Fabrazyme – Package Leaflet: InfoCons – Consumer Protection presents, in an accessible format, the official package leaflet of the medicine Fabrazyme, as authorised in the European Union and published in the Union Register of medicinal products.
Before anything else
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 leafle t 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 ing redients 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 s ide 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 occ urring.
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.
27 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 There is limited experience with the use of Fabrazyme in pregnant women. As a precaution, it is preferable to avoid the use of Fabrazyme during pregnancy. Fabrazyme get s into breast milk. Discuss with your doctor the risks and benefits of breastfeeding versus continuing Fabrazyme therapy.
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.
Fabrazyme contains sodium
This medicine contains less than 1 mmol sod ium (23 mg) per vial, that is to say essentially ‘sodium – free’.
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 reaction s”). Severe life -threatening allergic reactions (“anaphylactoid 28 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 peop le) 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 heartbeat • 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 or throat • decreased blood pressure • hot flush
- oedema in extremities • chest discomfort • feeling hot
- vertigo • face oedema • hyperthermia
- stomach discomfort • exacerbated difficulty in breathing • decreased mouth sensitivity
- 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 discolo uration
- itchy rash • skin discomfort • coldness of the extremities
- feeling hot and cold • musculoskeletal pain • injection site blood clotting
- difficulty swallowing • rhinitis • skin discolo uration
- 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 di rectly via the national reporting system 29 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 refer s to the last day of that month.
Unopened vials Store in a refrigerator (2 C – 8C).
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 – 8C.
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. After reconstitution each vial contains 5 mg of agalsidase beta per ml.
- The other ingredients are:
- Mannitol (E421)
- Sodium dihydrogen phosphate monohydrate (E339)
- Disodium phosphate heptahydrate (E339) .
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
Sanofi B.V., Paasheuvelweg 25, 1105 BP Amsterdam , The Netherlands
Manufacturer
Genzyme Ireland L imited , IDA Industrial Park, Old Kilmeaden Road, Waterford, Ireland 30 For any information about this medicine, please contact the local representative of the Marketing Authorisation Holder:
België/Belgique/Belgie n/ Luxembourg/Luxemburg Sanofi Belgium Tél/Tel: + 32 2 710 54 00 Lietuva Swixx Biopharma UAB Tel: +370 5 236 91 40 България Swixx Biopharma EOOD Тел.: +359 (0)2 4942 480 Magyarország SANOFI -AVENTIS Zrt.
Tel: +36 1 505 0050 Česká republika Sanofi s.r.o.
Tel: +420 233 086 111 Malta Sanofi S.r.l.
Tel: + 39 02 39394275 Danmark Sanofi A/S Tlf: +45 45 16 70 00 Nederland Sanofi B.V.
Tel: +31 20 245 4000 Deutschland Sanofi -Aventis Deutschland GmbH Tel.: 0800 04 36 996 Tel. aus dem Ausland: +49 69 305 70 13 Norge sanofi -aventis Norge AS Tlf: + 47 67 10 71 00 Eesti Swixx Biopharma OÜ Tel: +372 640 10 30 Österreich sanofi -aventis GmbH Tel: + 43 1 80 185 – 0 Ελλάδα Sanofi -Aventis Μονοπρόσωπη AEBE Τηλ: +30 210 900 1600 Polska Sanofi Sp. z o.o.
Tel.: +48 22 280 00 00 España sanofi -aventis, S.A.
Tel: +34 93 485 94 00 Portugal Sanofi – Produtos Farmacêuticos, Lda.
Tel: +351 21 35 89 400 France Sanofi Winthrop Industrie Tél: 0 800 222 555 Appel depuis l’étranger: +33 1 57 63 23 23 România Sanofi Romania SRL Tel: +40 (0) 21 317 31 36 Hrvatska Swixx Biopharma d.o.o.
Tel: +385 1 2078 500 Slovenija Swixx Biopharma d.o.o.
Tel: +386 1 235 51 00 Ireland sanofi -aventis Ireland Ltd. T/A SANOFI Tel: +353 (0) 1 403 56 00 Slovenská republika Swixx Biopharma s.r.o.
