Velmanase Alfa Dosage

Medically reviewed by Drugs.com. Last updated on Oct 30, 2023.

Usual Adult Dose for Mannosidosis

1 mg/kg (actual weight) via IV infusion once a week

Comments:

• Consider pre-treatment with antihistamines, antipyretics, and/or corticosteroids.
• See Other Comments/Administration Advice for recommended dilution volume, infusion rates, and recommendations for hypersensitivity reactions.

Use: For the treatment of non-central nervous system manifestations of alpha-mannosidosis in adult and pediatric patients

Usual Pediatric Dose for Mannosidosis

1 mg/kg (actual weight) via IV infusion once a week

Comments:

• Consider pre-treatment with antihistamines, antipyretics, and/or corticosteroids.
• See Other Comments/Administration Advice for recommended dilution volume, infusion rates, and recommendations for hypersensitivity reactions.

Use: For the treatment of non-central nervous system manifestations of alpha-mannosidosis in adult and pediatric patients

Renal Dose Adjustments

Data not available

Liver Dose Adjustments

Data not available

Dose Adjustments

DOSE MODIFICATIONS FOR HYPERSENSITIVITY OF INFUSION-ASSOCIATED REACTIONS:
For severe hypersensitivity or infusion-associated reactions:

• Immediately discontinue drug and initiate appropriate medical treatment.
• Consider the risk/benefit of readministering the drug after severe hypersensitivity reactions (including anaphylaxis).
• Patients may be rechallenged at slower infusion rates with closer monitoring; see dose adjustment recommendations for mild to moderate reactions.

For mild to moderate hypersensitivity or infusion-associated reactions:

• Consider temporarily holding infusion for 15 to 30 minutes, slowing the infusion rate by 25% to 50% of the recommended rate, and initiating appropriate medical treatment.
• If symptoms subside after holding or slowing infusion, resume at 25% to 50% of the recommended rate, increasing by increments of 25% as tolerated until recommended infusion rate is reached; monitor patient closely.
• If symptoms persist despite holding or slowing infusion, stop and monitor patient.
• If symptoms still persist, discontinue infusion, and consider reinitiating infusion in 7 to 14 days at 25% to 50% of the recommended rate with pretreatment.

Precautions

US BOXED WARNING: SEVERE HYPERSENSITIVITY REACTIONS

• Severe hypersensitivity reactions, including anaphylaxis, were reported in some patients during treatment with this drug. During administration, healthcare providers should be prepared to provide medical support measures; cardiopulmonary resuscitation equipment should be readily available. Patients should be observed closely during and after infusion; discontinue drug immediately if symptoms of severe hypersensitivity occur. Desensitization procedures may be considered in some patients with severe hypersensitivity reactions.

CONTRAINDICATIONS: None

Safety and efficacy have not been established in patients younger than 3 years.

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration advice:
For IV infusion:

• The manufacturer product information should be consulted prior to product administration.
• Administer diluted solution using a low-protein-binding infusion set with a pump and low-protein-binding 0.2 micrometer in-line filter.
• Patients weighing less than 49 kg: Infusion volume should be delivered over a minimum of 60 minutes; total volume is based on patient's actual body weight.
• Patients weighing 50 kg or more: Infusion volume should be delivered at a maximum rate of 25 mL/hour to control protein load.; total volume is based on patient's actual body weight.
• Infusion rates may be slowed, temporarily stopped, or discontinued in the event of hypersensitivity reactions.

Storage requirements:

• Do not freeze or shake
• Protect from light during refrigeration
• Concentrated vials: Store at 2C to 8C (36F to 46F) in original carton
• Reconstituted vial:
• If not used immediately, may store for up to 24 hours inclusive of infusion time at 2C to 8C (36F to 46F).
• Once removed from refrigerator for use the vial must be infused within 10 hours, inclusive of total infusion time; discard if not used within 10 hours.

Reconstitution/preparation techniques:

• The manufacturer product information should be consulted prior to product reconstitution.
• Drug should be reconstituted with Sterile Water for Injection; final vial concentration will be 2 mg/mL; solution should be clear to slightly opalescent, occasionally containing small proteinaceous strands.
• Avoid inversion or agitation of product during preparation to prevent foaming.

General:

• Closely observe patients during and after administration; infusions should occur in a setting equipped to treat anaphylaxis.
• Clinical studies did not include patients 65 years or older so it is unknown whether they respond differently.

Monitoring:

• Hypersensitivity: Signs/symptoms of allergic reactions and/or infusion-related reactions (during and after infusion)

Patient advice:

• Patients and caregivers should understand that infusion-related and hypersensitivity reactions including anaphylaxis may occur with therapy.
• Patients and caregivers should be informed of the signs and symptoms of anaphylaxis and hypersensitivity reactions and should be instructed to seek medical care should signs and symptoms occur.
• Female patients of reproductive potential should understand that this drug could cause fetal harm and should use effective contraception during treatment and for 14 days after last dose.

See also:

Further information

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

Medical Disclaimer