Kuvan 500 mg/1

sapropterin dihydrochloride · POWDER, FOR SOLUTION · BioMarin Pharmaceutical Inc.

No Recall History

Plain English

Kuvan is an oral powder medication used to treat phenylketonuria (PKU), a genetic disorder that affects how the body processes the amino acid phenylalanine. It works by helping the body reduce dangerously high levels of phenylalanine in the blood.

Key Facts

Brand Name: Kuvan
Generic Name: sapropterin dihydrochloride
NDC Code (Product): 68135-482
Manufacturer: BioMarin Pharmaceutical Inc.
Strength: 500 mg/1
Dosage Form: POWDER, FOR SOLUTION
Route: ORAL
Marketing Status
Application #: NDA205065
Marketing Start: 05/27/2015

Recall History

No Recall History

Side Effects Reported to FDA

FDA FAERS database · These are reported events, not confirmed side effects

drug ineffective356 reports

headache352 reports

therapy non-responder347 reports

vomiting291 reports

maternal exposure during pregnancy277 reports

diarrhoea268 reports

nausea216 reports

amino acid level increased204 reports

abdominal pain upper198 reports

anxiety180 reports

Full Prescribing Information

Source: FDA Drug Label (SPL)For healthcare professionals

Indications & Usage

1 INDICATIONS AND USAGE KUVAN ® is indicated to reduce blood phenylalanine (Phe) levels in adult and pediatric patients one month of age and older with hyperphenylalaninemia (HPA) due to tetrahydrobiopterin- (BH4-) responsive Phenylketonuria (PKU). KUVAN is to be used in conjunction with a Phe-restricted diet. KUVAN is a phenylalanine hydroxylase activator indicated to reduce blood phenylalanine (Phe) levels in adult and pediatric patients one month of age and older with hyperphenylalaninemia (HPA) due to tetrahydrobiopterin‑ (BH4‑) responsive Phenylketonuria (PKU). KUVAN is to be used in conjunction with a Phe‑restricted diet. ( 1 )

Dosage & Administration

2 DOSAGE AND ADMINISTRATION All patients with PKU who are being treated with KUVAN should also be treated with a Phe-restricted diet, including dietary protein and Phe restriction. ( 2.1 ) Starting Dosage Pediatric patients 1 month to 6 years: The recommended starting dosage of KUVAN is 10 mg/kg administered orally once daily. ( 2.2 ) Patients 7 years and older : The recommended starting dosage of KUVAN is 10 to 20 mg/kg administered orally once daily. ( 2.2 ) Dosage Adjustment Doses of KUVAN may be adjusted in the range of 5 to 20 mg/kg taken once daily. ( 2.2 ) Monitor blood Phe regularly, especially in pediatric patients. ( 2.2 , 5.3 ) Preparation and Administration See the full prescribing information for preparation and administration instructions. ( 2.3 ) 2.1 Recommendations Prior to KUVAN Treatment Treatment with KUVAN should be directed by physicians knowledgeable in the management of PKU. All patients with PKU who are being treated with KUVAN should also be treated with a Phe-restricted diet, including dietary protein and Phe restriction. 2.2 Recommended Dosage and Administration The recommended starting dosage of KUVAN is: Pediatric Patients 1 month to 6 years : 10 mg/kg …

Contraindications

4 CONTRAINDICATIONS None. None. ( 4 )

Drug Interactions

7 DRUG INTERACTIONS Table 4 includes drugs with clinically important drug interactions when administered with sapropterin dihydrochloride and instructions for preventing or managing them. Table 4: Clinically Relevant Drug Interactions Levodopa Clinical Impact Sapropterin dihydrochloride may increase the availability of tyrosine, a precursor of levodopa. Neurologic events were reported postmarketing in patients receiving sapropterin and levodopa concomitantly for a non-PKU indication [see Warnings and Precautions ( 5.5 )] . Intervention Monitor patients for a change in neurologic status. Inhibitors of Folate Synthesis (e.g., methotrexate, valproic acid, phenobarbital, trimethoprim) Clinical Impact In vitro and in vivo nonclinical data suggest that drugs that inhibit folate synthesis may decrease the bioavailability of endogenous BH4 by inhibiting the enzyme dihydrofolate reductase, which is involved in the recycling (regeneration) of BH4. This reduction in net BH4 levels may increase Phe levels. Intervention Consider monitoring blood Phe levels more frequently during concomitant administration. An increased dosage of KUVAN may be necessary to achieve a biochemical response. Drugs Af…

Adverse Reactions

6 ADVERSE REACTIONS Most common adverse reactions (≥4%) are: headache, rhinorrhea, pharyngolaryngeal pain, diarrhea, vomiting, cough, and nasal congestion. ( 6.1 ) To report SUSPECTED ADVERSE REACTIONS, contact BioMarin Pharmaceutical Inc. at 1-877-695-8826, or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch . 6.1 Clinical Trials Experience Because clinical trials are conducted under widely varying conditions, adverse reaction rates observed in the clinical trials of a drug cannot be directly compared to the rates in the clinical trials of another drug and may not reflect the rates observed in clinical practice. PKU Clinical Studies The safety of KUVAN was evaluated in 7 clinical studies in patients with PKU (aged 1 month to 50 years) [see Clinical Studies ( 14 )] . In Studies 1-4 (controlled and uncontrolled studies), 579 patients with PKU aged 4 to 49 years received KUVAN in doses ranging from 5 to 20 mg/kg per day for lengths of treatment ranging from 1 to 164 weeks. The patient population was evenly distributed in gender, and approximately 95% of patients were Caucasian. The most common adverse reactions (≥4% of patients) were headache, rhinorrhea, pharyngolaryngeal pain, diarrhe…

Frequently Asked Questions

What is Kuvan used for?

Is Kuvan a controlled substance?

Kuvan is not classified as a controlled substance by the DEA.

What is the generic name for Kuvan?

The generic name for Kuvan is sapropterin dihydrochloride. There are 11 other brand versions of sapropterin dihydrochloride.

What is the NDC code for Kuvan 500 mg/1?

The NDC (National Drug Code) for Kuvan 500 mg/1 is 68135-482, listed by BioMarin Pharmaceutical Inc..

Product NDC

68135-482

Package NDC

68135-482-11

Other Sapropterin Brands

See all →

Not medical advice. Always consult your doctor or pharmacist before making any medication decisions.

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