HIGHLIGHTS OF PRESCRIBING INFORMATION
These highlights do not include all the information needed to use KUVAN safely and effectively.
See full prescribing information for KUVAN.
KUVAN (sapropterin dihydrochloride) tablets, for oral use
KUVAN (sapropterin dihydrochloride) powder, for oral solution
Initial U.S. Approval: 2007
INDICATIONS AND USAGE
Kuvan is a phenylalanine hydroxylase activator indicated to reduce blood phenylalanine (Phe) levels in patients with hyperphenylalaninemia (HPA) due to tetrahydrobiopterin‑ (BH4‑) responsive Phenylketonuria (PKU). Kuvan is to be used in conjunction with a Phe‑restricted diet (1).
DOSAGE AND ADMINISTRATION
Starting dose:
Patients 1 month to 6 years: The recommended starting dose of Kuvan is 10 mg/kg taken once daily (2.1, 5.3).
Patients 7 years and older: The recommended starting dose of Kuvan is 10 to 20 mg/kg taken once daily (2.1).
Dose Adjustment:
Doses of Kuvan may be adjusted in the range of 5 to 20 mg/kg taken once daily. Blood Phe must be monitored regularly (2.1).
Instruct patients to take with a meal.
Swallow tablets whole or after mixing in a small amount of soft foods or dissolving in recommended liquid.  Swallow oral solution after mixing powder in a small amount of soft foods or dissolving in recommended liquids.
DOSAGE FORMS AND STRENGTHS
Tablets, 100 mg (3)
Powder for Oral Solution, 100 mg, 500 mg (3)
CONTRAINDICATIONS
None (4).
WARNINGS AND PRECAUTIONS
Hypersensitivity reactions including anaphylaxis have occurred (5.1).
Gastritis was reported in clinical trials. Monitor patients for signs of gastritis (5.2).
Children younger than 7 years treated with Kuvan doses of 20 mg/kg per day are at increased risk for low levels of blood Phe compared with children 7 years and older (5.3).
Monitor blood Phe levels during treatment to ensure adequate blood Phe control (5.4).
Identify non-responders to Kuvan treatment: Not all patients with PKU respond to treatment with Kuvan (5.5).
Treat all patients with a Phe-restricted diet: The initiation of Kuvan therapy does not eliminate the need for ongoing dietary management (5.6).
Monitor liver function tests in patients with liver impairment who are receiving Kuvan (5.7).
Monitor patients when co-administering Kuvan with medications known to inhibit folate metabolism, or with levodopa. Monitor patients for hypotension when co-administering Kuvan with medications known to affect nitric oxide-mediated vasorelaxation (5.8, 5.9, 5.10).
There have been post-marketing reports of hyperactivity with administration of Kuvan. Monitor patients for hyperactivity (5.11).
ADVERSE REACTIONS
The most common adverse reactions (incidence ≥4%) in patients treated with Kuvan are headache, rhinorrhea, pharyngolaryngeal pain, diarrhea, vomiting, cough, and nasal congestion (6.1).
To report SUSPECTED ADVERSE REACTIONS, contact BioMarin Pharmaceutical Inc. at 1-866-906-6100, or FDA at 1-800-FDA-1088 orwww.fda.gov/medwatch.
See 17 for PATIENT COUNSELING INFORMATION and FDA-approved patient labeling.
Revised: 7/2015
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FULL PRESCRIBING INFORMATION: CONTENTS*
1.  INDICATIONS AND USAGE
2.  DOSAGE AND ADMINISTRATION
2.1  Dosage 2.2  Administration 2.3 Instructions for Use 3.  DOSAGE FORMS AND STRENGTHS
4.  CONTRAINDICATIONS
5.  WARNINGS AND PRECAUTIONS
5.1