PROCYSBI 25 mg Enteric-Coated Hard Capsule

Зміст інструкції

1. PROCYSBI 25 mg gastro-resistant hard capsules, 75 mg gastro-resistant hard capsules
   1. What is PROCYSBI and what is it used for
   2. What you need to know before taking PROCYSBI
   3. How to take PROCYSBI
   4. Possible side effects
   5. How to store PROCYSBI
   6. Contents of the pack and other information
2. PROCYSBI 75 mg gastro-resistant granules, 300 mg gastro-resistant granules
   1. What is PROCYSBI and what is it used for
   2. What you need to know before taking PROCYSBI
   3. How to take PROCYSBI
   4. Possible side effects
   5. How to store PROCYSBI
   6. Package contents and other information

PROCYSBI 25 mg gastro-resistant hard capsules, 75 mg gastro-resistant hard capsules

cysteamine (mercaptamine bitartrate)

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

1. What is PROCYSBI and what is it used for

PROCYSBI contains the active substance cysteamine (also known as mercaptamine) and is taken for
the treatment of nephropathic cystinosis in children and adults. Cystinosis is a disease that affects
body functions, with an abnormal accumulation of the amino acid cystine in various organs of the body, such as
the kidneys, eyes, muscles, pancreas and brain. The accumulation of cystine damages the kidneys and causes
the excretion of excessive amounts of glucose, proteins and electrolytes. Different organs are affected at
different ages.
PROCYSBI is a medicine that reacts with cystine to decrease its level inside the
cells. To obtain the maximum benefit, therapy with cysteamine should be started immediately after
confirmation of the diagnosis of nephropathic cystinosis.

2. What you need to know before taking PROCYSBI

Do not take PROCYSBI

• if you are allergic to cysteamine (also known as mercaptamine) or to any of the other ingredients of this medicine (listed in section 6).
• if you are allergic to penicillamine (this is not “penicillin”, but a medicine used to treat Wilson’s disease).
• if you are breastfeeding.

Warnings and precautions
Talk to your doctor or pharmacist before taking PROCYSBI.

• Because oral cysteamine does not prevent the deposition of cystine crystals in the eyes, you must continue to use the cysteamine eye solution prescribed by your doctor.
• To avoid the risk of choking, whole cysteamine capsules should not be given to children under approximately 6 years of age (refer to section 3 “How to take PROCYSBI – Method of administration”).
• Severe skin lesions may occur in patients treated with high doses of cysteamine. Your doctor will routinely monitor your skin and bones and will reduce or discontinue treatment if necessary (see section 4).
• Ulcers and bleeding in the stomach and intestines may occur in patients taking cysteamine (see section 4).
• Other intestinal symptoms, including nausea, vomiting, anorexia and stomach pain, may occur with cysteamine. If these symptoms occur, your doctor may suspend and modify the dosage.
• Contact your doctor if you experience unusual stomach symptoms or changes in stomach symptoms.
• Symptoms such as dizziness, fatigue, drowsiness, depression and brain diseases (encephalopathy) may occur with cysteamine. If you experience these symptoms, contact your doctor who will adjust the dosage.
• Abnormalities in liver function or a reduction in white blood cell count (leukopenia) may occur with the use of cysteamine. Your doctor will routinely monitor your white blood cell count and your liver function.
• Your doctor will check you for benign intracranial hypertension (or pseudotumor cerebri [PTC]) and/or swelling of the optic nerve (papilledema) associated with cysteamine treatment. You will undergo regular eye examinations to identify this condition, as prompt treatment can prevent vision loss. Other medicines and PROCYSBITell your doctor or pharmacist if you are taking, have recently taken or might take any other medicines. If your doctor prescribes bicarbonate, do not take it at the same time as PROCYSBI; take the bicarbonate at least one hour before or at least one hour after the medicine.

PROCYSBI with food and drink
For at least 1 hour before and 1 hour after taking PROCYSBI, try to avoid meals rich in
fats or proteins and any food or liquid that may reduce stomach acidity, such as milk or
yogurt. If this is impossible, you can eat a small amount (about 100 grams) of food
(preferably carbohydrates, e.g. bread, pasta, fruit) during the hour before and after
taking PROCYSBI.
Take the capsule with an acidic drink (such as orange juice or any acidic fruit juice) or
water. For children and patients who have difficulty swallowing, refer to section 3
“How to take PROCYSBI – Method of administration”.

