PROCYSBI - Cysteamine uses, dose and side effects

PROCYSBI contains the active substance cysteamine (also called mercaptamine) and is taken to treat nephropathic cystinosis in children and adults. Cystinosis is a disease that affects the body’s function and causes an abnormal accumulation of the amino acid cystine in various organs in the body, e.g. kidneys, eyes, muscles, pancreas and brain. Cystine accumulation causes kidney damage and excessive secretion of glucose , proteins and electrolytes . Different organs are affected at different ages.

PROCYSBI is a drug that reacts with cystine and lowers the cystine content in the cells. Treatment with cysteamine should be initiated immediately after the diagnosis of cystinosis is confirmed to achieve maximum effect.

What you need to know before taking PROCYSBI

Do not take PROCYSBI

if you are allergic to cysteamine (also known as mercaptin) or any of the other ingredients of this medicine (listed in section 6),
if you are allergic to penicillamine,
if you are breast-feeding.

Warnings and cautions

Talk to your doctor or pharmacist before taking PROCYSBI.

As cysteamine taken by mouth does not prevent the accumulation of cystine crystals in the eye, you should continue to take cysteamine eye drops according to your doctor’s instructions.
Whole cysteam capsules should not be given to children under 6 years of age due to the risk of suffocation (see section 3 “How to take PROCYSBI” under “Method of administration”).
Patients treated with high- dose cysteamine may have severe skin lesions. Your doctor will check your skin and skeleton regularly and reduce or stop treatment if necessary (see section 4).
Ulcers or bleeding in the stomach and intestines may occur in patients receiving cysteamine (see section 4).
Other symptoms from the stomach and intestines, including nausea, vomiting, decreased appetite and stomach pain may occur when taking cysteamine. Your doctor may stop treatment and change your dose if these symptoms occur.
Talk to your doctor if you have unusual stomach symptoms or if your stomach symptoms change.
Symptoms such as seizures, fatigue, drowsiness, depression and brain disorders ( encephalopathy ) can occur when taking cysteamine. If you get such symptoms, tell your doctor, who will then adjust the dose .
There may be abnormal liver function or a lower number of white blood cells ( leukopenia ) when taking cysteamine. Your doctor will check your blood cell count and liver function regularly.
Your doctor will check you for benign intracranial hypertension (high pressure inside the skull) (also known as pseudotumor cerebri (PTC)) and / or swelling of the optic nerve (papilloedema) associated with cysteamine treatment. You must undergo regular eye examinations so that this condition can be detected as early treatment can prevent visual impairment.

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

Try to avoid meals that are high in fat or protein as well as foods and beverages that could reduce the acidity of the stomach at least 1 hour before and 1 hour after taking PROCYSBI, e.g. milk or yogurt. If this is not possible, you can eat a small amount (about 100 g) of food (preferably carbohydrates, eg bread, pasta or fruit) during the hour before and after PROCYSBI dose .

Take the capsule together with a sour drink (eg orange juice or other sour juice) or water. For information on children and patients with swallowing difficulties, see section 3 “How to take PROCYSBI” under “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, especially during the first three months of pregnancy. If you are a woman planning to become pregnant or become pregnant, tell your doctor immediately. Treatment with the drug may be stopped as continued treatment may be harmful to the unborn baby.

This medicine should not be used during breast-feeding (see section 2 under “Do not take PROCYSBI”).

Driving and using machines

This medicine may cause some drowsiness. When you start treatment, you should wait to drive, use machines and engage in other dangerous activities until you know how the medicine affects you.

PROCYSBI contains sodium

This medicine contains less than 1 mmol (23 mg) sodium per dose , ie essentially ‘sodium-free’. is next to “sodium-free”.

How to take PROCYSBI

Always take this medicine exactly as your doctor or pharmacist has told you. Ask your doctor or pharmacist if you are unsure.

The recommended dose for you or your child depends on your or your child’s age and body weight. The target for the maintenance dose is 1.3 g / m ² / day.

Dosage schedule

Take this medicine twice a day, every 12 hours. To get the most out of this medicine, you should try to avoid meals and dairy products for at least 1 hour before and 1 hour after a dose of PROCYSBI . If this is not possible, you can eat a small amount (about 100 grams) of food (preferably carbohydrates, eg bread pasta or fruit) during the hour before and after PROCYSBI dose .

It is important that you always take PROCYSBI in the same way.

Do not increase or decrease the dose of any of the medicines without your doctor’s consent.

The usual total dose should not exceed 1.95 g / m ² / day.

Duration of treatment

Treatment with PROCYSBI should be continued for life, according to the doctor’s instructions.

Method of administration

You should only take this medicine by mouth.

