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NEXVIAZYME is a brand name for Avalglucosidase Alfa, supplied as a powder for solution. The medicine, its uses, side effects and dosage are the same regardless of brand.
Used for: Nexviazyme™ (avalglucosidase alfa for injection) is an enzyme replacement therapy (ERT) indicated for: • the long-term treatment of patients with late-onset Pompe disease (acid α-glucosidase deficiency). 1.1 Pediatrics Pediatrics (>6 months of age): Based on the data submitted and reviewed by Health Canada, the safety…
Verbatim from this product's HC label. Tap a section to expand.
1 Dosing Considerations • Vials are single-use only. Any unused product should be discarded. 2 Recommended Dose and Dosage Adjustment Patients with late-onset Pompe disease (LOPD) The recommended dose of Nexviazyme is 20 mg/kg of body weight administered every other week (qow).
Pediatric patients (>6 months of age) Based on population-PK modeling, no dose adjustment is required in pediatric LOPD patients (see Section 7 WARNINGS AND PRECAUTIONS, Pediatrics). Geriatric patients (>65 years of age) Clinical studies with Nexviazyme included 11 patients aged 65-75 years and 2 patients over the age of 75 years.
There is no recommended dose adjustment for patients over the age of 65 (see 14 CLINICAL TRIALS). Hepatic impairment The safety and efficacy of Nexviazyme have not been studied in patients with hepatic impairment. Renal impairment Based on available data, including population-PK modeling, no dose adjustment is required in patients with mild renal impairment.
Nexviazyme has not been studied in patients with moderate or severe renal impairment (see 10 CLINICAL PHARMACOLOGY, Pharmacokinetics). 0 mL at 10 mg/mL of Nexviazyme. Each vial contains an overfill to compensate for liquid loss during preparation.
This overfill ensures that, after dilution with the entire content, there is solution containing 10 mg/mL Nexviazyme. 4 Administration Nexviazyme is intended for long-term, chronic use under the guidance and supervision of a health care professional who is knowledgeable in the treatment of Pompe disease.
Nexviazyme should be reconstituted, diluted and administered by a health care professional in a hospital or in an appropriate setting of outpatient care. Nexviazyme is for intravenous (IV) infusion only. Intravenous (IV) infusion Product Monograph NEXVIAZYME™ avalglucosidase alfa for injection Page 6 of 29 Internal Infusion should be administered incrementally as determined by patient response and comfort over approximately 4 hours for patients with LOPD.
For patients with LOPD, it is recommended that the infusion begins at an initial rate of 1 mg/kg/hour and be gradually increased by 2 mg/kg/hour every 30 minutes if there are no signs of infusion associated reactions (IARs) until a maximum rate of 7 mg/kg/hour is reached.
Vital signs should be obtained at each step, before increasing the infusion rate. Patients may be pretreated with antihistamines, antipyretics and/or corticosteroids to prevent or reduce allergic reactions. In the event of anaphylaxis or severe hypersensitivity reaction or severe infusion associated reactions (IARs), immediately discontinue administration of Nexviazyme and initiate appropriate medical treatment.
, Clinical Trial Adverse Reactions). Immune Hypersensitivity reactions including anaphylaxis Hypersensitivity reactions, including anaphylaxis, have been reported in Nexviazyme-treated patients. 6%) patients experienced hypersensitivity reactions including 7 patients who reported severe hypersensitivity reactions and 4 patients who experienced anaphylaxis.
Some of the hypersensitivity reactions were IgE mediated. Anaphylaxis signs and symptoms included hypotension, hypoxia, respiratory distress, chest pressure, generalized edema, flushing, cough, dizziness, nausea, redness on palms, swollen lower lip, decreased breath sounds, feeling hot, erythema, redness on feet, throat tightness, difficulty swallowing (dysphagia), difficulty speaking (dysarthria), pruritis, swollen tongue, itchy palms and feet, and oxygen desaturation.