Pregnancy and breastfeeding
If you are pregnant, suspect you may be pregnant, are planning a pregnancy or are breastfeeding,
ask your doctor or pharmacist for advice before taking this medicine.
This medicine should not be used during pregnancy, especially during the first trimester.
Before starting treatment, you must perform a pregnancy test and this must be negative, while during treatment you must use adequate contraception. If you are
planning a pregnancy or if you are pregnant, contact your doctor immediately to
request discontinuation of therapy with this medicine as continued treatment
may be harmful to the unborn child.
Do not use this medicine if you are breastfeeding (see section 2 under “Do not take
PROCYSBI”).

Driving and operating machinery
This medicine may cause some drowsiness. When you start therapy, avoid driving, using machinery, or performing other dangerous activities until you know the effects
of the medicine on your abilities.

PROCYSBI contains sodium
This medicine contains less than 1 mmol (23 mg) of sodium per dose, i.e. it is essentially ‘sodium-free’.

3. How to take PROCYSBI

Take this medicine following exactly the instructions of your doctor or pharmacist. If you
have any doubts, ask your doctor or pharmacist.
The recommended dose prescribed for you or for your child will depend on your age and weight or
on that of your child. The planned maintenance dose is 1.3 g/m²/day.

Dosage Schedule
Take this medicine twice a day, every 12 hours. To get the maximum benefit from
this medicine, try to avoid regularly meals and dairy products for at least 1 hour
before and 1 hour after taking PROCYSBI. If this is impossible, you can eat a small
amount (about 100 grams) of food (preferably carbohydrates, e.g. bread, pasta, fruit) during the hour
before and after taking PROCYSBI.
It is important to take PROCYSBI regularly over time.
Do not increase or decrease the amount of medicine without your doctor’s approval.
The usual total dose should not exceed 1.95 g/m²/day.

Duration of treatment
Treatment with PROCYSBI lasts for life, as established by your doctor.

Method of administration
This medicine can only be taken by mouth.
For this medicine to work correctly, follow these instructions:

• For patients who are able to swallow the whole capsule: Swallow the capsule whole with an acidic beverage (such as orange juice or any acidic fruit juice) or water. Do not crush or chew the capsules or their contents. Children under 6 years of age may not be able to swallow hard gelatin capsules and may therefore choke. PROCYSBI can be given to children under 6 years of age by opening the capsules and dispersing the contents in food or liquid, as instructed below.

For patients who are unable to swallow the whole capsule or who use a feeding tube:
Taking the medicine dispersed in food
Open the hard gelatin capsules and disperse the contents (granules) in about 100 grams of
food, such as applesauce or fruit jam.
Gently stir the granules into the soft food, creating a mixture of granules and food.
Ingest the entire mixture, then drink 250 mL of an acidic beverage (such as orange juice or
any acidic fruit juice) or water to make it easier to swallow.
If you do not consume the mixture immediately, it can be stored in the refrigerator (2 °C-8 °C)
from the time of preparation to the time of administration and consumed within 2 hours of
preparation. Any remaining mixture should not be stored for more than 2 hours.
Administration via a feeding tube
Open the hard gelatin capsules and disperse the contents (granules) in about 100 grams of
applesauce or fruit jam. Gently stir the granules into the soft food,
creating a mixture of granules and food. Administer the mixture through a
gastrostomy tube, a nasogastric tube, or a gastrojejunal tube using a
catheter syringe. Before administering PROCYSBI: open the gastrostomy button
and attach the feeding tube. Flush with 5 mL of water to clean the button. Aspirate the mixture
into the syringe. The use of a maximum of 60 mL of mixture in a catheter syringe
with a straight feeding tube or for bolus administration is recommended. Insert the opening of the syringe
containing the PROCYSBI and food mixture into the opening of the feeding tube and fill
completely with the mixture. To prevent possible obstruction problems, apply
gentle pressure to the syringe and keep the feeding tube in a horizontal position
during administration. To avoid the risk of obstruction, it is also recommended to use
a viscous food, such as applesauce or fruit jam, administering it at
a rate of about 10 mL every 10 seconds until the syringe is completely emptied. Repeat the
step above until all the mixture has been administered. After
administering PROCYSBI, aspirate 10 mL of fruit juice or water into another syringe and
flush the gastrostomy tube, making sure that no residue of the PROCYSBI and food mixture remains
blocked in the gastrostomy tube.
If you do not consume the mixture immediately, it can be stored in the refrigerator (2 °C-8 °C)
from the time of preparation to the time of administration and consumed within 2 hours of
preparation. Any remaining mixture should not be stored for more than 2 hours.
Consult your pediatrician for precise instructions on the correct methods of administering
the medicine through a feeding tube and in case of feeding tube obstruction problems.
Taking the medicine dispersed in orange juice or any acidic fruit juice or in
water
Open the hard gelatin capsules and disperse the contents (granules) in about 100-150 mL of
acidic fruit juice (such as orange juice or any acidic fruit juice) or water.
Gently mix the PROCYSBI mixture for 5 minutes, mixing it in a cup or
stirring it in a cup with a lid (e.g., a sippy cup), then drink the mixture.
If you do not drink the mixture immediately, it can be stored in the refrigerator (2 °C-8 °C) from
the time of preparation to the time of administration and drunk within 30 minutes of
preparation. Any remaining mixture should not be stored for more than 30 minutes.
Administering a mixture to drink using an oral syringe
Aspirate the mixture to drink into a dosing syringe and administer it directly into the mouth.
If you do not consume the mixture immediately, it can be stored in the refrigerator (2 °C-8 °C)
from the time of preparation to the time of administration and consumed within 30 minutes
of preparation. Any remaining mixture should not be stored for more than 30 minutes.
Your doctor may recommend or prescribe including, in addition to cysteamine, one or more
supplements to replace important electrolytes that are lost through the kidneys. It is important
to take these supplements exactly as instructed. Consult your doctor if you have
missed several doses of these supplements or if you experience weakness or drowsiness.
Regular blood tests to measure the amount of cystine within white blood cells and/or the
concentration of cysteamine in the blood are necessary to determine the correct dose of
PROCYSBI. You or your doctor will arrange for the blood tests to be performed.
These tests must be performed 12.5 hours after the evening dose the previous day, and
therefore 30 minutes after taking the morning dose the following day. Regular blood and urine tests are also needed to measure the levels of important electrolytes for
the body to help you or your doctor adjust the doses of these supplements correctly.

