Toggle navigation
Toggle search

Avalglucosidase Alfa-ngpt (Intravenous route)


ay-val-gloo-KOE-si-dase AL-fa - ngpt

Brand Names:

  • Nexviazyme

Dosage Forms:

  • Powder for Solution


Intravenous route(Powder for Solution)

Warning: Severe hypersensitivity reactions, infusion-associated reactions, and risk of acute cardiorespiratory failure in susceptible patientsHypersensitivity Reactions including AnaphylaxisPatients treated with avalglucosidase alfa-ngpt have experienced life-threatening hypersensitivity reactions, including anaphylaxis. Appropriate medical support measures, including cardiopulmonary resuscitation equipment, should be readily available during avalglucosidase alfa-ngpt administration. If a severe hypersensitivity reaction (e.g., anaphylaxis) occurs, avalglucosidase alfa-ngpt should be discontinued immediately and appropriate medical treatment should be initiated. 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 PatientsPatients 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 .



Enzyme Replacement


Avalglucosidase Alfa

Uses of This Medicine:

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 This Medicine:

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.

Older adults—

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


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.

Other 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.

Other interactions—

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:

  • Heart disease or
  • Lung or breathing problems—Use with caution. May make these conditions worse.

Proper Use of This Medicine:

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.

Precautions While Using This Medicine:

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 a rash, itching, hoarseness, trouble breathing, trouble swallowing, or any swelling of your hands, face, or mouth after using 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 fever, chills or shaking, dizziness, trouble breathing, itching or rash, lightheadedness, or fainting after receiving this medicine.

Side Effects of This Medicine:

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
Back pain
bluish lips or skin
blurred vision
chest discomfort or tightness
difficulty swallowing
dizziness, faintness, or lightheadedness when getting up suddenly from a lying or sitting position
fast, pounding, or irregular heartbeat or pulse
feeling of warmth
general feeling of discomfort or illness
hives, itching, skin rash
joint pain
loss of appetite
muscle aches and pains
pounding in the ears
puffiness or swelling of the eyelids or around the eyes, face, lips, or tongue
redness of the face, neck, arms, and occasionally, upper chest
runny nose
shakiness in the legs, arms, hands, or feet
slow or fast heartbeat
sore throat
trembling or shaking of the hands or feet
trouble breathing
trouble sleeping
unusual tiredness or weakness

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
Burning, crawling, itching, numbness, prickling, "pins and needles", or tingling feelings
difficulty in moving
swollen joints

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.

Last Updated: 3/30/2022
The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites.

All rights reserved

A.D.A.M. content is best viewed in IE9 or above, Firefox and Google Chrome browser.

We are physicians, hospitals and communities working together to help you live better.