Kuvan – Package Leaflet: InfoCons – Consumer Protection presents, in an accessible format, the official package leaflet of the medicine Kuvan, as authorised in the European Union and published in the Union Register of medicinal products.
Before anything else
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 pharmacis t.
- 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.
ℹ️ 1. What Kuvan is and what it is used for
Kuvan contains the active substa nce sapropterin which is a synthetic copy of a body’s own substance called tetrahydrobiopterin (BH4). BH4 is required by the body to use a n amino acid called phenylalanine in order to build another amino acid called tyrosine.
Kuvan is used to treat hyperpheny lalaninaemia (HPA) or phenylke tonuria (PKU) in patients of all ages. HPA and PKU are due to abnormally high levels of phenylalanine in t he 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 patie nts of all ages, in which the body cannot produce enough BH 4. Because of very low BH4 lev els 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 in creases the dietary tolerance to phenylalanine.
⚠️ 2. What you need to know before you take Kuvan
Do not take Kuvan
If you are allergic to sapropterin or any of the other ingredie nts of this medicine (listed in section 6).
Warnings and precautions
Talk to your doctor or pharmacist before taking Kuvan, particul arly:
- 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 du ring illness as blood phenylalanine levels may increase
- if you have predisposition to convulsions 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 do ctor. Do not change your diet without contacting your doctor. Even if you take Kuvan, if your phenyla lanine blood levels are not well controlled, you can develop severe neurologic problems. Your doctor should continue to monitor your blood phenylalanine levels often during your treatment with Kuvan, to make sure that your blood phenylalanine levels are not too high or too low.
Other medicines and Kuvan Tell your doctor or pha rmacist if you are taking, have recently taken or might take any other medicines. In particular you should tell your doctor if you are using:
- levodopa (used to treat Parkinson’s disease)
- medicines for treatment of cancer (e.g. methotrexate)
- medicines for treatment of bacterial infections (e.g. trimethop rim)
- medicines that cause dilation of blood vessels, (such as glyceryl trinitrate (GTN), isosorbide dinitrate (ISDN), sodium nitroprusside (SNP), molsidomin, minoxidil).
Pregnancy and breast feeding If you are pregnant or breast-feed ing, think you may be pregnant or are planning to have a baby, ask your doctor or pharmacist for advice before taking this medicine.
If you are pregnant your doctor will tell you how to control ph enylalanine levels adequately. If these are not strictly controlled before or when you become pregnant, this co uld be harmful to you and to your baby. Your doctor will monitor the restriction of dietary phenylalanine intake prior and during pregnancy.
If the strict diet does not adequately reduce phenylalanine amo unt in your blood your doctor will consider whether you must take this medicine. You should not take this medici ne if you are breast-feeding.
Driving and using machines
Kuvan is not expected to affect the ability to drive and use machines.
Important information about some of the ingredients of Kuvan This medicine contains less than 1 mmol sodium (23 mg) per tabl et, that is to say essentially ‘sodium-free’.
💊 3. How to take Kuvan
Always take this medicine exactly as your doctor has told you. Check with your doctor if you are not sure.
Dosing for PKU The recommended starting dose of Kuvan in patients with PKU is 10 mg for each kg of body weight. Take Kuvan as a single daily dose w ith a meal to increase the absorp tion, and at the same time each day, preferably in the morning. Your doctor may adjust your dose, us ually between 5 and 20 mg for each kg of body weight per day, depe nding on your condition.
Dosing for BH4 deficiency The recommended starting dose of Kuvan in patients with BH4 def iciency is 2 to 5 mg for each kg of body weight. Take Kuvan with a meal to increase the absorption. Divide the total daily dose into 2 or 3 doses, taken over the day. Your doctor may adjust your dose up to 20 m g for each kg of body weight per day, depending on your condition.
The table below is an example of how an appropriate dose is calculated Body weight (kg) Number of 100 mg tablets (dose 10 mg/kg) Number of 100 mg tablets (dose 20 mg/kg) 10 1 2 20 2 4 30 3 6 40 4 8 50 5 10 Method of administration For PKU patients, the total daily dose is taken once a day at t he same time each day, preferably in the morning.
