Nexviazyme (avalglucosidase alfa-ngpt)
Metabolic Disease Enzyme Replacement, Pompe Disease Intravenous Vial (Ea)
Last reviewed · Source: First Databank (FDB) drug data
Overview
Avalglucosidase alfa-ngpt is a hydrolytic lysosomal glycogen-specific recombinant human glucosidase alpha-glucosidase enzyme.
Avalglucosidase alfa-ngpt is a hydrolytic lysosomal glycogen-specific recombinant human alpha-glucosidase enzyme that is conjugated with multiple synthetic bis-mannose-6-phosphate (bis-M6P)-tetra mannose glycans.
Pompe disease, which is also known as glycogen storage disease type II, acid maltase deficiency, and glycogenosis type II, is a hereditary glycogen metabolic disorder caused by lysosomal enzyme acid alpha-glucosidase (GAA) deficiency, which results in intralysosomal accumulation of glycogen in various tissues. Use of avalglucosidase alfa provides an exogenous source of GAA. The mannose-6-phosphate (M6P) on avalglucosidase alfa mediates binding to M6P receptors on the cell surface.
After binding, the drug is internalized and transported into lysosomes where proteolysis causes increased GAA enzymatic activity. Avalglucosidase alfa then exerts enzymatic activity to cleave glycogen, which is measured in clinical studies by urinary levels of glucose tetrasaccharide (G1c4).
Exposure to avalglucosidase alfa is approximately dose proportional over a dosage range of 5-20 mg/kg (0.25-1, and 0.125-0.5 times the approved recommended dosage in patients weighing 30 kg or greater, and in patients weighing less than 30 kg, respectively). No accumulation of the drug has been observed following an every 2 week dosing interval. Patients weighing less than 30 kg receiving a dosage of 40 mg/kg every 2 weeks are expected to have a similar total exposure based on AUC as patients weighing at least 30 kg receiving a dosage of 20 mg/kg every 2 weeks.
The metabolic pathway of avalglucosidase alfa is not fully characterized; however, the protein component is expected to undergo metabolism to small peptides and amino acids via catabolic pathways. The mean plasma elimination half-life of avalglucosidase alfa in patients with LOPD is 1.6 hours. No clinically significant differences in avalglucosidase alfa pharmacokinetics have been observed based on age (1-78 years) or sex.
USES: This medication is used by people with a certain inherited condition (Pompe disease) to reduce high levels of a certain substance (glycogen) that builds up in the body. High levels of glycogen in the body may cause muscle weakness, heart problems, and breathing problems. Avalglucosidase alfa works by breaking down glycogen.
Generic name: avalglucosidase alfa-ngpt
Pronunciation: (AY-val-gloo-KOE-si-dase)
Drug class: Metabolic Disease Enzyme Replacement, Pompe Disease
Therapeutic class: Metabolic Disease Enzyme Replacement Agents
Reference product & biosimilars
No reference, biosimilar or interchangeable associated with this drug.
Today’s intelligence on Nexviazyme
Indications
The following indications are listed for Nexviazyme:
- Lysosomal Alpha-1,4-Glucosidase Deficiency
Also known as: Acid Maltase Deficiency, Glycogen Storage Disease Type 2, Glycogen Storage Disease Type Ii, Glycogenosis Type Ii, Pompe's Disease.
Dosing
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.
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.
| Drug label | Dosing type | Instructions |
|---|---|---|
| Nexviazyme 100 Mg Vial | Maintenance | 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 dosing information is available for this tab.
Drug interactions
No formal drug interaction studies have been conducted with avalglucosidase-alfa-ngpt.
There are no contraindication interactions for this drug.
There are no severe interactions for this drug.
There are no moderate interactions for this drug.
Contraindications
*None.
There are no contraindication contraindications for this drug.
There are no severe contraindications for this drug.
- Cardiopulmonary impairment
- Chronic heart failure
- Hypervolemia
Adverse reactions
The most common adverse reactions (>5%) were headache, fatigue, diarrhea, nausea, arthralgia, dizziness, myalgia, pruritus, vomiting, dyspnea, erythema, paresthesia, and urticaria.
More Frequent
- Hypersensitivity drug reaction
Less Frequent
- Acute respiratory distress syndrome
- Anaphylaxis
- Hypertension
- Hypotension
Rare or Very Rare
- Tongue swelling
More Frequent
- Arthralgia
- Dizziness
- Fatigue
- General weakness
- Headache disorder
- Myalgia
- Pain in extremities
- Paresthesia
- Urticaria
Less Frequent
- 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
Precautions
No enhanced messaging available.
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.
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 800-633-1610, option 1.
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 800-633-1610, option 1.
The manufacturer recommends the same dosage in geriatric patients used in younger adult patients.
Clinical studies of avalglucosidase alfa-ngpt included 4 patients 75 years of age and older, and 13 patients 65-74 years of age. Dosage recommendations in geriatric patients are the same as the recommended dosage in younger adult patients.
ICD codes
| Code | Type | Description |
|---|---|---|
| Lysosomal alpha-1,4-glucosidase deficiency | ||
| E74.02 | ICD10CM | Pompe disease |
ICD code information from First Databank (FDB).
Formulary status
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Last reviewed · Source: First Databank (FDB) drug data
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