Label: information for the user

PROCYSBI 25 mg hard-gastro-resistant capsules

PROCYSBI 75 mg hard-gastro-resistant capsules

cysteamine bitartrate

Read this label carefully before starting to take this medicine, as it contains important information for you.

• Keep this label, as you may need to read it again.
• If you have any doubts, consult your doctor or pharmacist.
• This medicine has been prescribed only for you, and you should not give it to other people

even if they have the same symptoms as you, as it may harm them.

• If you experience any adverse effects, consult your doctor or pharmacist, even if they are not listed in this label.

See section 4.

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 bodily function, with an abnormal accumulation of the amino acid cystine in various organs such as the kidneys, eyes, muscles, pancreas, and brain. The accumulation of cystine causes damage to the kidney and excessive elimination of glucose, proteins, and electrolytes. Different organs can be affected at different ages.

PROCYSBI is a medication that reacts with cystine and decreases its levels in cells. To achieve maximum benefit, cysteamine therapy should be initiated promptly after confirmation of the diagnosis of cystinosis.

Do not take PROCYSBI

• If you are allergic to cysteamine (also known as mercaptamine) or any of the other components of this medication (listed in section6).
• If you are allergic to penicillamine(not to be confused with “penicillin”, but a medication used to treat Wilson's disease).
• If you are breastfeeding.

Warnings and precautions

Consult your doctor or pharmacist before starting to take PROCYSBI.

• Since oral cysteamine does not prevent the accumulation of cystine crystals in the eyes, you should continue using the cysteamine eye drops prescribed by your doctor.
• Do not administer whole cysteamine capsules to children under 6years due to the risk of choking (see section3 “How to take PROCYSBI – Method of administration”).

• Severe skin lesions may occur in patients treated with high doses of cysteamine. Your doctor will perform regular skin and bone examinations and reduce or discontinue treatment as necessary (see section4).

• Ulcers and gastrointestinal bleeding may occur in patients treated with cysteamine (see section4).
• During cysteamine treatment, other gastrointestinal symptoms may occur, particularly nausea, vomiting, anorexia, and stomach pain. In these cases, your doctor may interrupt and modify the dose.
• Consult your doctor if you experience unusual stomach symptoms or changes in stomach symptoms.
• During cysteamine treatment, symptoms such as seizures, fatigue, drowsiness, depression, and cerebral disorders (encephalopathy) may occur. If these symptoms occur, notify your doctor, who will adjust the dose.
• During cysteamine treatment, abnormalities in liver function or a decrease in the number of white blood cells (leucopenia) may occur. Your doctor will monitor your hemoglobin and liver function periodically.
• Your doctor will monitor for the development of benign intracranial hypertension (or pseudotumor cerebri [PTC]) and optic nerve inflammation (papillary edema) associated with cysteamine treatment. You will undergo periodic eye examinations to identify this disorder, as immediate treatment may prevent vision loss.

Other medications and PROCYSBI

Inform your doctor or pharmacist if you are taking, have taken recently, or may need to take any other medication. If your doctor prescribes bicarbonate, do not take it at the same time as PROCYSBI; take it at least one hour before or one hour after taking the medication.

Taking PROCYSBI with food and drinks

Avoid eating fatty or protein-rich foods, as well as any food or liquid that may reduce stomach acidity, such as milk or yogurt, for at least 1hour before and 1hour after taking PROCYSBI. If this is not possible, you can eat a small amount (approximately 100grams) of food (preferably carbohydrates, e.g., bread, pasta, fruits) during the hour before and after taking PROCYSBI. Take the capsule with an acidic beverage (such as orange juice or any acidic juice) or water. For children and patients with swallowing difficulties, see section3 “How to take PROCYSBI – Method of administration”.

Pregnancy and breastfeeding

If you are pregnant or breastfeeding, or if you think you may be pregnant or plan to become pregnant, consult your doctor or pharmacist before using this medication.

