.
Make sure all of the water or formula drains through the feeding tube.
You can use 1 oral dosing syringe and 1 blue AdaptaCap® Bottle Adapter for all doses each day. You can also choose to use a new oral dosing syringe with each dose. Do not rinse the AdaptaCap® Bottle Adapter or oral dosing syringe between doses.
After the last dose of the day, dispose of the oral dosing syringe.
If you take too much RAVICTI, call your doctor or go to the nearest hospital emergency room right away.
You should consult your physician if you have missed a dose of RAVICTI.
For patients who require a volume of less than 1 mL per dose via nasogastric or gastrostomy tube, the delivered dosage may be less than anticipated due to adherence of RAVICTI to the plastic tubing. Therefore, these patients should be closely monitored using ammonia levels following initiation of RAVICTI dosing or dosage adjustments.
Horizon By Your Side
A patient support program for patients prescribed RAVICTI.
The dedicated members of the Horizon By Your Side team provide personalized service to patients living with UCDs.
The Patient Access Liaison (PAL) provides dedicated, one-on-one support for patients. They work directly with individual patients to answer nonmedical, logistical questions, and provide support upon enrollment. Additionally, the PAL educates about navigating insurance processes and accessing treatment on your patient’s behalf. The PAL has the expertise and tools to support patients by educating on patient benefits, prior authorization requirements, payor policies, and coding and claim submissions.
These services are offered to RAVICTI patients upon enrollment with the Patient Enrollment Form (PEF) and completed patient consent. The services are built around 3 components: connect, coordinate and champion.
The Horizon By Your Side team can:
It’s easy to
reach us.
Call 1-855-UCD-SUPT (1-855-823-7878) Monday–Friday 9 AM–8 PM (EST)
- Connect your patients to others living with UCDs through live events, peer mentor programs, and online resources
- Provide tools and resources to help your patients manage the day-to-day challenges of living with UCDs
- Introduce patients and their families to UCD advocacy groups for more support and inform them of
UCD-related events in their area
Coordinate
- Help patients understand their coverage and address financial barriers
- Assist in connecting patients with their specialty pharmacy to schedule the shipment of their medicine so they avoid running out of supply
Champion
- Serve as a dedicated personal resource and the main point of contact for your patients’ ongoing nonmedical, logistical needs
- Provide your patients with RAVICTI education and answer their nonclinical questions
- Help patients through changes that may impact their treatment
It’s easy to
reach us.
Call 1-855-UCD-SUPT (1-855-823-7878) Monday–Friday 9 AM–8 PM (EST)
UCD Mentors at RAVICTI.com
UCD Mentors are real patients and caregivers who understand what it means to live with a UCD. UCD Mentors are ready to connect with patients, share their experiences, and answer questions.
Explore other organizations that may provide support for patients with UCD
Your patients may be interested in learning more about independent organizations that are dedicated to improving the lives of patients with UCDs through support, education, advocacy, and research.
Find a Metabolic
Specialist
Locate a specialist experienced in treating patients with UCD near you or your patient
View Practice
Resources
Access resources for your practice to help in the management of UCDs
Order a Metabolite
Testing Kit
Receive a phenylbutyrate metabolite testing kit at no cost
Stay informed about RAVICTI
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.
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.