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Drug overview for NEXVIAZYME (avalglucosidase alfa-ngpt):
Generic name: avalglucosidase alfa-ngpt (AY-val-gloo-KOE-si-dase)
Drug class: Metabolic Disease Enzyme Replacement, Pompe Disease
Therapeutic class: Metabolic Disease Enzyme Replacement Agents
Avalglucosidase alfa-ngpt is a hydrolytic lysosomal glycogen-specific recombinant human glucosidase alpha-glucosidase enzyme.
No enhanced Uses information available for this drug.
Generic name: avalglucosidase alfa-ngpt (AY-val-gloo-KOE-si-dase)
Drug class: Metabolic Disease Enzyme Replacement, Pompe Disease
Therapeutic class: Metabolic Disease Enzyme Replacement Agents
Avalglucosidase alfa-ngpt is a hydrolytic lysosomal glycogen-specific recombinant human glucosidase alpha-glucosidase enzyme.
No enhanced Uses information available for this drug.
DRUG IMAGES
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The following indications for NEXVIAZYME (avalglucosidase alfa-ngpt) have been approved by the FDA:
Indications:
Lysosomal alpha-1,4-glucosidase deficiency
Professional Synonyms:
Acid maltase deficiency
Glycogen storage disease type 2
Glycogen storage disease type II
Glycogenosis type II
Pompe's disease
Indications:
Lysosomal alpha-1,4-glucosidase deficiency
Professional Synonyms:
Acid maltase deficiency
Glycogen storage disease type 2
Glycogen storage disease type II
Glycogenosis type II
Pompe's disease
The following dosing information is available for NEXVIAZYME (avalglucosidase alfa-ngpt):
If one or more doses are missed, restart treatment as soon as possible, maintaining the 2 week interval between infusions thereafter.
If a severe hypersensitivity reaction (including anaphylaxis), or severe IAR develops during the avalglucosidase alfa-ngpt infusion, immediately discontinue the infusion and administer appropriate medical treatment.
If a mild to moderate hypersensitivity reaction, or moderate IAR develops during the avalglucosidase alfa-ngpt infusion, consider temporarily holding or slowing the infusion rate and initiating appropriate medical treatment. If symptoms persist despite temporary interruption of the infusion, wait at least 30 minutes for symptom resolution before deciding to stop the infusion. If symptoms subside, the infusion may be resumed at half the rate at which the reaction occurred for 30 minutes, and subsequently increased by 50% for an additional 15-30 minutes.
If there is no symptom recurrence, then the infusion may be increased to the rate at which the reaction occurred. Consider further rate increases in a stepwise manner.
If a severe hypersensitivity reaction (including anaphylaxis), or severe IAR develops during the avalglucosidase alfa-ngpt infusion, immediately discontinue the infusion and administer appropriate medical treatment.
If a mild to moderate hypersensitivity reaction, or moderate IAR develops during the avalglucosidase alfa-ngpt infusion, consider temporarily holding or slowing the infusion rate and initiating appropriate medical treatment. If symptoms persist despite temporary interruption of the infusion, wait at least 30 minutes for symptom resolution before deciding to stop the infusion. If symptoms subside, the infusion may be resumed at half the rate at which the reaction occurred for 30 minutes, and subsequently increased by 50% for an additional 15-30 minutes.
If there is no symptom recurrence, then the infusion may be increased to the rate at which the reaction occurred. Consider further rate increases in a stepwise manner.
Avalglucosidase alfa-ngpt is administered as an IV infusion. It is recommended to use a sterile, low-protein binding, in-line, 0.2 micron filter to administer the drug.
To ensure full delivery of the avalglucosidase alfa-ngpt dose, flush the infusion line with 5% dextrose injection at the end of the infusion. Do not infuse the diluted solution in the same IV line with other products. Avalglucosidase alfa-ngpt is available as a lyophilized powder that must be reconstituted and further diluted before IV administration. Store unopened vials of avalglucosidase alfa-ngpt at 2-8oC.
