Procysbi 25 mg gastro-resistant hard capsules

Patient Information Leaflet

Introduction

Patient Information Leaflet

PROCYSBI 25 mg gastro-resistant hard capsules

PROCYSBI 75 mg gastro-resistant hard capsules

cysteamine (mercaptopamine bitartrate)

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

• Keep this leaflet, as you may need to read it again.
• If you have any questions, consult your doctor or pharmacist.
• This medicine has been prescribed for you only, and must not be given to other people, even if they have the same symptoms as you, because it could harm them.
• If you experience any side effects, consult your doctor or pharmacist, even if they are not listed in this leaflet. See section 4.

Leaflet Contents

1. What PROCYSBI is and what it is 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

1. What PROCYSBI is and what it is 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 function, causing 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 kidney damage and excessive loss of glucose, proteins, and electrolytes. Different organs may be affected at different ages.

PROCYSBI is a medicine that reacts with cystine and reduces its levels within cells. To achieve maximum benefit, cysteamine therapy should be initiated promptly following confirmation of the diagnosis of 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 starting to take PROCYSBI.

• Since oral cysteamine does not prevent the accumulation of cystine crystals in the eyes, you must continue using the cysteamine eye drops prescribed by your doctor.

• Whole cysteamine capsules must not be administered to children under 6 years of age due to the risk of choking (see 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 perform periodic examinations of the skin and bones and may reduce or discontinue treatment if necessary (see section 4).

• Gastrointestinal ulcers and bleeding may occur in patients treated with cysteamine (see section 4).

• During treatment with cysteamine, other gastrointestinal symptoms may occur, particularly nausea, vomiting, anorexia, and stomach pain. In such cases, your doctor may interrupt or adjust the dose.

• Consult your doctor if you experience unusual stomach symptoms or changes in your gastrointestinal symptoms.

• During treatment with cysteamine, symptoms such as seizures, fatigue, drowsiness, depression, and brain disorders (encephalopathy) may occur. If these symptoms occur, inform your doctor, who will adjust your dose.

• During treatment with cysteamine, abnormalities in liver function or a decrease in white blood cell count (leukopenia) may occur. Your doctor will periodically monitor blood counts and liver function.

• Your doctor will periodically monitor for benign intracranial hypertension (or pseudotumor cerebri [PTC]) and inflammation of the optic nerve (papilledema) associated with cysteamine treatment. You will undergo periodic eye examinations to detect this condition, as immediate treatment may prevent vision loss.

Other medicines and PROCYSBI

Inform your doctor or pharmacist if you are taking, have recently taken, or might need to take any other medicines. 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 medicine.

Taking PROCYSBI with food and drinks

For at least 1 hour before and 1 hour after taking PROCYSBI, try to avoid high-fat or high-protein meals, as well as any food or liquid that may reduce stomach acidity, such as milk or yogurt. If this is not possible, you may eat a small amount (approximately 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 juice) or water. For children and patients with swallowing difficulties, see section 3 “How to take PROCYSBI – Method of administration”.

Pregnancy and breastfeeding

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

Do not use this medicine 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 planning to become pregnant, consult your doctor about discontinuing treatment with this medicine, as it may be harmful to the fetus.

Do not use this medicine during breastfeeding (see section 2 “Do not take PROCYSBI”).

Driving and using machines

This medicine may cause drowsiness. When starting treatment, avoid driving, using machines, or performing other hazardous activities until you know how this medicine affects you.

PROCYSBI contains sodium

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

3. How to take PROCYSBI

Follow exactly the instructions for using this medicine given by your doctor or pharmacist. If you are unsure, 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 intended maintenance dose is 1.3 g/m²/day.

Dosage schedule

Take this medicine twice daily, every 12 hours. To obtain the maximum benefit from this medicine, try to avoid food and dairy products for at least one hour before and after taking PROCYSBI. If this is not possible, you may consume a small amount (approximately 100 grams) of food (preferably carbohydrates, e.g., bread, pasta, fruit) within one hour before and after administration of PROCYSBI.

It is important that you take PROCYSBI consistently over time.

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

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

Duration of treatment

Treatment with PROCYSBI should continue for life, as directed by your doctor.

Method of administration

This medicine is taken orally only (by mouth).