Do not use this medication if you are pregnant, especially during the first trimester. Before starting treatment, you must have a negative pregnancy test, and during treatment, you must use an appropriate contraceptive method. If you are a woman and plan to become pregnant, consult your doctor about suspending treatment with this medication, as it may be harmful to the fetus.

Do not use this medication during breastfeeding (see section2 “Do not take PROCYSBI”).

Driving and operating machinery

This medication may cause drowsiness. When starting treatment, avoid driving, operating machinery, or performing other hazardous activities until you know how it affects you.

PROCYSBI contains sodium

This medication contains less than 1mmol of sodium (23mg) per dose; it is essentially “sodium-free”.

Follow exactly the administration instructions of this medication as indicated by your doctor or pharmacist. If in doubt, consult your doctor or pharmacist again.

The recommended dose for you or your child will depend on your age and weight or that of your child. The maintenance dose is expected to be 1.3 g/m2/day.

Dosing Schedule

Take this medication twice a day, every 12 hours. To get the maximum benefit from this medication, try to avoid food and dairy products for at least an hour before and after taking PROCYSBI. If this is not possible, you can eat a small amount (approximately 100 grams) of food (preferably carbohydrates, e.g., bread, pasta, fruits) during the hour before and after administration of PROCYSBI.

It is essential to take PROCYSBI at a consistent time.

Do not increase or decrease the amount of medication without prior approval from your doctor.

The usual total dose should not exceed 1.95 g/m2/day.

Treatment Duration

The treatment with PROCYSBI should continue for the rest of your life, as indicated by your doctor.

Administration Form

It is taken only orally (mouth).

To ensure this medication works correctly:

• For patients who can swallow the entire capsule:

Swallow the entire capsule with an acidic beverage (such as orange juice or any acidic juice) or water. Do not crush or chew the capsules or the contents of the capsules. It is possible that children under 6 years of age may not be able to swallow the hard, gastro-resistant capsules and may choke on them. You can administer PROCYSBI to children under 6 years of age by opening the capsules and sprinkling their contents over food or liquids, following the instructions indicated below.

• For patients who cannot swallow the entire capsule or who use a feeding tube:

Sprinkle over food

Open the hard, gastro-resistant capsules and sprinkle the contents (granules) over approximately 100 grams of food, for example, apple sauce or fruit jam.

Remove the granules from the food gently, so that a mixture of granules and food is created. Consume the entire mixture. Then, drink approximately 250 ml of an acidic beverage (such as orange juice or any other acidic fruit juice) or water to facilitate the ingestion of the mixture.

If you do not consume the mixture immediately, you can refrigerate it (between 2°C and 8°C) from the time you prepare it until the time of administration and consume it within the 2 hours after preparation. Do not store anything from the mixture after 2 hours.

Administration through a feeding tube

Open the hard, gastro-resistant capsules and sprinkle the contents (granules) over approximately 100 grams of apple sauce or fruit jam. Remove the granules from the food gently, so that a mixture of granules and food is created. Administer the mixture through the gastrostomy tube, nasogastric tube, or gastrostomy-jejunostomy tube using a syringe with a catheter tip. Before administering PROCYSBI, open the button of the gastrostomy tube and attach the feeding tube. Rinse with 5 ml of water to wash the button. Aspirate the mixture into the syringe. It is recommended to use a maximum volume of 60 ml of the mixture in a syringe with a catheter tip for a straight feeding tube or a bolus tube. Place the opening of the syringe containing the PROCYSBI mixture and food into the feeding tube and fill it completely with the mixture: gently pressing the syringe and keeping the feeding tube in a horizontal position during administration may help avoid blockage problems. It is suggested to use a viscous food such as apple sauce or fruit jam at a rate of approximately 10 ml every 10 seconds until the syringe is completely empty, to avoid blockage. Repeat the above step until all the mixture is administered. After administering PROCYSBI, aspirate 10 ml of fruit juice or water into another syringe and rinse the gastrostomy tube, ensuring that no PROCYSBI mixture and food are left stuck in the gastrostomy tube.