If you take more PROCYSBI than you should
If you have taken more PROCYSBI than you should, contact your doctor or the emergency room immediately.
You may feel drowsy.

If you forget to take PROCYSBI
If you forget to take a dose of medicine, take it as soon as possible. However, if
less than 4 hours remain until the next dose, skip the missed dose and resume your regular dosing schedule.
Do not take a double dose to make up for a missed dose.
If you have any doubts about using 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.

Seek immediate medical or nursing attention if you notice any of the following side effects - you may need urgent medical treatment:

• Severe allergic reaction (uncommon): seek emergency medical assistance if you experience any of these signs of an allergic reaction: hives; difficulty breathing; swelling of the face, lips, tongue, or throat.

If you experience any of the following side effects, contact your doctor immediately. Because some of
these are serious, consult your doctor for a more detailed explanation of their warning signs.

Common side effects(may affect up to 1 in 10 people):

• Skin rash: tell your doctor immediately if you have a skin rash. It may be necessary to temporarily stop PROCYSBI until the rash clears up. If the rash is severe, your doctor may stop treatment with cysteamine.
• Abnormal liver function tests in the blood. Your doctor will monitor you for this.

Uncommon side effects(may affect up to 1 in 100 people):

• Skin lesions, bone lesions and joint problems: treatment with high doses of cysteamine can lead to the development of skin lesions. These include striae of the skin (which look like whip marks), bone lesions (such as fractures), bone deformities and joint problems. Check your skin while taking this medicine. Tell your doctor about any changes. Your doctor will monitor you for this.
• Decreased white blood cell count. Your doctor will monitor you for this.
• Central nervous system symptoms: some patients taking cysteamine have developed dizziness, depression and become too apathetic (excessive drowsiness). If you experience these symptoms, tell your doctor.
• Stomach and intestinal (gastrointestinal) problems: patients taking cysteamine have developed ulcers and bleeding. Tell your doctor immediately if you have stomach pain, or if you vomit blood.
• Benign intracranial hypertension, also called pseudotumor cerebri, has been reported with the use of cysteamine. This is a condition in which there is an increase in pressure of fluids around the brain. Tell your doctor immediately if you develop any of the following symptoms while taking PROCYSBI: ringing or “whooshing” sounds in the ear, dizziness, double vision, blurred vision, loss of vision, pain behind the eye or pain associated with eye movement. Your doctor will perform eye exams to detect and treat this problem promptly. This will help reduce the likelihood of vision loss.

The other side effects listed below are provided with an estimate of the frequency with which
they may occur with PROCYSBI.

Very common side effects(may affect more than 1 in 10 people):

• nausea
• vomiting
• loss of appetite
• diarrhea
• fever
• feeling sleepy

Common side effects

• headache
• encephalopathy
• abdominal pain
• dyspepsia
• bad breath and body odor
• heartburn
• fatigue

Uncommon side effects

• leg pain
• scoliosis (curvature of the spine)
• brittle bones
• hair discoloration
• seizures
• nervousness
• hallucinations
• effects on the kidneys that manifest with swelling of the extremities and weight gain

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 listed in Annex V. By reporting side effects you can help provide more information on the safety of this medicine.

