SAPROPTERINA 100 mg Tabletka Rozpuszczalna

Treść ulotki

1. Sapropterina Dipharma 100 mg soluble tablets
   1. What is Sapropterina Dipharma and what is it used for
   2. What you need to know before taking Sapropterina Dipharma
   3. How to take Sapropterin Dipharma
   4. Possible side effects
   5. How to store Sapropterin Dipharma
   6. Contents of the pack and other information
2. Sapropterina Dipharma 100 mg powder for oral solution, 500 mg powder for oral solution
   1. What is Sapropterina Dipharma and what is it used for
   2. What you need to know before taking Sapropterina Dipharma
   3. How to take Sapropterin Dipharma
3. Sapropterina Dipharma in water, the solution should appear clear, from colorless to yellow.
   1. Possible side effects
   2. How to store Sapropterin Dipharma
   3. Contents of the pack and other information

Sapropterina Dipharma 100 mg soluble tablets

sapropterina dicloridrato (sapropterini dihydrochloridum)

Read this leaflet carefully before you start taking this medicine as it contains
important information for you.

• Keep this leaflet. You may need to read it again.
• If you have any questions, ask your doctor or pharmacist.
• This medicine has been prescribed for you only. Do not pass it on to others, even if they have the same symptoms as you, as it may be harmful.
• If you get any side effects, even those not listed in this leaflet, tell your doctor or pharmacist. See section 4.

Contents of this leaflet

• 1. What Sapropterina Dipharma is and what it is used for
• 2. What you need to know before you take Sapropterina Dipharma
• 3. How to take Sapropterina Dipharma
• 4. Possible side effects
• 5. How to store Sapropterina Dipharma
• 6. Contents of the pack and other information

1. What is Sapropterina Dipharma and what is it used for

Sapropterina Dipharma contains the active ingredient sapropterin, which is a synthetic copy of a
substance present in our body called tetrahydrobiopterin (BH4). The function of the
BH4 molecule is to help the body use an amino acid called phenylalanine to
produce another amino acid called tyrosine.
Sapropterina Dipharma is used to treat hyperphenylalaninemia (HPA) or phenylketonuria (PKU) in
patients of any age. HPA and PKU are due to excessively high levels of phenylalanine
in the blood, which can be harmful. Sapropterina Dipharma reduces these levels in some patients
who respond to BH4 and can help to increase the amount of phenylalanine that can be included
in the dietary intake.
This medicine is also used to treat an inherited disease called BH4 deficiency, in
patients of any age, due to which our body cannot produce enough BH4. Because of very low levels of BH4, phenylalanine is not used appropriately and its
levels increase causing harmful effects. By replacing the BH4 that our body does not
produce, Sapropterina Dipharma reduces the harmful effects of excess phenylalanine in the blood and
increases tolerance to phenylalanine in the diet.

2. What you need to know before taking Sapropterina Dipharma

Do not take Sapropterin Dipharma

• if you are allergic to sapropterin or to any of the other ingredients of this medicine (listed in section 6).

Warnings and precautions
Talk to your doctor or pharmacist before taking Sapropterin Dipharma, especially:

• if you are over 65 years old
• if you have problems with your kidneys or liver
• if you are ill. If you become ill, consulting your doctor is recommended as phenylalanine levels may increase
• if you have a predisposition to convulsions

During treatment with Sapropterina Dipharma, your doctor will perform blood tests
to determine the levels of phenylalanine and tyrosine present in it and may decide, if
necessary, to modify the dosage of Sapropterina Dipharma or the diet.
You must continue your dietary treatment as recommended by your doctor. Do not change your
diet without contacting your doctor. Even if you take Sapropterina Dipharma, serious neurological problems may occur if phenylalanine levels in the blood are not kept under
control. During treatment with Sapropterin Dipharma, the doctor must continue to frequently monitor
blood phenylalanine levels, to ensure that they are not too high or too
low.

