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MYOZYME is a brand name for Alglucosidase Alfa, supplied as a powder for solution. The medicine, its uses, side effects and dosage are the same regardless of brand.
Used for: MYOZYME (alglucosidase alfa for injection) is indicated for: • use in patients with Pompe’s Disease (acid alpha-glucosidase [GAA] deficiency). 1.1 Pediatrics Pediatrics (<18 years of age): Based on the data submitted and reviewed by Health Canada, the safety and efficacy of Myozyme in pediatric patients has been…
Verbatim from this product's HC label. Tap a section to expand.
1 Dosing Considerations Myozyme (alglucosidase alfa for injection) is intended for long-term, chronic use under the guidance and supervision of a physician. Myozyme should be reconstituted, diluted and administered by a health care professional in a hospital or in an appropriate setting of outpatient care.
Because of the potential for fluid overload, appropriate medical support measures should be readily available when Myozyme is administered. (see 7 WARNINGS AND PRECAUTIONS, Cardiovascular) Myozyme (alglucosidase alfa for injection) Page 5 of 46 Protected B / Protégé B Because of the potential for significant hypersensitivity reactions, appropriate medical support measures, including cardiopulmonary resuscitation equipment, especially for patients with cardiac hypertrophy and patients with significantly compromised respiratory function, should be readily available when Myozyme is administered.
2 Recommended Dose and Dosage Adjustment The recommended dosage regimen of Myozyme is 20 mg/kg body weight administered every 2 weeks as an intravenous infusion. There was no additional clinical benefit with doses of Myozyme higher than 20 mg/kg of body weight in clinical trials.
3 Reconstitution Vials of Myozyme are stored under refrigerated conditions (at 2-8 °C). As Myozyme does not contain a preservative, strict aseptic techniques are to be used in the preparation of a patient’s dose. Once reconstituted, vials of Myozyme are to be used immediately for dilution into an infusion bag.
Diluted Myozyme into an infusion bag is also to be used immediately (within 3 hours). The reconstituted and diluted infusion solution should be protected from light. Any unused product should be discarded. Use aseptic technique during preparation.
Do not use filter needles during preparation. 1. Determine the number of vials to be reconstituted based on the individual patient’s weight and the recommended dose of 20 mg/kg. Round up to the nearest whole vial. Remove the required number of vials from the refrigerator and allow them to reach room temperature prior to reconstitution (approximately 30 minutes).
Patient weight (kg) x Dose (mg/kg) = Patient Dose (in mg) Patient dose (in mg) ÷ 50 mg/vial = number of vials to reconstitute. If the number of vials includes a fraction, round up to the next whole number. 4 vials; therefore, 7 vials should be reconstituted 2.
). Infusion reactions which were reported in ≥ 5% of Myozyme -treated late-onset patients included headache, nausea, urticaria, dizziness, chest discomfort, hyperhidrosis, flushing, blood pressure increased, and vomiting. Serious infusion-associated reactions included rales, bronchospasm, oxygen saturation decreased, tachypnea, tachycardia, periorbital edema, urticaria, hypertension, heart rate increased and fever.
Most infusion-associated reactions requiring intervention were ameliorated with slowing the infusion rate, temporarily stopping the infusion and/or administration of antipyretics, antihistamines or corticosteroids. All patients recovered without sequelae from the reactions.
) at the time of Myozyme infusion appear to be at greater risk for infusion-associated reactions. Careful consideration should be given to the patient’s clinical status prior to administration of Myozyme (see 7 WARNINGS AND PRECAUTIONS).
Infusion reactions are also more likely to occur with higher infusion rates. Patients with advanced Pompe’s Disease may have compromised cardiac and respiratory function, which may also predispose them to a higher risk of severe complications from infusion reactions.
In clinical trials, some patients were pre-treated with antihistamines, antipyretics and/or Myozyme (alglucosidase alfa for injection) Page 11 of 46 Protected B / Protégé B corticosteroids. Infusion reactions occurred in some patients after receiving antipyretics, antihistamines and/or corticosteroids.