To ensure full delivery of the avalglucosidase alfa-ngpt dose, flush the infusion line with 5% dextrose injection at the end of the infusion. Do not infuse the diluted solution in the same IV line with other products. Avalglucosidase alfa-ngpt is available as a lyophilized powder that must be reconstituted and further diluted before IV administration. Store unopened vials of avalglucosidase alfa-ngpt at 2-8oC.
| DRUG LABEL | DOSING TYPE | DOSING INSTRUCTIONS |
|---|---|---|
| NEXVIAZYME 100 MG VIAL | Maintenance | Adults infuse 20 mg/kg by intravenous route every 2 weeks at a rate of 1 mg/kg/hr for 30 minutes may increase up to a max rate of 7 mg/kg/hr |
No generic dosing information available.
The following drug interaction information is available for NEXVIAZYME (avalglucosidase alfa-ngpt):
There are 0 contraindications.
There are 0 severe interactions.
There are 0 moderate interactions.
The following contraindication information is available for NEXVIAZYME (avalglucosidase alfa-ngpt):
Drug contraindication overview.
None.
None.
There are 0 contraindications.
There are 0 severe contraindications.
There are 3 moderate contraindications.
Clinically significant contraindication, where the condition can be managed or treated before the drug may be given safely.
| Moderate List |
|---|
| Cardiopulmonary impairment |
| Chronic heart failure |
| Hypervolemia |
The following adverse reaction information is available for NEXVIAZYME (avalglucosidase alfa-ngpt):
Adverse reaction overview.
The most common adverse reactions (>5%) were headache, fatigue, diarrhea, nausea, arthralgia, dizziness, myalgia, pruritus, vomiting, dyspnea, erythema, paresthesia, and urticaria.
The most common adverse reactions (>5%) were headache, fatigue, diarrhea, nausea, arthralgia, dizziness, myalgia, pruritus, vomiting, dyspnea, erythema, paresthesia, and urticaria.
There are 6 severe adverse reactions.
| More Frequent | Less Frequent |
|---|---|
|
Hypersensitivity drug reaction |
Acute respiratory distress syndrome Anaphylaxis Hypertension Hypotension |
| Rare/Very Rare |
|---|
|
Tongue swelling |
There are 25 less severe adverse reactions.
| More Frequent | Less Frequent |
|---|---|
|
Arthralgia Dizziness Fatigue General weakness Headache disorder Myalgia Pain in extremities Paresthesia Urticaria |
Acute abdominal pain Chest discomfort Chills Cough Diarrhea Dysphagia Dyspnea Erythema Fever Flushing Nausea Ocular redness Pruritus of skin Skin rash Upper abdominal pain Vomiting |
| Rare/Very Rare |
|---|
| None. |
The following precautions are available for NEXVIAZYME (avalglucosidase alfa-ngpt):
The safety and efficacy of avalglucosidase alfa-ngpt for the treatment of late onset Pompe disease (LOPD) have been established in pediatric patients 1 year of age and older. Use for this indication is supported by evidence from 2 clinical trials including adults and 1 pediatric patient 16 years of age with Pompe disease, in addition to safety data in 19 pediatric patients with infantile-onset Pompe disease (IOPD) (1-12 years of age) treated with avalglucosidase alfa-ngpt. The use of avalglucosidase alfa-ngpt is not approved for the treatment of IOPD.
In pediatric patients 1-12 years of age with Pompe disease, the safety profile of avalglucosidase alfa-ngpt was similar to that in adults and older pediatric patients with LOPD. Safety and efficacy of avalglucosidase alfa-ngpt have not been established in pediatric patients <1 year of age.
Contraindicated
Severe Precaution
Management or Monitoring Precaution
In pediatric patients 1-12 years of age with Pompe disease, the safety profile of avalglucosidase alfa-ngpt was similar to that in adults and older pediatric patients with LOPD. Safety and efficacy of avalglucosidase alfa-ngpt have not been established in pediatric patients <1 year of age.
