Prescribing RAVICTI for your patients

Access treatment profiles, tools, and information that can help guide your decisions about RAVICTI® (glycerol phenylbutyrate) Oral Liquid

The RAVICTI choice

Evaluating RAVICTI for phenylbutyrate-naïve patients

Look over the patient treatment profile and administration features of RAVICTI

Sarah

A busy patient whose daily protein intake variesa

Characteristics:
22-year-old patient with ornithine transcarbamylase (OTC) deficiency
Current management:
Low-protein diet and prescription supplements
Other Considerations:
Works during the day, attends school at night; experiences nausea and fatigue

UCD management challenge:

“My patient is constantly on the go and her prescribed diet is not always sufficient to control ammonia.”

RAVICTI effectively controls ammonia with easy oral administration1



For patients with urea cycle disorders (UCDs), controlling ammonia is a process that typically includes dietary modifications, supplements, and sometimes nitrogen scavenger therapies. As the only oral liquid treatment for patients with UCDs with no pills or powders to prepare, RAVICTI is made to fit today’s busy lives.1

Selected Important Safety Information

RAVICTI must be used with dietary protein restriction and, in some cases, supplements (eg, essential amino acids, arginine, citrulline, protein-free calorie supplements).

Getting started: all it takes is 1 twist off and 1 twist on

Remove the childproof cap on the RAVICTI bottle by pushing down on the cap while twisting it to the left. Then, attach the blue AdaptaCap™ Bottle Adapter on to the bottle by twisting it to the right.

Fill the syringe: it’s as easy as “hold, flip, pull”

  • Hold the RAVICTI bottle securely as you place the tip of the oral dosing syringe into the AdaptaCap™ Bottle Adapter.
  • Flip the bottle upside down with the oral dosing syringe still inserted.
  • Pull the plunger of the oral dosing syringe back slowly to draw up the amount of RAVICTI prescribed by your doctor. Don’t forget to turn the bottle upright again. (Tip: Pulling slowly will help avoid large air bubbles from forming in the medicine.)

Squirt RAVICTI right into mouth

Once the oral dosing syringe is ready, place it into the mouth. Push the plunger to squirt all of the medicine directly into the mouth.

Wash RAVICTI down with water or food

Ensure the full dose of RAVICTI is swallowed completely by drinking liquid or eating some food. Breakfast, lunch, or dinner, RAVICTI can work with the timing of your patients’ meals.1

If you’re ready to prescribe RAVICTI to your patients, it’s important that you and your patients understand how to administer RAVICTI properly. Watch the videos below to review the complete directions for taking RAVICTI.

Selected Important Safety Information

  • 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.

Ready to start your patients on RAVICTI?

Review dosage guidelines for phenylbutyrate-naïve patients.

View Dosage Guide

Consider RAVICTI as an alternative to sodium phenylbutyrate (NaPBA)

Look over the patient treatment profile, see how RAVICTI works, and get helpful forms and tools for switching.

Patients switching from NaPBA to RAVICTI should receive the dosage of RAVICTI that contains the same amount of phenylbutyric acid.1

Jonas

A patient who has trouble eatinga

Characteristics:
18-month-old male with argininosuccinate lyase (ASL) deficiency
Current management:
Low-protein diet, prescription supplements, NaPBA powder
Other Considerations:
Patient does not like the smell and taste of NaPBA; caregiver reports “feeling stressed” about administering the medication

UCD management challenge:

“My patient does not like taking his medication, and his mother would prefer a more palatable option.”

RAVICTI offers convenient dosing, easy administration, and requires minimal preparation1,2

  • Nearly tasteless and odorless liquid2

  • No pill or powder preparation and minimal dosing steps required1

  • Taken with meals via oral dosing syringe1

  • RAVICTI is the only FDA-approved oral liquid for the treatment of patients with UCDs.1
  • RAVICTI can be taken orally, even in patients with a nasogastric tube or g-tube.1
    • For patients who require a volume of less than 1 mL per dose via nasogastric or gastrostomy tube, the delivered dose may be less than anticipated. Closely monitor these patients using ammonia levels.1
  • The total daily dosage of RAVICTI should be divided into equally divided doses according to age range.1
    • Patients 2 years of age and older: Give RAVICTI in 3 equally divided doses, each rounded up to the nearest 0.5 mL.1
    • Patients 2 months of age to less than 2 years: Give RAVICTI in 3 or more equally divided doses, each rounded up to the nearest 0.1 mL.1
  • The maximum daily dosage of RAVICTI (17.5 mL) is equivalent to 40 tablets of sodium phenylbutyrate.1,2

Selected Important Safety Information

RAVICTI must be used with dietary protein restriction and, in some cases, supplements (eg, essential amino acids, arginine, citrulline, protein-free calorie supplements).

