• Keep this leaflet. You may need to read it again.
• If you have any further questions, ask your doctor, pharmacist or nurse.
• If you get any side effects, talk to your doctor, pharmacist or nurse. This includes any possible side effects not listed in this leaflet. See section 4

1. What Myozyme is and what it is used for
2. What you need to know before you are given Myozyme
3. How Myozyme is given
4. Possible side effects
5. How to store Myozyme
6. Contents of the pack and other information

If you have experienced life-threatening allergic (hypersensitive) reactions to alglucosidase alfa or any of the other ingredients of this medicine (listed in section 6) and re-administration of the medicine was not successful.

Symptoms of life-threatening allergic reactions include, but are not limited to, low blood pressure, very fast heart rate, difficulty breathing, vomiting, facial swelling, hives or rash.

If you are treated with Myozyme, you may experience an infusion-associated reaction while you are being given the medicine or during the hours following the infusion.

Such a reaction comprises different symptoms like low blood pressure, chest discomfort, throat tightness, face, lips or tongue swelling (angioedema), hives (urticaria), dizziness, rash, itchy skin, nausea, vomiting, cough and bronchospasm (see section 4 for an overview of all infusion-associated reactions). An infusion-associated reaction can sometimes be very severe. If you experience a reaction like this, you should tell your doctor immediately. You may need to be given pre-treatment medicines to prevent an allergic reaction (e.g. antihistamines and/or corticosteroids) or to reduce fever (antipyretics).

In studies doctors have used medicines to suppress the immune system to reduce the production of antibodies. Because you have Pompe disease, there is a risk that you get a severe infection of your airways or lungs. Using these medicines to suppress the immune system may further increase this risk.

If you experience severe ulcerative lesions of your skin, please inform your doctor. If you experience swelling of your lower limbs or generalized swelling, please inform your doctor. Your doctor should consider discontinuation of the administration of Myozyme and initiate appropriate medical treatment. Your doctor should consider the risks and benefits of re-administering Myozyme.

Tell your doctor or pharmacist if you are using, have recently used or might use any other medicines.

There is limited experience of the use of Myozyme in pregnant women. You should not be using Myozyme during pregnancy unless clearly necessary. Tell your doctor if you are breastfeeding. There is limited experience suggesting that Myozyme passes into human breast milk in very small amounts. No effects on the breastfeed infant is expected. Therefore breastfeeding during the treatment may be considered. However, you can discuss with your doctor whether to interrupt breastfeeding as a precautionary measure for the first 24 hours after each dose of Myozyme. 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.

Take care when driving or using any tools or machines shortly after infusion of Myozyme, since you may experience dizziness, sleepiness, shaking, and/or low blood pressure.

This medicine contains less than 1 mmol sodium (23 mg) per vial, i.e. essentially ‘sodium free’.

The recommended dosage of Myozyme in children and adolescents is the same as in adults.

Myozyme 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.

If you are given Myozyme at a higher dose or infusion rate than recommended, you may experience infusion associated reactions. Such a reaction may include symptoms like:

• skin and lips turning blue because of a lack of oxygen in body tissues, increased heart rate, palpitations
• difficulty in breathing, cough
• dizziness, headache, taste disturbance
• high blood pressure, hot flush
• tongue swelling, vomiting, diarrhoea, feeling sick (nausea)
• chest pain, chest discomfort, throat tightness, fever, chills, feeling cold, redness at the infusion site
• muscle pain
• reddening of the skin

If you experience a reaction like this, you should tell your doctor immediately (see section 2). The rate of your infusion will be reduced or the infusion will be interrupted, and, as appropriate, you may receive a corrective treatment.

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

If you get any side effects, talk to your doctor, pharmacist or nurse. This includes any possible side effects not listed in this leaflet. You can also report side effects directly via the Yellow Card Scheme at: www.mhra.gov.uk/yellowcard or search for MHRA Yellow Card in the Google Play or Apple App Store.

By reporting side effects you can help provide more information on the safety of this medicine

• 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
  • mannitol (E421),
  • sodium dihydrogen phosphate monohydrate (E339)
  • disodium phosphate heptahydrate (E339)
  • polysorbate 80 (E433).

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.

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