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Fabrazyme (agalsidase beta) – Package leaflet - A16AB04

Updated on site: 06-Oct-2017

Medication nameFabrazyme
ATC CodeA16AB04
Substanceagalsidase beta
ManufacturerGenzyme Europe B.V.

Package leaflet: Information for the user

Fabrazyme 35 mg powder for concentrate for solution for infusion

Agalsidase beta

Read all of this leaflet carefully before you start using this medicine because it contains important information for you.

-Keep this leaflet. You may need to read it again.

-If you have any further questions, ask your doctor or pharmacist.

-This medicine has been prescribed for you only. Do not pass it on to others. It may harm them, even if their signs of illness are the same as yours.

-If you get any side effects, talk to your doctor or pharmacist. This includes any possible side effects not listed in this leaflet. See section 4.

What is in this leaflet

1.What Fabrazyme is and what it is used for

2.What you need to know before you use Fabrazyme

3.How to use Fabrazyme

4.Possible side effects

5.How to store Fabrazyme

6.Contents of the pack and other information

1.What Fabrazyme is and what it is used for

Fabrazyme contains the active substance agalsidase beta and is used as enzyme replacement therapy in Fabry disease, where the level of -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.

Fabrazyme is indicated in adults, children and adolescents aged 8 years and older.

2.What you need to know before you use Fabrazyme

Do not use Fabrazyme

-if you are allergic to agalsidase beta or any of the other ingredients of this medicine (listed in section 6).

Warnings and precautions

Talk to your doctor or pharmacist before using 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.

Children and adolescents

No clinical studies have been performed in children 0-4 years old. The risks and benefits of Fabrazyme in children aged 5 to 7 years have not yet been established and therefore no dose can be recommended for this age group.

Other medicines and Fabrazyme

Tell your doctor or pharmacist if you are taking, have recently taken or might take any other medicines.

Tell your doctor if you use any medicines containing chloroquine, amiodarone, benoquin or gentamicin. There is a theoretical risk of decreased agalsidase beta activity.

Pregnancy, breast-feeding and fertility

Use of Fabrazyme during pregnancy is not recommended. There is no experience with the use of Fabrazyme in pregnant women. Fabrazyme may get into breast milk. Use of Fabrazyme during breast- feeding is not recommended. Studies have not been performed to examine the effects of Fabrazyme on fertility.

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.

Driving and using machines

Do not drive or use machines if you experience dizziness, sleepiness, vertigo or fainting during or shortly after administration of Fabrazyme (see section 4). Talk to your doctor first.

3.How to use Fabrazyme

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 Professionals at the end of this leaflet).

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

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.

The recommended dose of Fabrazyme for adults is 1 mg/kg body weight, once every 2 weeks. No changes in dose are necessary for patients with kidney disease.

Use in children and adolescents

The recommended dose of Fabrazyme for children and adolescents 8 – 16 years is 1 mg/kg body weight, once every 2 weeks. No changes in dose are necessary for patients with kidney disease.

If you use more Fabrazyme than you should

Doses up to 3 mg/kg body weight have shown to be safe.

If you forget to use Fabrazyme

If you have missed an infusion of Fabrazyme, please contact your doctor.

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

4.Possible side effects

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

In clinical studies side effects were mainly seen while patients were being given the medicine or shortly after (“infusion related reactions”). Severe life-threatening allergic reactions (“anaphylactoid reactions”) have been reported in some patients. If you experience any serious side effect, you should contact your doctor immediately.

Very common symptoms (may affect more than 1 in 10 people) include chills, fever, feeling cold, nausea, vomiting, headache and abnormal feelings in the skin such as burning or tingling. Your doctor may decide to lower the infusion rate or give you additional medicines to prevent such reactions from occurring.