5. How to store PROCYSBI

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 pack and on the label of the
vial after “Exp.”. The expiry date refers to the last day of that month.
Do not use this medicine if the seal has been broken for more than 30 days. Discard the opened vial
and use a new vial.
Store in a refrigerator (2°C-8°C). Do not freeze.
After opening, do not store at a temperature above 25°C.
Keep the container tightly closed to protect the medicine from light and moisture.
Do not dispose of any medicine in wastewater. Ask your pharmacist how to dispose of medicines that
you no longer use. This will help protect the environment.

6. Contents of the pack and other information

What PROCYSBI contains

• The active ingredient is cysteamine (mercaptamine bitartrate). PROCYSBI 25 mg hard gastro-resistant capsules Each hard gastro-resistant capsule contains 25 mg of cysteamine.

PROCYSBI 75 mg hard gastro-resistant capsules
Each hard gastro-resistant capsule contains 75 mg of cysteamine.

• The other ingredients are: o In the capsules: microcrystalline cellulose, methacrylic acid-ethyl acrylate copolymer (1:1), hypromellose, talc, triethyl citrate, sodium lauryl sulfate (see paragraph “PROCYSBI contains sodium”). o In the capsule shell: gelatin, titanium dioxide (E171), indigo carmine (E132). o In the printing ink: shellac, povidone (K-17), titanium dioxide (E171).

Description of PROCYSBI and contents of the pack

• PROCYSBI 25 mg is presented as blue hard gastro-resistant capsules (size 15.9 x 5.8 mm). The light blue upper part has the logo “PRO” printed with white ink and the light blue body has “25 mg” printed with white ink. A white plastic bottle contains 60 capsules. The child-resistant closure has a foil seal. Each bottle contains two plastic cylindrical containers for additional protection from moisture and air.
• PROCYSBI 75 mg is presented as blue hard gastro-resistant capsules (size 21.7 x 7.6 mm). The dark blue upper part has the logo “PRO” printed with white ink and the light blue body has “75 mg” printed with white ink. A white plastic bottle contains 250 capsules. The child-resistant closure has a foil seal. Each bottle contains three plastic cylindrical containers for additional protection from moisture and air.
• During use of the bottle, the cylindrical containers should be left inside. The cylindrical containers can be discarded with the bottle after use.

Marketing Authorisation Holder
Chiesi Farmaceutici S.p.A.
Via Palermo 26/A
43122 Parma
Italy

Manufacturer
Chiesi Farmaceutici S.p.A.
Via San Leonardo 96
43122 Parma
Italy
For further information on this medicinal product, contact the local representative of the Marketing Authorisation Holder:

