Kuvan

Country: European Union

Language: English

Source: EMA (European Medicines Agency)

Active ingredient:

Sapropterin dihydrochloride

Available from:

BioMarin International Limited

ATC code:

A16AX07

INN (International Name):

sapropterin

Therapeutic group:

Other alimentary tract and metabolism products,

Therapeutic area:

Phenylketonurias

Therapeutic indications:

Kuvan is indicated for the treatment of hyperphenylalaninaemia (HPA) in adults and paediatric patients of all ages with phenylketonuria (PKU) who have been shown to be responsive to such treatment.Kuvan is also indicated for the treatment of hyperphenylalaninaemia (HPA) in adults and paediatric patients of all ages with tetrahydrobiopterin (BH4) deficiency who have been shown to be responsive to such treatment.

Product summary:

Revision: 20

Authorization status:

Authorised

Authorization date:

2008-12-02

Patient Information leaflet

38
B. PACKAGE LEAFLET
39
PACKAGE LEAFLET: INFORMATION FOR THE PATIENT
KUVAN 100 MG SOLUBLE TABLETS
Sapropterin dihydrochloride
READ ALL OF THIS LEAFLET CAREFULLY BEFORE YOU START TAKING THIS
MEDICINE BECAUSE IT CONTAINS IMPORTANT
INFORMATION FOR YOU.
-
Keep this leaflet. You may need to read it again.
-
If you have any further questions, ask your doctor or pharmacist.
-
This medicine has been prescribed for you only. Do not pass it on to
others. It may harm them, even if
their signs of illness are the same as yours.
-
If you get any side effects talk to your doctor or pharmacist. This
includes any possible side effects not
listed in this leaflet. See section 4.
WHAT IS IN THIS LEAFLET
1.
What Kuvan
_ _
is and what it is used for
2.
What do you need to know before you take Kuvan
3.
How to take Kuvan
_ _
4.
Possible side effects
5.
How to store Kuvan
_ _
6.
Contents of the pack and other information
1.
WHAT KUVAN IS AND WHAT IT IS USED FOR
Kuvan contains the active substance sapropterin which is a synthetic
copy of a body’s own substance called
tetrahydrobiopterin (BH4). BH4 is required by the body to use an amino
acid called phenylalanine in order to
build another amino acid called tyrosine.
Kuvan is used to treat hyperphenylalaninaemia (HPA) or phenylketonuria
(PKU) in patients of all ages. HPA
and PKU are due to abnormally high levels of phenylalanine in the
blood which can be harmful. Kuvan
reduces these levels in some patients who respond to BH4 and can help
increase the amount of phenylalanine
that can be included in the diet.
This medicine is also used to treat an inherited disease called BH4
deficiency in patients of all ages, in which
the body cannot produce enough BH4. Because of very low BH4 levels
phenylalanine is not used properly
and its levels rise, resulting in harmful effects. By replacing the
BH4 that the body cannot produce, Kuvan
reduces the harmful excess of phenylalanine in the blood and increases
the dietary tolerance to
phenylalanine.
2.
WHAT YOU NEED TO KNOW BEFORE YOU TAKE KUVAN
DO NOT T

Summary of Product characteristics

1
ANNEX I
SUMMARY OF PRODUCT CHARACTERISTICS
2
1.
NAME OF THE MEDICINAL PRODUCT
Kuvan 100 mg soluble tablets
_ _
2.
QUALITATIVE AND QUANTITATIVE COMPOSITION
Each soluble tablet contains 100 mg of sapropterin dihydrochloride
(equivalent to 77 mg of sapropterin).
For the full list of excipients, see section 6.1.
3.
PHARMACEUTICAL FORM
Soluble tablet
Off-white to light yellow soluble tablet with “177” imprinted on
one face.
4.
CLINICAL PARTICULARS
4.1
THERAPEUTIC INDICATIONS
Kuvan is indicated for the treatment of hyperphenylalaninaemia
_ _
(HPA) in adults and paediatric patients of all
ages with phenylketonuria (PKU) who have been shown to be responsive
to such treatment (see section 4.2).
Kuvan is also indicated for the treatment of hyperphenylalaninaemia
(HPA) in adults and paediatric patients
of all ages with tetrahydrobiopterin (BH4) deficiency who have been
shown to be responsive to such
treatment (see section 4.2).
4.2
POSOLOGY AND METHOD OF ADMINISTRATION
Treatment with Kuvan must be initiated and supervised by a physician
experienced in the treatment of PKU
and BH4 deficiency.
Active management of dietary phenylalanine and overall protein intake
while taking this medicinal product
is required to ensure adequate control of blood phenylalanine levels
and nutritional balance.
_ _
As HPA due to either PKU or BH4 deficiency is a chronic condition,
once responsiveness is demonstrated,
Kuvan is intended for long-term use (see section 5.1).
Posology
_PKU _
The starting dose of Kuvan in adult and paediatric patients with PKU
is 10 mg/kg body weight once daily.
The dose is adjusted, usually between 5 and 20 mg/kg/day, to achieve
and maintain adequate blood
phenylalanine levels as defined by the physician.
_BH4 deficiency _
The starting dose of Kuvan in adult and paediatric patients with BH4
deficiency is 2 to 5 mg/kg body weight
total daily dose. Doses may be adjusted up to a total of 20 mg/kg per
day.
_ _
_ _
Kuvan is provided as 100 mg tablets. The calculated daily dose based
on body weight should be rounded to