Contraindicated
| None |
Severe Precaution
| None |
Management or Monitoring Precaution
| None |
Data on avalglucosidase alfa-ngpt use in pregnant women are insufficient to assess the drug-associated risk of major birth defects, miscarriage, or adverse maternal or fetal outcomes. Postmarketing data and case reports on alglucosidase alfa, another enzyme replacement therapy, have not identified a drug-associated risk of adverse pregnancy outcomes. The continuation of treatment for Pompe disease during pregnancy should be individualized; untreated Pompe disease during pregnancy has been associated with worsening respiratory and musculoskeletal symptoms in some pregnant patients. Pregnant women or their healthcare providers should report exposure to avalglucosidase alfa-ngpt during pregnancy by calling 800-745-4447, extension 15500.
There are no data available on the presence of avalglucosidase alfa-ngpt in human or animal milk. The effects of the drug on milk production and on the breastfed infant are not known. Available data suggest that alglucosidase alfa (another hydrolytic lysosomal glycogen-specific enzyme replacement therapy) is present in human milk.
Consider the benefits of breastfeeding along with the potential for adverse effects from avalglucosidase alfa-ngpt exposure to the breast-fed infant and the mother's clinical need for the drug. Lactating women who receive avalglucosidase alfa-ngpt, or their healthcare providers, should report avalglucosidase alfa-ngpt exposure by calling 800-745-4447, extension 15500.
Consider the benefits of breastfeeding along with the potential for adverse effects from avalglucosidase alfa-ngpt exposure to the breast-fed infant and the mother's clinical need for the drug. Lactating women who receive avalglucosidase alfa-ngpt, or their healthcare providers, should report avalglucosidase alfa-ngpt exposure by calling 800-745-4447, extension 15500.
The manufacturer recommends the same dosage in geriatric patients used in younger adult patients. Clinical studies of avalglucosidase alfa-ngpt included 3 patients 75 years of age and older, and 14 patients 65-74 years of age. Dosage recommendations in geriatric patients are the same as the recommended dosage in younger adult patients.
The following prioritized warning is available for NEXVIAZYME (avalglucosidase alfa-ngpt):
WARNING: Avalglucosidase alfa may cause certain serious side effects, including allergic and infusion reactions. Your doctor may prescribe other medications (such as antihistamines, corticosteroids) before each treatment to help prevent these side effects. Get medical help right away if you have any symptoms of a serious allergic reaction or infusion reactions such as flushing/rash, itching/swelling (especially of the face, tongue, throat), severe dizziness, trouble breathing, trouble swallowing, cough, or chest discomfort/tightness.
Your doctor may slow down or stop your treatment for some time. Before starting treatment with this medication, tell your doctor if you have a history of heart problems (such as heart failure), breathing problems (such as fluid in the lungs), or swelling. These problems may get worse during the infusion. Your doctor may check your blood pressure, pulse (heart rate), and breathing more often during your infusion.
WARNING: Avalglucosidase alfa may cause certain serious side effects, including allergic and infusion reactions. Your doctor may prescribe other medications (such as antihistamines, corticosteroids) before each treatment to help prevent these side effects. Get medical help right away if you have any symptoms of a serious allergic reaction or infusion reactions such as flushing/rash, itching/swelling (especially of the face, tongue, throat), severe dizziness, trouble breathing, trouble swallowing, cough, or chest discomfort/tightness.
Your doctor may slow down or stop your treatment for some time. Before starting treatment with this medication, tell your doctor if you have a history of heart problems (such as heart failure), breathing problems (such as fluid in the lungs), or swelling. These problems may get worse during the infusion. Your doctor may check your blood pressure, pulse (heart rate), and breathing more often during your infusion.
The following icd codes are available for NEXVIAZYME (avalglucosidase alfa-ngpt)'s list of indications:
| Lysosomal alpha-1,4-glucosidase deficiency | |
| E74.02 | Pompe disease |
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