Document Details

Generated Narrative: Bundle TEST PURPOSES ONLY - procysbi

Document Subject

Generated Narrative: MedicinalProductDefinition mp8fe556bb25526e379f33fd00eb2af422

identifier: http://ema.europa.eu/identifier/EU/1/13/861/001

type: Medicinal Product

domain: Human use

status: active

legalStatusOfSupply: Medicinal product subject to medical prescription

name

productName: PROCYSBI 25 mg gastro-resistant hard capsules

type: Full name

part

part: nan

type: Invented name part

part

part: nan

type: Scientific name part

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part: nan

type: Strength part

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part: nan

type: Pharmaceutical dose form part

Usages

-	Country	Jurisdiction	Language
*	EU	EU	en

Document Content

Generated Narrative: Composition composition-en-8fe556bb25526e379f33fd00eb2af422

identifier: http://ema.europa.eu/identifier/EU/1/13/861/001

status: Final

type: Package Leaflet

category: Raw

date: 2022-02-16 13:28:17+0000

author: Organization ACME industry

title: TEST PURPOSES ONLY - procysbi

Attesters

What is in 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

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 affecting how the body functions, with an abnormal accumulation of the amino acid cystine in various organs of the body such as the kidney, eye, muscle, pancreas, and brain. Cystine build-up causes kidney damage and excretion of excess 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 within the cells. Cysteamine therapy should be initiated promptly after confirmation of the diagnosis of cystinosis to achieve maximum benefit.

Do not take PROCYSBI

If you are allergic to cysteamine (also known as mercaptamine) or 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 for the treatment of Wilson s disease).

If you are breast-feeding.

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

Since oral cysteamine doesn t prevent deposits of cystine crystals in the eye, you should continue taking cysteamine eye drops as prescribed by your doctor.

Whole cysteamine capsules should not be given to children under the age of 6 years due to the risk of choking (refer to section 3 How to take PROCYSBI Method of administration ).

Serious skin lesions can occur in patients treated with high doses of cysteamine. Your doctor will routinely monitor your skin and bones and reduce or stop your treatment if needed (see section 4).

Stomach and intestinal ulcers and bleeding can occur in patients receiving cysteamine (see section 4).

Other intestinal symptoms including nausea, vomiting, anorexia and stomach ache can occur with cysteamine. Your doctor may interrupt and change your dose if these occur.

Talk to your doctor if you have any unusual stomach symptoms or changes in stomach symptoms.

Symptoms such as seizures, tiredness, sleepiness, depression, and brain disorders (encephalopathy) can occur with cysteamine. If such symptoms develop, tell your doctor who will adjust your dose.

Abnormal liver function or reduced white blood cell count (leukopenia) can occur with use of cysteamine. Your doctor will routinely monitor your blood counts and liver function.

Your doctor will monitor you for benign intracranial hypertension (or pseudotumor cerebri [PTC]) and/or swelling of the optic nerve (papilledema) associated with cysteamine treatment. You will receive regular eye examinations to identify this condition as early 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 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, which are rich in fat or proteins as well as any food or liquid that could decrease the acidity in your stomach, like milk or yogurt. If this is not possible, you can eat a small amount (about 100 grams) of food (preferably carbohydrates e.g. bread, pasta, fruits) 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 who have problems to swallow, please refer to section 3 How to take PROCYSBI
Method of administration .

Pregnancy and breastfeeding If you are pregnant or breast-feeding, think you may be pregnant or are planning to have a baby, ask your doctor or pharmacist for advice before taking this medicine.

You should not use this medicine if you are pregnant, particularly during the first trimester. Before starting the treatment, you should have a pregnancy test with negative result, while during the course of treatment you should use an adequate method of contraception. If you are a woman planning a pregnancy or become pregnant, seek immediate advice from your doctor about stopping therapy with this medicine as continued treatment may be harmful to the unborn baby.

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 starting therapy, you should not drive, use machines, or engage in other dangerous activities until you know how the medicine affects you.

PROCYSBI contains sodium This medicine contains less than 1 mmol sodium (23 mg) per dose, that is to say essentially sodium-free

Always take this medicine exactly as your doctor or pharmacist has told you. Check with your doctor or pharmacist if you are not sure.

The recommended dose for you or your child will depend on your or your child s age and weight. The targeted maintenance dose is 1.3 g/m2/day.

Dosing schedule Take this medicine two times a day, every 12 hours. To get the most benefit from this medicine, try to avoid meals and dairy products for at least 1 hour before and 1 hour after PROCYSBI dosing. If this is not possible, you can eat a small amount (about 100 grams) of food (preferably carbohydrates e.g. bread, pasta, fruits) during the hour before and after PROCYSBI administration.

It is important to take PROCYSBI in a consistent way over time.

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

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

Duration of treatment Treatment with PROCYSBI should continue life-long, as instructed by your doctor.

Method of administration You should take this medicine only by mouth.

