Details for: MYOZYME
Company: SANOFI GENZYME, A DIVISION OF SANOFI-AVENTIS CANADA INC
| DIN | DIN name | Active Ingredient(s) | Strength | Dosage Form | Route of Administration |
|---|---|---|---|---|---|
| 02284863 | MYOZYME | ALGLUCOSIDASE ALFA | 50 MG / VIAL | POWDER FOR SOLUTION | INTRAVENOUS |
Summary Reports
Consumer Information
This information was provided by the drug’s manufacturer when this drug product was approved for sale in Canada. It is designed for consumers and care givers. It is a summary of information about the drug and will not tell you everything about the drug. Contact your doctor or pharmacist if you have any questions about the drug.
What the medication is used for
- MYOZYME® is a medicine used for patients with Pompe’s Disease (GAA deficiency). MYOZYME® is used to treat adults, children and adolescents of all ages who have a confirmed diagnosis of Pompe disease.
What it does
People with Pompe’s Disease have low levels of an enzyme called alpha-glucosidase (GAA). This enzyme helps the body control levels of glycogen (a type of carbohydrate). Glycogen provides the body with energy, but in Pompe’s Disease the levels can get too high. Glycogen accumulation in Pompe’s Disease occurs in various tissues, particularly cardiac, respiratory and skeletal muscle, leading to the development of cardiomyopathy and progressive muscle weakness, including impairment of respiratory function.
MYOZYME® is an artificial enzyme called alglucosidase alfa – this can replace the natural enzyme which is lacking in Pompe disease.
It is postulated that MYOZYME® will restore lysosomal GAA activity resulting in stabilization or restoration of cardiac and skeletal muscle function (including respiratory muscles). Due to the blood-brain barrier effect and the enzyme’s size, uptake of alglucosidase alfa in the central nervous system is unlikely.
When it should not be used
- You have any allergies to this drug or its ingredients or components of the container
What the medicinal ingredient is
alglucosidase alfa
What the non-medicinal ingredients are
Mannitol, Polysorbate 80, Sodium phosphate dibasic heptahydrate, Sodium phosphate monobasic monohydrate
What dosage form it comes in
Sterile lyophilized powder for reconstitution to be used as intravenous infusion, 50 mg
Warnings and precautions
Serious Warnings and Precautions
Do not use MYOZYME® if you are severely allergic to alglucosidase alfa or any other ingredient of MYOZYME®.
If you are treated with MYOZYME® you may experience an infusion-associated reaction. An infusion-associated reaction is defined as any related side effect occurring during the infusion or during the 2 hours following infusion. Life-threatening allergic reactions, including anaphylactic shock, have been observed in patients during MYOZYME® infusion. Because of the potential for severe infusion reactions, appropriate medical support should be readily available when MYOZYME® is administered.
Individuals with an acute underlying illness [e.g fever, pneumonia or sepsis (severe infection), wheezing/difficulty in breathing, heart failure] at the time of MYOZYME® infusion appear to be at greater risk for infusion reactions. Careful consideration should be given to your clinical status prior to administration of MYOZYME®.
Precaution must be observed when administering general anesthesia to individuals with infantile-onset Pompe’s Disease. Reports of intraoperative cardiac arrest following anesthesia induction for invasive procedures have been reported, some of which were fatal. The presence of severe hypertrophic cardiomyopathy in infantile-onset Pompe’s Disease may increase the risk of general anesthesia complications.
Infantile onset Pompe patients with heart or breathing problems may be at risk for increasing the seriousness of these problems as a result of MYOZYME® administration, and may require additional monitoring.
To help avoid side effects and ensure proper use, talk to your healthcare professional before you take MYOZYME®. Talk about any health conditions or problems you may have, including if you:
- Have an acute underlying illness
- Need general anaesthesia for central venous catheter placement
- Have had a severe hypersensitivity or anaphylactic reaction to administration of MYOZYME®
- Have experienced infusion-associated reactions
- Are at increased risk of lung infections due to the progressive effects of the disease on the lung muscles
- Have underlying heart enlargement
- Are pregnant or plan to become pregnant or are breast feeding
- Are above the age of 65
Interactions with this medication
Tell your healthcare professional about all the medicines you take, including any drugs, vitamins, minerals, natural supplements or alternative medicines.
The following may interact with MYOZYME®:
No formal interaction studies have been conducted. Please inform your doctor if you are using any other medicinal products, due to the potential risk of interference with the uptake of alglucosidase alfa.
Proper use of this medication
MYOZYME® will be given to you under the supervision of a doctor who is knowledgeable in the treatment of Pompe’s Disease.
