Inborn errors of metabolism:

Indications for KUVAN POWDER:

In conjunction with a phenylalanine (Phe)-restricted diet: To reduce blood Phe levels in hyperphenylalaninemia (HPA) due to tetrahydrobiopterin- (BH4-) responsive phenylketonuria (PKU).

Adults and Children:

<1 month: not established. Take with food at same time each day. Tabs: swallow whole or may dissolve in 120–240mL water or apple juice (may crush tab) and drink within 15mins (tab may not completely dissolve). Powder: dissolve in 120–240mL water or apple juice (5mL if child weighs ≤10kg) and drink within 30mins. 1 month–6yrs: initially 10mg/kg once daily; if inadequate response may increase to 20mg/kg once daily; if inadequate response after 1 month, discontinue. ≥7yrs: initially 10–20mg/kg once daily; if inadequate response after 1 month, discontinue. Usual range 5–20mg/kg per day; adjust based on blood Phe levels.

Warnings/Precautions:

Monitor blood levels of Phe after 1 week to assess effect, then periodically. Discontinue if anaphylaxis occurs. Maintain Phe-restricted diet. Pregnancy (Cat.C). Nursing mothers.

See Also:

Pharmacologic Class:

Phenylalanine hydroxylase activator.

Interactions:

Monitor neurologic status with concomitant levodopa. Caution with drugs that can inhibit folate metabolism (eg, methotrexate). Concomitant drugs that affect nitric oxide-mediated vasorelaxation (eg, PDE-5 inhibitors); monitor BP.

Adverse Reactions:

Headache, rhinorrhea, pharyngolaryngeal pain, diarrhea, vomiting, cough, nasal congestion; hypersensitivity reactions, gastritis (monitor), hypophenylalaninemia, hyperactivity (monitor).

Generic Availability:

NO

How Supplied:

Tabs—120; Packet—1, 30