List of other side effects:

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

 

 

 

chest pain

 

sleepiness

 

fatigue

 

difficulty in breathing

 

increased heart beat

 

flushing

 

pallor

 

abdominal pain

 

pain

 

itching

 

back pain

 

throat tightness

 

abnormal tear secretion

 

rash

 

dizziness

 

feeling weak

 

low heart rate

 

palpitations

 

tinnitus

 

lethargy

 

decreased sensitivity to pain

 

nasal congestion

 

syncope

 

burning sensation

 

diarrhoea

 

cough

 

wheezing

 

redness

 

abdominal discomfort

 

urticaria

 

muscle pain

 

swelling face

 

pain at the extremities

 

increased blood pressure

 

joint pain

 

nasopharyngitis

 

sudden swelling of the face

 

decreased blood pressure

 

hot flush

 

or throat

 

 

 

 

 

oedema in extremities

 

chest discomfort

 

feeling hot

 

vertigo

 

face oedema

 

hyperthermia

 

stomach discomfort

 

exacerbated difficulty in

 

decreased mouth sensitivity

 

 

 

breathing

 

 

 

muscle spasms

 

muscle tightness

 

musculoskeletal stiffness

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

 

 

 

tremor

 

itching eyes

 

low heart rate due to conduction

 

 

 

 

 

disturbances

 

red eyes

 

ear swelling

 

increased sensitivity to pain

 

ear pain

 

bronchospasm

upper respiratory tract congestion

 

throat pain

 

runny nose

 

red rash

 

fast breathing

 

heart burn

(mottled purplish) skin discoloration

 

itchy rash

 

skin discomfort

 

coldness of the extremities

 

feeling hot and cold

 

musculoskeletal pain

 

injection site blood clotting

 

difficulty swallowing

 

rhinitis

 

skin discoloration

 

infusion site pain

 

influenza-like illness

 

oedema

 

infusion site reaction

 

malaise

 

 

Not known (frequency cannot be estimated from the available data):lower blood oxygen levels serious inflammation of the

vessels

In some patients initially treated at the recommended dose, and whose dose was later reduced for an extended period, some symptoms of Fabry disease were reported more frequently.

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.

5.How to store Fabrazyme

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 label after ‘EXP’. The expiry date refers to the last day of that month.

Unopened vials

Store in a refrigerator (2 C – 8 C).

Reconstituted and diluted solutions

The reconstituted solution cannot be stored and should be promptly diluted. The diluted solution can be held for up to 24 hours at 2 C – 8 C.

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

6.Contents of the pack and other information

What Fabrazyme contains

-The active substance is agalsidase beta, one vial contains 35 mg.

-The other ingredients are:

-Mannitol

-Sodium phosphate monobasic, monohydrate

-Sodium phosphate dibasic, heptahydrate.

What Fabrazyme looks like and contents of the pack

Fabrazyme is supplied as a white to off-white powder. After reconstitution it is a clear, colourless liquid, free from foreign matter. The reconstituted solution must be further diluted.

Package sizes: 1, 5 and 10 vials per carton. Not all pack sizes may be marketed.

Marketing Authorisation Holder and Manufacturer

Marketing authorisation holder

Genzyme Europe B.V., Gooimeer 10, 1411 DD Naarden, The Netherlands

Manufacturer

Genzyme Ltd., 37 Hollands Road, Haverhill, Suffolk CB9 8PU, United Kingdom

Genzyme Ireland Ltd., IDA Industrial Park, Old Kilmeaden Road, Waterford, Ireland

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

België/Belgique/Belgien/

Lietuva

Luxembourg/Luxemburg

UAB „SANOFI-AVENTIS LIETUVA“

Sanofi Belgium

Tel. +370 5 275 5224

Tél/Tel: + 32 2 710 54 00

 

България

Magyarország

SANOFI BULGARIA

SANOFI-AVENTIS Zrt.

Tел: +359 2 9705300

Tel: +36 1 505 0050

Česká republika

Malta

sanofi-aventis, s.r.o.

Sanofi Malta Ltd

Tel: +420 233 086 111

Tel: +356 21493022

Danmark

Nederland

sanofi-aventis Denmark A/S

Genzyme Europe B.V.

Tlf: +45 45 16 70 00

Tel: +31 35 699 1200

Deutschland

Norge

Genzyme GmbH

sanofi-aventis Norge AS

Tel: +49 (0)6102 3674 0

Tlf: + 47 67 10 71 00

Eesti

Österreich

sanofi-aventis Estonia OÜ

sanofi-aventis GmbH

Tel. +372 6 273 488

Tel: + 43 1 80 185 - 0

Ελλάδα

Polska

sanofi-aventis AEBE

sanofi-aventis Sp. z o.o.

Τηλ: +30 210 900 1600

Tel.: +48 22 280 00 00

España

Portugal

sanofi-aventis, S.A.

Sanofi – Produtos Farmacêuticos, Lda.

Tel: +34 93 485 94 00

Tel: +351 21 422 0100

France

România

Genzyme S.A.S.

