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.
KUVAN POWDER Warnings/Precautions:
Monitor blood Phe levels after 1 week to assess effect, then periodically. Maintain Phe-restricted diet. Monitor for upper GI mucosal inflammation, hyperactivity. Discontinue if anaphylaxis occurs. Pregnancy. Nursing mothers.
KUVAN POWDER Classification:
Phenylalanine hydroxylase activator.
KUVAN POWDER Interactions:
Monitor neurologic status with concomitant levodopa. Caution with drugs that can inhibit folate metabolism (eg, methotrexate, valproic acid, phenobarbital, trimethoprim). Concomitant drugs that affect nitric oxide-mediated vasorelaxation (eg, PDE-5 inhibitors); monitor BP.
Headache, rhinorrhea, pharyngolaryngeal pain, diarrhea, vomiting, cough, nasal congestion; hypersensitivity reactions, gastritis (monitor), hypophenylalaninemia.
Half-life: 6.7 hours (range: 3.9–17 hours).
Generic Drug Availability:
Tabs—120; Packets—1, 30