Other medicines and Sapropterin Dipharma
Tell your doctor or pharmacist if you are taking, have recently taken or might take
any other medicines. In particular, you should inform your doctor if you are using:

• levodopa (used to treat Parkinson's disease)
• medicines used for the treatment of cancer (e.g. methotrexate)
• medicines used for the treatment of bacterial infections (e.g. trimethoprim)
• medicines that cause dilation of blood vessels (such as glyceryl trinitrate (GTN), isosorbide dinitrate (ISDN), sodium nitroprusside (SNP), molsidomine, minoxidil).

Pregnancy, breastfeeding and fertility
If you are pregnant, suspect you are pregnant or are planning to become pregnant, or if you are breastfeeding,
ask your doctor or pharmacist for advice before taking this medicine.
If you are pregnant, your doctor will tell you how to adequately control the level of
phenylalanine. If the level of phenylalanine is not well controlled before or during pregnancy, this may be harmful to you and the baby. The doctor will monitor the restriction of
phenylalanine intake through diet before and during pregnancy.
If a strict diet does not allow you to adequately reduce the amount of phenylalanine in the blood, the
doctor will decide whether you should take this medicine.
You should not take this medicine during breastfeeding.

Driving and operating machinery
It is not believed that the use of Sapropterin Diphama interferes with the ability to drive or operate
machinery.

Sapropterina Dipharma contains sodium
This medicine contains less than 1 mmol of sodium (23 mg) per tablet, i.e. essentially
‘sodium-free’.

3. How to take Sapropterin Dipharma

Take this medicine following exactly the instructions of your doctor. If you have any doubts, ask your doctor.

Dosage for PKU
The recommended initial dose of Sapropterin Dipharma in patients affected by PKU is 10 mg per
each kg of body weight. Take Sapropterin Dipharma once a day, with meals to increase its
absorption, always at the same time and preferably in the morning. Your doctor may
modify your dose, usually between 5 and 20 mg per day for each kg of body weight, depending on your
needs.

Dosage for BH4 deficiency
The recommended initial dose of Sapropterin Dipharma in patients affected by BH4 deficiency is
between 2 and 5 mg per each kg of body weight. Take Sapropterin Dipharma with meals
to increase its absorption. Divide the total daily dose into 2 or 3
administrations, taken throughout the day. Your doctor may modify your dose up to 20 mg per
day for each kg of body weight, depending on your needs.

The table below is an example of how an appropriate dose is calculated
Method of administration
For patients affected by PKU, the total daily dose must be taken once a day, always
at the same time and preferably in the morning.
For patients with BH4 deficiency, the total daily dose is divided into 2 or 3 administrations
throughout the day.

Use in all patients
Transfer the prescribed number of tablets into a glass or cup of water, as described in
detail below, and mix until dissolved.
The tablets may take a few minutes to dissolve. To speed up dissolution, the
tablets may be crushed. Some small particles may be visible within the
solution but they will not affect the effectiveness of the medicine.
Drink the dissolved Sapropterin Dipharma preparation with a meal, within 15 or 20
minutes of preparation.

Use in patients with body weight over 20 kg
Place the tablets in a glass or cup of water (120 - 240 ml) stirring until
dissolved.

Use in children with body weight up to 20 kg
The dose depends on body weight, which changes as your child grows. Your doctor will tell you:

• the number of Sapropterin Dipharma tablets needed for a dose
• the amount of water needed to prepare a dose of Sapropterin Dipharma
• the amount of solution to give to your child for the prescribed dose.

Your child must drink the solution with a meal.
Give your child the prescribed amount of solution within 15 or 20 minutes after dissolving. If you cannot give the dose to your child within 15 or 20 minutes of dissolving the tablets, you will need to prepare
a new solution, because the unused solution should not be used after 20
minutes have elapsed.

