Prior Auth Protocol

FDA Approved Indications:

Kuvan 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.

Health Net Approved Indications and Usage Guidelines:

Patients with hyperphenylalaninemia (HPA) due to tetrahydrobiopterin (BH4-) responsive phenylketonuria (PKU).

Coverage is Not Authorized For:

Non-FDA approved indications, which are not listed in the Health Net Approved Indications and Usage Guidelines section, unless there is sufficient documentation of efficacy and safety in the published literature.

General Information:

Kuvan is to be used in conjunction with a Phe-restricted diet.
Baseline blood Phe measurements should be taken just prior to initiation of a Kuvan response test. Patients should be started at a dose of 10 mg/kg/day. Blood Phe levels should be checked after 1 week of Kuvan treatment and periodically for up to a month to determine response. A response to treatment with Kuvan may be determined by a decrease in blood Phe level compared to baseline level. If blood Phe level does not decrease at 10 mg/kg/day, the dose may be increased to 20 mg/kg/day. Patients whose blood Phe does not decrease from baseline after 1 month of treatment at 20 mg/kg/day are nonresponders, and treatment with Kuvan should be discontinued in these patients.
Doses of Kuvan above 20 mg/kg/day have not been evaluated in clinical trials.

Therapeutic Alternatives:

Drug	Dosing Regimen	Dose Limit/ Maximum Dose
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* Requires Prior Authorization

Recommended Dosing Regimen and Authorization Limit:

Drug	Dosing Regimen	Authorization Limit
Kuvan	Patients 1 month to 6 years: The recommended starting dose is 10 mg/kg PO QD. Patients 7 years and older: The recommended starting dose is 10 to 20 mg/kg PO QD Doses of Kuvan may be adjusted in the range of 5 to 20 mg/kg PO QD. Blood Phe must be monitored regularly.	One month initially then length of benefit if there is a response after one month (Patients whose blood Phe does not decrease from baseline after 1 month of treatment at 20 mg/kg/day are nonresponders, and treatment with Kuvan should be discontinued in these patients.)

Product Availability:

Kuvan: 100 mg tablet; powder packet containing 100 mg

References:

Kuvan [Prescribing information] Novato, CA: BioMarin Pharmaceutical Inc.; April 2014
Micromedex Healthcare Series [Internet Database]. Greenwood, Colo: Thomson Reuters (Healthcare) Inc. Updated periodically. Accessed May 28, 2015.
Sapropterin dihydrochloride. American Hospital Formulary Service Drug Information. Available at http://www.medicinescomplete.com/mc/ahfs/current/. Accessed May 28, 2015 .