BUPHENYL® (sodium phenylbutyrate tablets and powder) ISI | Prescribing Information | Medication Guide

Download this form to initiate switching

If your patients are ready to make the switch, complete the Patient Enrollment Form.

Patient enrollment form

Ready to start your patients on RAVICTI?

Review dosage guidelines and calculate the dose for patients currently taking NaPBA.

Visit Dosage Guide

Knowing when to modify the dose of RAVICTI

Look over the patient treatment profile and important biomarkers, and order a metabolite testing kit

Miranda

10-year-old female with carbamyl phosphate synthetase 1 (CPS1) deficiencya

Current management:
Low-protein diet, prescription supplements, RAVICTI
Other Considerations:
Has recently had a growth spurt and is experiencing elevations in ammonia

UCD management challenge:

“My patient is nearing adolescence and her fasting ammonia level is a bit elevated. Is there any way to ensure I am giving her the correct dosage of RAVICTI®?”

Order phenylbutyrate metabolite testing at no cost

With this test, you can determine your patient’s plasma PAA:PAGN ratio and urinary PAGN level.

Order Kit

When modifying the dosage of RAVICTI, it is important to assess these biomarkers

Normal ammonia levels: If patients experience symptoms of vomiting, nausea, headache, somnolence, or confusion in the absence of high ammonia levels or other intercurrent illnesses, reduce the RAVICTI dosage and monitor patients clinically. If available, obtain measurements of plasma phenylacetate (PAA) concentrations and the ratio of plasma PAA to phenylacetylglutamine (PAGN) to guide dosing.1

Elevated ammonia levels: When plasma ammonia is elevated, increase the RAVICTI dosage to reduce the fasting plasma ammonia level to less than half the upper limit of normal (ULN) in patients 6 years of age and older. In infants and pediatric patients (generally <6 years of age) in whom obtaining fasting ammonia is problematic due to frequent feedings, adjust the dosage to keep the first ammonia of the morning below the ULN.1,b

PAA and PAGN: PAA is the major metabolite of RAVICTI and is associated with neurotoxicity. The ratio of PAA to PAGN in plasma may provide additional information to guide dosing adjustments. A high PAA:PAGN ratio may indicate that saturation of the conjugation reaction to form PAGN. The PAA:PAGN ratio has been shown to be less than 1 in patients without significant PAA accumulation, while a ratio >2.5 may predict PAA levels nearing toxicity, which would necessitate dosage reduction.1,2

Urinary PAGN (U-PAGN): This measurement may be used to determine whether the RAVICTI dosage is providing enough ammonia control. Each gram of U-PAGN excreted over 24 hours covers waste nitrogen generated from 1.4 grams of dietary protein. If U-PAGN excretion is insufficient to cover daily dietary protein intake and the fasting ammonia is greater than half the ULN, the RAVICTI dosage should be adjusted upward. The amount of dosage adjustment should factor in the amount of dietary protein that has not been covered, as indicated by the 24-hour U-PAGN level and the estimated RAVICTI dose needed per gram of dietary protein ingested, and the maximum total daily dosage (ie, 17.5 mL).1

Consider a patient’s use of concomitant medications, such as probenecid, when making dosing adjustment decisions based on urinary PAGN and PAA. Probenecid may result in a decrease of the urinary excretion of PAGN.1

The maximum total daily dosage for RAVICTI is 17.5 mL (19 g).1

Assess how your patients are doing on RAVICTI

This assessment tool can help you evaluate treatment of your patients and ensure that they are staying on track with their UCD management.

Patient assessment follow-up

Should you modify the RAVICTI dosage?

Learn more about optimizing RAVICTI dosage and how changes in body surface area may impact the appropriate dosage.

Visit Dosage Guide

aHypothetical case study.

bInstruct patients to abstain from eating or drinking any foods or liquids 4 to 6 hours before plasma ammonia levels are to be measured.3

References:    1. RAVICTI [package insert]. Lake Forest, IL: Horizon Pharma USA, Inc.; 2017.   2. Mokhtarani M, Diaz GA, Rhead W, et al. Mol Genet Metab. 2013;110(4):446-453. doi:10.1016/j.ymgme.2013.09.017.   3. Häberle J. Eur J Pediatr. 2011;170(1):21-34. doi:10.1007/s00431-010-1369-2.

Prescribing RAVICTI for your patients

Access treatment profiles, tools, and information that can help guide your decisions about RAVICTI® (glycerol phenylbutyrate) Oral Liquid

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