Material needed to prepare and give your child the dose of Sapropterin Dipharma

• The number of Sapropterin Dipharma tablets needed for a dose
• A graduated glass with markings at 20, 40, 60 and 80 ml.
• A glass or cup
• A teaspoon or clean utensil for stirring
• An oral dispensing syringe (with 1 ml markings) (10 ml syringe for administering volumes ≤ 10 ml or 20 ml syringe for administering volumes > 10 ml)

If you do not have the graduated glass for dissolving the tablets and the 10 ml or 20 ml syringe, ask your doctor for them.

Steps for preparing and taking the dose:

• Transfer the prescribed number of tablets into the graduated glass. Pour into the graduated glass the amount of water indicated by your doctor (for example, your doctor told you to use 20 ml to dissolve one Sapropterin Dipharma tablet). Make sure the amount of liquid corresponds to the amount indicated by your doctor. Mix with the teaspoon or clean utensil until the tablets are dissolved.
• If your doctor told you to administer only a part of the solution, immerse the tip of the oral dispensing syringe into the measuring glass. Slowly retract the plunger to draw up the amount indicated by your doctor.
• Transfer the solution by slowly pressing on the plunger until all the solution contained in the oral use syringe is transferred into a glass or cup for administration (for example, if your doctor told you to dissolve two Sapropterin Dipharma tablets in 40 ml of water and give 30 ml to your child, you must use the 20 ml oral use syringe twice to aspirate 30 ml (for example 20 ml + 10 ml) of solution and transfer it to a glass or cup for administration). Use a 10 ml oral use syringe for administering volumes ≤ 10 ml and a 20 ml oral use syringe for administering volumes > 10 ml.
• If your child is too small to drink from a glass or cup, you can administer the solution with the oral dispensing syringe. Aspirate the prescribed volume from the solution prepared in the graduated glass and place the tip of the oral dispensing syringe in your child's mouth. Direct the tip of the oral dispensing syringe towards the cheek. Slowly press on the plunger and administer the quantity little by little, until the total administration of the solution contained in the oral dispensing syringe.
• Discard any remaining solution. Remove the plunger from the body of the oral dispensing syringe. Wash the two components of the oral dispensing syringe and the graduated glass with warm water and let them air dry. When the oral dispensing syringe is dry, reinsert the plunger into the body of the syringe. Store the oral dispensing syringe and the measuring glass for subsequent use.

If you take more Sapropterin Dipharma than you should
If you take a dose of Sapropterin Dipharma higher than that prescribed, side effects may occur
including headache and dizziness. If you take a dose of Sapropterin
Dipharma higher than that prescribed, contact your doctor or pharmacist immediately.

If you forget to take Sapropterin Dipharma
Do not take a double dose to make up for the missed dose. Take the next dose
at the usual time.

If you stop taking Sapropterin Dipharma
Do not stop taking Sapropterin Dipharma without first consulting your doctor
as this may lead to an increase in phenylalanine levels in the blood.
If you have any doubts about the use of this medicine, ask your doctor or pharmacist.

4. Possible side effects

Like all medicines, this medicine can cause side effects, although not everybody gets them.
Few cases of allergic reactions (such as, for example, skin rashes and severe reactions) have been reported.
Their frequency is not known (frequency cannot be estimated based on available data).
If you experience reddened, itchy, and raised skin areas (urticaria), runny nose, rapid or irregular
pulse, swelling of the tongue and throat, sneezing, wheezing, severe difficulty breathing, or
dizziness, this may indicate a severe allergic reaction to the medicine. If you observe these
symptoms, seek medical attention 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 blocked nose, cough, diarrhea, vomiting, stomach pain, low
levels of phenylalanine in the blood, indigestion and nausea (see section 2: “Warnings and
precautions”).
Side effects with unknown frequency (frequency cannot be estimated based on available data)
Gastritis (inflammation of the inner lining of the stomach), esophagitis (inflammation of the
inner lining of the esophagus).

Reporting of side effects
If you get any side effects, even those not listed in this leaflet, tell your doctor or pharmacist.
You can also report side effects directly through the national reporting system as indicated in
Appendix V. By reporting side effects you can help provide more information on the safety of this
medicine.