Tel: +421 2 208 33 600 Ísland Vistor hf.
Sími: +354 535 7000 Suomi/Finland Sanofi Oy Puh/Tel: + 358 201 200 300 Italia Sanofi S.r.l.
Tel: 800536389 Sverige Sanofi AB Tel: +46 (0)8 634 50 00 31 Κύπρος C.A. Papaellinas Ltd.
Τηλ: +357 22 741741 United Kingdom (Northern Ireland) sanofi -aventis Ireland Ltd. T/A SANOFI Tel: +44 (0) 800 035 2525 Latvija Swixx Biopharma SIA Tel: +371 6 616 47 50
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 solution for injection and t hen 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. The number of vials should be determined to be reconstituted based on the individual patient’s weight and the required vials should be removed 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 o nly.
Reconstitution 2. Each vial of Fabrazyme 35 mg has to be reconstituted with 7.2 ml water for injections . Forceful impact of the water for injections on the powder and foaming should be avoided . This is done by slow drop -wise addition of the water for injection down the inside of the vial and not directly onto the lyophilisate. Each vial should be rolled and tilted gently. The vial should not be invert ed, swirl ed or shake n.
3. The reconstituted s olution 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, the reconstituted solution in each vial should be visually inspected for particulat e matter and discolo uration. The solution should not be used 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.
32 Dilution 6. Prior to adding the reconstituted volume of Fabrazyme required for the patient dose, i t is recommended to remove an equal volume of 0.9% sodium chloride solution for injection , from the infusion bag.
7. The airspace within the infusion bag should be removed to minimi se the air/liquid interface.
8. 7.0 ml (equal to 35 mg) of the reconstituted solution from each vial up to the total volume required should be slowly withdrawn for the patient dose . Filter needles should not be used and foaming should be avoided .
9. The reconstituted solution should slowly be injected directly into the 0.9% sodium chloride solution for injection (not in any remaining airspace) to a final concentration between 0.05 mg/ml and 0.7 mg/ml. The total volume of sodium chloride 0.9% solution for infusion (between 50 and 500 ml) should be determined based on the individual dose. For doses lower than 35 mg a minimum of 50 ml should be used , for doses 35 to 70 mg a minimum of 100 ml should be used , for doses 70 to 100 mg a minimum of 250 ml should be used and for doses greater than 100 mg only 500 ml should be used . The infusion bag should be gently inverted or lightly massaged to mix the diluted solution. The infusion bag should not be shake n or excessively agitate d.
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 IV infusion rate should be no more than 0.25 mg/min (15 mg/hour). The infusion ra te may be slowed in the event of infusion -associated reactions.
After patient tolerance is well established, the infusion rate may be increased in increments of 0.05 to 0.08 3 mg/min (increments of 3 to 5 mg/hr) with each subsequent infusion. In clinical trials with classic patients , the infusion rate was increased incrementally to reach a minimum of 2 hours. This was achieved after 8 initial infusions at 0.25 mg/min (15 mg/hr), without any IARs, change in infusion rate, o r infusion interruption. A further decrease of infusion time to 1.5 hours was allowed for patients without new IARs during the last 10 infusions or reported serious adverse events within the last 5 infusions. Each rate increment of 0.083 mg/min (~5 mg/hr) was maintained for 3 consecutive infusions, without any new IARs, change in infusion rate, or infusion interruption, before subsequent rate increases.
For patients weighing < 30 kg, the maximum infusion rate should remain at 0.25 mg/min (15 mg/hr).
33 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 you rs.
- 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.
ℹ️ 1. What Fabrazyme is and what it is used for
Fabrazyme contains the active substance agalsidase beta and is used as enzyme replacemen t 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 adolesce nts 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 Fabrazy me in children aged 5 to 7 years have not yet been established and therefore no dose can be recommended for this age group.
34 Other medicines and Fabrazyme Tell your doctor or pharmacist if you are taking, have recently taken or might take any other medicin es.
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 There is limited experience with the use of Fabrazyme in pregnant women. As a precaution, it is preferable to avoid the use of Fabrazyme during pregnancy. Fabrazyme get s into breast milk. Discuss with your doctor the risks and benefits of breastfeeding versus continuing Fabrazyme therapy. 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, a sk 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.