Symptoms of severe hypersensitivity reactions included swollen tongue, respiratory failure, respiratory distress, generalized edema, erythema, urticaria, and rash. 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 Nexviazyme is administered.
Product Monograph NEXVIAZYME™ avalglucosidase alfa for injection Page 10 of 29 Internal If severe hypersensitivity or anaphylaxis occur, Nexviazyme should be discontinued immediately, and appropriate medical treatment should be initiated.
The risks and benefits of re-administering Nexviazyme following anaphylaxis or severe hypersensitivity reaction should be considered. Some patients have been re-challenged using slower infusion rates at a dose lower than the recommended dose.
In patients with severe hypersensitivity, desensitization procedure to Nexviazyme may be considered. If the decision is made to re-administer the product, extreme caution should be exercised, with appropriate resuscitation measures available.
). Infusion-Associated Reactions (IARs) • Patients treated with NEXVIAZYME have experienced severe IARs. If severe IARs occur, consider immediate discontinuation of NEXVIAZYME, initiation of appropriate medical treatment, and the benefits and risks of readministering NEXVIAZYME following severe IARs.
Patients with an acute underlying illness at the time of NEXVIAZYME infusion may be at greater risk for IARs. Patients with advanced Pompe disease may have compromised cardiac and respiratory function, which may predispose them to a higher risk of severe complications from IARs (see 7 WARNINGS AND PRECAUTIONS).
1 Dosing Considerations • Vials are single-use only. Any unused product should be discarded. 2 Recommended Dose and Dosage Adjustment Patients with late-onset Pompe disease (LOPD) The recommended dose of Nexviazyme is 20 mg/kg of body weight administered every other week (qow).
Pediatric patients (>6 months of age) Based on population-PK modeling, no dose adjustment is required in pediatric LOPD patients (see Section 7 WARNINGS AND PRECAUTIONS, Pediatrics). Geriatric patients (>65 years of age) Clinical studies with Nexviazyme included 11 patients aged 65-75 years and 2 patients over the age of 75 years.
There is no recommended dose adjustment for patients over the age of 65 (see 14 CLINICAL TRIALS). Hepatic impairment The safety and efficacy of Nexviazyme have not been studied in patients with hepatic impairment. Renal impairment Based on available data, including population-PK modeling, no dose adjustment is required in patients with mild renal impairment.
Nexviazyme has not been studied in patients with moderate or severe renal impairment (see 10 CLINICAL PHARMACOLOGY, Pharmacokinetics). 0 mL at 10 mg/mL of Nexviazyme. Each vial contains an overfill to compensate for liquid loss during preparation.
This overfill ensures that, after dilution with the entire content, there is solution containing 10 mg/mL Nexviazyme. 4 Administration Nexviazyme is intended for long-term, chronic use under the guidance and supervision of a health care professional who is knowledgeable in the treatment of Pompe disease.
Life-threatening hypersensitivity to the active substance or to any of the excipients when re-challenge was unsuccessful (see WARNINGS AND PRECAUTIONS, Immune, Hypersensitivity reactions including anaphylaxis). For a complete listing, see 6 DOSAGE FORMS, STRENGTHS, COMPOSITION AND PACKAGING.
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In the event of mild to moderate hypersensitivity reactions or IARs, the infusion rate may be slowed or temporarily stopped and/or appropriate medical treatment initiated (see 7 WARNINGS AND PRECAUTIONS, Immune, Hypersensitivity reactions including anaphylaxis and Infusion Associated Reactions).
Symptoms may persist despite temporarily stopping the infusion; therefore, the treating physician should wait at least 30 minutes for symptoms of the reactions to resolve before deciding to stop the infusion for the remainder of the day.
If symptoms subside, resume infusion rate for 30 minutes at half the rate, or less, of the rate at which the reactions occurred, followed by an increase in infusion rate by 50% for 15 to 30 minutes. If symptoms do not recur, increase the infusion rate to the rate at which the reactions occurred and consider continuing to increase the rate in a stepwise manner until the maximum rate is achieved.