For this medicine to work properly:

• For patients who can swallow the capsule whole:

Swallow the capsule whole with an acidic drink (such as orange juice or any acidic 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 the hard gastro-resistant capsules and may choke on them. PROCYSBI may be administered to children under 6 years of age by opening the capsules and sprinkling the contents onto food or liquids, as described below.

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

Sprinkling onto food

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

Gently mix the granules into the soft food to form a mixture of granules and food. Consume the entire mixture. Then drink approximately 250 ml of an acidic drink (for example, orange juice or any other acidic fruit juice) or water to help facilitate ingestion of the mixture.

If you do not take the mixture immediately, it may be refrigerated (between 2°C and 8°C) from the time of preparation until administration, and must be consumed within 2 hours of preparation. Do not keep any of the mixture after 2 hours.

Administration via feeding tube

Open the hard gastro-resistant capsules and sprinkle the contents (granules) onto approximately 100 grams of apple sauce or fruit jam. Gently mix the granules into the soft food to form a mixture of granules and soft food. Administer the mixture through a gastrostomy tube, nasogastric tube, or gastrostomy-jejunostomy tube using a syringe with a catheter tip. Before administering PROCYSBI, open the gastrostomy tube port and connect the feeding tube. Flush with 5 ml of water to clean the port. Aspirate the mixture into the syringe. A maximum mixture volume of 60 ml in a catheter-tip syringe is recommended for a straight or bolus feeding tube. Place the opening of the syringe containing the PROCYSBI and food mixture into the feeding tube port and completely fill the tube with the mixture: gently pushing the syringe plunger and keeping the feeding tube horizontal during administration may help prevent blockage. It is suggested to use a viscous food such as apple sauce or fruit jam at a rate of about 10 ml every 10 seconds until the syringe is completely empty, to help prevent blockage. Repeat this step until the entire mixture has been administered. After administering PROCYSBI, aspirate 10 ml of fruit juice or water into another syringe and flush the gastrostomy tube, ensuring that no residue of the PROCYSBI and food mixture remains stuck in the feeding tube.

If you do not consume the mixture immediately, it may be refrigerated (between 2°C and 8°C) from the time of preparation until administration and must be consumed within 2 hours of preparation. Do not keep any of the mixture after 2 hours.

Consult your child’s doctor for complete instructions on how to properly administer the product through feeding tubes or if you experience tube blockage.

Sprinkling into orange juice or any other acidic fruit juice or water

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

If you do not take the mixture immediately, it may be refrigerated (between 2°C and 8°C) from the time of preparation until administration and must be consumed within 30 minutes of preparation. Do not keep any of the mixture after 30 minutes.

Administration of a drinkable mixture 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 may be refrigerated (between 2°C and 8°C) from the time of preparation until administration and must be consumed within 30 minutes of preparation. Do not keep any of the mixture after 30 minutes.

Your doctor may recommend or prescribe, in addition to cysteamine, one or more supplements to replace important electrolytes lost through the kidneys. It is important that these supplements are taken exactly as instructed. If several doses of the supplements are missed or if weakness or drowsiness occurs, consult your doctor for further instructions.

Regular blood tests are required to measure the amount of cystine in leukocytes 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 tests should be performed 12.5 hours after the previous evening's dose and then 30 minutes after the morning dose. Periodic blood and urine tests are also required to measure levels of important electrolytes to help your doctor or you correctly adjust the doses of these supplements.

If you take more PROCYSBI than you should

If you have taken more PROCYSBI than prescribed, contact your doctor immediately or go to the nearest hospital emergency department. You may experience drowsiness.

If you forget to take PROCYSBI

If you forget to take a dose, take it as soon as possible. If less than four hours remain before the next dose, skip the missed dose and return to your regular schedule.

Do not take a double dose to make up for a missed dose.

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

4. Possible adverse effects

Like all medicines, this medicine can cause adverse effects, although not everyone experiences them.

Contact a doctor or nurse immediately if you notice any of the following adverse effects, as you may require urgent medical treatment:

• Severe allergic reactions (uncommon): go to emergency services if you experience any of the following signs of an allergic reaction: hives, difficulty breathing, swelling of the face, lips, tongue, or throat.