Lietuva
Chiesi Pharmaceuticals GmbH
Tel: + 43 1 4073919

България
Chiesi Bulgaria EOOD
Тел.: + 359 29201205

België/Belgique/Belgien
Chiesi sa/nv
Tél/Tel: + 32 (0)2 788 42 00

Luxembourg/Luxemburg
Chiesi sa/nv
Tél/Tel: + 32 (0)2 788 42 00

Česká republika
Chiesi CZ s.r.o.
Tel: + 420 261221745

Magyarország
Chiesi Hungary Kft.
Tel.: + 36-1-429 1060

Danmark
Chiesi Pharma AB
Tlf: + 46 8 753 35 20

Malta
Chiesi Farmaceutici S.p.A.
Tel: + 39 0521 2791

Deutschland
Chiesi GmbH
Tel: + 49 40 89724-0

Nederland
Chiesi Pharmaceuticals B.V.
Tel: + 31 88 501 64 00

Eesti
Chiesi Pharmaceuticals GmbH
Tel: + 43 1 4073919

Norge
Chiesi Pharma AB
Tlf: + 46 8 753 35 20

Ελλάδα
Chiesi Hellas AEBE
Τηλ: + 30 210 6179763

Österreich
Chiesi Pharmaceuticals GmbH
Tel: + 43 1 4073919

España
Chiesi España, S.A.U.
Tel: + 34 93 494 8000

Polska
Chiesi Poland Sp. z.o.o.
Tel.: + 48 22 620 1421

France
Chiesi S.A.S.
Tél: + 33 1 47688899

Portugal
Chiesi Farmaceutici S.p.A.
Tel: + 39 0521 2791

Hrvatska
Chiesi Pharmaceuticals GmbH
Tel: + 43 1 4073919

România
Chiesi Romania S.R.L.
Tel: + 40 212023642

Ireland
Chiesi Farmaceutici S.p.A.
Tel: + 39 0521 2791

Slovenija
Chiesi Slovenija d.o.o.
Tel: + 386-1-43 00 901

Ísland
Chiesi Pharma AB
Sími: +46 8 753 35 20

Slovenská republika
Chiesi Slovakia s.r.o.
Tel: + 421 259300060

Italia
Chiesi Italia S.p.A.
Tel: + 39 0521 2791

Suomi/Finland
Chiesi Pharma AB
Puh/Tel: +46 8 753 35 20

Κύπρος
Chiesi Farmaceutici S.p.A.
Τηλ: + 39 0521 2791

Sverige
Chiesi Pharma AB
Tel: +46 8 753 35 20

Latvija
Chiesi Pharmaceuticals GmbH
Tel: + 43 1 4073919

United Kingdom (Northern Ireland)
Chiesi Farmaceutici S.p.A.
Tel: + 39 0521 2791
More detailed information on this medicinal product is available on the website of the European Medicines Agency, http://www.ema.europa.eu .

PROCYSBI 75 mg gastro-resistant granules, 300 mg gastro-resistant granules

cysteamine (mercaptamine bitartrate)

Read this leaflet carefully before you start to use 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 PROCYSBI is and what it is used for
• 2. What you need to know before you take PROCYSBI
• 3. How to take PROCYSBI
• 4. Possible side effects
• 5. How to store PROCYSBI
• 6. Contents of the pack and other information

1. What is PROCYSBI and what is it used for

PROCYSBI contains the active ingredient cysteamine (also known as mercaptamine) and is taken for
the treatment of nephropathic cystinosis in children and adults. Cystinosis is a disease that affects
body functions, with an abnormal accumulation of the amino acid cystine in various organs of the body, such as
the kidneys, eyes, muscles, pancreas and brain. The accumulation of cystine damages the kidneys and causes
the excretion of excessive amounts of glucose, proteins and electrolytes. Different organs are affected at
different ages.
PROCYSBI is a medicine that reacts with cystine to decrease its level inside the
cells. To obtain the maximum benefit, therapy with cysteamine should be started immediately after
confirmation of the diagnosis of nephropathic cystinosis.

2. What you need to know before taking PROCYSBI

Do not take PROCYSBI

• if you are allergic to cysteamine (also known as mercaptamine) or to any of the other ingredients of this medicine (listed in section 6).
• if you are allergic to penicillamine (this is not “penicillin”, but a medicine used to treat Wilson's disease).
• if you are breastfeeding.

Warnings and precautions
Talk to your doctor or pharmacist before taking PROCYSBI.

• Because oral cysteamine does not prevent the deposition of cystine crystals in the eyes, you must continue to use the cysteamine eye solution prescribed by your doctor.
• Severe skin lesions may occur in patients treated with high doses of cysteamine. Your doctor will routinely monitor your skin and bones and will reduce or discontinue treatment if necessary (see section 4).
• Ulcers and bleeding in the stomach and intestines may occur in patients taking cysteamine (see section 4).
• Other gastrointestinal symptoms, including nausea, vomiting, anorexia and stomach pain, may occur with cysteamine. If these symptoms occur, your doctor may suspend and modify the dosage.
• Contact your doctor if you experience unusual stomach symptoms or changes in stomach symptoms.
• Symptoms such as dizziness, fatigue, drowsiness, depression and brain diseases (encephalopathy) may occur with cysteamine. If these symptoms occur, contact your doctor who will adjust the dosage.
• Abnormalities in liver function or a decrease in white blood cell count (leukopenia) may occur with the use of cysteamine. Your doctor will routinely monitor your white blood cell count and your liver function.
• Your doctor will check you for possible benign intracranial hypertension (or pseudotumor cerebri [PTC]) and/or swelling of the optic nerve (papilledema) associated with cysteamine treatment. You will undergo regular eye examinations to identify this condition, as prompt treatment can prevent vision loss.

Other medicines and PROCYSBI
Tell your doctor or pharmacist if you are taking, have recently taken or might take
any other medicines. If your doctor prescribes bicarbonate, do not take it at the same time
as PROCYSBI; take the bicarbonate at least one hour before or at least one hour after the medicine.

PROCYSBI with food and drink
For at least 1 hour before and 1 hour after taking PROCYSBI, try to avoid meals rich in
fats or proteins and any food or liquid that may reduce stomach acidity, such as milk or
yogurt. If this is impossible, you can eat a small amount (about 100 grams) of food
(preferably carbohydrates, e.g. bread, pasta, fruit) during the hour before and after
taking PROCYSBI.
See also section 3 “How to take PROCYSBI – Method of administration”.

