CYSTAGON 50 mg Hard Capsule

Зміст інструкції

1. Cystagon 50 mg Hard Capsules, 150 mg Hard Capsules
   1. What is CYSTAGON and what is it used for
   2. What you need to know before using CYSTAGON
   3. How to use CYSTAGON
   4. Possible side effects
   5. How to store CYSTAGON
   6. Package contents and other information

Cystagon 50 mg Hard Capsules, 150 mg Hard Capsules

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

1. What is CYSTAGON and what is it used for

Cystinosis is a metabolic disease called “nephropathic cystinosis”, characterized by 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.
CYSTAGON is prescribed for the treatment of this rare hereditary disorder. CYSTAGON is a
medicine that reacts with cystine to reduce its level inside the cells.

2. What you need to know before using CYSTAGON

Do not use CYSTAGON

• if you - or the child - are allergic to cysteamine bitartrate or penicillamine or to any of the other ingredients of this medicine (listed in section 6).
• if you are pregnant, especially during the first trimester.
• if you are breastfeeding.

Warnings and precautions

• If the disease has been confirmed in you or the child, by measuring cystine levels in white blood cells, therapy with CYSTAGON should be started as soon as possible.
• Some cases of skin lesions on the elbow, in the form of small hard masses, have been reported in children treated with high doses of various cysteamine-based preparations. These lesions were associated with skin striae and bone lesions, such as fractures or bone deformities, and joint laxity. The doctor may require periodic medical visits and X-rays for the skin and bones, in order to monitor the effects of the medicine. Self-examination of the skin is also recommended, for both you and the child. If skin or bone abnormalities appear, inform your doctor immediately.
• The doctor may require periodic monitoring of blood cell counts.
• It has not been demonstrated that CYSTAGON prevents the accumulation of cystine crystals in the eye. If a cysteamine ophthalmic solution has been used for this purpose, continue its use.
• Unlike phosphocysteamine, another active ingredient similar to cysteamine bitartrate, CYSTAGON does not contain phosphate. You may already be taking phosphate supplements and their dose may require changes when CYSTAGON replaces phosphocysteamine.
• To avoid the risk of aspiration into the lungs, capsules should not be given to children under approximately 6 years of age.
• Do not ingest the desiccant container placed inside the bottle.

Other medicines and CYSTAGON
Tell your doctor or pharmacist if you are taking, have recently taken or might take any other
medicine.

CYSTAGON with food and drink
For children under approximately six years of age, the hard capsule can be opened and its contents
poured onto food (e.g. milk, potatoes or starch-based foods) or mixed into formula. Do not
add it to acidic drinks such as orange juice. Consult your doctor for precise instructions.

Pregnancy
Do not use CYSTAGON if you are pregnant. Consult your doctor if you are planning to become
pregnant.

Breastfeeding
CYSTAGON should not be used during breastfeeding.

Driving and operating machinery
CYSTAGON may cause some drowsiness. At the beginning of therapy, it is advisable that you or your
child avoid engaging in potentially dangerous activities until the effects of the medicine are
fully known.

3. How to use CYSTAGON

Use this medicine following the instructions of your doctor or pediatrician exactly. If you have
any doubts, consult your doctor.
The dose of CYSTAGON prescribed for you or your child will depend on their age and weight or
on those of your child.
For children up to 12 years of age, the dose will be based on body surface area; the usual dose is
1.30 g/m of body surface area per day.
For patients over 12 years of age and weighing more than 50 kg, the usual dose is 2 g/day.
In any case, the usual dose should not exceed 1.95 g/m /day.
CYSTAGON must be taken or administered only orally and exactly as directed
by your doctor or pediatrician. For CYSTAGON to work correctly, follow these instructions:

