Soluble tablets (100 mg)
Consumer Medicine Information
What is in this leaflet
Please read this leaflet carefully before you start using KUVAN.
This leaflet answers some common questions about KUVAN.
It does not contain all the available information.
It does not take the place of talking to your doctor or pharmacist.
All medicines have risks and benefits. Your doctor has weighed the risks of you taking KUVAN against the benefits they expect it will have for you.
If you have any concerns about taking this medicine, ask your doctor or pharmacist.
Keep this leaflet with the medicine. You may need to read it again.
What KUVAN is used for
KUVAN is used to reduce phenylalanine (Phe) in the blood of people with phenylketonuria (PKU) or tetrahydrobiopterin (BH4) deficiency. Phe is a necessary amino acid but it is harmful in excessive amounts. People with PKU or BH4 deficiency cannot process Phe normally. A Phe restricted diet will reduce Phe levels in the body and some people also need treatment with KUVAN.
KUVAN contains sapropterin dihydrochloride which is identical to the body's own natural BH4 except that it is made in a laboratory. BH4 is used by the body to process Phe in order to build another amino acid called tyrosine, which is a building block for several important brain chemicals.
People with PKU or BH4 deficiency have abnormally high levels of Phe in the blood, which can be harmful. KUVAN is used together with dietary restrictions to reduce Phe in the blood. Not everyone with PKU or BH4 deficiency responds to KUVAN but if you do respond, you may be able to tolerate more Phe in your diet than you could without treatment.
Your doctor may have prescribed KUVAN for another reason. Ask your doctor if you have any questions about why KUVAN has been prescribed for you.
This medicine is available only with a doctor's prescription.
Before you use KUVAN
When you must not use it
Do not take KUVAN if you have an allergy to:
- sapropterin dihydrochloride
- any of the other ingredients listed at the end of this leaflet
Symptoms of an allergic reaction may include:
- shortness of breath, wheezing, difficulty breathing or a tight feeling in your chest
- swelling of the face, lips, tongue or other parts of the body
- rash, itching, hives or flushed, red skin
Do not take KUVAN after the expiry date printed on the label of the bottle and the carton. If you take this medicine after the expiry date has passed, it may not work as well.
Do not take KUVAN if the packaging is torn or shows signs of tampering. If it has expired or is damaged, return it to your pharmacist or doctor for disposal.
If you are not sure whether you should start taking KUVAN, talk to your doctor or pharmacist.
Before you start to use it
Tell your doctor or pharmacist if you have allergies to:
- any other medicines
- any other substances, such as foods, preservatives or dyes
Tell your doctor if:
- you do not feel well before you start to take KUVAN. The levels of Phe in your blood may be higher if you are currently ill and your doctor may want to check that.
- you have or have had kidney or liver disease
- you have or have had convulsions ('fits')
- you have gastritis (inflammation of the lining of the stomach)
- you are over 50 years old
Tell your doctor if your child is to be treated with KUVAN and he or she is less than 4 years old. More frequent monitoring is recommended in children, particularly those less than 4 years old.
Tell your doctor if you are pregnant or intend to become pregnant. It is very important to control blood Phe during pregnancy as high Phe can harm you or your baby. Dietary management is the first option. Your doctor will consider the use of KUVAN only if strict dietary management does not adequately reduce your blood Phe levels.
Tell your doctor if you are breastfeeding or intend to breastfeed. It is not known whether KUVAN passes into breast milk. You should not take KUVAN if you are breastfeeding.
If you have not told your doctor about any of the above, tell them before you start taking KUVAN.
Taking other medicines
Tell your doctor or pharmacist if you are taking any other medicines, including medicines that you buy without a prescription from your pharmacy, supermarket or health food shop. You should also tell any health professional who is prescribing a new medication for you that you are taking KUVAN.
Tell your doctor or pharmacist if you are taking any of the following medicines:
- methotrexate (a medicine used to treat arthritis, psoriasis and some cancers)
- trimethoprim (an antibiotic)
- any heart medicine or angina treatment, e.g. glyceryl trinitrate, isosorbide dinitrate or sodium nitroprusside
- any medicine for the treatment of impotence, such as sildenafil, vardenafil or tadalafil
- minoxidil (a medicine used to treat hair loss and high blood pressure)
- levodopa (a medicine used to treat Parkinson's disease)
These medicines may be affected by KUVAN, or may affect how well it works. You may need to use different amounts of your medicines, or take different medicines. Your doctor will advise you.
