What is it and how is it used?

Kuvan 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 for the treatment of hyperphenylalaninaemia (HPA) or phenylketonuria (PKU), 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.

Kuvan is also used for the treatment of an inherited disease called BH4 deficiency, 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.

Table of Contents
What do you have to consider before using it?
How is it used?
What are possible side effects?
How should it be stored?
Further information

What do you have to consider before using it?

Do not take KuvanIf you are allergic (hypersensitive) to sapropterin or any of the other ingredients of Kuvan.

Take special care with Kuvan

You should consult your doctor:

  • if your child that was prescribed Kuvan is under 4 years of age
  • if you are 65 years of age or older
  • if you have problems with your kidney or liver
  • if you are ill. Consultation with a physician is recommended during illness as blood phenylalanine levels may increase
  • if you have predisposition to convulsions
  • if you are using any of the medicines listed below under ?Taking other medicines?

When you are treated with Kuvan, your doctor will test your blood to verify how much phenylalanine and tyrosine it contains and may decide to adjust the dose of Kuvan or your diet if needed.

You must continue your diet treatment as recommended by your doctor. Do not change your diet without contacting your doctor.

Taking other medicines

You should consult your doctor if you are taking:

  • levodopa (used to treat Parkinson?s disease)
  • inhibitors of dihydrofolate reductase (e.g. methotrexate, trimethoprim)
  • agents that cause vasodilation, including those administered topically, by affecting nitric oxide (NO) metabolism or action, including the classical NO donors (such as glyceryl trinitrate (GTN), isosorbide dinitrate (ISDN), sodium nitroprusside (SNP) and molsidomin), phosphodiesterase type 5 (PDE-5) inhibitors and minoxidil.

Please tell your doctor or pharmacist if you are taking or have recently taken any other medicines, including medicines obtained without a prescription.

Taking Kuvan with food and drink

Kuvan should be taken with food at the same time each day preferably in the morning.

Pregnancy and breast-feeding

For Kuvan no information is available for human pregnancies. Animal studies do not indicate direct or indirect harmful effects with respect to pregnancy , embryonal/foetal development, birth or postnatal development.

If maternal phenylalanine levels are not strictly controlled before and during pregnancy, this could be harmful to the mother and the foetus. In case of pregnancy your doctor will tell you how to control phenylalanine levels adequately. Physician-supervised restriction of dietary phenylalanine intake prior to and throughout pregnancy is the first choice of treatment in this patient group.
The use of Kuvan should be considered only if strict dietary management does not adequately reduce blood phenylalanine levels.

Be sure to tell your doctor if you are pregnant or breast-feeding, if you think you might be pregnant, or if you are planning to become pregnant or are planning to begin breast-feeding.
You should not take Kuvan if you are breast-feeding.

Ask your doctor or pharmacist for advice before taking any medicine.

Driving and using machines
No studies on the effects on the ability to drive and use machines have been performed. Kuvan is not expected to affect the ability to drive and use machines.'

How is it used?

Always take Kuvan exactly as your doctor has told you. You should check with your doctor if you are not sure.

Dosage

PKU
The usual starting dose of Kuvan in adult and paediatric patients with PKU is 10 mg for each kg of body weight. Take the soluble tablets as a single daily dose with a meal to increase the absorption, and at the same time each day, preferably in the morning. Your doctor may adjust your dose, usually between 5 and 20 mg for each kg of body weight per day, depending on your condition.

BH4 deficiency
The usual starting dose of Kuvan in adult and paediatric patients with BH4 deficiency is 2 to 5 mg for each kg of body weight. Take the soluble tablets as a single daily dose, with a meal to increase the absorption, and at the same time each day, preferably in the morning. Your doctor may adjust your dose up to 20 mg for each kg of body weight per day, depending on your condition. It may be necessary to divide the total daily dose into 2 or 3 doses, distributed over the day, to achieve the best therapeutic effect.

The table below is an example of how an appropriate dose is calculated:

Body weight kg Number of tablets Kuvan dose 10 mgkg Number of tablets Kuvan dose 20 mgkg 10 1 2 20 2 4 30 3 6 40 4 8 50 5 10

Method of Administration

Adults
Place the tablets in a glass or cup (120 to 240 ml) of water and stir until dissolved.

Paediatric patients
The prescribed number of tablets should be placed in a glass or cup (up to 120 ml) of water and stir until dissolved.

For doses below 100 mg, one tablet should be dissolved in 100 ml of water. Your doctor will tell you to administer only a certain volume of the solution corresponding to the appropriate dose. An accurate measuring device with suitable graduations should be used for this purpose.

It may take a few minutes for the tablets to dissolve. To make the tablets dissolve faster you can crush them. Small particles may be visible in the solution, but they will not affect the effectiveness of the medicine. Drink the dissolved preparation of Kuvan with a meal, at the same time each day, preferably in the morning within 15 to 20 minutes of its preparation.

Make sure you do not swallow the desiccant capsule contained in the bottle.

If you take more Kuvan than you should

If you take more Kuvan than prescribed, you may experience side effects that could include headache and dizziness. You should immediately contact your doctor or pharmacist if you take more Kuvan than prescribed.

If you forget to take Kuvan
Do not take a double dose to make up for a forgotten dose.

If you stop taking Kuvan

Do not stop taking Kuvan without prior discussion with your doctor, as phenylalanine levels in the blood may increase.

If you have any further questions on the use of this product, ask your doctor or pharmacist.

What are possible side effects?

Like all medicines, Kuvan can cause side effects, although not everybody gets them.