• Strictly adhere to the doctor's instructions. Do not increase or decrease the amount of medicine without the doctor's approval.
• Hard capsules should not be given to children under approximately six years of age as they may not be able to swallow them and therefore choke. For children under approximately six years of age, the hard capsule can be opened and its contents poured onto food (e.g. milk, potatoes or starch-based foods) or mixed into formula. Do not add it to acidic beverages such as orange juice. Consult your doctor for specific instructions.
• Your or your child's treatment may include, in addition to CYSTAGON, one or more supplements to replace important electrolytes that are lost through the kidneys. It is important to administer or take these supplements exactly as directed. 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 leukocytes help determine the correct dosage of CYSTAGON. Your doctor or pediatrician will arrange the necessary

blood tests. Also, regular blood and urine tests to measure the levels of
electrolytes important for the body help the doctor to correctly establish the doses of
the supplements.
CYSTAGON must be taken 4 times a day, every 6 hours, preferably immediately after meals
or during meals. It is important to take the dose respecting the 6-hour intervals as much as possible.
Treatment with CYSTAGON is of indefinite duration, as established by the doctor.
To easily open and close the Cystagon bottle, follow the instructions below.
Instructions for opening and closing the container

OPEN	r a CLOSE F l e d
To open Place a pen or similar object between the raised sections of the protective cap and rotate in the direction shown (counterclockwise)	a To close Place a pen or similar object between the raised sections of the protective cap and rotate in the direction shown (clockwise)

If you use more CYSTAGON than you should
Contact your doctor or pediatrician or the hospital emergency room immediately if the medicine
has been taken in doses higher than those prescribed, in case of drowsiness.

If you forget to take CYSTAGON
If you forget to take a dose of medicine, take it as soon as possible. However, if
less than two hours remain until the next dose, skip the missed dose and resume the normal dosing schedule. Do not take a double dose to make up for a missed dose.

4. Possible side effects

Like all medicines, this medicine can cause side effects, although not everybody gets them.
CYSTAGON may cause some people to feel sleepy or have a lower level of alertness than
normal. Before driving or operating machinery, or doing anything that could be dangerous if you are
not fully alert, make sure you know how you or your child react to the medicine.
The following side effects have been reported with the following frequencies: very common (may affect
more than 1 in 10 people), common (may affect up to 1 in 10 people), uncommon (may affect up to 1
in 1000 people), rare (may affect up to 1 in 10,000 people), very rare (may affect up to 1 in 100,000

• people).
• Very common: vomiting, nausea, diarrhoea, loss of appetite, fever and feeling sleepy.
• Common: abdominal pain or discomfort, bad breath and altered body odour, rash, gastroenteritis, fatigue, headache, encephalopathy (brain disorder) and altered liver function tests.
• Uncommon: stretch marks, skin lesions (small hard lumps on the elbows), joint laxity, leg pain, bone fractures, scoliosis (curvature of the spine), bone deformities and fragility, hair discoloration, severe allergic reactions, drowsiness, seizures, nervousness, hallucinations, decreased white blood cells, gastrointestinal ulcer manifesting with bleeding from the digestive tract and effects on the kidneys manifesting with swelling of the extremities and weight gain. Some of the above side effects are serious. Therefore, we invite you to consult your doctor or paediatrician for a more detailed explanation of the related symptoms.

Reporting of side effects
If you get any side effects, talk to your doctor or pharmacist. This includes any possible side effects not
listed in this leaflet. You can also report side effects directly through the national reporting system listed
in Appendix V. By reporting side effects you can help provide more information on the safety of this

5. How to store CYSTAGON

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 label after Exp. The expiry
date refers to the last day of that month.
Do not store at a temperature above 25°C and keep the container tightly closed and protected from light
and humidity.
Do not dispose of any medicine in waste water or household waste. Ask your pharmacist how to
dispose of medicines you no longer use. This will help protect the environment.

6. Package contents and other information

What CYSTAGON contains

• The active ingredient is cysteamine bitartrate (mercaptamine bitartrate). Each hard capsule of CYSTAGON 50 mg contains 50 mg of cysteamine (as mercaptamine bitartrate). Each hard capsule of CYSTAGON 150 mg contains 150 mg of cysteamine (as mercaptamine bitartrate).
• The excipients are: microcrystalline cellulose, pregelatinized starch, magnesium stearate/sodium lauryl sulfate, colloidal silicon dioxide, cross-linked sodium carboxymethyl cellulose, capsule shell: gelatin, titanium dioxide, black ink on the hard capsules (E 172).