Your doctor or pharmacist has more information on medicines to be careful with or avoid while taking KUVAN.
How to use KUVAN
Follow carefully all directions given to you by your doctor, dietician or pharmacist.
KUVAN is taken as a tablet or tablets dissolved in water. Do not use any liquid other than water to dissolve KUVAN.
It is not possible to know whether or not KUVAN will work for you until you start taking it. Your doctor will monitor your blood Phe levels when you start taking KUVAN to see if it is working.
How much to take and how often to take it
Your doctor will tell you how many tablets to take. You must take KUVAN exactly as explained by your doctor.
If you have PKU
Take the tablet(s) dissolved in water as a single daily dose with a meal and at the same time each day, preferably in the morning.
The usual starting dose of KUVAN in adults and children with PKU is 10 mg for each kg of body weight per day. Your doctor may adjust your dose depending on your condition and response to treatment. The recommended daily dose is between 5 mg and 20 mg for each kg of body weight per day.
If you have BH4 deficiency
Take the tablet(s) dissolved in water with a meal. It may be necessary to divide the total daily dose into 2 or 3 doses, distributed over the day, to achieve the best treatment effect.
The usual starting dose of KUVAN in adults and children with BH4 deficiency is 2 mg to 5 mg for each kg of body weight per day. Your doctor may adjust your dose depending on your condition and response to treatment. The recommended daily dose is between 2 mg and 20 mg for each kg of body weight per day.
How to use KUVAN
Dissolve KUVAN tablets in water while stirring. It may take a few minutes for the tablets to dissolve. To make the tablets dissolve faster you can crush them.
Don't worry if there are small particles in the solution. This will not affect how well it works.
Drink the solution with a meal.
Only prepare the solution when you are ready to take it. KUVAN solution should be drunk within 15 to 20 minutes after preparing it.
Place the number of tablet(s) your doctor has told you to take in a glass or cup (120 – 240 mL) of water and stir until dissolved.
For doses above 100 mg, place the number of tablet(s) your doctor has told you to take in a glass or cup (up to 120 mL) of water and stir until dissolved.
For doses below 100 mg, dissolve one tablet in 100 mL of water. Your doctor or pharmacist will tell you the volume of solution needed to give the correct dose. You will need to use an accurate measuring device (e.g. medicine cup or oral syringe) with suitable graduations for this purpose. If only a portion of this solution needs to be administered, an oral syringe should be used to withdraw the volume of solution to be administered. The solution may be transferred to another cup for administration of the medicine. Ask your pharmacist for an accurate measuring device. Discard any unused portion.
If necessary, KUVAN tablets can be dissolved in smaller volumes, e.g. for young children. The minimum volume required to dissolve each tablet is 20 mL, e.g. 1 tablet in 20 mL, 2 tablets in 40 mL and so on.
Ask your doctor or pharmacist if you have any questions about how to prepare KUVAN or how much to take.
Do not swallow the desiccant capsule contained in the bottle.
How long to take KUVAN
Continue taking KUVAN for as long as your doctor tells you.
Do not stop taking KUVAN without telling your doctor. Your doctor will need to carefully monitor your blood Phe levels if you stop taking KUVAN.
If you forget to take it
If you forget to take KUVAN at the right time, take it as soon as you remember on the same day.
Do not take a double dose to make up for a forgotten dose.
At your next appointment, tell your doctor if you forgot to take KUVAN.
If you take too much (Overdose)
Immediately contact your doctor or the Poisons Information Centre (In Australia telephone 131 126. In New Zealand telephone 0800 POISON or 0800 764 766) if you think you or someone else may have taken too much KUVAN.
Symptoms of an overdose with KUVAN may include headache and dizziness.
While you are taking KUVAN
Things you must do
Keep all your doctor's appointments so your progress can be checked. While you are taking KUVAN, your doctor will test your blood regularly to check your levels of Phe and tyrosine and may decide to adjust the dose of KUVAN or your diet if needed.
