KUVAN POWDER Rx
Generic Name and Formulations:
Sapropterin dihydrochloride 100mg; pwd for oral soln.
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: consult manufacturer. Take with food at same time each day. Dissolve powder in 4–8oz water or apple juice (5oz if child weighs ≤10kg) and drink within 30 mins. May stir powder in small amount of apple sauce or pudding. 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: 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.
Monitor blood levels of Phe after 1 week to assess effect, then periodically. Discontinue if anaphylaxis occurs. Maintain Phe-restricted diet. Monitor BP and change in neurologic status. Hepatic or renal impairment; monitor. Pregnancy (Cat.C). Nursing mothers.
Caution with drugs that can inhibit folate metabolism (eg, methotrexate), PDE-5 inhibitors (eg, sildenafil, tadalafil, vardenafil), other nitric oxide donors, levodopa; monitor. May potentiate drugs that are P-gp or BCRP substrates.
Enzyme cofactor (BH4).
Headache, rhinorrhea, pharyngolaryngeal pain, diarrhea, vomiting, cough, nasal congestion, gastritis, hypophenylalaninemia, hyperactivity (monitor).
Tabs—120; Packet—1, 30
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