Sanofi Romania SRL

Information médicale: tél: +33(0) 800 100 499

Tel: +40 (0) 21 317 31 36

Hrvatska

Slovenija

sanofi-aventis Croatia d.o.o.

sanofi-aventis d.o.o.

Tel: +385 1 600 34 00

Tel: +386 1 560 4800

Ísland

Slovenská republika

Vistor hf.

sanofi-aventis Pharma Slovakia s.r.o.

Sími: +354 535 7000

Tel.: +421 2 33 100 100

Italia

Suomi/Finland

Genzyme Srl

Sanofi Oy

Tel: +39 059 349 811

Puh/Tel: + 358 201 200 300

Κύπρος

Sverige

sanofi-aventis Cyprus Ltd.

Sanofi AB

Τηλ: +357 22 871600

Tel: +46 (0)8 634 50 00

Latvija

United Kingdom/Ireland

sanofi-aventis Latvia SIA

Genzyme Therapeutics Ltd. (United Kingdom)

Tel: +371 67 33 24 51

Tel: +44 (0) 1865 405200

This leaflet was last revised in

 

Other sources of information

 

Detailed information on this medicine is available on the European Medicines Agency web site: http://www.ema.europa.eu. There are also links to other websites about rare diseases and treatments.

---------------------------------------------------------------------------------------------------------------------------

The following information is intended for healthcare professionals only:

Instructions for use – reconstitution, dilution and administration

The powder for concentrate for solution for infusion has to be reconstituted with water for injections, diluted with 0.9% sodium chloride intravenous solution and then administered by intravenous infusion.

From a microbiological point of view, the product should be used immediately. If not used immediately, in-use storage and conditions are the responsibility of the user. The reconstituted solution cannot be stored and should be promptly diluted; only the diluted solution can be held for up to 24 hours at 2ºC -8ºC.

Use Aseptic Technique

1.Determine the number of vials to be reconstituted based on the individual patient's weight and remove the required vials from the refrigerator in order to allow them to reach room temperature (in approximately 30 minutes). Each vial of Fabrazyme is intended for single use only.

Reconstitution

2.Reconstitute each vial of Fabrazyme 35 mg with 7.2 ml water for injections. Avoid forceful impact of the water for injections on the powder and avoid foaming. This is done by slow drop- wise addition of the water for injection down the inside of the vial and not directly onto the lyophilized cake. Roll and tilt each vial gently. Do not invert, swirl or shake the vial.

3.The reconstituted solution contains 5 mg agalsidase beta per ml, and appears as a clear colourless solution. The pH of the reconstituted solution is approximately 7.0. Before further dilution, visually inspect the reconstituted solution in each vial for particulate matter and discoloration. Do not use the solution if foreign particles are observed or if the solution is discoloured.

4.After reconstitution it is recommended to promptly dilute the vials, to minimise protein particle formation over time.

5.Any unused medicinal product or waste material should be disposed of in accordance with local requirements.

Dilution

6.Prior to adding the reconstituted volume of Fabrazyme required for the patient dose, it is recommended to remove an equal volume of 0.9% sodium chloride intravenous solution, from the infusion bag.

7.Remove the airspace within the infusion bag to minimize the air/liquid interface.

8.Slowly, withdraw 7.0 ml (equal to 35 mg) of the reconstituted solution from each vial up to the total volume required for the patient dose. Do not use filter needles and avoid foaming.

9.Then slowly inject the reconstituted solution directly into the 0.9% sodium chloride intravenous solution (not in any remaining airspace) to a final concentration between 0.05 mg/ml and

0.7 mg/ml. Determine the total volume of sodium chloride 0.9% solution for infusion (between 50 and 500 ml) based on the individual dose. For doses lower than 35 mg use a minimum of 50 ml, for doses 35 to 70 mg use a minimum of 100 ml, for doses 70 to 100 mg use a minimum of 250 ml and for doses greater than 100 mg use only 500 ml. Gently invert or lightly massage the infusion bag to mix the diluted solution. Do not shake or excessively agitate the infusion bag.

Administration

10.It is recommended to administer the diluted solution through an in-line low protein-binding 0.2 µm filter to remove any protein particles which will not lead to any loss of agalsidase beta activity. The initial infusion rate should be no more than 0.25 mg/min (15 mg/hour) to minimise the potential occurrence of infusion-associated reactions. After patient tolerance is established, the infusion rate may be increased gradually with subsequent infusions.

