What is Ravicti?
Ravicti helps eliminate nitrogen by binding with substances in the kidneys and liver. Hyperammonemia is a buildup of ammonia that can be caused by excess nitrogen. Ammonia can be toxic in the blood and tissues and cause brain damage, coma, or death.Ravicti prevents ammonia from building up in the blood of adults and children who have urea cycle disorders. This medicine does not treat hyperammonemia.The treatment of Ravicti usually includes a low-protein diet and, in some cases, dietary supplements.
Side effects of Ravicti
If you experience any of the following symptoms of an allergic response to Ravicti, you should seek immediate medical attention: itching, coughing, wheezing, or difficulty breathing; feeling as if you may pass out; swelling of your lips, face, tongue, or throat.
If you notice any symptoms of hyperammonemia (high blood ammonia levels), call your doctor immediately.
- Weakness is a lack of energy.
- Thinking problems, behavioral changes, feeling irritable
- Breathing problems
- Feeding problems or other issues
- Seizure (convulsions).
Ravicti may affect your nervous system. If you experience:
- Drowsiness is a feeling of unusual fatigue.
- Memory problems
- A feeling of lightheadedness, as if you could pass out.
- Confusion, ongoing headache, vomiting;
- You may experience numbness or burning in your feet or hands.
- Changed sense of taste
- Hearing problems are a common problem.
Some of the common side effects of Ravicti include:
- Occasionally headache
- Nausea, vomiting, diarrhea;
- Loss of appetite, stomach pain, and gas;
- Dizziness, tiredness;
- Rash;
- Fever, cold or flu symptoms (in children under 2 years), cough, and small bumps on the skin
There may be other side effects. For medical advice regarding side effects, always contact your physician first. For reporting side effects to the FDA directly, call them at 1-800-FDA-1088.
Warnings
Ravicti may affect your nervous system. Contact your doctor immediately if you experience confusion, headaches, or memory problems. You experience nausea.
Before you take this drug
If you have an allergy to glycerol-phenylbutyrate, then you should avoid using Ravicti.
Tell your doctor about any of the following to ensure that Ravicti will be safe for you:
- Liver or kidney disease
- A pancreas disorder;
- Stomach or intestinal disorder
- A condition known as NAGS deficiency (N-acetylglutamate synthase); or
- If you are taking other medications,
Unknown is whether glycerol-phenylbutyrate can harm a baby.Notify your doctor immediately if you are pregnant or planning on becoming pregnant.You may find your name on a registry if you get pregnant. This will allow you to monitor the pregnancy's outcome and evaluate the effects of glycerol and phenylbutyrate.Ravicti should not be used while breastfeeding.
How to take Ravicti?
Follow the directions on your prescription label and read all medication guides or instructions sheets. Read all the instructions and directions that come with your prescription. Sometimes your doctor will change the dose.Ravicti can be taken up to three times a day. Take it with food or infant formula.When breastfeeding a child who is taking the medicine, administer each dose just before nursing.Measuring Ravicti liquid with care is important. Use the provided dosing device or a dose-measuring tool (not a spoon).The following is how to administer Ravicti through a gastric or nasogastric feeding tube: Measuring the correct dosage in an oral syringe and attaching it to the tube, then pushing the plunger to empty the syringe. Allow the tube to drain after flushing it with 10 milliliters of water. Then, add 10 mL of water to the tube and rinse it down.Your dose will change if you switch from sodium butyrate to Ravicti. Keep your doctor's instructions in mind at all times.
The doses of glycerol and phenylbutyrate are determined by the body surface area, which is a combination of height and weight. You may need to adjust your dose if you lose or gain weight, or if you're still growing.Ravicti may require you to adhere to a special eating plan. Please follow all directions from your doctor or dietitian. Learn which foods you should eat and avoid in order to control your condition.An ammonia buildup in the bloodstream can cause death or brain damage. You'll need to have frequent blood tests. A urea-cycle disorder requires that you remain under the supervision of your doctor.You may find your name on the registry for urea cycle disorder. The registry is used to gather information on people who have these disorders and to assess the effectiveness of treatment.Store Ravicti away from moisture, heat, and humidity at room temperature.