5. How to store Sapropterin Dipharma

Keep this medicine out of the sight and reach of children.
Do not use this medicine after the expiry date which is stated on the bottle and the pack after
EXP.
The expiry date refers to the last day of that month.
This medicine does not require any particular storage temperature.
Keep the bottle tightly closed to protect the medicine from moisture.
Do not dispose of any medicine in wastewater or household waste. Ask your pharmacist how to
dispose of medicines you no longer use. This will help protect the environment.

6. Contents of the pack and other information

What Sapropterin Dipharma contains

• The active ingredient is sapropterin dihydrochloride. Each tablet contains 100 mg of sapropterin dihydrochloride equivalent to 77 mg of sapropterin
• The other ingredients are: mannitol (E421), crospovidone type A, copovidone K 28, ascorbic acid (E300), sodium stearyl fumarate, riboflavin (E101), anhydrous colloidal silica (E551). See section 2 “Sapropterina Dipharma contains sodium”

Description of the appearance of Sapropterin Dipharma and contents of the pack
Sapropterina Dipharma 100 mg soluble tablets are from white to off-white in colour, approximately
10 mm x 3.65 mm, with "11" imprinted on one side and the notch on the other side.
The notch is not designed for breaking the tablet.
It is available in a child-resistant screw-cap bottle containing a desiccant
(silica).
Pack sizes of 30 or 120 soluble tablets.
Not all pack sizes may be marketed.

Marketing Authorisation Holder
Dipharma B.V.
Prins Bernhardplein 200
1097JB Amsterdam
Netherlands

Manufacturer
Depo-Pack S.r.l.
Via Giovanni Morandi 28
21047 Saronno (VA)
Italy
or
Pharmadox Healthcare Ltd.
KW20A Kordin Industrial Park
Paola PLA3000
Malta

Other sources of information
More detailed information on this medicine is available on the European Medicines Agency website, http://www.ema.europa.eu/ .

Sapropterina Dipharma 100 mg powder for oral solution, 500 mg powder for oral solution

sapropterina dihydrochloride (sapropterini dihydrochloridum)

Read this leaflet carefully before you start to take this medicine as it contains
important information for you.

• Keep this leaflet. You may need to read it again.
• If you have any questions, ask your doctor or pharmacist.
• This medicine has been prescribed for you only. Do not pass it on to others, even if their symptoms are the same as yours, as it could be harmful.
• If you get any side effects, even those not listed in this leaflet, tell your doctor or pharmacist. See section 4.

Contents of this leaflet

• 1. What Sapropterina Dipharma is and what it is used for
• 2. What you need to know before you take Sapropterina Dipharma
• 3. How to take Sapropterina Dipharma
• 4. Possible side effects
• 5. How to store Sapropterina Dipharma
• 6. Contents of the pack and other information

1. What is Sapropterina Dipharma and what is it used for

Sapropterina Dipharma contains the active ingredient sapropterin, which is a synthetic copy of a
substance present in our body called tetrahydrobiopterin (BH4). The function of the BH4 molecule is to
help the body use an amino acid called phenylalanine to produce another amino acid called tyrosine.
Sapropterina Dipharma is used to treat hyperphenylalaninemia (HPA) or phenylketonuria (PKU) in
patients of any age. HPA and PKU are due to excessively high levels of phenylalanine in the blood,
which can be harmful. Sapropterina Dipharma reduces these levels in some patients who respond to BH4
and can help to increase the amount of phenylalanine that can be included in the dietary intake.
This medicine is also used to treat an inherited disease called BH4 deficiency, in patients of any age,
due to which our body cannot produce enough BH4. Because of very low levels of BH4, phenylalanine is
not used appropriately and its levels increase causing harmful effects. By replacing the BH4 that our
body does not produce, Sapropterina Dipharma reduces the harmful effects of excess phenylalanine in the
blood and increases tolerance to phenylalanine in the diet.