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

Frequent adverse effects (may affect up to 1 in 10 people):

• Skin rash: inform your doctor immediately. Treatment with PROCYSBI may need to be temporarily discontinued until the rash resolves. If the rash is severe, your doctor may discontinue treatment with cysteamine.
• Abnormal liver function tests. Your doctor will monitor this effect.

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

• Skin, bone, and joint lesions: treatment with high doses of cysteamine may cause skin lesions. These include stretch marks (striae), bone lesions (such as fractures), bone deformities, and joint problems. Examine your skin regularly while taking this medicine. Report any changes to your doctor. Your doctor will monitor for 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 (excessive somnolence). Inform your doctor if you experience these symptoms.

• Gastrointestinal (stomach and intestinal) problems: patients taking cysteamine have developed ulcers and bleeding. Inform your doctor immediately if you experience stomach pain or vomit blood.

• Benign intracranial hypertension, also known as pseudotumor cerebri, has also been reported with the use of cysteamine. This is a condition characterized by increased pressure of the fluid surrounding the brain. Inform your doctor immediately if you experience any of the following symptoms while taking PROCYSBI: ringing in the ears, dizziness, double vision, blurred vision, vision loss, pain behind the eye, or pain when moving the eye. Your doctor will perform eye examinations to identify and treat this problem in its early stages, helping to reduce the risk of vision loss.

The remaining adverse effects listed below are provided with an estimate of how frequently they may occur with PROCYSBI.

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

• nausea
• vomiting
• loss of appetite
• diarrhea
• fever
• drowsiness

Common adverse effects:

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

Uncommon adverse effects:

• leg pain
• scoliosis (spinal curvature)
• bone fragility
• change in hair color
• seizures
• nervousness
• hallucinations
• kidney-related effects manifested by swelling of the limbs and weight gain

Reporting of adverse effects

If you experience any adverse effect, consult your doctor or pharmacist, even if it is a possible adverse effect not listed in this leaflet. You may also report them directly via the Spanish Pharmacovigilance System for Human Medicinal Products: https://www.notificaram.es. By reporting adverse effects, you can help provide more information on the safety of this medicine.

5. Storage of PROCYSBI

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

Do not use this medicine after the expiry date stated on the carton and on the label of the bottle after EXP. The expiry date refers to the last day of the month indicated.

Do not take this medicine if the aluminium seal has been open for more than 30 days. Discard the opened bottle and use a new one.

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

Medicines should not be disposed of via wastewater. Ask your pharmacist how to dispose of medicines and packaging you no longer need. This helps protect the environment.

6. Contents of the pack and other information

Composition of PROCYSBI

• The active substance is cysteamine (as mercaptamine bitartrate).

PROCYSBI 25 mg gastro-resistant hard capsules

Each gastro-resistant hard capsule contains 25 mg of cysteamine.

PROCYSBI 75 mg gastro-resistant hard capsules

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

• The other components are:

• Of the capsules: microcrystalline cellulose, methacrylic acid-ethyl acrylate copolymer (1:1), hypromellose, talc, triethyl citrate and sodium lauryl sulfate (see section “PROCYSBI contains sodium”).

• Of the capsule shell: gelatin, titanium dioxide (E171) and indigo carmine (E132).

• Of the printing ink: shellac glaze, povidone (K-17) and titanium dioxide (E171).

Presentation of the product and contents of the pack

• PROCYSBI 25 mg is available as gastro-resistant hard capsules (size 15.9 x 5.8 mm). The light blue cap is printed with the logo «PRO» in white ink and the light blue body is printed with «25 mg» in white ink. A white plastic bottle contains 60 capsules. The cap is child-resistant and has an aluminium seal. Each bottle contains two plastic cylinders used to provide additional protection against moisture and air.

• PROCYSBI 75 mg is available as gastro-resistant hard capsules (size 21.7 x 7.6 mm). The dark blue cap is printed with the logo «PRO» in white ink and the light blue body is printed with «75 mg» in white ink. A white plastic bottle contains 250 capsules. The cap is child-resistant and has an aluminium seal. Each bottle contains three plastic cylinders used to provide additional protection against moisture and air.

• Keep the cylinders inside each bottle during use. The cylinders may be discarded together 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

Further information on this medicinal product is available upon request to the local representative of the Marketing Authorisation Holder:

Date of the most recent review of this leaflet:

Detailed information on this medicine is available on the website of the European Medicines Agency http://www.ema.europa.eu.