Kuvan

KUVAN- sapropterin dihydrochloride tablet
KUVAN- sapropterin dihydrochloride powder, for solution
BioMarin Pharmaceutical Inc.

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.

2 DOSAGE AND ADMINISTRATION

2.1 Dosage

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.

Starting Dosage

Pediatric Patients 1 month to 6 years: The recommended starting dose of Kuvan is 10 mg/kg taken once daily.

Patients 7 years and older: The recommended starting dose of Kuvan is 10 to 20 mg/kg taken once daily.

Dosage Adjustment (Evaluation Period)

Existing dietary protein and Phe intake should not be modified during the evaluation period.

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 do not show a biochemical response 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 show a biochemical response (blood Phe does not decrease) after 1 month of treatment at 20 mg/kg per day [see Warnings and Precautions (5.4)].

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 biochemical response to therapy (blood Phe). Periodic blood Phe monitoring is recommended to assess blood Phe control, especially in pediatric patients [see Warnings and Precautions (5.3)].

2.2 Preparation and Administration Instructions

Take Kuvan orally with a meal , preferably at the same time each day [see Clinical Pharmacology (12.3)]. A missed dose should be taken as soon as possible, but two doses should not be taken on the same day.

Kuvan Tablets

Kuvan tablets may be swallowed either as whole tablets or dissolved in 120 to 240 mL of water or apple juice and taken orally within 15 minutes of dissolution. It may take a few minutes for the tablets to dissolve. To make the tablets dissolve faster, tablets may be stirred or crushed. The tablets may not dissolve completely. Patients may see small pieces floating on top of the water or apple juice. This is normal and safe for patients to swallow. If after drinking the medicine patients still see pieces of the tablet in the container, more water or apple juice can be added to make sure all of the medicine is consumed. Kuvan tablets may also be crushed and then mixed in a small amount of soft foods such as apple sauce or pudding.

Kuvan Powder for Oral Solution

Patients weighing greater than 10 kg

Kuvan powder for oral solution should be dissolved in 120 to 240 mL of water or apple juice and taken orally within 30 minutes of dissolution. Kuvan powder for oral solution may also be stirred in a small amount of soft foods such as apple sauce or pudding. Empty the contents of the packet(s) in water, apple juice, or a small amount of soft foods and mix thoroughly. The powder should dissolve completely.

Patients weighing 10 kg or less (use 100 mg packets)

For infants weighing 10 kg or less, Kuvan powder for oral solution can be dissolved in as little as 5 mL of water or apple juice and a portion of this solution corresponding to a 10 mg/kg dose may be administered orally via an oral dosing syringe. Table 1 provides dosing information for infants at the recommended starting dose of 10 mg/kg per day. Refer to Table 2 for dosing information at 20 mg/kg per day if dosage adjustment is needed.

Table 1: 10 mg/kg per day Dosing Table for Infants Weighing 10 kg or less

Patient

Weight (kg)

Starting Dose: 10 mg/kg per day*

Dose (mg)

Kuvan Powder for Oral Solution

100 mg Packets Dissolved

Dilution Volume (mL)

Administered Dose volume

(mL)§

1

10

1

10

1

2

20

1

10

2

3

30

1

10

3

4

40

1

10

4

5

50

1

10

5

6

60

1

5

3

7

70

1

5

3.5

8

80

1

5

4

9

90

1

5

4.5

10

100

1

5

5

*Starting dose for infants is 10 mg/kg per day. Dosing information for 20 mg/kg per day is provided in Table 2.

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.

Table 2: 20 mg/kg per day Dosing Table for Infants Weighing 10 kg or less

Patient

Weight (kg)

20 mg/kg per day

Dose (mg)

Kuvan Powder for Oral Solution

100 mg Packets* Dissolved

Dilution Volume (mL)

Administered Dose volume

(mL)§

1

20

1

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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