For BH4 deficiency patients, the total daily dose is divided in to 2 or 3 doses over the day.
Use in all patients Place the prescribed number of tablets in a glass or cup of water as accurately described below and stir until dissolved. 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 i n the solution, but they will n ot affect the effectiveness of the medicine.
Drink the dissolved preparation of Kuvan with a meal within 15 to 20 minutes of its preparation.
Do not swallow the desiccant cap sule contained in the bottle.
Use in patients above 20 kg body weight Place the tablets in a glass or cup (120 to 240 ml) of water an d stir until dissolved.
Use in children up to 20 kg body weight The dose is based on body weight. T his will change as your child grows. Your doctor will tell you: the number of Kuvan tablets needed for one dose the amount of water needed to mix one dose of Kuvan the amount of solution you will need to give your child for the ir prescribed dose Your child should drink the solution with a meal.
Give your child the prescribed amount of solution within 15 to 20 minutes after dissolving. If you are not able to give your child’s dose within 15 to 20 minutes after di ssolving the tablets, you will need to prepare a new solution as the unused solution should not be used beyond 2 0 minutes.
Supplies needed to prepare and give your child’s dose of Kuvan The number of Kuvan tablets needed for one dose A medicine cup with graduation markings at 20, 40, 60 and 80 ml A glass or cup Small spoon or clean utensil for stirring Oral syringe (graduated in 1 ml divisions) (10 ml syringe for a dministration of volumes of ≤10 ml or 20 ml syringe for administration of volumes of >10 ml) Ask your doctor for the medicine cup for dissolving the tablets and the 10 ml or 20 ml oral syringe if you do not have these supplies.
Steps for preparing and taking your dose: Place the prescribed number of tablets in the medicine cup. Pou r the amount of water into the medicine cup, as instructed by your doctor (e.g. your doctor told you to use 20 ml for dissolving one Kuvan tablet). Check to make sure that the amount of liquid lin es up with the amount that your doctor tells you. Stir with the small s poon or clean utensil until the tablets dissolve. If your doctor told you to administer only a portion of the sol ution, point the tip of the oral syringe into the medicine cup. Slowly pull back the plunger to withdraw the amount as instructed by your doctor. Transfer the solution by pushing on the plunger slowly until al l of the solution in the oral syringe is transferred to a glass or cup for administration (e.g. if your doctor told you to dissolve two Kuvan tablets in 40 ml water and administer 30 ml to your child, you would have to use the 20 ml oral syringe two times to draw up 30 ml (e.g. 20 ml + 10 ml) of the solution and transfer it to a glass or cup for administration). Use a 10 ml oral syringe for administration of volumes ≤10 ml or a 20 ml oral syringe for administration of volumes >10 ml. If your baby is too small to dri nk from a glass or a cup you ma y administer the solution via the oral syringe. Draw up the prescribed volume from the solution prepar ed in the medicine cup and place the tip of the oral syringe into your baby’s mouth. Point the tip o f the oral syringe towards either cheek.
Push on the plunger slowly, a small amount at a time, until all of the solution in the oral syringe is given. Throw away any remaining solution. Remove the plunger from the barrel of the oral syringe. Wash both parts of the oral syringe and the medicine cup with warm w ater and air dry. When the oral syringe is dry, put the plunger back into the barrel. Store the oral syringe and the medicine cup for next use.
If you take more Kuvan than you should
If you take more Kuvan than prescr ibed, you may experience side effects that could include headache and dizziness. 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 mak e up for a forgotten dose. Take the next dose at the usual time.
If you stop taking Kuvan
Do not stop taking Kuvan without prior discussion with your doc tor, as phenylalanine levels in your blood may increase.
If you have any further questions on the use of this medicine, ask your doctor or pharmacist.
❗ 4. Possible side effects
Like all medicines, this medicine can cause side effects, altho ugh not everybody gets them.
Few cases of allergic reactions (such as skin rash and serious reactions) have been reported. Their frequency is not known (frequency cannot be estimated from the available data).
If you have red, itchy, raised ar eas (hives), runny nose, fast or uneven pulse, swelli ng of your tongue and throat, sneezing, wheezing, serious difficulty in breathing or dizziness, you may be having a serious allergic reaction to the medicine. If you notice these signs, contact yo ur doctor immediately.