Pregnancy and breastfeeding
If you are pregnant, suspect you may be pregnant, are planning a pregnancy or are breastfeeding
with breast milk, ask your doctor or pharmacist for advice before taking this medicine.
This medicine should not be used during pregnancy, especially during the first trimester.
Before starting treatment, you must perform a pregnancy test and this must be negative, while during treatment you must use adequate contraception. If you are
planning a pregnancy or if a pregnancy has occurred, contact your doctor immediately to
request discontinuation of therapy with this medicine as continued treatment
may be harmful to the unborn child.
Do not use this medicine if you are breastfeeding (see section 2 under “Do not take
PROCYSBI”).

Driving and using machines
This medicine may cause some drowsiness. When you start therapy, avoid driving, using machines, or engaging in other dangerous activities until you know the effects
of the medicine on your abilities.

PROCYSBI contains sodium
This medicine contains less than 1 mmol (23 mg) of sodium per dose, i.e. it is essentially ‘sodium-free’.

3. How to take PROCYSBI

Take this medicine following exactly the instructions of your doctor or pharmacist. If you
have any doubts, ask your doctor or pharmacist.
The recommended dose prescribed for you or for your child will depend on your age and weight or
on those of your child. The planned maintenance dose is 1.3 g/m²/day.

Dosage schedule
Take this medicine twice a day, every 12 hours. To get the maximum benefit from
this medicine, try to avoid regularly meals and dairy products for at least 1 hour
before and 1 hour after taking PROCYSBI. If this is impossible, you can eat a small
amount (about 100 grams) of food (preferably carbohydrates, e.g. bread, pasta, fruit) during the hour
before and after taking PROCYSBI.
It is important to take PROCYSBI regularly over time.
Do not increase or decrease the amount of medicine without your doctor's approval.
The usual total dose should not exceed 1.95 g/m²/day.

Duration of treatment
Treatment with PROCYSBI lasts for life, as established by your doctor.

Method of administration
This medicine can only be taken by mouth.
Each sachet must be used only once.
For this medicine to work correctly, follow these instructions:
Open the sachet and disperse all the granules in food (such as applesauce or fruit jam), to take it by ingestion or administer it through feeding tubes, or
mix it within an acidic beverage (such as orange juice or any acidic fruit juice)
or water and drink the mixture. Do not crush or chew the granules.
Taking the drug dispersed in food
Open the sachet and disperse all the granules in about 100 grams of food, such as applesauce or
fruit jam. Gently stir the granules into the soft food, creating a
mixture of granules and food. Swallow the entire mixture, then drink about 250 mL of an acidic beverage
(such as orange juice or any acidic fruit juice) or water to make it easier to
swallow. If you do not consume the mixture immediately, it can be stored in the refrigerator (2 °C-8 °C)
from the time of preparation to the time of administration and consumed within 2 hours of
preparation. Any residue of the mixture must not be stored for more than 2 hours.
Administration through a feeding tube
Open the sachet and disperse the granules in about 100 grams of applesauce or fruit jam.
Gently stir the granules into the soft food, creating a mixture of granules and
food. Administer the mixture through a gastrostomy tube, a nasogastric tube or
a gastrojejunal tube using a syringe with a catheter tip. Before
administering PROCYSBI: open the gastrostomy button and connect the feeding tube. Flush with 5 mL of water to clean the button. Aspirate the mixture into the syringe. It is
recommended to use a maximum of 60 mL of mixture in a syringe with a catheter tip with
straight feeding tube or bolus administration. Insert the opening of the syringe
containing the PROCYSBI and food mixture into the opening of the feeding tube and fill
completely with the mixture. To prevent possible obstruction problems, apply
light pressure on the syringe and keep the feeding tube in a horizontal position
during administration. To avoid the risk of obstruction, it is also recommended to use
a viscous food, such as applesauce or fruit jam, administering it at a
rate of about 10 mL every 10 seconds until the syringe is completely emptied. Repeat the
above step until all the mixture has been administered. After
administering PROCYSBI, aspirate 10 mL of fruit juice or water into another syringe and
flush the gastrostomy tube, making sure that no residue of the PROCYSBI and food mixture remains
blocked in the gastrostomy tube.
If you do not consume the mixture immediately, it can be stored in the refrigerator (2 °C-8 °C)
from the time of preparation to the time of administration and consumed within 2 hours of
preparation. Any residue of the mixture must not be stored for more than 2 hours.
Consult your pediatrician for precise instructions on the correct methods of administering
the medicine through a feeding tube and in case of feeding tube obstruction problems.
Dispersion in orange juice or any acidic fruit juice or water
Open the sachet and disperse the granules in about 100-150 mL of acidic fruit juice (such as orange
juice or any acidic fruit juice) or water. Gently mix the PROCYSBI mixture for 5 minutes, stirring it in a cup or shaking it in a cup with a lid
(e.g., a sippy cup), then drink the mixture.
If you do not drink the mixture immediately, it can be stored in the refrigerator (2 °C-8 °C) from
the time of preparation to the time of administration and drunk within 30 minutes of
preparation. Any residue of the mixture must not be stored for more than 30 minutes.
Administering a mixture to drink using an oral syringe
Aspirate the drinkable mixture into a dosing syringe and administer it directly into the mouth.
If you do not consume the mixture immediately, it can be stored in the refrigerator (2 °C-8 °C)
from the time of preparation to the time of administration and consumed within 30 minutes
from preparation. Any residue of the mixture must not be stored for more than 30 minutes.
Your doctor may recommend or prescribe including, in addition to cysteamine, one or more supplements to
replace important electrolytes that are lost through the kidneys. It is important to take these
supplements following the instructions exactly. Consult your doctor if you have missed taking several
doses of these supplements or in case of weakness or drowsiness.
Regular blood tests to measure the amount of cystine inside white blood cells and/or the
concentration of cysteamine in the blood are necessary to determine the correct dose of PROCYSBI.
You or your doctor will arrange for the blood tests to be performed. These tests must
be performed 12.5 hours after the evening dose the previous day, and therefore 30 minutes after
taking the morning dose the next day. Regular blood and urine tests are also needed to measure the levels of important electrolytes for the body to help you or your doctor to
adjust the doses of these supplements correctly.

