Avalglucosidase Alfa

Avalglucosidase Alfa is a prescription medication used for the treatment of late-onset Pompe disease.

• Avalglucosidase Alfa is available under the following different brand names: Nexviazyme, Avalglucosidase Alfa-ngpt

Common side effects of Avalglucosidase Alfa include:

• headache
• dizziness
• tiredness
• nausea
• vomiting
• diarrhea
• itching
• rash
• redness
• muscle or joint pain
• trouble breathing
• numbness, tingling, or burning pain

Serious side effects of Avalglucosidase Alfa include:

• hives
• difficulty breathing
• swelling of the face, lips, tongue, or throat
• severe dizziness
• redness or tingling
• chest discomfort
• itching
• wheezing
• cough
• chest discomfort
• pale skin
• blue lips or fingernails
• swelling in your tongue
• trouble swallowing
• nausea
• rash
• flushing (sudden warmth, redness, or tingly feeling)

Rare side effects of Avalglucosidase Alfa include:

• none

Seek medical care or call 911 at once if you have the following serious side effects:

• Severe headache, confusion, slurred speech, arm or leg weakness, trouble walking, coordination loss, unsteady, very stiff muscles, high fever, profuse sweating, or tremors
• Serious eye symptoms such as sudden vision loss, blurred vision, tunnel vision, eye pain or swelling, or seeing halos around lights
• Serious heart symptoms include fast, irregular, or pounding heartbeats; fluttering in the chest; shortness of breath; sudden dizziness, light-headedness, or passing out

This is not a complete list of side effects and other serious side effects or health problems that may occur because of the use of this drug. Call your doctor for medical advice about serious side effects or adverse reactions. You may report side effects or health problems to FDA at 1-800-FDA-1088.

Adult and pediatric dosage

Injection, lyophilized powder for reconstitution

• 100 mg/vial

Pompe disease

Adult dosage

• Dose based on actual body weight
• Weighing 30 kg or more: 20 mg/kg IV every 2 weeks

Pediatric dosage

• Dose based on actual body weight.
• Children weighing below 30 kg: 40 mg IV every 2 weeks
• Children weighing 30 kg or more: 20 mg/kg IV every 2 weeks

Dosage Considerations – Should be Given as Follows: 

• See “Dosages”

If your medical doctor is using this medicine to treat your pain, your doctor or pharmacist may already be aware of any possible drug interactions and may be monitoring you for them. Do not start, stop, or change the dosage of any medicine before checking with your doctor, healthcare provider, or pharmacist first.

• Avalglucosidase Alfa has no noted severe interactions with any other drugs.
• Avalglucosidase Alfa has no noted serious interactions with any other drugs.
• Avalglucosidase Alfa has no noted moderate interactions with any other drugs.
• Avalglucosidase Alfa has no noted minor interactions with any other drugs.

This information does not contain all possible interactions or adverse effects. Visit the RxList Drug Interaction Checker for any drug interactions. Therefore, before using this product, tell your doctor or pharmacist about all the products you use. Keep a list of all your medications with you and share this information with your doctor and pharmacist. Check with your healthcare professional or doctor for additional medical advice or health questions or concerns.

Contraindications

• None

Effects of drug abuse

• None

Short-Term Effects

• See “What Are Side Effects Associated with Using Avalglucosidase Alfa?”

Long-Term Effects

• See “What Are Side Effects Associated with Using Avalglucosidase Alfa?”

Cautions

• Also, see Black Box Warnings and Dosage Modifications
• Severe hypersensitivity reaction (eg, anaphylaxis) reported; if this occurs, discontinue immediately and administer appropriate medical treatment; may consider desensitization
• IAR reported; immediate discontinuation required if severe; milder reactions may be managed by slowing infusion rate; antihistamines, antipyretics, and/or corticosteroids may be administered before the infusion to reduce risk; some patients have been rechallenged using slower infusion rates at a dose lower than recommended dose; once a patient tolerates infusion, the dose may be increased to reach a recommended approved dose
• Risk for acute cardiorespiratory failure reported in patients susceptible to fluid overload and those with acute underlying respiratory illness or compromised cardiac/respiratory function; monitor vital signs more frequently
• Patients and their caregivers should be encouraged to participate in the Pompe Registry and advised that their participation is voluntary and may involve long-term follow-up; for more information regarding the registry program, visit www.registrynxt.com or call 1-800-745-4447, extension 15500

Pregnancy and Lactation

• Data are insufficient to evaluate the drug-associated risk for major birth defects, miscarriage, or adverse maternal or fetal outcomes; however, available data from postmarketing reports and published case reports on Alglucosidase alfa (another hydrolytic lysosomal glycogen-specific enzyme replacement therapy) use in pregnant women have not identified drug-associated risk for adverse pregnancy outcomes
• Pregnant women exposed, or their healthcare providers should report exposure by calling 1-800-745-4447, extension 15500
• Clinical considerations
  • Untreated Pompe disease may worsen respiratory and musculoskeletal symptoms in pregnant women
• Lactation
  • Data are unavailable regarding the presence of human or animal milk, effects on breastfed infants, or effects on milk production
  • Available published literature suggests the presence of Alglucosidase alfa (another hydrolytic lysosomal glycogen-specific enzyme replacement therapy) in human milk
  • Lactating women exposed, or their healthcare providers, should report exposure by calling 1-800-745-4447, extension 15500