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Nexviazyme (Intravenous)

Generic name: avalglucosidase alfa-ngptay-val-gloo-KOE-si-dase-AL-fa-- ngpt ]
Drug class: Lysosomal enzymes

Medically reviewed by Last updated on Dec 16, 2023.

Intravenous route(Powder for Solution)

Warning: Severe hypersensitivity reactions, infusion-associated reactions, and risk of acute cardiorespiratory failure in susceptible patients

Hypersensitivity Reactions including Anaphylaxis

Patients treated with avalglucosidase alfa-ngpt have experienced life-threatening hypersensitivity reactions, including anaphylaxis. Appropriate medical monitoring and support measures, including cardiopulmonary resuscitation equipment, should be readily available during avalglucosidase alfa-ngpt administration. If a severe hypersensitivity reaction (e.g., anaphylaxis) occurs, discontinue avalglucosidase alfa-ngpt immediately and initiate appropriate medical treatment. In patients with severe hypersensitivity reaction, a desensitization procedure to avalglucosidase alfa-ngpt may be considered.

Infusion-Associated Reactions (IARs)Patients treated with avalglucosidase alfa-ngpt have experienced severe IARs. If severe IARs occur, consider immediate discontinuation of avalglucosidase alfa-ngpt, initiation of appropriate medical treatment, and the benefits and risks of readministering avalglucosidase alfa-ngpt following severe IARs. Patients with an acute underlying illness at the time of avalglucosidase alfa-ngpt 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.Risk of Acute Cardiorespiratory Failure in Susceptible Patients

Patients susceptible to fluid volume overload, or those with acute underlying respiratory illness or compromised cardiac or respiratory function for whom fluid restriction is indicated may be at risk of serious exacerbation of their cardiac or respiratory status during avalglucosidase alfa-ngpt infusion. More frequent monitoring of vitals should be performed during avalglucosidase alfa-ngpt infusion in such patients .

Uses for Nexviazyme

Avalglucosidase alfa-ngpt injection is an enzyme used to treat late-onset of Pompe disease, which is also called glycogen storage disease type II. It contains a human enzyme called acid alpha-glucosidase, which helps with the digestion and absorption of glycogen. People with Pompe disease are not able to make enough of this enzyme.

This medicine is to be given only by or under the direct supervision of your doctor.

Before using Nexviazyme

In deciding to use a medicine, the risks of taking the medicine must be weighed against the good it will do. This is a decision you and your doctor will make. For this medicine, the following should be considered:


Tell your doctor if you have ever had any unusual or allergic reaction to this medicine or any other medicines. Also tell your health care professional if you have any other types of allergies, such as to foods, dyes, preservatives, or animals. For non-prescription products, read the label or package ingredients carefully.


Appropriate studies performed to date have not demonstrated pediatric-specific problems that would limit the usefulness of avalglucosidase alfa-ngpt injection in children 1 year of age and older. However, safety and efficacy have not been established in children younger than 1 year of age.


Appropriate studies performed to date have not demonstrated geriatric-specific problems that would limit the usefulness of avalglucosidase alfa-ngpt injection in the elderly.

Breast Feeding

There are no adequate studies in women for determining infant risk when using this medication during breastfeeding. Weigh the potential benefits against the potential risks before taking this medication while breastfeeding.

Interactions with Medicines

Although certain medicines should not be used together at all, in other cases two different medicines may be used together even if an interaction might occur. In these cases, your doctor may want to change the dose, or other precautions may be necessary. Tell your healthcare professional if you are taking any other prescription or nonprescription (over-the-counter [OTC]) medicine.

Interactions with Food/Tobacco/Alcohol

Certain medicines should not be used at or around the time of eating food or eating certain types of food since interactions may occur. Using alcohol or tobacco with certain medicines may also cause interactions to occur. Discuss with your healthcare professional the use of your medicine with food, alcohol, or tobacco.

Other Medical Problems

The presence of other medical problems may affect the use of this medicine. Make sure you tell your doctor if you have any other medical problems, especially:

Proper use of Nexviazyme

A nurse or other trained health professional will give you or your child this medicine in a hospital. This medicine is given through a needle placed in one of your veins. The medicine must be injected slowly, so your IV tube will need to stay in place for 4 to 7 hours. It is usually given every 2 weeks.

Your doctor will give you other medicines (eg, allergy, fever, or steroid medicine) before you receive this medicine to help prevent unwanted effects.

Missed Dose

This medicine needs to be given on a fixed schedule. If you miss a dose or forget to use your medicine, call your doctor or pharmacist for instructions.

Precautions while using Nexviazyme

It is very important that your doctor check your or your child's progress closely and at regular visits to make sure this medicine is working properly and to decide if you should continue to receive it. Blood tests may be needed to check for unwanted effects.

This medicine may cause serious allergic reactions, including anaphylaxis, which can be life-threatening and require immediate medical attention. Tell your doctor right away if you have blurred vision, chest discomfort or tightness, confusion, dizziness, faintness, or lightheadedness when getting up suddenly from a lying or sitting position, fast heartbeat, flushing, redness of the skin, rash, itching, hoarseness, sweating, trouble breathing, trouble swallowing, unusually warm skin, any swelling of your hands, face, or mouth, or unusual tiredness or weakness after receiving this medicine.

This medicine may cause infusion-related reactions, which can be life-threatening and require immediate medical attention. Tell your doctor right away if you start to have chest discomfort, fever, chills or shaking, confusion, difficulty swallowing, dizziness, fast heartbeat, flushing, redness of the skin, increase or decrease blood pressure, trouble breathing, itching or rash, unusually warm skin, or lightheadedness, or fainting after receiving this medicine.

Side Effects of Nexviazyme

Along with its needed effects, a medicine may cause some unwanted effects. Although not all of these side effects may occur, if they do occur they may need medical attention.

Check with your doctor or nurse immediately if any of the following side effects occur:

More common

Some side effects may occur that usually do not need medical attention. These side effects may go away during treatment as your body adjusts to the medicine. Also, your health care professional may be able to tell you about ways to prevent or reduce some of these side effects. Check with your health care professional if any of the following side effects continue or are bothersome or if you have any questions about them:

More common

Other side effects not listed may also occur in some patients. If you notice any other effects, check with your healthcare professional.

Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088.

Commonly used brand name(s)

In the U.S.

Available Dosage Forms:

Therapeutic Class: Enzyme Replacement

Pharmacologic Class: Avalglucosidase Alfa

Further information

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.