In order for this medicine to work correctly, you must do the following:

• For patients who can swallow the whole capsule: Swallow the whole capsule with an acidic drink (such as orange juice or any acidic juice) or water. Do not crush or chew capsules or capsule contents. Children under 6 years of age may not be able to swallow gastro-resistant hard capsules and may choke them. You can give PROCYSBI to children under 6 years of age by opening the capsules and sprinkling the contents on food or liquid, as per the instructions given below.

For patients who cannot swallow the whole capsule or who use a feeding tube: Sprinkling on food Open the gastro-resistant hard capsules and sprinkle the contents (granules) onto approximately 100 grams of food such as apple sauce or fruit jam. Gently stir the granules into the soft food, creating a mixture of granules and food. Eat the entire mixture. Then drink about 250 mL of an acidic drink (such as orange juice or any acidic juice) or water to ease the swallowing of the mixture. If you don t eat the mixture immediately, you may refrigerate (2 C-8 C) it from the time of preparation to the time of administration and eat it within 2 hours of preparation. Nothing of the mixture should be saved beyond 2 hours.

Administering through feeding tube

Open the gastro-resistant hard capsules and sprinkle the contents (granules) onto approximately 100 grams of apple sauce or fruit jam. Gently stir the granules into the soft food, creating a mixture of granules and the soft food. Administer the mixture by gastrostomy tube, nasogastric tube or gastrostomy jejunostomy tube using a catheter tip syringe. Before PROCYSBI administration: Unclasp the G-tube button and attach the feeding tube. Flush with 5 mL of water to clear the button. Draw the mixture up into the syringe. A maximum 60 mL mixture volume in a catheter tip syringe is recommended for use with a straight or bolus feeding tube. Place the opening of the syringe containing the PROCYSBI and food mixture into the opening of the feeding tube and fill completely with the mixture: pressing gently on the syringe and keeping the feeding tube horizontal during administration can help to avoid clogging issues. Using 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 is suggested to avoid clogging. Repeat the above step until all of the mixture is given. After PROCYSBI administration, draw 10 mL of fruit juice or water up into another syringe and flush the G-tube ensuring that none of the PROCYSBI and food mixture gets stuck in the G-tube. If you don t consume the mixture immediately, you may refrigerate (2 C-8 C) it from the time of preparation to the time of administration and consume it within 2 hours of preparation. Nothing of the mixture should be saved beyond 2 hours. Consult your child s doctor for complete instructions on how to properly administer the product through feeding tubes and if you experience clogging issues.

Sprinkling in orange juice or any acidic fruit juice or water

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

If you don t drink the mixture immediately, you may refrigerate (2 C-8 C) it from the time of preparation to the time of administration and drink it within 30 minutes after preparation. Nothing of the mixture should be saved beyond 30 minutes.

Administering a drink mixture by oral syringe

Aspirate the drink mixture into a dosing syringe and administer it into the mouth directly.

If you don t consume the mixture immediately, you may refrigerate (2 C-8 C) it from the time of preparation to the time of administration and consume it within 30 minutes after preparation. Nothing of the mixture should be saved beyond 30 minutes.

Your doctor may recommend or prescribe to include, in addition to cysteamine, one or more supplements to replace important electrolytes lost through the kidneys. It is important to take these supplements exactly as instructed. If several doses of the supplements are missed or weakness or drowsiness develops, call your doctor for instructions.

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 help determine the correct dose of PROCYSBI. You or your doctor will arrange for these blood tests to be performed. These tests must be obtained 12.5 hours after the evening dose the day before, and therefore 30 minutes after the following morning dose is given. Regular blood and urine tests to measure the levels of the body s important electrolytes are also necessary to help you or your doctor correctly adjust the doses of these supplements.

If you take more PROCYSBI than you should You should contact your doctor or the hospital emergency department immediately if you have taken more PROCYSBI than you should. You may become drowsy.

If you forget to take PROCYSBI If you missed a dose of medicine, you should take it as soon as possible. However, if it is within 4 hours of the next dose, skip the missed dose and go back to the regular dosing schedule.

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

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

Like all medicines, this medicine can cause side effects, although not everybody gets them.

Tell your doctor or nurse straight away if you notice any of the following side effects you may need urgent medical treatment:

Severe allergic reaction (seen uncommonly): Get emergency medical help if you have any of these signs of an allergic reaction: hives; difficulty breathing; swelling of face, lips, tongue, or throat.

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

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

Skin rash: Tell the doctor right away if you get a skin rash. PROCYSBI may need to be temporarily stopped until the rash goes away. If the rash is severe, your doctor may discontinue cysteamine treatment.

Abnormal liver function on blood tests. 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 cause skin lesions to develop. These include skin striae (which are like stretch marks), bone injuries (such as fractures), bone deformities, and joint problems. Examine your skin while taking this medicine. Report any changes to your doctor. Your doctor will monitor you for these problems.

Low white blood cell count. Your doctor will monitor you for this.

Central nervous system symptoms: Some patients taking cysteamine have developed seizures, depression, and become too sleepy (excessive sleepiness). Tell your doctor if you have these symptoms.