Package leaflet: Information for the user

Fabrazyme 5 mg powder for concentrate for solution for infusion

Agalsidase beta

Read all of this leaflet carefully before you start using this medicine because it contains important information for you.

-Keep this leaflet. You may need to read it again.

-If you have any further questions, ask your doctor or pharmacist.

-This medicine has been prescribed for you only. Do not pass it on to others. It may harm them, even if their signs of illness are the same as yours.

-If you get any side effects, talk to your doctor or pharmacist. This includes any possible side effects not listed in this leaflet. See section 4.

What is in this leaflet

1.What Fabrazyme is and what it is used for

2.What you need to know before you use Fabrazyme

3.How to use Fabrazyme

4.Possible side effects

5.How to store Fabrazyme

6.Contents of the pack and other information

1.What Fabrazyme is and what it is used for

Fabrazyme contains the active substance agalsidase beta and is used as enzyme replacement therapy in Fabry disease, where the level of -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.

Fabrazyme is indicated in adults, children and adolescents aged 8 years and older.

2.What you need to know before you use Fabrazyme

Do not use Fabrazyme

-if you are allergic to agalsidase beta or any of the other ingredients of this medicine (listed in section 6).

Warnings and precautions

Talk to your doctor or pharmacist before using 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.

Children and adolescents

No clinical studies have been performed in children 0-4 years old. The risks and benefits of Fabrazyme in children aged 5 to 7 years have not yet been established and therefore no dose can be recommended for this age group.

Other medicines and Fabrazyme

Tell your doctor or pharmacist if you are taking, have recently taken or might take any other medicines.

Tell your doctor if you use any medicines containing chloroquine, amiodarone, benoquin or gentamicin. There is a theoretical risk of decreased agalsidase beta activity.

Pregnancy, breast-feeding and fertility

Use of Fabrazyme during pregnancy is not recommended. There is no experience with the use of Fabrazyme in pregnant women. Fabrazyme may get into breast milk. Use of Fabrazyme during breast- feeding is not recommended. Studies have not been performed to examine the effects of Fabrazyme on fertility.

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.

Driving and using machines

Do not drive or use machines if you experience dizziness, sleepiness, vertigo or fainting during or shortly after administration of Fabrazyme (see section 4). Talk to your doctor first.

3.How to use Fabrazyme

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 Professionals at the end of this leaflet).

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

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.

The recommended dose of Fabrazyme for adults is 1 mg/kg body weight, once every 2 weeks. No changes in dose are necessary for patients with kidney disease.

Use in children and adolescents

The recommended dose of Fabrazyme for children and adolescents 8 – 16 years is 1 mg/kg body weight, once every 2 weeks. No changes in dose are necessary for patients with kidney disease.

If you use more Fabrazyme than you should

Doses up to 3 mg/kg body weight have shown to be safe.

If you forget to use Fabrazyme

If you have missed an infusion of Fabrazyme, please contact your doctor.

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

4.Possible side effects

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

In clinical studies side effects were mainly seen while patients were being given the medicine or shortly after (“infusion related reactions”). Severe life-threatening allergic reactions (“anaphylactoid reactions”) have been reported in some patients. If you experience any serious side effect, you should contact your doctor immediately.

Very common symptoms (may affect more than 1 in 10 people) include chills, fever, feeling cold, nausea, vomiting, headache and abnormal feelings in the skin such as burning or tingling. Your doctor may decide to lower the infusion rate or give you additional medicines to prevent such reactions from occurring.

List of other side effects:

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

 

 

 

chest pain

 

sleepiness

 

fatigue

 

difficulty in breathing

 

increased heart beat

 

flushing

 

pallor

 

abdominal pain

 

pain

 

itching

 

back pain

 

throat tightness

 

abnormal tear secretion

 

rash

 

dizziness

 

feeling weak

 

low heart rate

 

palpitations

 

tinnitus

 

lethargy

 

decreased sensitivity to pain

 

nasal congestion

 

syncope

 

burning sensation

 

diarrhoea

 

cough

 

wheezing

 

redness

 

abdominal discomfort

 

urticaria

 

muscle pain

 

swelling face

 

pain at the extremities

 

increased blood pressure

 

joint pain

 

nasopharyngitis

 

sudden swelling of the face

 

decreased blood pressure

 

hot flush

 

or throat

 

