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Avalglucosidase Alfa Dosage

Medically reviewed by Drugs.com. Last updated on Sep 10, 2021.

Applies to the following strengths: ngpt 100 mg

Usual Adult Dose for:

Usual Pediatric Dose for:

Additional dosage information:

Usual Adult Dose for Pompe disease

Weight: 30 kg or greater: 20 mg/kg IV infusion every 2 weeks
Weight: Less than 30 kg: 40 mg/kg IV infusion every 2 weeks

Initial infusion rate is 1 mg/kg/hour; gradually increase infusion rate every 30 minutes if there are no signs of infusion-associated reactions

Comments:

  • Consider pretreating with antihistamines, antipyretics, and/or corticosteroids.
  • Weight based dosing is based on actual body weight.

Use: For the treatment of patients with late-onset Pompe disease.

Usual Pediatric Dose for Pompe disease

1 year or older:

  • Weight: 30 kg or greater: 20 mg/kg IV infusion every 2 weeks
  • Weight: Less than 30 kg: 40 mg/kg IV infusion every 2 weeks

Initial infusion rate is 1 mg/kg/hour; gradually increase infusion rate every 30 minutes if there are no signs of infusion-associated reactions

Comments:
  • Consider pretreating with antihistamines, antipyretics, and/or corticosteroids.
  • Weight based dosing is based on actual body weight.

Use: For the treatment of patients 1 year of age and older with late-onset Pompe disease.

Renal Dose Adjustments

Data not available

Liver Dose Adjustments

Data not available

Dose Adjustments

Severe Hypersensitivity Reaction or Severe Infusion Associated Reaction:

  • Immediately discontinue and initiate appropriate medical treatment

Mild to Moderate Hypersensitivity or Moderate Infusion Associated Reaction:
  • Temporarily hold or slow the infusion rate and initiate appropriate medical treatment
  • If symptoms persist despite temporarily holding the infusion, wait at least 30 minutes for symptoms to resolve before deciding to stop the infusion for the day
  • If symptoms subside, resume infusion at half the rate for 30 minutes, then, increase the infusion by 50% for 15 to 30 minutes; if symptoms do not recur, may resume infusion rate at which reaction occurred with subsequent increases in a stepwise manner as tolerated.

Precautions

US BOXED WARNINGS: SEVERE HYPERSENSITIVITY REACTIONS, INFUSION ASSOCIATED REACTIONS, and RISK OF ACUTE CARDIORESPIRATORY FAILURE IN SUSCEPTIBLE PATIENTS

  • Hypersensitivity Reactions Including Anaphylaxis: Patients treated with this drug have experienced life-threatening hypersensitivity reactions, including anaphylaxis. Appropriate medical support measures, including cardiopulmonary resuscitation equipment, should be readily available during administration. If a severe hypersensitivity reaction (e.g., anaphylaxis) occurs, immediately discontinue and initiate appropriate medical treatment. In patients with severe hypersensitivity reaction, a desensitization procedure may be considered.
Infusion-Associated Reactions (IARs): Patients treated have experienced severe IARs. If severe IARs occur, consider immediate discontinuation and initiation of appropriate medical treatment; consider the benefits and risks of readministering this drug following severe IARs. Patients with an acute underlying illness at the time of 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 Patients: Patients 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 infusion. More frequent monitoring of vitals should be performed during infusion in such patients.

CONTRAINDICATIONS: None

Safety and efficacy have not been established in patients younger than 1 year of age.

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration advice:

  • Administer via IV infusion using an in-line low protein binding, 0.2 micrometer filter; consider pretreatment with antihistamines, antipyretics, and/or corticosteroids
  • Initial Infusion Rate:1 mg/kg/hour; gradually increase every 30 minutes if there are no signs of IARs, then
  • For a Dose of 20 mg/kg: Initial and Subsequent Infusions: Gradually increase the infusion rate every 30 minutes in each of the following three steps: 3 mg/kg/hour, 5 mg/kg/hour, and then 7 mg/kg/hour; then, maintain the infusion rate at 7 mg/kg/hour until the infusion is complete; approximate total infusion duration is 4 hours to 5 hours
  • For a Dose of 40 mg/kg: Initial Infusion: Gradually increase the infusion rate every 30 minutes in each of the following three steps: 3 mg/kg/hour, 5 mg/kg/hour, and then 7 mg/kg/hour; then, maintain the infusion rate at 7 mg/kg/hour until the infusion is complete (4-step process). The approximate total infusion duration is 7 hours. Subsequent Infusions: Continue with the 4-step process or use a 5-step process: 3 mg/kg/hour, 6 mg/kg/hour, 8 mg/kg/hour, and then 10 mg/kg/hour; maintain the infusion rate at 10 mg/kg/hour until the infusion is complete. The approximate total 5¬ step infusion duration is 5 hours

See DOSAGE ADJUSTMENTS for modifications due to hypersensitivity reactions or IARs

Storage requirements:
  • Reconstituted solution: May store refrigerated for up to 24 hours (36F to 46F [2C to 8C]); do not freeze
  • Diluted solution must be infused within 9 hours after removal from the refrigerator
  • Once diluted solution is removed from the refrigerator, it should not be placed back in the refrigerator
  • Discard diluted solution if refrigerated for more than 24 hours or if the diluted solution is not able to be completely infused within 9 hours after removal from the refrigerator

Reconstitution/preparation techniques:
  • Must be reconstituted and diluted prior to use
  • Allow vials to reach room temperature (approximately 30 minutes) prior to reconstitution
  • Reconstitute each vial with 10 mL of sterile water for injection by slowing adding diluent down the inside of the vial and not directly onto lyophilized powder; avoid forceful impact of diluent on lyophilized powder and avoid foaming
  • Allow solution to dissolve; do not invert, swirl, or shake
  • Diluting the Reconstituted Solution: Slowly withdraw reconstituted solution and slowly add to infusion bag of D5W; avoid foaming or agitation of the infusion bag and avoid adding air to the infusion bag
  • Contents of infusion bag can be mixed by gently inverting or massaging the infusion bag; do not shake
  • Consult manufacturer's product information for additional information including a table with total infusion volumes according to patient weight

IV compatibility: Do not infuse with other products

General:
  • A Pompe Registry has been established to better understand the variability and progression of Pompe disease, and to continue to monitor and evaluate long-term effects of this drug; 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.

Monitoring:
  • Monitor for IARs and hypersensitivity reactions

Patient advice:
  • Patients/caregivers should be instructed to read the approved patient labeling.
  • Patients/caregivers should be advised that reactions related to the infusion may occur during and after treatment and immediate medical care should be sought if signs and symptoms occur.
  • Patients/caregivers should be aware that the manufacturer of this drug has established registries to monitor and evaluate effects of this drug; participation is voluntary.

Further information

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