Stomach and intestinal (gastrointestinal) problems: Patients taking cysteamine have developed ulcers and bleeding. Tell your doctor right away if you get stomach ache, or throw up blood.

Benign intracranial hypertension, also called pseudotumor cerebri, has been reported with cysteamine use. This is a condition where there is high pressure in the fluid around the brain. Tell your doctor right away if you develop any of the following symptoms while taking PROCYSBI: buzzing or whooshing sound in the ear, dizziness, double vision, blurry vision, loss of vision, pain behind the eye or pain with eye movement. Your doctor will monitor you with eye examinations to find and treat this problem early. This will help lessen the chance of loss of eyesight.

The other side effects listed below are given with an estimation 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

diarrhoea

fever

sensation of sleep

Common side effects:

headache

encephalopathy

abdominal pain

dyspepsia

unpleasant breath and body odour

heartburn

tiredness

Uncommon side effects:

leg pain

scoliosis (deviation of the vertebral column)

bone fragility

hair discolouration

fits

nervousness

hallucination

effect on the kidney manifested by swelling of the extremities and weight gain

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

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 carton and the bottle label after EXP. The expiry date refers to the last day of that month.

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

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

Do not throw away any medicines via wastewater. Ask your pharmacist how to throw away the medicines you no longer use. These measures will help protect the environment.

What PROCYSBI contains

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

What PROCYSBI looks like and contents of the pack

PROCYSBI 25 mg is presented as blue gastro-resistant hard capsules (of size 15.9 x 5.8 mm). The light blue cap is imprinted with PRO in white ink and the light blue body is imprinted with 25 mg in white ink. A white plastic bottle contains 60 capsules. The cap is child resistant and has a foil seal. Each bottle contains two plastic cylinders used for additional moisture and air protection

PROCYSBI 75 mg is presented as blue gastro-resistant hard capsules (of size 21.7 x 7.6 mm). The dark blue cap is imprinted with PRO in white ink and the light blue body is imprinted with 75 mg in white ink. A white plastic bottle contains 250 capsules. The cap is child resistant and has a foil seal. Each bottle contains three plastic cylinders used for additional moisture and air protection.

Please keep the cylinders in each bottle during the use of the bottle. The cylinders may 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 43122 Parma
Italy

For any information about this medicine, please contact the local representative of the Marketing Authorisation Holder:

Belgi /Belgique/Belgien Chiesi sa/nv
T l/Tel: + 32 (0)2 788 42 Lietuva Chiesi Pharmaceuticals GmbH
Tel: + 43 1 4073
Chiesi Bulgaria EOOD
Te .: + 359 29201Luxembourg/Luxemburg Chiesi sa/nv
T l/Tel: + 32 (0)2 788 42 esk republika Chiesi CZ s.r.o.
Tel: + 420 261221Magyarorsz g Chiesi Hungary Kft.
Tel.: + 36-1-429 1Danmark Chiesi Pharma AB
Tlf: + 46 8 753 35 Malta Chiesi Farmaceutici S.p.A.
Tel: + 39 0521 2Deutschland Chiesi GmbH
Tel: + 49 40 89724-0

Nederland Chiesi Pharmaceuticals B.V.
Tel: + 31 88 501 64 Eesti Chiesi Pharmaceuticals GmbH
Tel: + 43 1 4073Norge Chiesi Pharma AB
Tlf: + 46 8 753 35
Chiesi Hellas AEBE
: + 30 210 6179 sterreich Chiesi Pharmaceuticals GmbH
Tel: + 43 1 4073Espa a Chiesi Espa a, S.A.U.
Tel: + 34 93 494 8Polska Chiesi Poland Sp. z.o.o.
Tel.: + 48 22 620 1France Chiesi S.A.S.
T l: + 33 1 47688Portugal Chiesi Farmaceutici S.p.A.
Tel: + 39 0521 2Hrvatska Chiesi Pharmaceuticals GmbH
Tel: + 43 1 4073Rom nia Chiesi Romania S.R.L.
Tel: + 40 212023Ireland Chiesi Farmaceutici S.p.A.
Tel: + 39 0521 2Slovenija Chiesi Slovenija d.o.o.
Tel: + 386-1-43 00 sland Chiesi Pharma AB
S mi: +46 8 753 35 Slovensk republika Chiesi Slovakia s.r.o.
Tel: + 421 259300Italia Chiesi Italia S.p.A.
Tel: + 39 0521 2Suomi/Finland Chiesi Pharma AB
Puh/Tel: +46 8 753 35
Chiesi Farmaceutici S.p.A.
: + 39 0521 2Sverige Chiesi Pharma AB
Tel: +46 8 753 35 Latvija Chiesi Pharmaceuticals GmbH
Tel: + 43 1 4073United Kingdom (Northern Ireland)
Chiesi Farmaceutici S.p.A.
Tel: + 39 0521 2This leaflet was last revised in

Detailed information on this medicine is available on the website of the European Medicines Agency