Velmanase Alfa Dosage

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

Applies to the following strengths: tycv 10 mg

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.