Very common side effects
(may affect more than 1 in 10 people) Headache and runny nose.
Common side effects
(may affect up to 1 in 10 people) Sore throat, nasal congestion or stuffy nose, cough, diarrhoea, vomiting, stomach ache, too low levels of phenylalanine in blood tests, indigestion and feeling sick (nau sea) (see section 2: “Warnings and precautions”).
Not known side effects (frequency cannot be estimated from the available data)
Gastritis (inflammation of the lining of the stomach), oesophag itis (inflammation of the lining of the gullet).
Reporting of side effects
If you get any side effects, talk to your doctor or pharmacist or nurse. This includes any possible side effects not listed in this leaflet. You can also report side effects di rectly via the national reporting system listed in Appendix V . By reporting side effects you can help provide more informati on on the safety of this medicine.
🌡️ 5. How to store Kuvan
Keep this medicine out of th e sight and reach of children.
Do not use this medicine after th e 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 moistur e.
Do not throw away any medicines via wastewater or household was te. Ask your pharmacist how to throw away medicines you no longer use. These measures will help prot ect the environment.
📦 6. Contents of the pack and other information
What Kuvan contains
- The active substance is sapropt erin dihydrochloride. Each table t contains 100 mg of sapropterin dihydrochloride (equivalent to 77 mg of sapropterin).
- The other ingredients are mannitol (E421), calcium hydrogen phosphate anhydrous, c rospovidone type A, ascorbic acid (E300), sodium stearyl fumarate, and riboflavi n (E101).
What Kuvan looks like and contents of the pack
Kuvan 100 mg soluble tablets are off-white to light yellow with “177” imprinted on one face.
It is 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
BioMarin International Limited Shanbally, Ringaskiddy County Cork Ireland
Manufacturer
BioMarin International Limited Shanbally, Ringaskiddy County Cork Ireland
This leaflet was last revised in month/YYYY
Other sources of information Detailed information on this med icine is available on the Europ ean Medicines Agency web site: http://www.ema.europa.eu . There are also links to othe r websites about rare diseases an d treatments.
Package leaflet: Information for the patient Kuvan 100 mg powder for oral solution 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 pharmacis t.
- 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 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 substa nce sapropterin which is a synthetic copy of a body’s own substance called tetrahydrobiopterin (BH4). BH4 is required by the body to use a n amino acid called phenylalanine in order to build another amino acid called tyrosine.
Kuvan is used to treat hyperpheny lalaninaemia (HPA) or phenylke tonuria (PKU) in patients of all ages. HPA and PKU are due to abnormally high levels of phenylalanine in t he 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 patie nts of all ages, in which the body cannot produce enough BH 4. Because of very low BH4 lev els 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 in creases the dietary tolerance to phenylalanine. 2. What you need to know before you take Kuvan Do not take Kuvan
- if you are allergic to sapropterin or any of the other ingredients of this medicine (listed in section 6).
Warnings and precautions
Talk to your doctor or pharmacist before taking Kuvan, particul arly:
- 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 du ring illness as blood phenylalanine levels may increase
- if you have predisposition to convulsions 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 do ctor. Do not change your diet without contacting your doctor. Even if you take Kuvan, if your phenyla lanine blood levels are not well controlled, you can develop severe neurologic problems. Your doctor should continue to monitor your blood phenylalanine levels often during your treatment with Kuvan, to make sure that your blood phenylalanine levels are not too high or too low.
Other medicines and Kuvan
Tell your doctor or pha rmacist if you are taking, have recently taken or might take any other medicines. In particular you should tell your doctor if you are using: – levodopa (used to treat Parkinson’s disease)
- medicines for treatment of cancer (e.g. methotrexate)
- medicines for treatment of bacterial infections (e.g. trimethop rim)
- medicines that cause dilation of blood vessels, (such as glyceryl trinitrate (GTN), isosorbide dinitrate (ISDN), sodium nitroprusside (SNP), molsidomin, minoxidil).
Pregnancy and breast-feeding
If you are pregnant or breast-feed ing, think you may be pregnant or are planning to have a baby, ask your doctor or pharmacist for advice before taking this medicine.