 

 

 

 

oedema in extremities

 

chest discomfort

 

feeling hot

 

vertigo

 

face oedema

 

hyperthermia

 

stomach discomfort

 

exacerbated difficulty in

 

decreased mouth sensitivity

 

 

 

breathing

 

 

 

muscle spasms

 

muscle tightness

 

musculoskeletal stiffness

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

 

 

 

tremor

 

itching eyes

 

low heart rate due to conduction

 

 

 

 

 

disturbances

 

red eyes

 

ear swelling

 

increased sensitivity to pain

 

ear pain

 

bronchospasm

upper respiratory tract congestion

 

throat pain

 

runny nose

 

red rash

 

fast breathing

 

heart burn

(mottled purplish) skin discoloration

 

itchy rash

 

skin discomfort

 

coldness of the extremities

 

feeling hot and cold

 

musculoskeletal pain

 

injection site blood clotting

 

difficulty swallowing

 

rhinitis

 

skin discoloration

 

infusion site pain

 

influenza-like illness

 

oedema

 

infusion site reaction

 

malaise

 

 

Not known (frequency cannot be estimated from the available data):lower blood oxygen levels serious inflammation of the

vessels

In some patients initially treated at the recommended dose, and whose dose was later reduced for an extended period, some symptoms of Fabry disease were reported more frequently.

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.

5.How to store Fabrazyme

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 label after ‘EXP’. The expiry date refers to the last day of that month.

Unopened vials

Store in a refrigerator (2 C – 8 C).

Reconstituted and diluted solutions

The reconstituted solution cannot be stored and should be promptly diluted. The diluted solution can be held for up to 24 hours at 2 C – 8 C.

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

6.Contents of the pack and other information

What Fabrazyme contains

-The active substance is agalsidase beta, one vial contains 5 mg.

-The other ingredients are:

-Mannitol

-Sodium phosphate monobasic, monohydrate

-Sodium phosphate dibasic, heptahydrate.

What Fabrazyme looks like and contents of the pack

Fabrazyme is supplied as a white to off-white powder. After reconstitution it is a clear, colourless liquid, free from foreign matter. The reconstituted solution must be further diluted.

Package sizes: 1, 5 and 10 vials per carton. Not all pack sizes may be marketed.

Marketing Authorisation Holder and Manufacturer

Marketing authorisation holder

Genzyme Europe B.V., Gooimeer 10, 1411DD Naarden, The Netherlands

Manufacturer

Genzyme Ltd., 37 Hollands Road, Haverhill, Suffolk CB9 8PU, United Kingdom

Genzyme Ireland Ltd., IDA Industrial Park, Old Kilmeaden Road, Waterford, Ireland

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

België/Belgique/Belgien/

Lietuva

Luxembourg/Luxemburg

UAB „SANOFI-AVENTIS LIETUVA“

Sanofi Belgium

Tel. +370 5 275 5224

Tél/Tel: + 32 2 710 54 00

 

България

Magyarország

SANOFI BULGARIA EOOD

SANOFI-AVENTIS Zrt.

Tел: +359 2 9705300

Tel: +36 1 505 0050

Česká republika

Malta

sanofi-aventis, s.r.o.

Sanofi Malta Ltd

Tel: +420 233 086 111

Tel: +356 21493022

Danmark

Nederland

sanofi-aventis Denmark A/S

Genzyme Europe B.V.

Tlf: +45 45 16 70 00

Tel: +31 35 699 1200

Deutschland

Norge

Genzyme GmbH

sanofi-aventis Norge AS

Tel: +49 (0)6102 3674 0

Tlf: + 47 67 10 71 00

Eesti

Österreich

sanofi-aventis Estonia OÜ

sanofi-aventis GmbH

Tel. +372 6 273 488

Tel: + 43 1 80 185 - 0

Ελλάδα

Polska

sanofi-aventis AEBE

sanofi-aventis Sp. z o.o.

Τηλ: +30 210 900 1600

Tel.: +48 22 280 00 00

España

Portugal

sanofi-aventis, S.A.

Sanofi – Produtos Farmacêuticos, Lda. Tel:

Tel: +34 93 485 94 00

+351 21 422 0100

France

România

Genzyme S.A.S.

