Prospect: Information for the User

CYSTAGON 50 mg Hard Capsules

CYSTAGON 150 mg Hard Capsules

bitartrate of cysteamine (bitartrate of mercaptamine)

Read this prospect carefully before starting to use the medicine, because it contains important information for you.

1. How to use CYSTAGON
2. Possible adverse effects
3. Storage of CYSTAGON
4. Contents of the package and additional information

Cystinosis is a metabolic disease called ‘cystinotic nephropathy’ that is characterized by the 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 produces lesions in the kidney and the elimination of excessive amounts of sugar (glucose), proteins, and electrolytes. Different organs may be affected at different ages.

CYSTAGON is prescribed to treat this rare hereditary disorder. CYSTAGON is a medication that reacts with cystine, reducing its levels in cells.

Do not take CYSTAGON:

• if you or your child are allergic to cysteamine bitartrate or penicillamine or any of the other components of this medication (listed in section 6).
• if you are pregnant, this is especially important during the first trimester.
• if you are breastfeeding.

Warnings and precautions:

• When your disease or your child's disease has been confirmed through cystine leukocyte measurements, CYSTAGON treatment should be started as soon as possible.
• Some cases of skin lesions in the form of small hard lumps, mainly on the elbows, have been reported in children treated with high doses of different cysteamine preparations. These lesions were associated with skin striations and bone lesions, such as fractures and bone deformities, and with loose joints.

Your doctor may ask you to have periodic physical skin examinations and X-ray examinations of the bones to monitor the effects of the medication. It is recommended that the patient or parents perform self-examination of the skin. If any skin or bone anomaly appears, you should inform your doctor immediately.

• Your doctor may ask you to have periodic blood tests to monitor the blood cell count.

• CYSTAGON does not prevent the accumulation of cystine crystals in the eyes. If you have used cysteamine eye drops for this purpose, you should continue using them...

• Unlike cysteamine phosphate, another active principle similar to cysteamine bitartrate, CYSTAGON does not contain phosphate. You may be taking phosphate supplements and it is possible that your doctor will modify your dose when you switch from CYSTAGON to cysteamine phosphate.

• Do not administer the capsules to children under approximately 6 years old to avoid the risk of aspiration in the lungs.
• Do not ingest the desiccant container in the bottle.

Other medications and CYSTAGON

Inform your doctor or pharmacist if you are using, have used recently, or may need to use any other medication.

CYSTAGON with food and drinks:

For children under six years old, the hard capsule can be opened and its contents can be spread on food (such as milk, potatoes, or starchy foods like flours), or mixed with the bottle. Do not mix with acidic drinks, such as orange juice. For complete instructions, consult your doctor.

Pregnancy

Do not use CYSTAGON during pregnancy. You should consult your doctor if you plan to become pregnant.

Lactation

CYSTAGON should not be used during breastfeeding.

Driving and operating machines:

CYSTAGON may cause drowsiness. When starting treatment, your child or you should avoid performing potentially hazardous activities until you are familiar with the effects of the medication.

Follow exactly the administration instructions of this medication as indicated by your doctor or your child's doctor. In case of doubts, consult your doctor again.

The CYSTAGON dose prescribed for you or your child will depend on your weight and age.

For children up to 12 years old, the dose will be based on body size (surface area), with the usual dose being 1.30 g/m2of body surface area per day.

For patients over 12 years old and over 50 kg in weight, the usual dose is 2 g/day.

In any case, the usual dose should not exceed 1.95 g/m2/day.

CYSTAGON must be taken orally and exactly as indicated by your doctor or your child's doctor. To ensure that CYSTAGON works correctly, it is necessary:

• Follow exactly the doctor's instructions. Do not increase or decrease the amount of medication without the doctor's approval.

• Do not administer the hard capsules to children under six years of age because they may not swallow them and choke. For children under six years old, the hard capsule can be opened and its contents can be spread on food (such as milk, potatoes, or starchy foods like flour), or mixed with the bottle. Do not mix with acidic beverages, such as orange juice. For the recommended guidelines, consult your doctor.

• The medical treatment prescribed for you or your child will include, in addition to CYSTAGON, one or more supplements to replace the important electrolytes eliminated by the kidneys. It is essential to take or give those supplements exactly as instructed. If several doses of one of the supplements are forgotten, or if weakness or drowsiness is noted, the doctor should be consulted immediately.
• To determine the correct dose of CYSTAGON, it is necessary to perform periodic blood tests to measure the amount of cystine within the leukocytes. Your doctor or your child's doctor will determine when the blood tests should be performed. It is also necessary to perform periodic blood and urine tests to measure the concentrations of the important electrolytes in the body, so that your doctor can adjust the doses of the electrolyte supplements needed.