2. What you need to know before taking Sapropterina Dipharma

Do not take Sapropterin Dipharma

• if you are allergic to sapropterin or to any of the other ingredients of this medicine (listed in section 6).

Warnings and precautions
Talk to your doctor or pharmacist before taking Sapropterin Dipharma, especially:

• if you are over 65 years old
• if you have problems with your kidneys or liver
• if you are ill. If you become ill, consulting your doctor is recommended as phenylalanine levels may increase
• if you are prone to convulsions

During treatment with Sapropterina Dipharma, your doctor will perform blood tests
to determine the levels of phenylalanine and tyrosine present in it and may decide, if
necessary, to modify the dosage of Sapropterina Dipharma or your diet.
You must continue your dietary treatment as recommended by your doctor. Do not change your
diet without contacting your doctor. Even if you take Sapropterina Dipharma, serious neurological problems may occur if phenylalanine levels in the blood are not kept under
control. During treatment with Sapropterin Dipharma, the doctor must continue to frequently monitor
phenylalanine levels in the blood, to ensure that they are not too high or too
low.

Other medicines and Sapropterin Dipharma
Tell your doctor or pharmacist 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 used for the treatment of cancer (e.g. methotrexate)
• medicines used for the treatment of bacterial infections (e.g. trimethoprim)
• medicines that cause dilation of blood vessels (such as glyceryl trinitrate (GTN), isosorbide dinitrate (ISDN), sodium nitroprusside (SNP), molsidomine, minoxidil).

Pregnancy, breastfeeding and fertility
If you are pregnant, suspect you are pregnant, are planning to become pregnant or are breastfeeding with
breast milk, ask your doctor or pharmacist for advice before taking this medicine.
If you are pregnant, your doctor will tell you how to adequately control your phenylalanine level.
If the phenylalanine level is not well controlled before or during pregnancy, this may be harmful to you and to the baby. The doctor will monitor the limitation of
phenylalanine intake through diet before and during pregnancy.
If a strict diet does not allow you to adequately reduce the amount of phenylalanine in the blood, the
doctor will decide whether you should take this medicine.
You should not take this medicine during breastfeeding.

Driving and operating machinery
It is not believed that the use of Sapropterin Diphama interferes with the ability to drive or operate
machinery.

Sapropterina Dipharma contains potassium

Sapropterina Dipharma 100 mg powder for oral solution
This medicine contains 0.3 mmol (11.7 mg) of potassium per sachet. This should be taken into consideration in
people with reduced kidney function or who are following a low-potassium diet.

Sapropterina Dipharma 500 mg powder for oral solution
This medicine contains 1.6 mmol (62.6 mg) of potassium per sachet. This should be taken into consideration in
people with reduced kidney function or who are following a low-potassium diet.

3. How to take Sapropterin Dipharma

Take this medicine following the instructions of your doctor exactly. If you have any doubts, ask your
doctor.
Sapropterina Dipharma 500 mg should be used exclusively in patients with a body weight
greater than 25 kg.

Dosage for PKU
The recommended initial dose of Sapropterina Dipharma in patients with PKU is 10 mg per
kg of body weight. Take Sapropterina Dipharma once a day, with meals to increase absorption, always at the same time and preferably in the morning. Your doctor may
modify your dose,
usually between 5 and 20mg per day for each kg of body weight, depending on your needs.

Dosage for BH4 deficiency
The recommended initial dose of Sapropterina Dipharma in patients with BH4 deficiency is
between 2 and 5 mg per kg of body weight. Take Sapropterina Dipharma with meals to increase absorption. Divide the total daily dose into 2 or 3
administrations, taken throughout the day. Your doctor may modify your dose up to 20 mg per
day for each kg of body weight, depending on your needs.

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

Method of administration
For patients with PKU, the total daily dose should be taken once a day, always
at the same time and preferably in the morning.
For patients with BH4 deficiency, the total daily dose is divided into 2 or 3 administrations
throughout the day.