If you are pregnant your doctor will tell you how to control ph enylalanine levels adequately. If these are not strictly controlled before or when you become pregnant, this co uld be harmful to you and to your baby. Your doctor will monitor the restriction of dietary phenylalanine intake prior and during pregnancy.
If the strict diet does not adequately reduce phenylalanine amo unt in your blood your doctor will consider whether you must take this medicine. You should not take this medici ne if you are breast-feeding.
Driving and using machines
Kuvan is not expected to affect the ability to drive and use machines.
Kuvan contains potassium citrate (E332)
This medicine contains 0.3 mmo l (12.6 mg) potassium per sachet. To be taken into consideration by patients with reduced kidney function or patients on a controlled potass ium diet.
3. How to take Kuvan Always take this medicine exactly as your doctor has told you. Check with your doctor if you are not sure.
Dosing for PKU The recommended starting dose of Kuvan in patients with PKU is 10 mg for each kg of body weight. Take Kuvan as a single daily dose w ith a meal to increase the absorp tion, and at the same time each day, preferably in the morning. Your doctor may adjust your dose, us ually between 5 and 20 mg for each kg of body weight per day, depe nding on your condition.
Dosing for BH4 deficiency The recommended starting dose of Kuvan in patients with BH4 def iciency is 2 to 5 mg for each kg of body weight. Take Kuvan with a meal to increase the absorption. Divide the total daily dose into 2 or 3 doses, taken over the day. Your doctor may adjust your dose up to 20 m g for each kg of body weight per day, depending on your condition.
The table below is an example of how an appropriate dose is calculated Body weight (kg) Number of 100 mg sachets (dose 10 mg/kg) Number of 100 mg sachets (dose 20 mg/kg) 10 1 2 20 2 4 30 3 6 40 4 8 Method of administration For PKU patients, the total daily dose is taken once a day at t he same time each day, preferably in the morning.
For BH4 deficiency patients, the total daily dose is divided in to 2 or 3 doses over the day.
Use in patients above 20 kg body weight Be sure that you know what dose of Kuvan powder your doctor pre scribed. For higher doses, your doctor may also prescibe Kuvan 500 mg powder for oral solution. Be sur e you know whether to use Kuvan 100 mg powder for oral solution, or both medicines to prepare your dos e. Open the sachet(s) only when you are ready to use them.
Preparing the sachet(s) Open the sachet(s) of Kuvan powder for oral solution by folding and tearing, or cutting at the dotted line in the upper right corner of the sachet. Empty the contents of the sachet(s) into 120 ml to 240 ml of water. After dissolving Kuvan powder in water, the solution must be clear, colourless to yellow.
Taking the medicine Drink the solution within 30 minutes.
Use in children up to 20 kg body weight Only use the 100 mg sachets to prepare Kuvan for children weigh ing up to 20 kg body weight.
The dose is based on body weight. T his will change as your child grows. Your doctor will tell you: the number of Kuvan 100 mg sachets needed for one dose the amount of water needed to mix one dose of Kuvan the amount of solution you will need to give your child for the ir prescribed dose Your child should drink the solution with a meal. Give your child the prescribed amount of solution within 30 min utes after dissolving. If you are not able to give your child’s dose within 30 minutes after dissolving the p owder, you will need to prepare a new solution as the unused solution should not be used beyond 30 minutes.
Supplies needed to prepare and give your child’s dose of Kuvan The number of Kuvan 100 mg sachets needed for one dose A medicine cup with graduation markings at 20, 40, 60 and 80 ml A glass or cup Small spoon or clean utensil for stirring Oral syringe (graduated in 1 ml divisions) (10 ml syringe for a dministration of volumes of ≤10 ml or 20 ml syringe for administration of volumes of >10 ml) Ask your doctor for the medicine cup for dissolving the powder and the 10 ml or 20 ml oral syringe if you do not have these supplies.