Home Infusion Infusion of Nexviazyme at home may be considered for patients who are tolerating their infusions well and have no history of moderate or severe IARs. The decision to have a patient move to home infusion should be made after evaluation and upon recommendation by the prescribing and or treating physician.
A patient’s underlying co-morbidities and ability to adhere to the home infusion requirements need to be taken into account when evaluating the patient for eligibility to receive home infusion. The following criteria should be considered: • The patient must have no ongoing concurrent condition that, in the opinion of the physician, may affect patient’s ability to tolerate the infusion.
• The patient is considered medically stable. A comprehensive evaluation must be completed before the initiation of home infusion. • The patient must have received Nexviazyme infusions for few months in a hospital or in another appropriate setting of outpatient care.
Documentation of a pattern of well-tolerated infusions with no IARs, or mild IARs that have been controlled with premedication, is a prerequisite for the initiation of home infusion. • The patient must have a history of adherence to the prescribed infusion schedule.
• Home infusion infrastructure, resources, and procedures, including training, must be established and available to the home infusion staff. The home infusion staff should be available at all times during the home infusion and a specified time after-infusion, depending on patient’s tolerance prior to starting home infusion.
Product Monograph NEXVIAZYME™ avalglucosidase alfa for injection Page 7 of 29 Internal If the patient experiences adverse reactions during the home infusion, stop the infusion process immediately and initiate appropriate medical treatment (see 7 WARNINGS AND PRECAUTIONS).
Subsequent infusions may need to occur in a hospital or in an appropriate setting of outpatient care until no such adverse reactions occur. Dose and infusion rate must not be changed without consulting the prescribing and or […]
Once a patient tolerates the infusion, the dose may be increased to reach the approved dose. If mild or moderate hypersensitivity reactions occur, the infusion rate may be slowed or temporarily stopped. Infusion Associated Reactions In clinical studies, IARs were reported to occur at any time during and/or within a few hours after the infusion of Nexviazyme and were more likely with higher infusion rates.
4%) patients treated with Nexviazyme in clinical studies. The majority of IARs were assessed as mild to moderate and symptoms reported in more than one patient included chills, cough, diarrhea, erythema, somnolence, sluggishness, fatigue, pyrexia, flushing, feeling hot or cold, cyanosis and pallor, headache, influenza like illness, nausea, ocular hyperemia, eyelid edema, face edema, pain in extremity, pruritus, rash, rash erythematous, increased or decreased blood pressure, tachycardia, urticaria, vomiting, respiratory distress, chest discomfort, dyspnea, dizziness, hyperhidrosis, skin plaque, lip swelling, oxygen saturation decreased, throat irritation, dyspepsia, burning sensation, pain, palmar erythema, swollen tongue and tremor.
2%) patients reported severe IARs including symptoms of respiratory distress, hypoxia, chest discomfort, generalized edema, tongue edema, dysphagia, nausea, erythema, urticaria, and increased or decreased blood pressure. Patients with an acute underlying illness at the time of Nexviazyme infusion appear to be at greater risk for IARs.
Patients with advanced Pompe disease may have compromised cardiac and respiratory function, which may predispose them to a higher risk of severe complications from IARs. Antihistamines, antipyretics, and/or corticosteroids can be given to prevent or reduce IARs.
However, IARs may still occur in patients after receiving pre-treatment. If severe IARs occur, immediate discontinuation of the administration of Nexviazyme should be considered and appropriate medical treatment should be initiated.
The benefits and risks of re- administering Nexviazyme following severe IARs should be considered. Some patients have been re- challenged using slower infusion rates at a dose lower than the recommended dose. Once a patient tolerates the infusion, the dose may be increased to reach the approved dose.