Use in patients with body weight greater than 20 kg
Make sure you know the dose of Sapropterina Dipharma powder prescribed by your doctor.

Sapropterina Dipharma 100 mg powder for oral solution
For higher doses, your doctor may also prescribe Sapropterina Dipharma 500 mg powder for
oral solution.

Sapropterina Dipharma 500 mg powder for oral solution
For the exact dose, your doctor may also prescribe Sapropterina Dipharma 100 mg powder for
oral solution.
Make sure you know whether you need to use only Sapropterina Dipharma 100 mg or 500 mg powder for
oral solution, or both medicines to prepare the dose.
Open the sachet(s) only when you are ready to use it/them.

Preparation of the sachet(s)

• Open the Sapropterina Dipharma powder for oral solution sachet(s) by folding and tearing or cutting along the dotted line in the upper right corner of the sachet.
• Empty the contents of the sachet(s) into 120 - 240 ml of water. After dissolving the powder of

Sapropterina Dipharma in water, the solution should appear clear, from colorless to yellow.

Taking the medicine

• Drink the solution within 30 minutes.

Use in children with body weight up to 20 kg
For the preparation of Sapropterina Dipharma for children with body weight up to 20 kg, use only
100 mg sachets.
The dose depends on the body weight, which changes as your child grows. The doctor will tell you:

• the number of Sapropterina Dipharma 100 mg sachets needed for a dose
• the amount of water needed to prepare a dose of Sapropterin Dipharma
• the amount of solution to administer to your child for the prescribed dose.

Your child must drink the solution in conjunction with a meal.
Give your child the prescribed amount of solution within 30 minutes after dissolution. If you cannot
give the dose to your child within 30 minutes of dissolving the powder, you will need to prepare a new
solution, because the unused solution should not be used after 30 minutes have elapsed.

Materials needed to prepare and give your child the dose of Sapropterin Dipharma

• The number of Sapropterina Dipharma 100 mg sachets needed for a dose
• A graduated glass with markings at 20, 40, 60 and 80 ml.
• A glass or cup
• A teaspoon or clean utensil for mixing
• An oral administration syringe (with markings of 1 ml) (10 ml syringe for administering volumes ≤ 10 ml or 20 ml syringe for administering volumes > 10 ml)

If you do not have the graduated glass to dissolve the sachets and the 10 ml or 20 ml syringe, request them
from your doctor.

Steps for preparing and taking the dose:

• Transfer the prescribed number of Sapropterina Dipharma 100 mg sachets into the graduated glass. Pour the amount of water indicated by the doctor into the graduated glass (for example, the doctor told you to use 20 ml to dissolve one sachet of Sapropterin Dipharma). Make sure that the amount of liquid corresponds to the amount indicated by the doctor. Mix with the teaspoon or clean utensil until the sachet dissolves. After dissolving the powder in water, the solution should appear clear, from colorless to yellow.
• If the doctor told you to administer only a part of the solution, immerse the tip of the oral administration syringe into the measuring glass. Slowly retract the plunger to draw up the amount indicated by the doctor.
• Transfer the solution by slowly pressing on the plunger until all the solution contained in the oral syringe is transferred into a glass or cup for administration (for example, if the doctor told you to dissolve two sachets of Sapropterina Dipharma 100 mg in 40 ml of water and give 30 ml to your child, you must use the 20 ml oral syringe twice to draw up 30 ml (for example 20 ml + 10 ml) of solution and transfer it to a glass or cup for administration). Use a 10 ml oral syringe for administering volumes ≤ 10 ml and a 20 ml oral syringe for administering volumes > 10 ml.
• If your child is too young to drink from a glass or cup, you can administer the solution with the oral administration syringe. Draw up the prescribed volume from the prepared solution in the graduated glass and place the tip of the oral administration syringe in your child's mouth. Direct the tip of the oral administration syringe towards the cheek. Press slowly on the plunger and administer the quantity little by little, until the total administration of the solution contained in the oral administration syringe.
• Discard any remaining solution. Remove the plunger from the body of the oral administration syringe. Wash the two components of the oral administration syringe and the graduated glass with warm water and let them air dry. When the oral administration syringe is dry, reinsert the plunger into the body of the syringe. Store the oral administration syringe and the measuring glass for subsequent use.

