COMING SOON

INDICATION

ZELVYSIA™ (sapropterin dihydrochloride) is a phenylalanine hydroxylase activator indicated to reduce blood phenylalanine (Phe) levels in adult and pediatric patients 1 month of age and older with hyperphenylalaninemia (HPA) due to tetrahydrobiopterin- (BH4-) responsive Phenylketonuria (PKU). ZELVYSIA is to be used in conjunction with a Phe-restricted diet.

IMPORTANT SAFETY INFORMATION

WARNINGS AND PRECAUTIONS

• Hypersensitivity Reactions, Including Anaphylaxis: Avoid use in patients with a history of anaphylaxis to sapropterin. Discontinue treatment if anaphylaxis occurs and initiate appropriate medical care. Continue dietary Phe restrictions.
• Upper Gastrointestinal (GI) Mucosal Inflammation: Serious GI adverse reactions (eg, esophagitis, gastritis, ulcer, bleeding) have been reported. Monitor for signs and symptoms.
• Hypophenylalaninemia: May occur; increased risk in children <7 years of age treated with doses of 20 mg/kg per day. Monitor closely.
• Frequent Blood Phe Monitoring: Frequent blood Phe monitoring is required to ensure levels remain in the desirable range. Prolonged high Phe can cause severe neurologic damage (eg, intellectual disability, developmental delay, microcephaly, delayed speech, seizures, behavioral abnormalities). Prolonged low Phe can lead to catabolism and adverse developmental outcomes. Active management of dietary protein and Phe restriction is required.
• Lack of Biochemical Response: Not all patients with PKU respond. Assess response through a therapeutic trial; response cannot be pre-determined by laboratory testing.
• Interactions with Levodopa: May cause seizures, exacerbation of seizures, overstimulation, or irritability. Monitor for changes in neurologic status.
• Hyperactivity: Monitor for signs of hyperactivity.

ADVERSE REACTIONS

The most common adverse reactions (incidence ≥4%) are headache, rhinorrhea, pharyngolaryngeal pain, diarrhea, vomiting, cough, and nasal congestion.

DRUG INTERACTIONS

• Folate Synthesis Inhibitors (eg, methotrexate, valproic acid, phenobarbital, trimethoprim): May decrease BH4 and increase Phe. Monitor Phe more frequently; adjust dosage as needed.
• PDE-5 Inhibitors (eg, sildenafil, vardenafil, tadalafil): Both ZELVYSIA and PDE-5 inhibitors may induce vasorelaxation and hypotension. Monitor blood pressure.

To report SUSPECTED ADVERSE REACTIONS, contact Aucta Pharmaceuticals, Inc. at 1-800-655-9902, or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.

Please see full Prescribing Information and Instructions for Use.