Description of the appearance of CYSTAGON and contents of the pack
Hard capsules

• Cystagon 50 mg: opaque, white-colored hard capsules, with CYSTA 50 imprinted on the body of the capsule and RECORDATI RARE DISEASES on the cap. Bottles of 100 or 500 hard capsules. Not all pack sizes may be marketed.
• Cystagon 150 mg: opaque, white-colored hard capsules, with CYSTAGON 150 imprinted on the body of the capsule and RECORDATI RARE DISEASES on the cap. Bottles of 100 or 500 hard capsules. Not all pack sizes may be marketed.

Marketing Authorisation Holder
Recordati Rare Diseases
Tour Hekla
52, Avenue du Général de Gaulle
F-92800 Puteaux
France

Manufacturer
Recordati Rare Diseases
Tour Hekla
52, Avenue du Général de Gaulle
F-92800 Puteaux
France
or
Recordati Rare Diseases
Eco River Parc
30, rue des Peupliers
F-92000 Nanterre
France
For further information on CYSTAGON, contact the local representative of the
Marketing Authorisation Holder:

Belgique/België/Belgien
Recordati

Lietuva
Recordati AB.
Tel: + 46 8 545 80 230
Sweden

България
Recordati Rare Diseases
Тел.: +33 (0)1 47 73 64 58
France

Luxembourg/Luxemburg
Recordati
Tél/Tel: +32 2 46101 36
Belgique/Belgien

Česká republika
Recordati Rare Diseases
Tel: +33 (0)1 47 73 64 58
France

Magyarország
Recordati Rare Diseases
Tel: +33 (0)1 47 73 64 58
France

Danmark
Recordati AB.
Tlf. : +46 8 545 80 230
Sweden

Malta
Recordati Rare Diseases
Tel: +33 1 47 73 64 58
France

Deutschland
Recordati Rare Diseases Germany GmbH
Tel: +49 731 140 554 0

Nederland
Recordati
Tel: +32 2 46101 36
België

Eesti
Recordati AB.
Tel: + 46 8 545 80 230
Sweden

Norge
Recordati AB.
Tlf : +46 8 545 80 230
Sweden

ΕλλάδαÖsterreich
Recordati Rare Diseases
Τηλ: +33 1 47 73 64 58
Greece
Recordati Rare Diseases Germany GmbH
Tel: +49 731 140 554 0
Germany

España
Recordati Rare Diseases Spain S.L.U.
Tel: + 34 91 659 28 90

Polska
Recordati Rare Diseases
Tel: +33 (0)1 47 73 64 58
France

France
Recordati Rare Diseases
Tél: +33 (0)1 47 73 64 58

Portugal
Recordati Rare Diseases SARL
Tel: +351 21 432 95 00

Hrvatska
Recordati Rare Diseases
Tél: +33 (0)1 47 73 64 58
France

România
Recordati Rare Diseases
Tel: +33 (0)1 47 73 64 58
France

Ireland
Recordati Rare Diseases
Tel: +33 (0)1 47 73 64 58
France

Slovenija
Recordati Rare Diseases
Tel: +33 (0)1 47 73 64 58
France

Ísland
Recordati AB.
Simi:+46 8 545 80 230
Sweden

Slovenská republika
Recordati Rare Diseases
Tel: +33 (0)1 47 73 64 58
France

Italia
Recordati Rare Diseases Italy Srl
Tel: +39 02 487 87 173

Suomi/Finland
Recordati AB.
Puh/Tel : +46 8 545 80 230
Sweden

Κύπρος
Recordati Rare Diseases
Τηλ : +33 1 47 73 64 58
France

Sverige
Recordati AB.
Tel : +46 8 545 80 230

Latvija
Recordati AB.
Tel: + 46 8 545 80 230
Sweden

This leaflet was last updated
More detailed information on this medicine is available on the European Medicines Agency website, https://www.ema.europa.eu
Also, links to other websites on rare diseases and related therapeutic treatments are provided.