Patients should be closely monitored during the Myozyme infusion. Infusion reactions may occur at any time during, or shortly after completion of Myozyme infusion. If the patient experiences an infusion reaction during the Myozyme infusion, the patient should be managed according to general standards of care consistent with treatment of the presenting symptom(s).
2 BREAST-FEEDING 11/2022 7 WARNINGS AND PRECAUTIONS, Immunogenicity 7/2024 TABLE OF CONTENTS Sections or subsections that are not applicable at the time of authorization are not listed. RECENT MAJOR LABEL CHANGES ............................................................................................
2 TABLE OF CONTENTS .............................................................................................................. 2 PART I: HEALTH PROFESSIONAL INFORMATION .....................................................................
4 1 INDICATIONS .............................................................................................................. 4 2 CONTRAINDICATIONS .................................................................................................
4 3 SERIOUS WARNINGS AND PRECAUTIONS BOX ............................................................ 4 4 DOSAGE AND ADMINISTRATION ................................................................................. 8 5 OVERDOSAGE..............................................................................................................
8 6 DOSAGE FORMS, STRENGTHS, COMPOSITION AND PACKAGING ................................. 8 7 WARNINGS AND PRECAUTIONS .................................................................................. 14 8 ADVERSE REACTIONS ................................................................................................
27 9 DRUG INTERACTIONS ............................................................................................... 28 10 CLINICAL PHARMACOLOGY .......................................................................................
28 11 STORAGE, STABILITY AND DISPOSAL ......................................................................... 29 PART II: SCIENTIFIC INFORMATION .......................................................................................
• Myozyme is contraindicated in patients who are hypersensitive to this drug or to any ingredient in the formulation, including any non-medicinal ingredient, or component of the container. For a complete listing, see 6 DOSAGE FORMS, STRENGTHS, COMPOSITION AND PACKAGING.
Not medical advice. Always read the patient information leaflet and follow your prescriber or pharmacist.
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3 mL of Sterile Water for Injection, USP to the inside wall of each vial. Avoid forceful impact of the water for injection 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.
Tilt and roll each vial gently. Do not invert, swirl or shake. Each vial will yield 5 mg/mL. The total extractable dose per vial is 50 mg, 10 mL. The reconstituted Myozyme should be protected from light. 3. Visually inspect the reconstituted vials.
The vials should contain a clear, colourless to pale yellow solution. The reconstituted solution may also contain a few particles in the form of thin white strands or translucent fibers. These particles have been shown to be composed of alglucosidase alfa which can be easily filtered during the infusion.
Do not use vials that contain foreign matter or if discoloured. 4 Administration. 4 Administration Myozyme treatment should be supervised by a physician who is knowledgeable in the treatment of Pompe’s disease. Myozyme is for intravenous (IV) administration only.
3 Reconstitution have been completed before continuing. 4. 9 % Sodium Chloride Injection, USP to the volume recommended in Table 1. The total volume of infusion is determined by the patient’s body weight and should be administered over approximately 4 hours.
Avoid foaming in the syringe. 5 to 4 mg/mL. Remove airspace from the infusion bag to minimize particle formation due to the sensitivity of Myozyme to air-liquid interface. Inject the reconstituted Myozyme solution directly into the sodium chloride solution rather than into the air within the infusion bag.
Avoid foaming in the infusion bag. Discard any vial with unused reconstituted solution. The diluted Myozyme should be protected from light. Patient dose (in mg) ÷ 5 mg/mL = number of mL of reconstituted Myozyme required for patient dose.
1 – 200 1000 5. Gently invert the infusion solution bag to mix. Avoid any vigorous shaking and agitation. Myozyme (alglucosidase alfa for injection) Page 7 of 46 Protected B / Protégé B 6. Myozyme should not be infused in the same intravenous line with other products.
7. 2 m filter during administration to remove any visible particles 8. Infusions should be administered in a step-wise manner using an infusion pump. The initial infusion rate should be no more than 1 mg/kg/h. The infusion rate may be increased by 2 mg/kg/h every 30 minutes, after patient tolerance to the infusion rate is established, until a maximum rate of 7 mg/kg/h is reached; see Table 2 below.