In order for this medicine to have the right effect, be sure to do the following:

Swallow the capsule whole with a sour drink (eg orange juice or other sour juice) or water. The capsules or capsule contents must not be crushed or chewed. Do not give hard enteric capsules to children under 6 years of age, as they may have difficulty swallowing and may suffocate. For patients who cannot swallow the whole capsule, you can open the hard enteric capsule and sprinkle the contents over food (eg apple puree or jam) and eat or give via a tube or mix it in a sour drink (eg orange juice or other acidic juice) or water. Ask your child’s doctor for complete instructions.
In addition to cysteamine, your treatment may consist of one or more supplements to replace important electrolytes (salts) that are lost in the urine. It is important that you take these supplements exactly as instructed. If you miss several doses of the supplements or if you become weak or lethargic, contact your doctor for instructions.
Regular blood tests are necessary to measure the amount of cystine in the white blood cells and / or the concentration of cysteamine in the blood to facilitate the correct dosing of PROCYSBI. You or your doctor will arrange for these blood tests to be performed. These tests must be done 12.5 hours after the evening dose the day before, ie 30 minutes after you take the next morning dose. Regular blood and urine tests are also necessary to measure the levels of important electrolytes in your body so that your doctor can adjust the doses of these supplements correctly.

If you take more PROCYSBI than you should

Contact your doctor or hospital emergency department immediately if you have taken too much PROCYSBI. You can get drowsy.

If you forget to take PROCYSBI

If you miss a dose of the medicine, take it as soon as possible. If there are less than 4 hours left until the next dose , you can skip the missed dose and return to normal medication times.

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

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

Possible side effects

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

Tell your doctor or nurse immediately if you get any of the following side effects – you may need urgent medical attention:

Severe allergic reaction (uncommon): Seek emergency medical attention if you have any of these signs of an allergic reaction: hives; hard to breathe; swelling of the face, lips, tongue or throat.

If you get any of these side effects , talk to your doctor immediately. As some of these side effects are serious, you should ask your doctor to explain any warning signs.

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

Skin rash: Tell your doctor immediately if you get a rash. PROCYSBI may need to be stopped temporarily until the rash disappears. If the rash is severe, your doctor may stop your cysteamine treatment.
Abnormal liver function during blood tests. Your doctor will check you for this.

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

Skin damage, skeletal damage and joint problems: Treatment with high doses of cysteamine can cause damage to the skin. These include striae (looks like stretch marks), skeletal injuries (eg fractures ), skeletal deformities and joint problems. Examine your skin while taking this medicine. Report any changes to your doctor. Your doctor will check you for these problems.
Low white blood cell count. Your doctor will check you for this.
Central nervous system symptoms: Some patients taking cysteamine have had seizures, depression and become very drowsy (excessive sleepiness). Tell your doctor if you get these symptoms.
Gastrointestinal problems (gastrointestinal): Patients taking cysteamine have ulcers and bleeding. Tell your doctor immediately if you have a stomach ache, nausea, vomiting, decreased appetite or if you vomit blood.
Benign intracranial hypertension (benign high pressure in the skull), also known as pseudotumor cerebri , has been reported with the use of cysteamine. This is a condition in which a high pressure occurs in the fluid that surrounds the brain. Tell your doctor immediately if you get any of the following symptoms while taking PROCYSBI: headache, buzzing or “roaring” sounds in the ears, dizziness, nausea, double vision, blurred vision, impaired vision, pain behind the eyes or pain with eye movements. Your doctor will monitor you with eye examinations to detect and treat this problem in good time. This reduces the risk of visual impairment.

Other side effects listed below are listed with an estimate of how often they may occur

arise with PROCYSBI.

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

diarrhea
fever
feeling sleepy

Common side effects:

unpleasant breath and body odor
heartburn
fatigue

Uncommon side effects are :

leg pain
scoliosis (curvature of the spine)
osteoporosis
discolored hair
seizures
nervousness
hallucination
effect on the kidneys which manifests as swollen arms and legs and weight gain

How to store PROCYSBI

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 bottle label after EXP. The expiration date is the last day of the specified month.

Do not take this medicine if the foil seal has been broken 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. Opened packaging is stored at a maximum of 25 ° C. Close the package tightly. Sensitive to light. Moisture sensitive.

Medicines should not be disposed of via wastewater. Ask your pharmacist how to dispose of medicines no longer required. These measures will help to protect the environment.

Contents of the pack and other information

Content declaration

The active substance is cysteamine (as mercaptamine bitartrate). Each hard enteric capsule contains 25 mg or 75 mg of cysteamine.
Other ingredients are:
- In the capsules: microcrystalline cellulose, methacrylic acid-ethyl acrylate copolymer (1: 1), hypromellose, talc, triethyl citrate, sodium lauryl sulfate.
- In the capsule shell: gelatin, titanium dioxide (E171), indigo carmine (E132).
- In the ink: shellac, povidone (K-17), titanium dioxide (E171).

What the medicine looks like and contents of the pack

PROCYSBI 25 mg is designed as blue enteric capsules, hard. The light blue cap is marked “PRO” with white ink and the light blue capsule body is printed with “25 mg” in white ink. A white plastic bottle contains 60 capsules. The lid is childproof and has a foil seal. Each bottle contains two plastic containers that are used for extra protection against moisture and air.
PROCYSBI 75 mg is designed as blue enteric capsules, hard. The dark blue cap is marked “PRO” with white ink and the light blue capsule body is printed with “75 mg” in white ink. A white plastic bottle contains 250 capsules. The lid is childproof and has a foil seal. Each bottle contains three plastic containers that are used for extra protection against moisture and air.
Leave the containers in the bottle for the entire period of use. The containers can be discarded together with the bottle after use.

Marketing Authorisation Holder

Chiesi Farmaceutici SpA

Via Palermo, 26 / A

43122 Parma

Italy

Manufacturer