If you take more Sapropterina Dipharma than you should
If you take a dose of Sapropterina Dipharma higher than that prescribed, you may
experience adverse effects including headache and dizziness. If you take a dose of Sapropterina
Dipharma higher than that prescribed, contact your doctor or pharmacist immediately.

If you forget to take Sapropterin Dipharma
Do not take a double dose to make up for a missed dose. Take the next dose
at the usual time.

If you stop taking Sapropterin Dipharma
Do not stop taking Sapropterina Dipharma without first consulting your doctor
as this may lead to an increase in the level of phenylalanine in the blood.
If you have any doubts about the use of this medicine, ask your doctor or pharmacist.

4. Possible side effects

Like all medicines, this medicine can cause side effects, although not everybody gets them.
Few cases of allergic reactions (such as for example skin rashes and severe reactions) have been reported.
Their frequency is not known (frequency cannot be estimated from the available data).
If you experience reddened, itchy, and raised skin areas (urticaria), runny nose, rapid or irregular
pulse, swelling of the tongue and throat, sneezing, wheezing, severe difficulty breathing or
dizziness, this may indicate a severe allergic reaction to the medicine. If you observe these
symptoms, seek medical attention 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 blocked nose, cough, diarrhoea, vomiting, stomach pain, low levels of
phenylalanine in the blood, indigestion and nausea (see section 2: “Warnings and
precautions”).
Side effects with unknown frequency (frequency cannot be estimated from the available data)
Gastritis (inflammation of the inner lining of the stomach), esophagitis (inflammation of the
inner lining of the esophagus).

Reporting of side effects
If you get any side effects, talk to your doctor or pharmacist. You can also report side effects directly
through the national reporting system as indicated in Annex V. By reporting side effects you can help
to provide more information on the safety of this medicine.

5. How to store Sapropterin Dipharma

Keep this medicine out of the sight and reach of children.
Do not use this medicine after the expiry date which is stated on the sachet and on the pack after
EXP. The expiry date refers to the last day of that month.
This medicine does not require any particular storage conditions.
Do not dispose of any medicine in waste water or household waste. Ask your pharmacist how
to eliminate medicines that you no longer use. This will help protect the environment.

6. Contents of the pack and other information

What Sapropterin Dipharma contains

• The active ingredient is sapropterin dihydrochloride. Sapropterina Dipharma 100 mg: Each sachet contains 100 mg of sapropterin dihydrochloride equivalent to 77 mg of sapropterin. Sapropterina Dipharma 500 mg: Each sachet contains 500 mg of sapropterin dihydrochloride equivalent to 384 mg of sapropterin.
• The other ingredients are: mannitol (E421), potassium citrate (E332), sucralose (E955) and ascorbic acid (E300). See section 2 “Sapropterina Dipharma contains potassium”

Description of the appearance of Sapropterin Dipharma and contents of the pack
The oral solution powder is from white to yellowish in color. The powder is contained in single-dose sachets containing 100 mg or 500 mg of sapropterin dihydrochloride.
Packs of 30 sachets.

Marketing authorisation holder
Dipharma B.V.
Prins Bernhardplein 200
1097JB Amsterdam
Netherlands

Manufacturer
Depo-Pack S.r.l.
Via Giovanni Morandi 28
21047 Saronno (VA)
Italy
or
Pharmadox Healthcare Ltd.
KW20A Kordin Industrial Park
Paola PLA3000
Malta

Other sources of information
More detailed information on this medicine is available on the website of the European
Medicines Agency, http://www.ema.europa.eu/ .