Sanofi Romania SRL

Information médicale: tél: +33(0) 800 100 499

Tel: +40 (0) 21 317 31 36

Hrvatska

Slovenija

sanofi-aventis Croatia d.o.o.

sanofi-aventis d.o.o.

Tel: +385 1 600 34 00

Tel: +386 1 560 4800

Ísland

Slovenská republika

Vistor hf.

sanofi-aventis Pharma Slovakia s.r.o.

Sími: +354 535 7000

Tel.: +421 2 33 100 100

Italia

Suomi/Finland

Genzyme Srl

Sanofi Oy

Tel: +39 059 349 811

Puh/Tel: + 358 201 200 300

Κύπρος

Sverige

sanofi-aventis Cyprus Ltd.

Sanofi AB

Τηλ: +357 22 871600

Tel: +46 (0)8 634 50 00

Latvija

United Kingdom/Ireland

sanofi-aventis Latvia SIA

Genzyme Therapeutics Ltd. (United Kingdom)

Tel: +371 67 33 24 51

Tel: +44 (0) 1865 405200

This leaflet was last revised in

 

Other sources of information

 

Detailed information on this medicine is available on the European Medicines Agency web site: http://www.ema.europa.eu. There are also links to other websites about rare diseases and treatments.

---------------------------------------------------------------------------------------------------------------------------

The following information is intended for healthcare professionals only:

Instructions for use – reconstitution, dilution and administration

The powder for concentrate for solution for infusion has to be reconstituted with water for injections, diluted with 0.9% sodium chloride intravenous solution and then administered by intravenous infusion.

From a microbiological point of view, the product should be used immediately. If not used immediately, in-use storage and conditions are the responsibility of the user. The reconstituted solution cannot be stored and should be promptly diluted; only the diluted solution can be held for up to 24 hours at 2ºC -8ºC.

Use Aseptic Technique

1.Determine the number of vials to be reconstituted based on the individual patient's weight and remove the required vials from the refrigerator in order to allow them to reach room temperature (in approximately 30 minutes). Each vial of Fabrazyme is intended for single use only.

Reconstitution

2.Reconstitute each vial of Fabrazyme 5 mg with 1.1 ml water for injections. Avoid forceful impact of the water for injections on the powder and avoid foaming. This is done by slow drop- wise addition of the water for injection down the inside of the vial and not directly onto the lyophilized cake. Roll and tilt each vial gently. Do not invert, swirl or shake the vial.

3.The reconstituted solution contains 5 mg agalsidase beta per ml, and appears as a clear colourless solution. The pH of the reconstituted solution is approximately 7.0. Before further dilution, visually inspect the reconstituted solution in each vial for particulate matter and discoloration. Do not use the solution if foreign particles are observed or if the solution is discoloured.

4.After reconstitution it is recommended to promptly dilute the vials, to minimise protein particle formation over time.

5.Any unused medicinal product or waste material should be disposed of in accordance with local requirements.

Dilution

6.Prior to adding the reconstituted volume of Fabrazyme required for the patient dose, it is recommended to remove an equal volume of 0.9% sodium chloride intravenous solution, from the infusion bag.

7.Remove the airspace within the infusion bag to minimize the air/liquid interface.

8.Slowly, withdraw 1.0 ml (equal to 5 mg) of the reconstituted solution from each vial up to the total volume required for the patient dose. Do not use filter needles and avoid foaming.

9.Then slowly inject the reconstituted solution directly into the 0.9% sodium chloride intravenous solution (not in any remaining airspace) to a final concentration between 0.05 mg/ml and

0.7 mg/ml. Determine the total volume of sodium chloride 0.9% solution for infusion (between 50 and 500 ml) based on the individual dose. For doses lower than 35 mg use a minimum of 50 ml, for doses 35 to 70 mg use a minimum of 100 ml, for doses 70 to 100 mg use a minimum of 250 ml and for doses greater than 100 mg use only 500 ml. Gently invert or lightly massage the infusion bag to mix the diluted solution. Do not shake or excessively agitate the infusion bag.

Administration

10.It is recommended to administer the diluted solution through an in-line low protein-binding 0.2 µm filter to remove any protein particles which will not lead to any loss of agalsidase beta activity. The initial infusion rate should be no more than 0.25 mg/min (15 mg/hour) to minimise the potential occurrence of infusion-associated reactions. After patient tolerance is established, the infusion rate may be increased gradually with subsequent infusions.

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