If you do not consume the mixture immediately, you can refrigerate it (between 2°C and 8°C) from the time you prepare it until the time of administration and consume it within the 2 hours after preparation. Do not store anything from the mixture after 2 hours.

Consult your child's doctor for complete instructions on how to correctly administer the product through feeding tubes or if you have problems with tube blockage.

Sprinkle in orange juice or any other acidic fruit juice or water

Open the hard, gastro-resistant capsules and sprinkle the contents (granules) over approximately 100 to 150 ml of acidic fruit juice (such as orange juice or any other acidic fruit juice) or water. Mix the PROCYSBI drinkable mixture gently for 5 minutes, mixing it in a cup or shaking it in a covered cup (e.g., a "sippy" cup), and drink the mixture.

If you do not consume the mixture immediately, you can refrigerate it (between 2°C and 8°C) from the time you prepare it until the time of administration and consume it within the 30 minutes after preparation. Do not store anything from the mixture after 30 minutes.

Administration of a drinkable mixture with an oral syringe

Aspirate the drinkable mixture into an oral dosing syringe and administer it directly into the mouth.

If you do not consume the mixture immediately, you can refrigerate it (between 2°C and 8°C) from the time you prepare it until the time of administration and consume it within the 30 minutes after preparation. Do not store anything from the mixture after 30 minutes.

Your doctor may recommend or prescribe the inclusion, in addition to cysteamine, of one or more supplements to replace the essential electrolytes lost by the kidneys. It is essential to take these supplements exactly as instructed. If you forget several doses of the supplements or experience weakness or drowsiness, consult your doctor for instructions to follow.

It is essential to perform regular blood tests to measure the amount of cystine in white blood cells and/or the concentration of cysteamine in the blood to help determine the correct dose of PROCYSBI. You or your doctor will schedule these blood tests. These blood tests should be obtained 12.5 hours after the night dose of the previous day and then 30 minutes after the morning dose. It is also essential to perform periodic blood and urine tests to measure the levels of essential electrolytes to help your doctor or you adjust the doses of these supplements correctly.

If you take more PROCYSBI than you should

If you have ingested more PROCYSBI than prescribed, consult your doctor immediately or go to the emergency department of a hospital. You may experience drowsiness.

If you forgot to take PROCYSBI

If you forgot to take a dose, take it as soon as possible. If less than four hours have passed since the next dose, skip the missed dose and return to the normal schedule.

Do not take a double dose to compensate for the missed doses.

If you have any other questions about the use of this medication, ask your doctor or pharmacist.

Like all medicines, this medicine can cause side effects, although not everyone will experience them.

Consult a doctor or nurse immediately if you notice any of the following side effects, as you may need urgent medical treatment:

• Severe allergic reactions (rare): go to the emergency room if you have any of the following signs of an allergic reaction: hives, difficulty breathing, facial swelling, lip, tongue, or throat inflammation.

If any of the following side effects occur, contact your doctor immediately. Since some of these side effects are serious, ask your doctor to explain the warning signs.

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

• Skin rash: inform your doctor immediately. You may need to temporarily discontinue PROCYSBI treatment until the rash disappears. If the rash is severe, your doctor may withdraw you from cysteamine treatment.
• Abnormal liver function in blood tests. Your doctor will monitor this effect.

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

• Skin lesions, bone lesions, and joint problems: high-dose cysteamine treatment can cause skin lesions. These include skin stretch marks, bone fractures, bone deformities, and joint problems. Examine your skin while taking this medicine. Notify any changes to your doctor. Your doctor will monitor these problems.

