Hypersensitivity Reactions Including Anaphylaxis 5.2 Gastritis 5.3 Hypophenylalaninemia 5.4 Monitor Blood Phe Levels During Treatment 5.5 Identify Non-Responders to Kuvan Treatment 5.6 Treat All Patients with a Phe-restricted Diet 5.7 Monitor Patients with Hepatic Impairment 5.8 Monitor Patients when Co-administering Kuvan and Medications Known to Inhibit Folate Metabolism 5.9 Monitor Patients for Hypotension when Co-administering Kuvan and Drugs Known to Affect Nitric Oxide-Mediated Vasorelaxation 5.10 Monitor Patients when Co-administering Kuvan and Levodopa 5.11 Monitor Patients for Hyperactivity 6. ADVERSE REACTIONS
6.1 Clinical Trials Experience 6.2 Post-Marketing Experience 7. DRUG INTERACTIONS
8. USE IN SPECIFIC POPULATIONS
8.1 Pregnancy 8.3 Nursing Mother 8.4 Pediatric Use 8.5 Geriatric Use 8.6 Patients with Renal Impairment 10. OVERDOSAGE
11. DESCRIPTION
12. CLINICAL PHARMACOLOGY
12.1 Mechanism of Action 12.2 Pharmacodynamics 12.3 Pharmacokinetics 13. NONCLINICAL TOXICOLOGY
13.1 Carcinogenesis, Mutagenesis, Impairment of Fertility 14. CLINICAL STUDIES
14.1 Clinical Studies in PKU 16. HOW SUPPLIED/STORAGE AND HANDLING
17. PATIENT COUNSELING INFORMATION
* Sections or subsections omitted from the full prescribing information are not listed. Close
1. INDICATIONS AND USAGE
Kuvan® (sapropterin dihydrochloride) is 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.
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2. DOSAGE AND ADMINISTRATION
2.1 DosagePatients 1 month to 6 years: The recommended starting dose of Kuvan is 10 mg/kg taken once daily [see Warnings and Precautions (5.3)].
Patients 7 years and older: The recommended starting dose of Kuvan is 10 to 20 mg/kg taken once daily.
If a 10 mg/kg per day starting dose is used, then response to therapy is determined by change in blood Phe following treatment with Kuvan at 10 mg/kg per day for a period of up to 1 month. Blood Phe levels should be checked after 1 week of Kuvan treatment and periodically for up to a month. If blood Phe does not decrease from baseline at 10 mg/kg per day, the dose may be increased to 20 mg/kg per day. Patients whose blood Phe does not decrease after 1 month of treatment at 20 mg/kg per day are non-responders and treatment with Kuvan should be discontinued in these patients.
If a 20 mg/kg per day starting dose is used, then response to therapy is determined by change in blood Phe following treatment with Kuvan at 20 mg/kg per day for a period of 1 month. Blood Phe levels should be checked after 1 week of Kuvan treatment and periodically during the first month. Treatment should be discontinued in patients who do not respond to Kuvan.
Once responsiveness to Kuvan has been established, the dosage may be adjusted within the range of 5 to 20 mg/kg per day according to response to therapy. Periodic blood Phe monitoring is recommended to assess blood Phe control [see Warnings and Precautions (5.3, 5.6)].
2.2 AdministrationKuvan is available as tablets and as powder for oral solution. Kuvan should be taken orally with a meal to increase absorption, preferably at the same time each day. A missed dose should be t
