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Avalglucosidase alfa

Generic name: avalglucosidase alfa [ AY-val-gloo-KOE-si-dase-AL-fa ]
Brand name: Nexviazyme
Dosage form: intravenous injection (ngpt 100 mg)
Drug class: Lysosomal enzymes

Medically reviewed by Drugs.com on Sep 8, 2021. Written by Cerner Multum.

What is avalglucosidase alfa?

Avalglucosidase alfa helps replace an enzyme that is missing in people with a genetic disorder called late-onset Pompe disease, (also called GAA deficiency) in children at least 1 year old.

Avalglucosidase alfa may also be used for purposes not listed in this medication guide.

Warnings

Tell your caregivers or get emergency medical help right away if you have any signs of a severe allergic reaction, such as itching, rash, redness or tingling, chest discomfort, wheezing, trouble breathing or swallowing, swelling in your face, pale skin, and blue lips or fingernails.

Before taking this medicine

Tell your doctor if you have ever had:

  • heart disease; or

  • lung disease or breathing problems.

Tell your doctor if you are pregnant or plan to become pregnant. Having untreated Pompe disease during pregnancy may increase your risk of worsened breathing problems and joint or muscle problems. The benefit of treating Pompe disease may outweigh any risks.

Ask a doctor if it is safe to breastfeed while using avalglucosidase alfa.

Your name may be listed on the Pompe Registry. This is to track the progress of your disease and the outcome of your treatment with avalglucosidase alfa.

How is avalglucosidase alfa given?

Avalglucosidase is injected into a vein by a healthcare provider, usually once every 2 weeks.

This medicine must be given slowly over 4 to 5 hours.

You may be given other medications to help prevent serious side effects or allergic reaction. Keep taking these medicines for as long as your doctor has prescribed.

What happens if I miss a dose?

Call your doctor for instructions if you miss an appointment for your avalglucosidase alfa injection.

What happens if I overdose?

Seek emergency medical attention or call the Poison Help line at 1-800-222-1222.

What should I avoid while receiving avalglucosidase alfa?

Follow your doctor's instructions about any restrictions on food, beverages, or activity.

Avalglucosidase alfa side effects

Get emergency medical help if you have signs of an allergic reaction: hives, itching; difficult breathing; swelling of your face, lips, tongue, or throat.

Some reactions may occur during the injection, or within a few hours afterward. Tell your medical caregiver or get emergency medical help right away if you have:

  • wheezing, cough, chest discomfort, trouble breathing;

  • pale skin, blue lips or fingernails;

  • swelling in your tongue;

  • trouble swallowing;

  • dizzines, nausea;

  • rash; or

  • flushing (sudden warmth, redness, or tingly feeling).

Tell your caregivers or get emergency medical help right away if you have any signs of a severe allergic reaction, such as itching, rash, redness or tingling, chest discomfort, wheezing, trouble breathing or swallowing, swelling in your face, pale skin, and blue lips or fingernails.

Common side effects of avalglucosidase alfa may include:

This is not a complete list of side effects and others may occur. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.

Avalglucosidase alfa dosing information

Usual Adult Dose for Pompe disease:

Less than 30 kg: 40 mg/kg IV every 2 weeks
At least 30 kg: 20 mg/kg every 2 weeks

Comments:
-The patient's actual body weight should be used to calculate the dose.

Use: For the treatment of patients with late-onset Pompe disease (lysosomal acid alpha-glucosidase [GAA] deficiency)

Usual Pediatric Dose for Pompe disease:

1 year or older:
-Less than 30 kg: 40 mg/kg IV every 2 weeks
-At least 30 kg: 20 mg/kg every 2 weeks

Comments:
-The patient's actual body weight should be used to calculate the dose.

Use: For the treatment of patients with late-onset Pompe disease (lysosomal GAA deficiency)

What other drugs will affect avalglucosidase alfa?

Other drugs may affect avalglucosidase, including prescription and over-the-counter medicines, vitamins, and herbal products. Tell your doctor about all other medicines you use.

Where can I get more information?

Remember, keep this and all other medicines out of the reach of children, never share your medicines with others, and use this medication only for the indication prescribed.

More about avalglucosidase alfa

Patient resources

Other brands

Nexviazyme

Professional resources

Related treatment guides

Further information

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