Description and Brand Names

Drug information provided by: IBM Micromedex

US Brand Name

  1. Nexviazyme

Descriptions


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.

This product is available in the following dosage forms:

  • Powder for Solution

Before Using

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:

Allergies

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.

Pediatric

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.

Geriatric

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

Breastfeeding

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.

Drug Interactions

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

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

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

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

  1. Back pain
  2. bluish lips or skin
  3. blurred vision
  4. chest discomfort or tightness
  5. chills
  6. confusion
  7. cough
  8. diarrhea
  9. difficulty swallowing
  10. dizziness, faintness, or lightheadedness when getting up suddenly from a lying or sitting position
  11. fast, pounding, or irregular heartbeat or pulse
  12. feeling of warmth
  13. fever
  14. general feeling of discomfort or illness
  15. headache
  16. hives, itching, skin rash
  17. joint pain
  18. loss of appetite
  19. muscle aches and pains
  20. nausea
  21. nervousness
  22. pounding in the ears
  23. puffiness or swelling of the eyelids or around the eyes, face, lips, or tongue
  24. redness of the face, neck, arms, and occasionally, upper chest
  25. runny nose
  26. shakiness in the legs, arms, hands, or feet
  27. shivering
  28. slow or fast heartbeat
  29. sore throat
  30. sweating
  31. trembling or shaking of the hands or feet
  32. trouble breathing
  33. trouble sleeping
  34. unusual tiredness or weakness
  35. vomiting

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

  1. Burning, crawling, itching, numbness, prickling, "pins and needles", or tingling feelings
  2. difficulty in moving
  3. 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.