Recommended Dosage

OLPRUVA treatment should be supervised by a healthcare provider experienced in the treatment of urea cycle disorders.

The recommended dosage of OLPRUVA for patients with urea cycle disorders is 9.9 –13 g/m2/day orally. Divide the calculated total daily dose into three to six doses. Administer as three to six divided doses and take with food.

Round each individual dose of OLPRUVA to the nearest available dosage strength. The maximum dosage is 20 grams per day. Combine OLPRUVA with dietary protein restriction and, in some cases, amino acid supplementation (e.g., essential amino acids, arginine, citrulline, and protein-free calorie supplements).

If a dose is missed, take the missed dose as soon as possible on the same day.

Dosage Administration and Monitoring

Monitor plasma ammonia levels to determine the need for dosage adjustment. Adjust the OLPRUVA dosage to maintain the plasma ammonia level within the normal range for the patient's age, taking into consideration their clinical condition (e.g., nutritional requirements, protein intake, growth parameters, etc.).

Monitor patients for potential neurotoxicity and obtain measurements of plasma phenylacetate and phenylacetylglutamine levels [see Warnings and Precautions (5.1), Adverse Reactions (6)]. If neurologic symptoms (e.g., vomiting, nausea, headache, somnolence, or confusion) are present in the absence of high ammonia levels or other incurrent illnesses, consider reducing the dose of OLPRUVA.

Dosage Adjustment in Patients with Hepatic Impairment

For patients with hepatic impairment, start at the lower end of the recommended dosing range and maintain patients on the lowest dose necessary to control plasma ammonia levels [see Use in Specific Populations (8.7)].

Preparation and Administration Instructions

For oral administration only. Do not administer via gastrostomy or nasogastric tubes.

1. Pour the entire contents of the Mix-Aid packet into approximately 4 ounces of water in a cup and stir, forming a suspension.
2. Pour the entire contents of the OLPRUVA packet(s) into the suspension and stir.
3. Drink the entire suspension within 5 minutes after stirring to minimize dissolution of coating. After 30 minutes, the suspension should be discarded.
4. Pour another 4 ounces of water into the cup and drink to make sure that any OLPRUVA remaining in the cup is consumed.