If you take more PROCYSBI than you should
If you have taken more PROCYSBI than you should, contact your doctor or the emergency room immediately.
You may feel drowsy.

If you forget to take PROCYSBI
If you forget to take a dose of medicine, take it as soon as possible. However, if
less than 4 hours remain until the next dose, skip the missed dose and resume your regular dosing schedule.
Do not take a double dose to make up for a missed dose.
If you have any questions about using 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.

Seek immediate medical or nursing attention if you notice any of the following
side effects - you may need urgent medical treatment:

• Severe allergic reaction (uncommon): seek emergency medical assistance if you experience any of these signs of an allergic reaction: hives; difficulty breathing; swelling of the face, lips, tongue, or throat.

If any of the following side effects occur, contact your doctor immediately. Because some of
them are serious, consult your doctor for a more detailed explanation of their warning signs.

Common side effects(may affect up to 1 in 10 people):

• Skin rash: tell your doctor immediately if you have a skin rash. It may be necessary to temporarily stop PROCYSBI until the rash clears up. If the rash is severe, your doctor may stop treatment with cysteamine.
• Abnormal liver function tests in the blood. Your doctor will monitor you for this.

Uncommon side effects(may affect up to 1 in 100 people):

• Skin lesions, bone lesions and joint problems: treatment with high doses of cysteamine can lead to the development of skin lesions. These include striae of the skin (which look like whip marks), bone lesions (such as fractures), bone deformities and joint problems. Check your skin while taking this medicine. Tell your doctor about any changes. Your doctor will monitor you for this.
• Decreased white blood cell count. Your doctor will monitor you for this.
• Central nervous system symptoms: some patients taking cysteamine have developed dizziness, depression and become too apathetic (excessive drowsiness). If you experience these symptoms, tell your doctor.
• Problems with the stomach and intestines (gastrointestinal): patients taking cysteamine have developed ulcers and bleeding. Tell your doctor immediately if you have stomach pain, or if you vomit blood.
• Benign intracranial hypertension, also called pseudotumor cerebri, has been reported with the use of cysteamine. This is a condition in which there is an increase in pressure of the fluids around the brain. Tell your doctor immediately if you develop any of the following symptoms while taking PROCYSBI: ringing or “whooshing” sounds in the ear, dizziness, double vision, blurred vision, vision loss, pain behind the eye, or pain associated with eye movement. Your doctor will perform eye exams to detect and treat this problem promptly. This will help reduce the possibility of vision loss.

The other side effects listed below are provided with an estimate of the frequency with
which they may occur with PROCYSBI.

Very common side effects(may affect more than 1 in 10 people):

• nausea
• vomiting
• loss of appetite
• diarrhea
• fever
• feeling sleepy

Common side effects

• headache
• encephalopathy
• abdominal pain
• dyspepsia
• bad breath and body odor
• heartburn
• fatigue

Uncommon side effects

• leg pain
• scoliosis (curvature of the spine)
• brittle bones
• hair discoloration
• seizures
• nervousness
• hallucinations
• effects on the kidneys that manifest with swelling of the extremities and weight gain

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 detailed in Annex V. By reporting side effects you can help provide
more information on the safety of this medicine.