Details on dosage
Adult dose of Ravicti to treat urea cycle disorders:
Initial Doses
Dosage: 3 doses, evenly divided and rounded to the nearest 0.5 mL.
Phenylbutyrate-Naive Patients: 4.5 to 11.2 mL/m2/day (5 to 12.4 g/m2/day)
Phenylbutyrate-Naive Patients with Some Residual Enzyme Activity: 4.5 mL/m2/day
Patients switching from sodium phenylbutyrate should use the following conversion:
Tables: Total daily dosage of glycerol butyrate (mL) = total daily dose (g) of sodium phenylbutyrate tablet (g)
Powder: Total daily dose (mL) of glycerol butyrate = total daily dose (g) of sodium phenylbutyrate.
MAINTENANCE DOSES: Monitor clinically and with plasma ammonia levels when changing doses; for those experiencing increased fasting plasma ammonia levels, increase dose to bring fasting plasma ammonia down below half the upper limit of normal; if available, utilize PAA:PAGN ratio measurement or urinary phenylacetylglutamine measurements as additional dosing guides; additionally product labeling may provide useful additional details.
MAXIMUM DOSE: 17.5 mg (19 g). Take one 17.5 milliliter capsule daily.
The usual pediatric dose of Ravicti to treat urea cycle disorders is:
Doses for Initial Doses
Patients less than 2 years of age: Give 3 or more doses equally divided, rounded to the nearest 0.01 mL.
Patients 2 years and older: Give 3 doses equally divided, rounded to the nearest 0.5 mL.
Phenylbutyrate Naive Patients: 4.5–11.2 mL/m2/day (5.0–12.4 g/m2/day).
Patients who are phenylbutyrate naive but have some residual enzyme activity: 4.5mL/m2/day
If you are switching from sodium phenylbutyrate to another drug, please use this conversion:
Tables: Total daily dosage of glycerol butyrate (mL) = total daily dose (g) of sodium phenylbutyrate tablet (g)
Powder: Total daily dose (mL) of glycerol butyrate = total daily dose (g) of sodium phenylbutyrate.
MAINTENANCE DOSES:
Patients 6 years of age or older: follow clinically and with plasma ammonia levels, especially with changing doses; for elevated plasma ammonia, increase dose to maintain fasting plasma ammonia to Dosage should not exceed half the upper limit of normal; using PAA-to-PAGN ratio and urinary phenylacetylglutamine measurements as guidelines, dosing will become easier;product labeling may be consulted for additional information.
Patients less than six years old: Follow the above instructions. However, if fasting ammonia cannot be obtained due to frequent feedings, then adjust the dose so that the first morning ammonia remains below the upper age limit.
MAXIMUM DOSE: 17.5 mg (19 grams) per day.
Comments:
Consider the residual urea synthesizing capacity of the patient, the dietary requirements for protein, and the adherence to the diet when determining the initial dose. You may use an estimated dose of 0.6 mL per gram ingested of dietary proteins per 24-hour period.
Use with protein-restricted diets and, in some cases, with supplements (e.g., essential amino acids; arginine; citrulline; calorie-free protein supplements).
It is not indicated in the treatment of acute hyperammonemia, as rapid interventions are recommended.
The safety and efficacy of treatment for N-acetylglutamate synthase deficiency (NAGS) have not been proven.
Use: As an agent that binds nitrogen for the chronic management of urea-cycle disorders (UCDs), which cannot be managed with amino acid supplements and/or dietary protein restrictions alone.
What happens if I miss the dose?
If you are almost due for your next dose, skip the missed one. Never take two doses of the same medicine at once.
What happens if I overdose?
Reach out for help or immediate medical attention for poison-related symptoms by dialing 1-800-222-1222, or seek emergency assistance immediately.
What should be avoided?
If your doctor prescribes any restrictions regarding food, beverage, or activity, follow their instructions.
Interaction with other drug
Certain medications should never be taken together. Some other medications can cause hyperammonemia or make Ravicti less efficient.Glycerol phenylbutyrate can interact with many drugs. These include prescription and over-the-counter medicines, vitamins, and herbal products. The list is not exhaustive. Tell your doctor about all the medications that you currently take and any new ones you plan to start or stop using.