9. […]
Regardless of pre- treatment, reduction of the infusion rate, temporarily interrupting the infusion, and/or administration of antihistamines, antipyretics and/or corticosteroids may ameliorate the symptoms. If severe infusion reactions occur, immediate discontinuation of the Myozyme infusion should be considered, and appropriate medical support measures, including cardiopulmonary resuscitation equipment, should be available.
Severe infusion reactions are generally managed with administration of antihistamines, corticosteroids, intravenous fluids, and/or oxygen, when clinically indicated. In sosme cases of anaphylactic reactions epinephrine was administered.
Patients who have experienced infusion reactions should be treated with caution when they are readministered Myozyme. Immunogenicity The effect of IgG antibody formation on safety and efficacy has been evaluated in clinical trials and post-marketing experience.
In clinical studies, the majority of patients developed IgG antibodies to alglucosidase alfa and seroconversion typically occurred within 3 months of treatment. Thus seroconversion is expected to occur in most patients treated with Myozyme.
Overall, a correlation was not observed between the onset of IARs and the time of IgG antibody formation. IARs can occur across all levels of antibody titres, however a trend was observed for more frequent IARs with higher titres of IgG antibody.
The development of high and sustained IgG antibody titres is one of several factors that may contribute to diminished clinical efficacy. With regard to IOPD, a tendency was observed for patients treated with a higher dose (40 mg/kg) to develop higher titres of IgG antibodies.
Furthermore, Cross Reactive Immunologic Material (CRIM) status has been shown to be associated with immunogenicity and patients’ responses to enzyme replacement therapies. Negative CRIM status, indicating no endogenous enzyme is detected, is a risk factor to develop high and sustained IgG antibody titres.
This risk is higher among CRIM negative patients versus CRIM-positive patients and is a contributing factor to reduced clinical efficacy. However, high and sustained IgG antibody titres has also occurred in a limited number of CRIM-positive patients, generally with very low endogenous enzyme.
With respect to LOPD patients, the majority showed either stabilizing or decreasing antibody titres over time. The development of high and sustained IgG antibody titres is infrequent in LOPD patients. Thus, the impact of IgG antibodies is more limited for LOPD patients.
Some IgG positive infantile-onset and late-onset patients in clinical trials who were retrospectively evaluated for the presence of inhibitory antibodies tested positive for inhibition of enzyme activity and/or uptake in in vitro assays.
However, the clinical relevance of this in vitro inhibition is unclear (see 8 ADVERSE REACTIONS, Clinical Trial Adverse Drug Reactions, Infantile-Onset Disease, Immunogenicity; Late-Onset Disease, Immunogenicity sections). IgG antibody titres should be monitored based on clinical phenotype.
Baseline serum sample collection prior to the first infusion is strongly encouraged. For IOPD patients, regular monitoring during first year of treatment (example: every 3 months) is suggested and subsequent monitoring depending on clinical Myozyme (alglucosidase alfa for injection) Page 12 of 46 Protected B / Protégé B outcomes and antibody titres level.
For LOPD patients, antibody development should be assessed within 6 months and subsequent monitoring as clinically warranted based on safety and efficacy considerations. A small number of patients who were evaluated tested positive for alglucosidase alfa-specific IgE antibodies, some of whom experienced anaphylactic reactions.
Testing was typically performed for IARs suggestive of hypersensitivity reactions. Some patients have been successfully rechallenged using slower […]
31 13 PHARMACEUTICAL INFORMATION ............................................................................ 31 14 CLINICAL TRIALS .......................................................................................................
33 15 MICROBIOLOGY ........................................................................................................ 39 16 NON-CLINICAL TOXICOLOGY .....................................................................................
39 PATIENT MEDICATION INFORMATION .................................................................................. 42 Myozyme (alglucosidase alfa for injection) Page 4 of 46 Protected B / Protégé B PART I: HEALTH PROFESSIONAL INFORMATION 1 INDICATIONS MYOZYME (alglucosidase alfa for injection) is indicated for: • use in patients […]