Steps for preparing and taking your dose: Place the prescribed number of Kuvan 100 mg sachets in the medi cine cup. Pour the amount of water into the cup, as instructed by your doctor (e.g. your doctor told you to use 20 ml for dissolving one Kuvan sachet). Check to make sure that the amount of liquid lines up with the amount that your doctor tells you. Stir with the small s poon or clean utensil until the powder dissolves. After dissolving the powder in water, the solution must be clear, colourless to yell ow. If your doctor told you to administer only a portion of the sol ution, point the tip of the oral syringe into the medicine cup. Slowly pull back the plunger to withdraw the amount as instructed by your doctor. Transfer the solution by pushing on the plunger slowly until al l of the solution in the oral syringe is transferred to a glass or cup for administration (e.g. if your doctor told you to dissolve two Kuvan 100 mg sachets in 40 ml water and administer 30 ml to your chil d, you would have to use the 20 ml oral syringe two times to draw up 30 ml (e.g. 20 ml + 10 ml) of the solution and transfer it to a glass or cup for administration). Use a 10 ml oral syringe for administr ation of volumes ≤10 ml or a 20 ml oral syringe for administration of volumes >10 ml. If your baby is too small to dri nk from a glass or a cup you ma y administer the solution via the oral syringe. Draw up the prescribed volume from the solution prepar ed in the medicine cup and place the tip of the oral syringe into your baby’s mouth. Point the tip o f the oral syringe towards either cheek.
Push on the plunger slowly, a small amount at a time, until all of the solution in the oral syringe is given. Throw away any remaining solution. Remove the plunger from the barrel of the oral syringe. Wash both parts of the oral syringe and the medicine cup with warm w ater and air dry. When the oral syringe is dry, put the plunger back into the barrel. Store the oral syringe and the medicine cup for next use.
If you take more Kuvan than you should
If you take more Kuvan than prescr ibed, you may experience side effects that could include headache and dizziness. 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 mak e up for a forgotten dose. Take the next dose at the usual time.
If you stop taking Kuvan
Do not stop taking Kuvan without prior discussion with your doc tor, as phenylalanine levels in your blood may increase.
If you have any further questions on the use of this medicine, ask your doctor or pharmacist.
4. Possible side effects Like all medicines, this medicine can cause side effects, altho ugh not everybody gets them.
Few cases of allergic reactions (such as skin rash and serious reactions) have been reported. Their frequency is not known (frequency cannot be estimated from the available data).
If you have red, itchy, raised ar eas (hives), runny nose, fast or uneven pulse, swelli ng of your tongue and throat, sneezing, wheezing, serious difficulty in breathing or dizziness, you may be having a serious allergic reaction to the medicine. If you notice these signs, contact yo ur doctor immediately.
Very common side effects
(may affect more than 1 in 10 people) Headache and runny nose.
Common side effects
(may affect up to 1 in 10 people) Sore throat, nasal congestion or stuffy nose, cough, diarrhoea, vomiting, stomach ache, too low levels of phenylalanine in blood tests, indigestion and feeling sick (nau sea) (see section 2: “Warnings and precautions”).
Not known side effects (frequency cannot be estimated from the available data)
Gastritis (inflammation of the lining of the stomach), oesophag itis (inflammation of the lining of the gullet).
Reporting of side effects If you get any side effects, talk to your doctor or pharmacist or nurse. This includes any possible side effects not listed in this leaflet. You can also report side effects directly via the national repo rting system listed in Appendix V . By reporting side effects you can help provide more information on the safety of this medicine.
5. How to store Kuvan Keep this medicine out of th e sight and reach of children.
Do not use this medicine after th e expiry date which is stated on the sachet and the carton after “EXP”. The expiry date refers to the last day of that month. Store below 25°C. Do not throw away any medicines via wastewater or household was te. Ask your pharmacist how to throw away medicines you no longer use. These measures will help prot ect the environment.
6. Contents of the pack and other information
What Kuvan contains
- The active substance is sapropterin dihydrochloride. Each sache t contains 100 mg of sapropterin dihydrochloride (equivalent to 77 mg of sapropterin).
- The other ingredients are mannitol (E421), potassium citrate (E332), sucralose (E955), ascorbic acid (E300).
What Kuvan looks like and contents of the pack
The powder for oral solution is clear, off-white to light yellow. The powder is filled in unit dose sachets containing 100 mg sapropterin dihydrochloride.
Each carton contains 30 sachets. Marketing Authorisation Holder and Manufacturer BioMarin International Limited Shanbally, Ringaskiddy County Cork Ireland This leaflet was last revised in MM/YYYY .
