Ravicti

Take with food or formula. Administer dose via oral syringe; may give by NG or gastrostomy tube: see full labeling. <2yrs: give in 3 or more equally divided doses, rounded to nearest 0.1mL. ≥2yrs: give in 3 equally divided doses, rounded to nearest 0.5mL. Max 17.5mL (19g) daily. Must be used with dietary protein restriction and, in some cases, dietary supplements (eg, essential amino acids, arginine, citrulline, protein-free calorie supplements). Phenylbutyrate-naïve: 4.5–11.2mL/m²/day (5–12.4g/m²/day); patients with some residual enzyme activity not adequately controlled with protein restrictions: 4.5mL/m²/day. To determine starting dose in treatment-naïve patients: consider the patient’s residual urea synthetic capacity, dietary protein requirements, and diet adherence. Switching from sodium phenylbutyrate: give dose that contains the same amount of phenylbutyric acid; convert as follows: Total daily dose of Ravicti (mL) = total daily dose of sodium phenylbutyrate tablets (g) × 0.86 or total daily dose of sodium phenylbutyrate powder (g) × 0.81. Moderate to severe hepatic impairment: start at the lower end of dosing range. Dose adjustment and monitoring: see full labeling.