If a mild or moderate IARs occur regardless of pre-treatment, decreasing the infusion rate or temporarily stopping the infusion may ameliorate the symptoms. Guide for healthcare professionals (HCPs) for immunosurveillance service A “Guide for healthcare professionals (HCPs) for immunosurveillance service” is available to help manage the following safety concerns for Nexviazyme: • Infusion associated reactions including hypersensitivity and anaphylactic reactions, with or without development of IgG and IgE antibodies • Immunogenicity leading to loss of response (High Sustained IgG Antibody Titers and/or neutralizing antibodies) Product Monograph NEXVIAZYME™ avalglucosidase alfa for injection Page 11 of 29 Internal The guide also provides information about the immunosurveillance service made available by Sanofi in Canada.
ca). Immunogenicity Treatment emergent anti-drug antibodies (ADA) were reported in both treatment naïve (95%) and treatment experienced patients (62%) (see 8 ADVERSE REACTIONS, Clinical Trial Adverse Reactions, Immunogenicity). IARs and hypersensitivity reactions may occur independent of the development of ADA.
The majority of IARs and hypersensitivity reactions were mild or moderate and were managed with standard clinical practices. 3% in patients with […]
Nexviazyme should be reconstituted, diluted and administered by a health care professional in a hospital or in an appropriate setting of outpatient care. Nexviazyme is for intravenous (IV) infusion only. Intravenous (IV) infusion Product Monograph NEXVIAZYME™ avalglucosidase alfa for injection Page 6 of 29 Internal Infusion should be administered incrementally as determined by patient response and comfort over approximately 4 hours for patients with LOPD.
For patients with LOPD, it is recommended that the infusion begins at an initial rate of 1 mg/kg/hour and be gradually increased by 2 mg/kg/hour every 30 minutes if there are no signs of infusion associated reactions (IARs) until a maximum rate of 7 mg/kg/hour is reached.
Vital signs should be obtained at each step, before increasing the infusion rate. Patients may be pretreated with antihistamines, antipyretics and/or corticosteroids to prevent or reduce allergic reactions. In the event of anaphylaxis or severe hypersensitivity reaction or severe infusion associated reactions (IARs), immediately discontinue administration of Nexviazyme and initiate appropriate medical treatment.
In the event of mild to moderate hypersensitivity reactions or IARs, the infusion rate may be slowed or temporarily stopped and/or appropriate medical treatment initiated (see 7 WARNINGS AND PRECAUTIONS, Immune, Hypersensitivity reactions including anaphylaxis and Infusion Associated Reactions).
Symptoms may persist despite temporarily stopping the infusion; therefore, the treating physician should wait at least 30 minutes for symptoms of the reactions to resolve before deciding to stop the infusion for the remainder of the day.
If symptoms subside, resume infusion rate for 30 minutes at half the rate, or less, of the rate at which the reactions occurred, followed by an increase in infusion rate by 50% for 15 to 30 minutes. If symptoms do not recur, increase the infusion rate to the rate at which the reactions occurred and consider continuing to increase the rate in a stepwise manner until the maximum rate is achieved.
Home Infusion Infusion of Nexviazyme at home may be considered for patients who are tolerating their infusions well and have no history of moderate or severe IARs. The decision to have a patient move to home infusion should be made after evaluation and upon recommendation by the prescribing and or treating physician.
A patient’s underlying co-morbidities and ability to adhere to the home infusion requirements need to be taken into account when evaluating the patient for eligibility to receive home infusion. The following criteria should be considered: • The patient must have no ongoing concurrent condition that, in the opinion of the physician, may affect patient’s ability to tolerate the infusion.
• The patient is considered medically stable. A comprehensive evaluation must be completed before the initiation of home infusion. • The patient must have received Nexviazyme infusions for few months in a hospital or in another appropriate setting of outpatient care.
Documentation of a pattern of well-tolerated infusions with no IARs, or mild IARs that have been controlled with premedication, is a prerequisite for the initiation of home infusion. • The patient must have a history of adherence to the prescribed infusion schedule.
• Home infusion infrastructure, resources, and procedures, including training, must be […]