• Low white blood cell count. Your doctor will monitor this effect.
• Central nervous system symptoms: some patients taking cysteamine have experienced seizures, depression, and excessive sleepiness. Notify your doctor if you have these symptoms.
• Gastrointestinal problems: patients taking cysteamine have developed ulcers and bleeding. Notify your doctor immediately if you experience stomach pain or vomit blood.
• Benign intracranial hypertension, also known as pseudotumor cerebri, has also been reported with cysteamine use. This is a condition characterized by increased pressure on the cerebrospinal fluid. Inform your doctor immediately if you experience any of the following symptoms while taking PROCYSBI: ringing in the ears, dizziness, double vision, blurred vision, loss of vision, eye pain, or pain when moving your eye. Your doctor will perform eye exams to describe and treat this problem in its initial phase, which will contribute to reducing the likelihood of vision loss.

The following side effects are listed below 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
• Drowsiness

Common side effects:

• Headache
• Encephalopathy
• Abdominal pain
• Indigestion
• Bad breath and body odor
• Stomach burning
• Fatigue

Rare side effects:

• Leg pain
• Scoliosis (spinal column deviation)
• Bone fragility
• Change in hair color
• Seizures
• Nervousness
• Hallucinations
• Renal effects manifesting as swelling of the extremities and weight gain

Reporting side effects

If you experience any type of side effect, consult your doctor or pharmacist, even if it is a possible side effect not listed in this prospectus. You can also report them directly through theSpanish System for Pharmacovigilance of Medicines for Human Usehttps://www.notificaram.es. By reporting side effects, you can contribute to providing more information on the safety of this medicine.

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

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

Do not take this medication if the aluminum seal has remained open for more than 30 days. Dispose of the opened bottle and use a new one.

Store in refrigerator (between 2°C and 8°C). Do not freeze. After opening, do not store at a temperature above 25°C. Keep the container perfectly closed to protect it from light and moisture.

Medicines should not be thrown away through drains. Ask your pharmacist how to dispose of the containers and medications that you no longer need. In this way, you will help protect the environment.

Composition of PROCYSBI

• The active ingredient is cysteamine (in the form of mercaptamine bitartrate).

PROCYSBI 25mg hard-gastrorresistant capsules

Each hard-gastrorresistant capsule contains 25mg of cysteamine.

PROCYSBI 75mg hard-gastrorresistant capsules

Each hard-gastrorresistant capsule contains 75mg of cysteamine.

• The other components are:

• From the capsules: microcrystalline cellulose, copolymer of methacrylic acid-ethyl acrylate (1:1), hypromellose, talc, triethyl citrate, and sodium lauryl sulfate(see section “PROCYSBI contains sodium”).
• From the capsule coating: gelatin, titanium dioxide (E171), and indigo carmine (E132).
• From the printing ink: shellac, povidone (K-17), and titanium dioxide (E171).

Appearance of the product and contents of the package

• PROCYSBI 25mg is presented in the form of hard-gastrorresistant capsules (size 15.9x5.8mm). The blue-colored cap with a white "PRO" logo and the blue-colored body with "25mg" in white ink. A white plastic bottle contains 60 capsules. The child-resistant cap has an aluminum seal. Each bottle contains two additional plastic cylinders for extra protection against humidity and air.

• PROCYSBI 75 mg is presented in the form of hard-gastrorresistant capsules (size 21.7x7.6mm). The dark blue cap with a white "PRO" logo and the light blue body with "75 mg" in white ink. A white plastic bottle contains 250 capsules. The child-resistant cap has an aluminum seal. Each bottle contains three additional plastic cylinders for extra protection against humidity and air.

• Keep the cylinders inside each bottle during use. The cylinders can be discarded with the bottle after use.

Marketing Authorization Holder

Chiesi Farmaceutici S.p.A.

Via Palermo 26/A

43122 Parma

Italy

Responsible for manufacturing

Chiesi Farmaceutici S.p.A.

Via San Leonardo 96

43122 Parma

Italy

For more information about this medicine, please contact the local representative of the marketing authorization holder:

Last revision date of this leaflet:

The detailed information about this medicine is available on the website of the European Medicines Agency

http://www.ema.europa.eu.