Fabrazyme 5 mg/35 mg Solution

FABRAZYME contains the active substance agalsidase beta. FABRAZYME is used as enzyme replacement therapy in Fabry disease, where the level of α - galactosidase enzyme activity is absent or lower than normal. If you suffer from Fabry disease, a fat substance called globotriaosylceramide (GL-3) is not removed from the cells of your body and starts to accumulate in the walls of the blood vessels of your organs. FABRAZYME is indicated for use as long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry disease.

Do not use FABRAZYME

• If you have experienced an allergic anaphylactic reaction to agalsidase beta or if you are allergic (hypersensitive) to any of the other ingredients of FABRAZYME (listed in section 6 of this leaflet).

Warnings and precautions

Take special care with FABRAZYME:

• If you are treated with FABRAZYME, you may develop infusion-associated reactions. An infusion-associated reaction is any side effect occurring during the infusion or until the end of the infusion day (see section 4). If you experience a reaction like this, you should tell your doctor immediately. You may need to be given additional medicines to prevent such reactions from occurring.
• If you have advanced kidney disease.

Children and adolescents

The safety and efficacy of FABRAZYME have not been evaluated in patients younger than 8 years old.

Do not share medicines prescribed for you with any other person. Always use FABRAZYME exactly as your doctor has instructed you. You should check with your doctor or pharmacist if you are unsure.

FABRAZYME is only used under the supervision of a doctor who is knowledgeable in the treatment of Fabry disease. Your doctor may advise that you can be treated at home provided you meet certain criteria. Please contact your doctor if you would like to be treated at home.

FABRAZYME is given through a drip into a vein (by intravenous infusion). It is supplied as a powder which will be mixed with sterile water before it is given (see information for health care providers at the end of this leaflet).

The recommended dose of FABRAZYME is 1 mg/kg body weight, once every 2 weeks. The rate of infusion will be decided by your doctor and may not be changed without the supervision of your doctor.

If you have the impression that the effect of FABRAZYME is too strong or too weak, talk to your doctor or health care provider.

If you receive more FABRAZYME than you should

Your doctor or health care provider will administer FABRAZYME to you and make sure you receive the correct dosage at the correct time. However, in the event of an overdosage your doctor will manage the overdosage.

If you forget to use FABRAZYME

If you have missed an infusion of FABRAZYME please contact your doctor or health care provider.

FABRAZYME can have side effects. Not all side effects reported for FABRAZYME are included in this leaflet. Should your general health worsen or if you experience any untoward effects while receiving FABRAZYME, please consult your doctor, pharmacist or other health care provider for advice.

If any of the following happens, stop receiving FABRAZYME and tell your doctor immediately or go to the casualty department at your nearest hospital:

• Swelling of your hands, feet, ankles, face, lips, mouth or throat, which may cause difficulty in swallowing or breathing.
• Rash, hives or itching.
• Fainting.

These are all serious side effects. If you have them, you may have had a serious allergic reaction to FABRAZYME. You may need urgent medical attention or hospitalisation.

Tell your doctor immediately or go to the casualty department at your nearest hospital if you notice any of the following:

Frequent:

• Yellowing of your skin and eyes, also called jaundice.
• Heart palpitations.
• Increased heart rate.
• Shortness of breath.
• Fever and chills.

Less frequent:

• Decreased heart rate.
• Infusion-associated reactions (such as fever, chills, flushing, itching, difficulty breathing, throat tightness, chest discomfort, itchy rash, runny nose, abdominal pain, nausea, vomiting, muscle pain and headache). See section “Warnings and precautions”.

Frequency unknown:

• Serious inflammation of the blood vessels, appearing as purple raised itchy lesions on the skin. Your organs may also be affected and cause symptoms such as fever, muscle aches, joint pain, blood in the urine or stool, abdominal pain, vomiting, cough, numbness and weakness.
• Serious kidney disease which may cause kidney failure, with symptoms such as swelling in the legs and ankles, weight gain, fatigue, poor appetite and urine that looks foamy.
• Lack of oxygen affecting your tissue, causing changes in the colour of your skin, ranging from blue to cherry red.

These are all serious side effects. You may need urgent medical attention.

Tell your doctor as soon as possible if you notice any of the following:

Frequent:

• Nausea, vomiting.
• Headache.
• Abnormal sensations such as tingling anywhere in the body.
• Ringing/buzzing in the ears.
• Motion sickness.
• Increased tear formation.
• Cold symptoms.
• Muscle pain/spasms, pain in the extremities, back pain, joint pain, muscle stiffness.
• Dizziness, drowsiness, reduced sensation to touch, burning sensation, feeling of exhaustion.
• Nasal congestion, cough, wheezing.
• Redness of the skin.
• Flushing, increase/decrease in blood pressure, pale skin colour.

Less frequent:

• Swelling or pain of the ear area.
• Reddening of the eyes.
• Indigestion, difficulty swallowing.
• Influenza-like symptoms.
• Stuffy nose.
• Increased sensitivity to stimulation.
• Pain in the throat and airways.
• Purplish discolouration of the skin.
• Skin discomfort.
• Infusion site pain.
• Blood clotting at injection site.

Frequency unknown:

• Blood test results showing lower blood oxygen levels.

If you notice any side effects not mentioned in this leaflet, please inform your doctor or pharmacist.

Reporting of side effects

If you get side effects, talk to your doctor or pharmacist. You can also report side effects to:

• The Pharmacovigilance Unit at Sanofi: [email protected] (email) or 011 256-3700 (tel), or
• SAHPRA via the “6.04 Adverse Drug Reaction Reporting Form”, found online under SAHPRA’s publications: https://www.sahpra.org.za/Publications/Index/8

By reporting side effects, you can help provide more information on the safety of FABRAZYME.

Unopened vials: Store in a refrigerator (2 °C – 8 °C).

Reconstituted and diluted solutions: FABRAZYME contains no preservatives. From a microbiological point of view, FABRAZYME should be used immediately. If not used immediately, in-use storage and conditions prior to use are the responsibility of the user. The reconstituted solution cannot be stored and should be promptly diluted. Only the diluted solution can be stored for up to 24 hours at 2 °C to 8 °C.

• STORE ALL MEDICINES OUT OF REACH OF CHILDREN.
• Do not use after the expiry date printed on the label or carton.
• Return all unused medicine to your pharmacist.
• Do not dispose of unused medicine in drains and sewerage systems (e.g. toilets).