The dose you receive is based on your body weight. MYOZYME® should be administered 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 infusion associated reactions (IARs) until a maximum rate of 7 mg/kg/h is reached.
Usual dose
The recommended dosage regimen of MYOZYME® is 20 mg/kg body weight administered every 2 weeks as an intravenous infusion.
Overdose
There is no experience with overdoses of MYOZYME® for doses up to 40 mg/kg of body weight.
Missed Dose
If you have missed a MYOZYME® infusion, please contact your doctor. It is important to have your infusion on a regular basis to avoid the accumulation of GAA. The total dose administered each month should remain substantially unchanged.
Side effects and what to do about them
These are not all the possible side effects you may feel when taking MYOZYME®. If you experience any side effects not listed here, contact your healthcare professional. Please also see Warnings and Precautions.
Side effects were mainly seen while patients were being given the medicine or shortly after (“infusion related effects”). Some of these infusion related side effects became serious. Should you experience any reaction like this, please tell your doctor immediately. Regardless of pre-treatment, your infusion may need to be slowed or stopped and you may need to be given additional medicines to treat an allergic reaction.
The most significant infusion reactions included allergic reactions and allergic shock to MYOZYME®. Other serious infusion reactions included hives, abnormal breathing sounds, elevated heart rate, difficulty in breathing, elevated respiration, swelling around the eyes, high blood pressure, decreased oxygen concentration in blood and fever, heart attack, chest pain, abdominal pain, low blood pressure, shortness of breath.
Some patients have experienced infusion related side effects in the form of flu-like symptoms or a combination of events such as fever, chills, muscle pain, joint pain, pain or fatigue, which lasted for a few days after completion of the infusion.
In addition, patients also experienced the following non-serious events:
cough, infusion site reaction including pain and bruising, feeling unwell, itching, diarrhea, nausea, vomiting, dry heaves, constipation, stomach bloating, indigestion, inability to sleep, agitation, irritability, restlessness, tremor, headache, dizziness, tingling sensation, lack of energy, feeling sleepy, ringing in the ears and blood in the urine.
| Symptom / effect | Talk with your doctor or pharmacist | ||
|---|---|---|---|
| Common (occurred in ≥ 5% of patients) | |||
| fever, decreased oxygen concentration in blood, hives, flushing, elevated heart rate, rash, shivering, low blood pressure, high blood pressure, cough, elevated respiration, agitation, irritability, vomiting, chest discomfort | ✔ | ||
| Uncommon (occurred in <5% of patients) | |||
| increased sweating, mottling, itching, rash, fever, pallor, cyanosis, restlessness, retching, tremor, chest pain, throat tightness, tongue swelling | ✔ | ||
If you have a troublesome symptom or side effect that is not listed here or becomes bad enough to interfere with your daily activities, talk to your healthcare professional.
If you opt for the infusion of MYOZYME® through central catheter, discuss with your doctor potential complications related to use of such a delivery system.
How to store
Store MYOZYME® in a refrigerator between 2°-8°C. DO NOT FREEZE OR SHAKE. DO NOT USE MYOZYME® after the expiration date on the vial.
It is recommended that MYOZYME® is used immediately after it has been mixed with sterile water. However it can be kept for up to 24 hours if it is kept cool (2°C – 8°C) and in the dark.
Keep out of reach and sight of children.
Reporting side effects
You can help improve the safe use of health products for Canadians by reporting serious and unexpected side effects to Health Canada. Your report may help to identify new side effects and change the product safety information.
3 ways to report:
- Online at MedEffect;
- By calling 1-866-234-2345 (toll-free);
- By completing a Consumer Side Effect Reporting Form and sending it by:
- Fax to 1-866-678-6789 (toll-free), or
- Mail to: Canada Vigilance Program
Health Canada, Postal Locator 0701E
Ottawa, ON
K1A 0K9
NOTE: Contact your health professional if you need information about how to manage your side effects. The Canada Vigilance Program does not provide medical advice.
More information
Pompe Registry:
Sanofi Genzyme informs all patients with Pompe’s Disease that a registry has been established in order to better understand the variability and progression of Pompe’s Disease and to continue to monitor and evaluate the safety and efficacy of MYOZYME® treatments. All patients are encouraged to participate and advised that their participation may involve long-term follow-up. Information regarding the registry program may be found at
www.pomperegistry.com or by calling 1-800-745-4447.
If you want more information about MYOZYME®:
- Talk to your healthcare professional
- Find the full product monograph that is prepared for healthcare professionals and includes this Patient Medication Information by visiting the Health Canada website; the manufacturer’s website, or by calling 1-877-220-8918.
This leaflet was prepared by Sanofi Genzyme, a division of sanofi-aventis Canada Inc.
Last Revised: Dec-16-2016