Kuvan
(sapropterin dihydrochloride)Dosage & 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.
Starting Dosage
Dosage Adjustment
Preparation and Administration
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Kuvan Prescribing Information
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.
Recommended Dosage and Administration
The recommended starting dosage of KUVAN is:
Pediatric Patients 1 month to 6 years: 10 mg/kg (actual body weight) administered orally once daily.
Patients 7 years and older: 10 to 20 mg/kg (actual body weight) administered orally once daily.
Administer KUVAN with a meal, preferably at the same time each day [see Clinical Pharmacology (12.3)].
A missed dose should be administered as soon as possible, but two doses should not be administered on the same day.
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 ].
Dosage Adjustment
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 ].
KUVAN tablets are for oral use. Each tablet contains 100 mg of sapropterin dihydrochloride. 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 and as a unit dose packet containing 500 mg of sapropterin dihydrochloride. The powder is off-white to yellow in color.
Pediatric Use
Pediatric patients with PKU, ages 1 month to 16 years, have been treated with KUVAN in clinical trials [see Clinical Studies ].
The efficacy and safety of KUVAN have not been established in neonates. The safety of KUVAN has been established in children younger than 4 years in trials of 6 months duration and in children 4 years and older in trials of up to 3 years in length [see Adverse Reactions ].
In children aged 1 month and older, the efficacy of KUVAN has been demonstrated in trials of 6 weeks or less in duration [see Clinical Studies ].
In a multicenter, open-label, single arm study, 57 patients aged 1 month to 6 years who were defined as KUVAN responders after 4 weeks of KUVAN treatment and Phe dietary restriction were treated for 6 months with KUVAN at 20 mg/kg per day. The effectiveness of KUVAN alone on reduction of blood Phe levels beyond 4 weeks could not be determined due to concurrent changes in dietary Phe intake during the study. Mean (±SD) blood Phe values over time for patients aged 1 month to <2 years and 2 to <7 years are shown in Figure 1.
| Figure 1: Mean Blood Phe Level Over Time by Age (years) (N=57) |
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| *Error bars indicate 95% confidence interval. |
None.
Geriatric Use
Clinical studies of KUVAN in patients with PKU did not include patients aged 65 years and older. It is not known whether these patients respond differently than younger patients.