Fabrazyme contains sodium
This medicine contains less than 1 mmol sodium (23 mg) per vial, that is to say essentially ‘sodium – free’.
💊 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 supervi sion 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 35 reactions”) have been reported in some patients . If yo u 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 o ther side effects:
Common (may affect up to 1 in 10 people)
- chest pain • sleepiness • fatigue
- difficulty in breathing • increased heartbeat • 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 or throat • decreased blood pressure • hot flush
- oedema in extremities • chest discomfort • feeling hot
- vertigo • face oedema • hyperthermia
- stomach discomfort • exacerbated difficulty in breathing • decreased mouth sensitivity
- 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 discolo uration
- itchy rash • skin discomfort • coldness of the extremities
- feeling hot and cold • musculoskeletal pain • injection site blood clotting
- difficulty swallowing • rhinitis • skin discolo uration
- 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 36 listed in Appendix V . By reporting si de 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 – 8C).
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 – 8C.
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. After reconstitution each vial contains 5 mg agalsidase beta per ml.
- The other ingredients are:
- Mannitol (E421)
- Sodium dihydrogen phosphate monohydrate (E339)
- Disodium phosphate heptahydrate (E339) .
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
Sanofi B.V., Paasheuvelweg 25, 1105 BP Am sterdam , The Netherlands
Manufacturer
Genzyme Ireland L imited , IDA Industrial Park, Old Kilmeaden Road, Waterford, Ireland 37 For any information about this medicine, please contact the local representative of the Marketing Authorisation Holder:
België/Belgique/Belgien/ Luxembourg/Luxemburg Sanofi Belgium Tél/Tel: + 32 2 710 54 00 Lietuva Swixx Biopharma UAB Tel: +370 5 236 91 40 България Swixx Biopharma EOOD Тел.: +359 (0)2 4942 480 Magyarország SANOFI -AVENTIS Zrt.
Tel: +36 1 505 0050 Česká republika Sanofi s.r.o.
Tel: +420 233 086 111 Malta Sanofi S.r.l.
Tel: + 39 02 39394275 Danmark Sanofi A/S Tlf: +45 45 16 70 00 Nederland Sanofi B.V.
Tel: +31 20 245 4000 Deutschland Sanofi -Aventis Deutschland GmbH Tel.: 0800 04 36 996 Tel. aus dem Ausland: +49 69 305 70 13 Norge sanofi -aventis Norge AS Tlf: + 47 67 10 71 00 Eesti Swixx Biopharma OÜ Tel: +372 640 10 30 Österreich sanofi -aventis GmbH Tel: + 43 1 80 185 – 0 Ελλάδα Sanofi -Aventis Μονοπρόσωπη AEBE Τηλ: +30 210 900 1600 Polska Sanofi Sp. z o.o.
Tel.: +48 22 280 00 00 España sanofi -aventis, S.A.
Tel: +34 93 485 94 00 Portugal Sanofi – Produtos Farmacêuticos, Lda.
Tel: +351 21 35 89 400 France Sanofi Winthrop Industrie Tél: 0 800 222 555 Appel depuis l’étranger: +33 1 57 63 23 23 România Sanofi Romania SRL Tel: +40 (0) 21 317 31 36 Hrvatska Swixx Biopharma d.o.o.
Tel: +385 1 2078 500 Slovenija Swixx Biopharma d.o.o.
Tel: +386 1 235 51 00 Ireland sanofi -aventis Ireland Ltd. T/A SANOFI Tel: +353 (0) 1 403 56 00 Slovenská republika Swixx Biopharma s.r.o.
Tel: +421 2 208 33 600 Ísland Vistor hf.
Sími: +354 535 7000 Suomi/Finland Sanofi Oy Puh/Tel: + 358 201 200 300 Italia Sanofi S.r.l.
Tel: 800536389 Sverige Sanofi AB Tel: +46 (0)8 634 50 00 38 Κύπρος C.A. Papaellinas Ltd.