Other sources of information Detailed information on this med icine is available on the Europ ean Medicines Agency web site: http://www.ema.europa.eu . There are also links to othe r websites about rare diseases an d treatments.
Package leaflet: Information for the patient Kuvan 500 mg powder for oral solution 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 pharmacis t.
- 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 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 substa nce sapropterin which is a synthetic copy of a body’s own substance called tetrahydrobiopterin (BH4). BH4 is required by the body to use a n amino acid called phenylalanine in order to build another amino acid called tyrosine.
Kuvan is used to treat hyperpheny lalaninaemia (HPA) or phenylke tonuria (PKU) in patients of all ages. HPA and PKU are due to abnormally high levels of phenylalanine in t he 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 patie nts of all ages, in which the body cannot produce enough BH 4. Because of very low BH4 lev els 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 in creases the dietary tolerance to phenylalanine. 2. What you need to know before you take Kuvan Do not take Kuvan
- if you are allergic to sapropterin or any of the other ingredients of this medicine (listed in section 6).
Warnings and precautions
Talk to your doctor or pharmacist before taking Kuvan, particul arly:
- 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 du ring illness as blood phenylalanine levels may increase
- if you have predisposition to convulsions 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 do ctor. Do not change your diet without contacting your doctor. Even if you take Kuvan, if your phenyla lanine blood levels are not well controlled, you can develop severe neurologic problems. Your doctor should continue to monitor your blood phenylalanine levels often during your treatment with Kuvan, to make sure that your blood phenylalanine levels are not too high or too low.
Other medicines and Kuvan
Tell your doctor or pha rmacist if you are taking, have recently taken or might take any other medicines. In particular you should tell your doctor if you are using: – levodopa (used to treat Parkinson’s disease)
- medicines for treatment of cancer (e.g. methotrexate)
- medicines for treatment of bacterial infections (e.g. trimethop rim)
- medicines that cause dilation of blood vessels, (such as glyceryl trinitrate (GTN), isosorbide dinitrate (ISDN), sodium nitroprusside (SNP), molsidomin, minoxidil).
Pregnancy and breast-feeding
If you are pregnant or breast-feed ing, think you may be pregnant or are planning to have a baby, ask your doctor or pharmacist for advice before taking this medicine.
If you are pregnant your doctor will tell you how to control ph enylalanine levels adequately. If these are not strictly controlled before or when you become pregnant, this co uld be harmful to you and to your baby. Your doctor will monitor the restriction of dietary phenylalanine intake prior and during pregnancy.
If the strict diet does not adequately reduce phenylalanine amo unt in your blood your doctor will consider whether you must take this medicine. You should not take this medici ne if you are breast-feeding.
Driving and using machines
Kuvan is not expected to affect the ability to drive and use machines.
Kuvan contains potassium citrate (E332)
This medicine contains 1.6 mmo l (62.7 mg) potassium per sachet. This should be taken into consideration by patients with reduced kidney function or patients on a controll ed potassium diet.
3. How to take Kuvan Kuvan 500 mg is for use in patie nts above 25 kg body weight onl y.
Always take this medicine exactly as your doctor has told you. Check with your doctor if you are not sure.
Dosing for PKU The recommended starting dose of Kuvan in patients with PKU is 10 mg for each kg of body weight. Take Kuvan as a single daily dose w ith a meal to increase the absorp tion, and at the same time each day, preferably in the morning. Your doctor may adjust your dose, us ually between 5 and 20 mg for each kg of body weight per day, depe nding on your condition.
Dosing for BH4 deficiency The recommended starting dose of Kuvan in patients with BH4 def iciency is 2 to 5 mg for each kg of body weight. Take Kuvan with a meal to increase the absorption. Divide the total daily dose into 2 or 3 doses, taken over the day. Your doctor may adjust your dose up to 20 m g for each kg of body weight per day, depending on your condition.
Method of administration For PKU patients, the total daily dose is taken once a day at t he same time each day, preferably in the morning.
For BH4 deficiency patients, the total daily dose is divided in to 2 or 3 doses over the day.