Contact your doctor if you become ill for any reason even if the illness is not related to the reason you are taking KUVAN. Your doctor may want to check your Phe levels as other illnesses may increase blood Phe.
Tell any other doctors, dentists and pharmacists who are treating you that you are taking KUVAN.
If you are about to be started on any new medicine, vitamin or supplement, tell your doctor, dentist or pharmacist that you are taking KUVAN.
Things you must not do
Do not change your diet without contacting your doctor. Any change you make to your diet may affect your blood Phe level. You must continue your diet treatment as recommended by your doctor.
Do not stop taking KUVAN unless your doctor tells you to. The Phe levels in your blood may increase. Your doctor will need to carefully monitor your blood Phe levels if you stop taking KUVAN.
Do not give KUVAN to anyone else, even if they have the same condition as you.
Do not take KUVAN to treat any other complaints unless your doctor tells you to.
Things to be careful of
It is not known if KUVAN affects your ability to drive or to operate machinery. Be careful driving or operating machinery until you know if KUVAN affects you.
Tell your doctor or pharmacist as soon as possible if you do not feel well while you are taking KUVAN.
All medicines can have side effects. Sometimes they are serious, most of the time they are not. You may need medical treatment if you get some of the adverse effects.
The levels of Phe in your blood may be higher if you are currently ill and your doctor may want to check that.
Like other medicines, KUVAN may have unwanted side effects.
Ask your doctor or pharmacist to answer any questions you may have.
Tell your doctor or pharmacist if you notice any of the following and they worry you:
- runny nose
- sore throat
- blocked or stuffy nose
- throat infection (pharyngitis)
- mouth and throat pain (oropharyngeal pain)
- diarrhoea (loose stools)
- upset stomach
- gastritis (inflammation of the lining of the stomach)
- oesophageal pain
- stomach ache
- allergic reactions (rash)
Gastritis may be severe. Call your doctor right away if you have any of these signs or symptoms: severe upper stomach area (abdominal) discomfort or pain, nausea and vomiting, blood in your vomit or stool, black or tarry stools.
Some side effects may be found only by having a blood test, e.g. levels of Phe in blood tests that are too low.
Other side effects not listed above may occur in some patients.
Tell your doctor or pharmacist if you notice anything that is making you feel unwell.
After taking KUVAN
Keep the tablets in the bottle in a cool dry place where the temperature stays below 25°C.
Do not leave them in the car on hot days or in the bathroom.
Keep the bottle tightly closed to protect the tablets from moisture.
Keep the desiccant tube in the bottle. Do not use the tablets if more than 2 months has passed since first opening the bottle.
Keep KUVAN where young children cannot reach it. A locked cupboard at least one-and-a-half metres above the ground is a good place to store medicines.
Once the bottle is opened, use the contents within two months or dispose.
Return any unused KUVAN to your pharmacist.
Also dispose of KUVAN as directed by your pharmacist if your doctor tells you to stop taking KUVAN, or the pack has passed its expiry date.
What it looks like
KUVAN is supplied as soluble tablets. The soluble tablets are off-white to light yellow with "177" printed on one face.
KUVAN is supplied in bottles with child-resistant closure containing 30 soluble tablets. Each bottle contains a small plastic tube of desiccant (silica gel).
- sapropterin dihydrochloride
- calcium hydrogen phosphate
- ascorbic acid
- sodium stearylfumarate
KUVAN is supplied in Australia by:
BioMarin Pharmaceutical Australia Pty Ltd
119 Willoughby Road
Crows Nest NSW 2065
Telephone (02) 8520 3255
KUVAN is supplied in New Zealand by:
Pharmacy Retailing (NZ) Limited t/a Healthcare Logistics
58 Richard Pearse Drive
Airport Oaks 2022
Telephone (09) 918 5100
For enquiries about KUVAN, contact email@example.com or call BioMarin:
Australia: 1800 387 876
New Zealand: 0800 882 012
To report adverse events, contact firstname.lastname@example.org or call BioMarin:
Australia: 1800 387 876
New Zealand: 0800 882 012
Australian Registration Number:
KUVAN 100mg AUST R 165738
This leaflet was prepared in October 2017.
® Registered trademark of BioMarin Pharmaceutical Inc.
Published by MIMS January 2018