Τηλ: +357 22 741741 United Kingdom (Northern Ireland) sanofi -aventis Ireland Ltd. T/A SANOFI Tel: +44 (0) 800 035 2525 Latvija Swixx Biopharma SIA Tel: +371 6 616 47 50
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. —————————————————————————————————————————
🩺 Information intended for healthcare professionals only
Instructions for use – reconstitution, dilution and admin istration The powder for concentrate for solution for infusion has to be reconstituted with water for injections, diluted with 0.9% sodium chloride solution for injection and then administered by intravenous infusion.
From a microbiological point of vie w, 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 hel d for up to 24 hours at 2ºC -8ºC.
Use aseptic technique 1. The number of vials should be determined to be reconstituted based on the individual patient’s weight and the required vials should be removed from the refrigerator in order to allow them to reach room temp erature (in approximately 30 minutes) . Each vial of Fabrazyme is intended for single use only.
Reconstitution 2. Each vial of Fabrazyme 5 mg has to be reconstituted with 1.1 ml water for injections . Forceful impact of the water for injections on the powder and foaming should be avoided . This is done by slow drop -wise addition of the water for injection down the inside of the vial and not directly onto the lyophili sate. Each vial should be rolled and tilted gently. The vial should not be inverted, swir led or shaken.
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, the reconstituted solution in each vial should be visually inspe cted for particulate matter and discolo uration. The solution should not be used 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 formatio n over time.
5. Any unused medicinal product or waste material should be disposed of in accordance with local requirements.
39 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 solution for injection , from the infusion bag.
7. The airspace within the infusion bag should be removed to minimi se the air/liquid interface.
8. 1.0 ml (equal to 5 mg) of the reconstituted solution from each vial up to the tota l volume required should be slowly withdrawn for the patient dose. Filter needles should not be used and foaming should be avoided .
9. The reconstituted solution should slowly be injected directly into the 0.9% sodium chloride solution for injection (not in any remaining airspace) to a final concentration between 0.05 mg/ml and 0.7 mg/ml. The total volume of sodium chloride 0.9% solution for infusion (between 50 and 500 ml) should be determined based on the individual dose. For doses lower than 35 mg a minimum of 50 ml should be used , for doses 35 to 70 mg a minimum of 100 ml should be used , for doses 70 to 100 mg a minimum of 250 ml should be used and for doses greater than 100 mg only 500 ml should be used . The infusion bag should be g ently inverted or lightly massaged to mix the diluted solution. The infusion bag should not be shaken or excessively agitated.
Administration 10. It is recommended to administer the diluted solution through an in -line low protein -binding 0.2 µm filter to rem ove any protein particles which will not lead to any loss of agalsidase beta activity. The initial IV infusion rate should be no more than 0.25 mg/min (15 mg/hour) . The infusion rate may be slowed in the event of infusion -associated reactions.
After patient tolerance is well established, the infusion rate may be increased in increments of 0.05 to 0.08 3 mg/min (increments of 3 to 5 mg/hr) with each subsequent infusion. In clinical trials with classic patients , the infusion rate was increased incrementa lly to reach a minimum of 2 hours. This was achieved after 8 initial infusions at 0.25 mg/min (15 mg/hr), without any IARs, change in infusion rate, or infusion interruption. A further decrease of infusion time to 1.5 hours was allowed for patients without new IARs during the last 10 infusions or reported serious adverse events within the last 5 infusions. Each rate increment of 0.083 mg/min (~5 mg/hr) was maintained for 3 consecutive infusions, without any new IARs, change in infusion rate, or infusion int erruption, before subsequent rate increases.
For patients weighing < 30 kg, the maximum infusion rate should remain at 0.25 mg/min (15 mg/hr).
📄 Package Leaflet – official source
This article reproduces the information in the official package leaflet, as authorised in the European Union. You can consult the original document here: Product information Fabrazyme (PDF, ec.europa.eu).
You can check this medicine at any time in the Union Register of medicinal products (EU number EU/1/01/188).
⚠️ Important: This article is for information purposes only and does not replace medical advice or the package leaflet inside the medicine box. Read the leaflet carefully before use. For any questions about your treatment, talk to your doctor or pharmacist. If you experience side effects, tell your doctor or pharmacist and report them through your national reporting system.