What Myozyme contains
- The active substance is alglucosidase alfa. One vial contains 50 mg of alglucosidase alfa. After reconstitution, the solution contains 5 mg of alglucosidase alfa per ml and after dilution, the concentration varies from 0.5 mg to 4 mg/ml.
- The other ingredients are
- sodium dihydrogen phosphate monohydrate
- disodium phosphate heptahydrate
- polysorbate 80
What Myozyme looks like and contents of the pack
Myozyme is a powder for concentrate for solution for infusion in a vial (50 mg/vial). Each pack contains 1, 10 or 25 vials. Not all pack sizes may be marketed.
The powder is white to off-white. After reconstitution it is a clear, colourless to pale yellow solution, which may contain particles. The reconstituted solution must be further diluted.
Marketing Authorisation Holder and Manufacturer
Marketing Authorisation Holder
Genzyme Europe B.V., Gooimeer 10, 1411 DD, Naarden, The Netherlands
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:
ItaliaMalta Genzyme Srl ItaliaItalja, Tel 39 059 349811 BelgiëBelgiqueBelgien LuxemburgLuxembourg Genzyme Belgium N.V., Tel Tél 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 35 699 1200 eská RepublikaSlovenská Republika Slovenija Genzyme Czech s.r.o., Tel 420 221 722 511
Österreich Genzyme Austria GmbH, Tel 43 1 774 65 38 DanmarkNorgeSverigeSuomiFinland Ísland Genzyme AS, DanmarkTanskaDanmörk, TlfPuh.Sími 45 32 71 2600
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.U., Tel 34 91 6591670 România Genzyme CEE GmbH- Reprezentana pentru România, Tel 188.8.131.52.28
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
<----------------------------------------------------------------------------------------------------------------------------- The following information is intended for medical or healthcare professionals only:
Instructions for use ? reconstitution, dilution and administration
Myozyme has to be reconstituted with water for injections, then diluted with sodium chloride 9 mg/ml (0.9%) solution for injection and then administered by intravenous infusion. Reconstitution and dilution should be performed in accordance with good practice rules, particularly for the respect of asepsis.
Due to the proteinaceous nature of the product, particle formation may occur in the reconstituted solution and final infusion bags. Therefore, a 0.2 micron low protein binding in-line filter should be used for administration. It was demonstrated that the use of a 0.2 micron in-line filter removes visible particles and does not result in an apparent loss of protein or activity.
Determine the number of vials to be reconstituted based on the individual patient?s dose regimen (mg/kg) and remove the required vials from the refrigerator in order to allow them to reach room temperature (approximately 30 minutes). Each vial of Myozyme is for single use only.
Use aseptic technique
Reconstitute each 50 mg vial of Myozyme with 10.3 ml water for injections using a syringe with a needle diameter not larger than 20 gauge. Add the water for injections by slow drop-wise addition down the side of the vial and not directly onto the lyophilised cake. Tilt and roll each vial gently. Do not invert, swirl or shake the vial. The reconstituted volume is 10.5 ml containing 5 mg enzyme/ml, and appears as a clear, colourless to pale yellow solution which may contain particles in the form of thin white strands or translucent fibres. Perform an immediate inspection of the reconstituted vials for particulate matter and discoloration. If upon immediate inspection foreign particles other than those described above are observed, or if the solution is discoloured, do not use. The pH of the reconstituted solution is approximately 6.2.
After reconstitution it is recommended to promptly dilute the vials (see below).
When reconstituted as above, the reconstituted solution in the vial contains 5 mg alglucosidase alfa per ml. The reconstituted volume allows accurate withdrawal of 10.0 ml (equal to 50 mg) from each vial. This should then be further diluted as follows: Slowly withdraw the reconstituted solution from each vial until the volume for the patient?s dose is obtained using a syringe with a needle diameter not larger than 20 gauge. The recommended final concentration of alglucosidase in the infusion bags ranges from 0.5 mg/ml to 4 mg/ml. Remove airspace within the infusion bag. Also remove an equal volume of sodium chloride 9 mg/ml (0.9%) solution for injection, that will be replaced with reconstituted Myozyme. Slowly inject the reconstituted Myozyme directly into the sodium chloride 9 mg/ml (0.9%) solution for injection. Gently invert or massage the infusion bag to mix the diluted solution. Do not shake or excessively agitate the infusion bag.
The final infusion solution should be administered as close to preparation time as possible.
Any unused product or waste material should be disposed of in accordance with local requirements.
It is recommended to start the administration of the diluted solution within three hours. The total time between reconstitution and completion of the infusion should not exceed 24 hours.
The recommended dose regimen of Myozyme is 20 mg/kg of body weight administered once every 2 weeks as an intravenous infusion.
Infusions should be administered incrementally. It is recommended that the infusion begin at an initial rate of 1 mg/kg/h and be gradually increased by 2 mg/kg/h every 30 minutes if there are no signs of IARs (Infusion Associated Reactions) until a maximum rate of 7 mg/kg/h is reached.