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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

What is Avalglucosidase Alpha?

Avalglucosidase Alpha helps replace a missing enzyme in people who have a genetic disorder known as late-onset Pompe disease (also called GAA deficiency) in children over 1 year of age.Avalglucosidase a-l may be used in other ways not mentioned in this guide.

Side effects of Avalglucosidase alfa

If you experience any of the following symptoms of an allergic reaction, seek immediate medical attention: hives; itching; difficulty breathing; swelling in your face, lips, or throat; or hives.

Some reactions can occur immediately after the injection or during the procedure. If you experience:

  • Wheezing, coughing, chest discomfort, difficulty breathing
  • Pale skin, blue lips, or fingernails;
  • You may experience swelling in your tongue.
  • Dizzines, nausea;
  • Is a rash
  • Flushing (a sudden feeling of warmth, redness, or tingling sensation)

If you experience any of the following symptoms, tell your carers right away or seek emergency medical attention. These include itching, rash or redness, chest pain, wheezing or difficulty breathing, swelling on your face or lips, or blue fingernails or lips.

Avalglucosidase alfa may cause the following side effects:

  • Feeling tired, having a headache, or having dizziness?
  • Nausea, vomiting, diarrhoea;
  • Itching, rash, redness;
  • Joint or muscle pain
  • Trouble breathing
  • Numbness, tingling, and burning pain.

There may be other side effects. For medical advice on side effects, call your doctor. The FDA can be contacted at 1-800-FDA-1088 to report side effects.

Warnings

If you experience any of the following symptoms, tell your carers right away or seek emergency medical attention. These include itching, rash or redness, chest pain, wheezing or difficulty breathing, swelling on your face or lips, or blue fingernails or lips.

Prior to using this drug

Tell your doctor about any of the following:

  • Heart disease
  • Lung disease or breathing problems

Inform your doctor of any pregnancy plans or if you plan to get pregnant. Untreated Pompe disease in pregnancy can increase the risk of breathing difficulties and joint or muscular problems. Treatment of Pompe disease can be beneficial and outweigh the risks.Avalglucosidase is a safe product to use while breastfeeding.You may find your name on the Pompe Registry. The purpose of this is to monitor the progression of your disease and the results of your treatment using avalglucosidase.

How do I take Avalglucosidase Alfa?

Avalglucosidase injections are usually given by a healthcare professional once every two weeks.The medicine should be taken slowly, over a period of 4 to 5 hours.Other medications may be prescribed to prevent allergic reactions or serious side effects. Continue to take these medications as prescribed by your doctor.

Info on dosage

Adult dose for Pompe disease:

If you weigh less than 30 kg, 40 mg/kg IV is given every 2 weeks.
Minimum 30 kg: 20 mg/kg per 2 weeks
Comments:
Calculate the dose using the patient's weight.
Use: To treat patients with late-onset Pompe disease (lysosomal alpha-glucosidase deficiency [GAA]).

The usual paediatric dose for Pompe disease is:

One year old or older
Less than 30 kg: IV 40 mg/kg every 2 weeks
At least 30 kg: 20 mg/kg once every two weeks
Comments:
Calculate the dose using the patient's weight.
Use: To treat patients with late-onset Pompe disease (lysosomal GA deficiency).

What happens if I miss the dose?

If you are unable to attend your appointment for avalglucosidase, please call your doctor.

What happens if I overdose?

Call 1-800-222-1222 for poison help or seek immediate medical attention.

Aviod this

If your doctor prescribes any restrictions regarding food, beverage, or activity, follow their instructions.

Interaction with other drug

Avalglucosidase can be affected by other drugs, such as vitamins and herbal supplements. Inform your doctor of all the other medications you take.

 

DRUG STATUS

Availability

Prescription only

Pregnancy & Lactation

CSA Schedule*
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