Be sure that you know what dose of Kuvan powder your doctor pre scribed. For the exact dose your doctor may also prescibe Kuvan 100 mg powder for oral solution. Be sur e whether you should use Kuvan 500 mg powder for oral solution alone or both medicines to prepare you r dose. Open the sachet(s) only when you are ready to use them.
Preparing the sachet(s): Open the sachet(s) of Kuvan powder for oral solution by folding and tearing, or cutting at the dotted line in the upper right corner of the sachet. Empty the contents of the sachet(s) into 120 ml to 240 ml of wa ter. After dissolving powder in water, the solution must be clear, colourless to yellow.
Taking the medicine Drink the solution within 30 minutes.
If you take more Kuvan than you should
If you take more Kuvan than prescr ibed, you may experience side effects that could include headache and dizziness. 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 mak e up for a forgotten dose. Take the next dose at the usual time.
If you stop taking Kuvan
Do not stop taking Kuvan without prior discussion with your doc tor, as phenylalanine levels in your blood may increase.
If you have any further questions on the use of this medicine, ask your doctor or pharmacist.
4. Possible side effects Like all medicines, this medicine can cause side effects, altho ugh not everybody gets them.
Few cases of allergic reactions (such as skin rash and serious reactions) have been reported. Their frequency is not known (frequency cannot be estimated from the available data).
If you have red, itchy, raised ar eas (hives), runny nose, fast or uneven pulse, swelli ng of your tongue and throat, sneezing, wheezing, serious difficulty in breathing or dizziness, you may be having a serious allergic reaction to the medicine. If you notice these signs, contact yo ur doctor immediately.
Very common side effects
(may affect more than 1 in 10 people) Headache and runny nose.
Common side effects
(may affect up to 1 in 10 people) Sore throat, nasal congestion or stuffy nose, cough, diarrhoea, vomiting, stomach ache, too low levels of phenylalanine in blood tests, indigestion and feeling sick (nau sea) (see section 2: “Warnings and precautions”).
Not known side effects (frequency cannot be estimated from the available data)
Gastritis (inflammation of the lining of the stomach), oesophag itis (inflammation of the lining of the gullet).
52 Reporting of side effects If you get any side effects, talk to your doctor or pharmacist or nurse. This includes any possible side effects not listed in this leaflet. You can also report side effects directly via the national repo rting system listed in Appendix V . By reporting side effects you can help provide more information on the safety of this medicine.
5. How to store Kuvan Keep this medicine out of th e sight and reach of children.
Do not use this medicine after th e expiry date which is stated on the sachet and the carton after “EXP”. The expiry date refers to the last day of that month. Store below 25°C.
Do not throw away any medicines via wastewater or household was te. Ask your pharmacist how to throw away medicines you no longer use. These measures will help prot ect the environment.
6. Contents of the pack and other information
What Kuvan contains
- The active substance is sapropterin dihydrochloride. Each sache t contains 500 mg of sapropterin dihydrochloride (equivalent to 384 mg of sapropterin).
- The other ingredients are mannitol (E421), potassium citrate (E332), sucralose (E955), ascorbic acid (E300).
What Kuvan looks like and contents of the pack
The powder for oral solution is clear, off-white to light yellow. The powder is filled in unit dose sachets containing 500 mg sapropterin dihydrochloride.
Each carton contains 30 sachets.
Marketing Authorisation Holder and Manufacturer
BioMarin International Limited Shanbally, Ringaskiddy County Cork Ireland This leaflet was last revised in MM/YYYY .
Other sources of information Detailed information on this med icine is available on the Europ ean Medicines Agency web site: http://www.ema.europa.eu . There are also links to othe r websites about rare diseases an d treatments.
📄 Package Leaflet – official source
This article reproduces the information in the official package leaflet, as authorised in the European Union. You can consult the original document here: Product information Kuvan (PDF, ec.europa.eu).
You can check this medicine at any time in the Union Register of medicinal products (EU number EU/1/08/481).
⚠️ Important: This article is for information purposes only and does not replace medical advice or the package leaflet inside the medicine box. Read the leaflet carefully before use. For any questions about your treatment, talk to your doctor or pharmacist. If you experience side effects, tell your doctor or pharmacist and report them through your national reporting system.