Frequencies of side effects are defined as:

  • very common: affects more than 1 user in 10
  • common: affects 1 to 10 users in 100
  • uncommon: affects 1 to 10 users in 1,000
  • rare: affects 1 to 10 users in 10,000
  • very rare: affects less than 1 user in 10,000
  • not known: frequency cannot be estimated from the available data.

Very common side effects: headache and runny nose.

Common side effects: sore throat, nasal congestion or stuffy nose, cough, diarrhoea, vomiting, stomach ache and too low levels of phenylalanine in blood tests.

If any of the side effects gets serious, or if you notice any side effects not listed in this leaflet, please tell your doctor or pharmacist.

How should it be stored?

Keep out of the reach and sight of children.

Do not use Kuvan after the expiry date which is stated on the bottle and the carton after ?EXP?. The expiry date refers to the last day of that month.

Store below 25°C.
Keep the bottle tightly closed in order to protect from moisture.

Medicines should not be disposed of via wastewater or household waste. Ask your pharmacist how to dispose of medicines no longer required. These measures will help to protect the environment.

Further information

What Kuvan contains

  • The active substance is sapropterin dihydrochloride. Each tablet contains 100 mg of sapropterin dihydrochloride (equivalent to 77 mg of sapropterin).
  • The other ingredients are mannitol (E421), calcium hydrogen phosphate anhydrous, crospovidone typeA, ascorbic acid (E300), sodium stearyl fumarate, and riboflavin (E101).
What Kuvan looks like and contents of the pack

Kuvan is supplied as soluble tablets. The soluble tablets are off-white to light yellow and have ?177? imprinted on one face.

It may be available in bottles with child-resistant closure of 30, 120 or 240 soluble tablets. Each bottle contains a small plastic tube of desiccant (silica gel).
Not all pack sizes may be marketed.

Marketing Authorisation Holder

Merck Serono Europe Limited
56, Marsh Wall
London E14 9TP
United Kingdom

Manufacturer

Merck KGaA, Frankfurter Str. 250, 64293 Darmstadt, Germanyor
Merck KGaA & Co. Werk Spittal, Hösslgasse 20, 9800 Spittal / Drau, Austria

For any information about this medicinal product, please contact the local representative of the Marketing Authorisation Holder.

BelgiëBelgiqueBelgien MERCK NVSA Brusselsesteenweg 288 B-3090 Overijse TélTel 32-2-686 07 11 LuxembourgLuxemburg MERCK NVSA Brusselsesteenweg 288 B-3090 Overijse, BelgiqueBelgien TélTel 32-2-686 07 11

Magyarország Merck Kft. Bocskai út 134-146. H-1113 Budapest Tel 36-1-463-8100 . . 83 1582 Te 359 28075 111

eská republika Merck spol. s r.o. Na Hebenech II. 171810 CZ-140 00 Praha 4 Tel. 420 272084211 Malta Cherubino Ltd Delf Building Sliema Road MT-GZR 06 Gzira Malta Tel 356-21-3432701234

Danmark Merck AS Strandvejen 102 B, 4th DK-2900 Hellerup Tlf 45 35253550 Nederland Merck BV Tupolevlaan 41-61 NL-1119 NW Schiphol-Rijk Tel 31-20-6582800

Deutschland Merck Serono GmbH Alsfelder Straße 17 D-64289 Darmstadt Tel 49-6151-6285-0 Norge Merck Serono Norge Luhrtoppen 2 N-1470 Lørenskog Tlf 47 67 90 35 90

Eesti Merck Serono OÜ Tornimäe 7 - 132 EE-10145, Tallinn Tel 372 682 5882 Österreich Merck GesmbH. Zimbagasse 5 A-1147 Wien Tel 43 1 57600-0

Polska Merck Sp. z o.o. Al. Jerozolimskie 178 PL-02-486 Warszawa Tel. 48 22 53 59 700 Merck A.E. 41-45, GR-151 23 T 30-210-61 65 100

Portugal Merck, s.a. Rua Alfredo da Silva, 3-C P-1300-040 Lisboa Tel 351-21-361 35 00 España Merck S.L. María de Molina, 40 E-28006 Madrid Línea de Información 900 200 400 Tel 34-91-745 44 00

România MERCK d.o.o., Dunajska cesta 119 SI-1000 Lubliana, Slovenia Tel 386 1 560 3 800 France Merck Serono s.a.s. 37, rue Saint-Romain F-69379 Lyon cedex 08 Tél. 33-4-72 78 25 25 Numéro vert 0 800 888 024

Slovenija MERCK d.o.o. Dunajska cesta 119 SI-1000 Ljubljana Tel 386 1 560 3 800 Ireland Merck Serono Ltd Bedfont Cross, Stanwell Road Feltham, Middlesex TW14 8NX United Kingdom Tel 44-20 8818 7200

Ísland Icepharma hf Lynghálsi 13 IS-110 Reykjavík Tel 354 540 8000 Slovenská republika Merck spol. s r.o. Tuhovská 3 SK-831 06 Bratislava Tel 421 2 49 267 111

Italia Merck Serono S.p.A. Via Casilina 125 I-00176 Roma Tel 39-06-70 38 41 SuomiFinland Merck Oy Pihatörmä 1 C FIN-02240 Espoo PuhTel 358-9-8678 700

Sverige Merck AB S-195 87 Stockholm Tel 46-8-562 445 00 . . 35, CY-2234 , . 357 22490305

Latvija Merck Serono SIA Duntes iela 23A LV-1005, Rga Tel 371 67152500 United Kingdom Merck Serono Ltd Bedfont Cross, Stanwell Road Feltham, Middlesex TW14 8NX- UK Tel 44-20 8818 7200

Lietuva
Merck Serono UAB
Savanoriu pr. 192,
LT-44151 Kaunas
Tel: +370 37320603

This leaflet was last approved in

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