CYSTAGON should be taken 4 times a day, every 6 hours, preferably with meals or immediately after meals. It is essential to respect the rule of taking the dose every 6 hours as strictly as possible.

The CYSTAGON treatment should be continued indefinitely as indicated by your doctor.

To open and close the Cystagon container easily, follow the instructions included below:

Instructions for opening and closing the container

If you take more CYSTAGON than you should

If more medication than prescribed has been ingested, or if drowsiness is experienced, consult your doctor or your child's doctor immediately, or go to the emergency service of a hospital.

If you forgot to take CYSTAGON

If a dose of the medication has been forgotten, take it as soon as possible. However, if there are less than 2 hours left before the next dose, the missed dose should be skipped and the normal schedule resumed. Do not take a double dose to compensate for the missed doses.

Like all medications, this medication may produce adverse effects, although not all people may experience them.

CYSTAGON may produce drowsiness or a decrease in alertness in some people compared to their normal state. Be sure to see how you or your child reacts to this medication before engaging in any activity that may be hazardous if you are not alert.

The following adverse effects have been reported in the following manner: very frequent (occurs in at least one in ten patients), frequent (occurs in at least one in one hundred patients), infrequent (occurs in at least one in one thousand patients), rare (occurs in at least one in ten thousand patients), very rare (occurs in at least one in one hundred thousand patients).

• Very frequent: vomiting, nausea, diarrhea, loss of appetite, fever, and feeling of sleepiness.

• Frequent: abdominal pain or discomfort, bad breath, and unpleasant body odor, skin rash, gastroenteritis, fatigue, headache, encephalopathy (brain disorder), and alteration of liver function tests.

• Infrequent: striae on the skin, skin lesions (small hard lumps on the elbows), loose joints, leg pain, bone fractures, scoliosis (vertebral column deviation), deformities, and bone fragility, hair depigmentation, severe allergic reaction, drowsiness, seizures, nervousness, hallucinations, decrease in white blood cell count, gastrointestinal ulcer with secondary digestive hemorrhage, and renal alterations that manifest with swelling of the extremities and weight gain.

As some of these adverse effects are serious, ask your doctor or pediatrician to explain the warning signs.

Reporting Adverse Effects

If you experience any type of adverse effect, consult your doctor or pharmacist, even if it is a possible adverse effect that does not appear in this prospectus. You can also report them directly through the national notification system included in theAppendix V. By reporting adverse effects, you can contribute to providing more information about the safety of this medication.

Keep this medication out of the sight and reach of children.

Do not use this medication after the expiration date that appears on the label after CAD. The expiration date is the last day of the month indicated.

Do not store above 25°C.

Keep the container perfectly closed to protect it from light and moisture.

Medicines should not be disposed of through drains or in the trash. Ask your pharmacist how to dispose of the containers and medications that you no longer need. This will help protect the environment.

Composition of CYSTAGON

• The active ingredient is cysteamine bitartrate (cysteamine bitartrate). Each hard capsule of CYSTAGON 50 mg contains 50 mg of cysteamine (in the form of cysteamine bitartrate). Each hard capsule of CYSTAGON 150 mg contains 150 mg of cysteamine (in the form of cysteamine bitartrate).

• The other components are microcrystalline cellulose, pregelatinized starch, magnesium stearate/laurilsulfate sodium, colloidal silicon dioxide, croscarmellose sodium. The capsule shells: gelatin, titanium dioxide, black ink in hard capsules (E172).

Appearance of the product and contents of the package

Hard capsules

• Cystagon 50 mg: White hard capsules with the legend CYSTA 50 on the body and RECORDATI RARE DISEASES on the closure capsule.

Bottles of 100 or 500 hard capsules. Some package sizes may only be marketed.

• Cystagon 150 mg: White hard capsules with the legend CYSTAGON 150 on the body and RECORDATI RARE DISEASES on the closure capsule.

Bottles of 100 or 500 hard capsules. Some package sizes may only be marketed.

Marketing Authorization Holder

Recordati Rare Diseases

Immeuble “Le Wilson”

70, Avenue du Général de Gaulle

F-92800 Puteaux

France

Responsible for manufacturing

Recordati Rare Diseases

Immeuble “Le Wilson”

70, 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 more information about this medicine, please contact the local representative of the marketing authorization holder.

Last review date of this leaflet:07/2024

For detailed information about this medicine, please visit the website of the European Medicines Agency:http://www.ema.europa.eu/