5
1
2
40
1
5
2
3
60
1
5
3
4
80
1
5
4
5
100
1
5
5
6
120
2
5
3
7
140
2
5
3.5
8
160
2
5
4
9
180
2
5
4.5
10
200
2
5
5
† Powder for oral solution provided in single use packets containing 100 mg Kuvan per packet
† Volume of water or apple juice to dissolve Kuvan Powder for Oral Solution.
§Discard remainder of mixture after volume to be administered is drawn.
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3. DOSAGE FORMS AND STRENGTHS
Kuvan tablets are for oral use. Each tablet contains 100 mg of sapropterin dihydrochloride (equivalent to 76.8 mg of sapropterin base). Tablets are round, off-white to light yellow, mottled, and debossed with “177”.
Kuvan powder for oral solution is available as a unit dose packet containing 100 mg of sapropterin dihydrochloride (equivalent to 76.8 mg of sapropterin base) and as a unit dose packet containing 500 mg of sapropterin dihydrochloride (equivalent to 384 mg of sapropterin base). The powder is off-white to yellow in color.
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4. CONTRAINDICATIONS
None.
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5. WARNINGS AND PRECAUTIONS
5.1 Hypersensitivity Reactions Including AnaphylaxisKuvan is not recommended in patients with a history of anaphylaxis to Kuvan. Hypersensitivity reactions, including anaphylaxis and rash, have occurred [see Adverse Reactions (6.2)]. Signs of anaphylaxis include wheezing, dyspnea, coughing, hypotension, flushing, nausea, and rash. Discontinue treatment with Kuvan in patients who experience anaphylaxis and initiate appropriate medical treatment. Continue dietary Phe restrictions in patients who experience anaphylaxis.
5.2 GastritisDuring clinical studies, gastritis was reported as a serious adverse reaction. Monitor patients for signs and symptoms of gastritis.
5.3 HypophenylalaninemiaIn clinical trials, some patients have experienced low blood Phe levels. 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 patients 7 years and older [see Adverse Reactions (6.1)].
5.4 Monitor Blood Phe Levels During TreatmentTreatment with Kuvan should be directed by physicians knowledgeable in the management of PKU. Prolonged elevations in blood Phe levels in patients with PKU can result in severe neurologic damage, including severe mental retardation, microcephaly, delayed speech, seizures, and behavioral abnormalities. Conversely, prolonged levels of blood Phe that are too low have been associated with catabolism and protein breakdown. Active management of dietary Phe intake while taking Kuvan is required to ensure adequate Phe control and nutritional balance. Monitor blood Phe levels during treatment to ensure adequate blood Phe level control. Frequent blood monitoring is recommended in the pediatric population [