5. How to store PROCYSBI

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 pack and on the sachet after
“Exp.”. The expiry date refers to the last day of that month.
Store in a refrigerator (2°C-8°C). Do not freeze.
Keep the sachets in the outer packaging to protect the medicine from light and moisture.
Closed sachets can be stored for a single period of time with a maximum duration of
4 months outside the refrigerator at a temperature below 25°C. Afterwards, the medicine must be
discarded.
Each sachet is for single use only.
Do not dispose of any medicine in wastewater. Ask your pharmacist how to dispose of medicines that
you no longer use. This will help protect the environment.

6. Package contents and other information

What PROCYSBI contains

• The active ingredient is cysteamine (mercaptamine bitartrate). PROCYSBI 75 mg gastro-resistant granules Each sachet of gastro-resistant granules contains 75 mg of cysteamine.

PROCYSBI 300 mg gastro-resistant granules
Each sachet of gastro-resistant granules contains 300 mg of cysteamine.

• The other ingredients are: microcrystalline cellulose, methacrylic acid-ethyl acrylate copolymer (1:1), hypromellose, talc, triethyl citrate, sodium lauryl sulfate (see paragraph “PROCYSBI contains sodium”).

Description of the appearance of PROCYSBI and contents of the pack

• PROCYSBI 75 mg is presented as a gastro-resistant granule of colour from white to off-white in sachets. Each pack contains 120 sachets.
• PROCYSBI 300 mg is presented as a gastro-resistant granule of colour from white to off-white in sachets. Each pack contains 120 sachets.

Marketing Authorisation Holder
Chiesi Farmaceutici S.p.A.
Via Palermo 26/A
43122 Parma
Italy

Manufacturer
Chiesi Farmaceutici S.p.A.
Via San Leonardo 96
43122 Parma
Italy
For further information on this medicinal product, contact the local representative of the marketing
authorisation holder:

Lietuva
Chiesi Pharmaceuticals GmbH
Tel: + 43 1 4073919

България
Chiesi Bulgaria EOOD
Тел.: + 359 29201205

België/Belgique/Belgien
Chiesi sa/nv
Tél/Tel: + 32 (0)2 788 42 00

Luxembourg/Luxemburg
Chiesi sa/nv
Tél/Tel: + 32 (0)2 788 42 00

Česká republika
Chiesi CZ s.r.o.
Tel: + 420 261221745

Magyarország
Chiesi Hungary Kft.
Tel.: + 36-1-429 1060

Danmark
Chiesi Pharma AB
Tlf: + 46 8 753 35 20

Malta
Chiesi Farmaceutici S.p.A.
Tel: + 39 0521 2791

Deutschland
Chiesi GmbH
Tel: + 49 40 89724-0

Nederland
Chiesi Pharmaceuticals B.V.
Tel: + 31 88 501 64 00

Eesti
Chiesi Pharmaceuticals GmbH
Tel: + 43 1 4073919

Norge
Chiesi Pharma AB
Tlf: + 46 8 753 35 20

Ελλάδα
Chiesi Hellas AEBE
Τηλ: + 30 210 6179763

Österreich
Chiesi Pharmaceuticals GmbH
Tel: + 43 1 4073919

España
Chiesi España, S.A.U.
Tel: + 34 93 494 8000

Polska
Chiesi Poland Sp. z.o.o.
Tel.: + 48 22 620 1421

France
Chiesi S.A.S.
Tél: + 33 1 47688899

Portugal
Chiesi Farmaceutici S.p.A.
Tel: + 39 0521 2791

Hrvatska
Chiesi Pharmaceuticals GmbH
Tel: + 43 1 4073919

România
Chiesi Romania S.R.L.
Tel: + 40 212023642

Ireland
Chiesi Farmaceutici S.p.A.
Tel: + 39 0521 2791

Slovenija
Chiesi Slovenija d.o.o.
Tel: + 386-1-43 00 901

Ísland
Chiesi Pharma AB
Sími: +46 8 753 35 20

Slovenská republika
Chiesi Slovakia s.r.o.
Tel: + 421 259300060

Italia
Chiesi Italia S.p.A.
Tel: + 39 0521 2791

Suomi/Finland
Chiesi Pharma AB
Puh/Tel: +46 8 753 35 20

Κύπρος
Chiesi Farmaceutici S.p.A.
Τηλ: + 39 0521 2791

Sverige
Chiesi Pharma AB
Tel: +46 8 753 35 20

Latvija
Chiesi Pharmaceuticals GmbH
Tel: + 43 1 4073919

United Kingdom (Northern Ireland)
Chiesi Farmaceutici S.p.A.
Tel: + 39 0521 2791
More detailed information on this medicinal product is available on the website of the European
Medicines Agency, http://www.ema.europa.eu .