What Fabrazyme contains
- The active substance is agalsidase beta, one vial contains 5 mg.
- 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, NL-1411DD Naarden, The Netherlands.
Manufacturer
Genzyme Ltd., 37 Hollands Road, Haverhill, Suffolk CB9 8PU, United Kingdom.
For any information about this medicinal product, please contact the local representative of the Marketing Authorisation Holder:
ItaliaMalta Genzyme Srl ItaliaItalja, Tel 39 059 349811 BelgiëBelgiqueBelgien LuxemburgLuxembourg Genzyme Belgium N.V., TelTél 32 2 714 17 11
Magyarország Genzyme Europe B.V. Képviselet Tel 36 1 310 7440 Genzyme CEE GmbH . 359 2 971 1001
Nederland Genzyme Europe B.V., Tel 31 35 6991200 eská RepublikaSlovenská Republika Slovenija Genzyme Czech s.r.o., Tel 420 227 133 665
Österreich Genzyme Austria GmbH, Tel 43 1 774 65 38 DanmarkNorgeSverigeSuomiFinland Ísland Genzyme AS, DanmarkTanskaDanmörk, TlfPuh.Sími 45 32712600
Deutschland Genzyme GmbH, Tel 49 610236740 PolskaEestiLatvijaLietuva Genzyme Polska Sp. z o. o. PoolaPolijaLenkija, Tel 48 22 24 60 900
Genzyme Hellas Ltd. 30 210 99 49 270 Portugal Genzyme Portugal, S.A., Tel 351 21 422 0100
España Genzyme, S.L., Tel 34 91 6591670 România Genzyme CEE GmbH- Reprezentana pentru România Tel 40 21 243 42 28
France Genzyme S.A.S, Tél 33 0 825 825 863 United KingdomIreland Genzyme Therapeutics Ltd. United Kingdom, Tel 44 1865 405200
This leaflet was last approved in
Detailed information on this medicine is available on the European Medicines Agency (EMEA) web site: http://www.emea.europa.eu. There are also links to other websites about rare diseases and treatments.
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The following information is intended for medical or 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 -8º C.
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 minimize protein particle formation over time.
5. Any unused 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 intravenoussolution (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.
