Version May 2024
Patients treated with velmanase alfa have experienced hypersensitivity reactions, including anaphylaxis. Appropriate medical support measures, including cardiopulmonary resuscitation equipment, should be readily available during velmanase alfa administration. If a severe hypersensitivity reaction (eg, anaphylaxis) occurs, discontinue velmanase alfa immediately and initiate appropriate medical treatment. In patients with severe hypersensitivity reaction, a desensitization procedure to velmanase alfa may be considered.
Alpha-mannosidosis, noncentral nervous system manifestations: Note: Consider pretreatment with antihistamines, antipyretics, and/or corticosteroids to decrease risk of hypersensitivity and infusion-associated reactions (IARs); use actual body weight for dosing:
Infants, Children, and Adolescents: IV infusion: 1 mg/kg once weekly (Ref).
Dosing adjustment for toxicity:
Hypersensitivity reactions or IARs:
Mild to moderate: Hold infusion for 15 to 30 minutes and initiate appropriate medical care; then restart infusion at 25% to 50% of recommended rate. Monitor and follow appropriate next steps based on symptoms:
If symptoms subside after holding the infusion or decreasing the infusion rate : Resume infusion at 25% to 50% of recommended rate; if tolerated, increase infusion rate in increments of 25% of recommended rate until recommended infusion rate is reached.
If symptoms persist after holding the infusion or decreasing the infusion rate : Stop infusion and monitor; if symptoms persist, discontinue infusion and consider reinitiating infusion within 7 to 14 days at 25% to 50% of recommended rate with appropriate pretreatment.
Severe: Discontinue infusion immediately and administer appropriate medical treatment. Consider risks and benefits of therapy readministration. If therapy is continued, consider desensitization measures (for hypersensitivity reactions).
There are no dosage adjustments provided in the manufacturer's labeling.
There are no dosage adjustments provided in the manufacturer's labeling.
(For additional information see "Velmanase alfa: Drug information")
Note: Consider premedication with antihistamines, antipyretics, and/or corticosteroids.
Alpha-mannosidosis: IV: Dosing based on actual body weight: 1 mg/kg once weekly (Ref).
Missed dose: If 1 or more doses are missed and the next scheduled dose is ≥3 days away, restart treatment as soon as possible, then resume usual dosing schedule. If 1 or more doses are missed and next scheduled dose is within 3 days, only administer next scheduled dose, then resume usual dosing schedule.
There are no dosage adjustments provided in the manufacturer's labeling.
There are no dosage adjustments provided in the manufacturer's labeling.
Patients treated with velmanase alfa may commonly experience hypersensitivity reaction, including anaphylaxis and severe hypersensitivity reaction requiring medical treatment. Symptoms may include cyanosis, hypotension, emesis, urticaria, erythema of skin, facial swelling, fever, and tremor.
Mechanism: Non–dose-related; immediate allergic hypersensitivity reactions (eg, anaphylaxis) are typically IgE-mediated; delayed-type reactions may be T-cell mediated (Ref)
Risk factors:
• Presence of anti–velmanase alfa-tycv antibodies (anti-drug antibodies)
• Higher infusion rates
Infusion-related reaction, including fever, chills, erythema of skin, vomiting, cough, urticaria, skin rash, and conjunctivitis, have been reported with velmanase alfa.
Onset: Rapid; occurs during or shortly after infusion; first or subsequent reactions may occur several weeks into therapy (Ref)
Risk factors:
• Presence of anti–velmanase alfa-tycv antibodies (anti-drug antibodies)
• Higher infusion rates
The following adverse drug reactions are derived from product labeling unless otherwise specified. Reported adverse reactions are for adults and pediatrics.
Frequency not defined:
Cardiovascular: Syncope
Dermatologic: Erythema of skin, excoriation of skin, skin rash
Endocrine & metabolic: Weight gain
Gastrointestinal: Gastroenteritis, upper abdominal pain
Genitourinary: Urinary tract infection
Hematologic & oncologic: Bruise, Henoch-Schönlein purpura
Hypersensitivity: Facial swelling, hypersensitivity reaction (including anaphylaxis and severe hypersensitivity reaction), infusion-related reaction
Immunologic: Antibody development (including neutralizing antibodies)
Infection: Influenza
Nervous system: Chills, headache, hyperthermia, seizure
Neuromuscular & skeletal: Arthralgia, back pain
Ophthalmic: Conjunctivitis, eye pruritus
Renal: Acute kidney injury
Respiratory: Cough, oropharyngeal pain, rhinitis, upper respiratory tract infection
Miscellaneous: Fever
There are no contraindications listed in the manufacturer’s labeling.
Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Solution Reconstituted, Intravenous [preservative free]:
Lamzede: Velmanase alfa-tycv 10 mg (1 ea)
No
Solution (reconstituted) (Lamzede Intravenous)
10 mg (per each): $5,040.00
Disclaimer: A representative AWP (Average Wholesale Price) price or price range is provided as reference price only. A range is provided when more than one manufacturer's AWP price is available and uses the low and high price reported by the manufacturers to determine the range. The pricing data should be used for benchmarking purposes only, and as such should not be used alone to set or adjudicate any prices for reimbursement or purchasing functions or considered to be an exact price for a single product and/or manufacturer. Medi-Span expressly disclaims all warranties of any kind or nature, whether express or implied, and assumes no liability with respect to accuracy of price or price range data published in its solutions. In no event shall Medi-Span be liable for special, indirect, incidental, or consequential damages arising from use of price or price range data. Pricing data is updated monthly.
Note: Administer in setting with medical equipment readily available for the management of anaphylaxis or infusion-associated reactions. Consider pretreatment with antihistamines, antipyretics, and/or corticosteroids.
IV infusion: Use low protein binding, 0.2-micron, inline filter; do not shake syringe(s); administration rate based on weight:
Weight <50 kg: Administer over ≥60 minutes.
Weight ≥50 kg: Maximum infusion rate: 25 mL/hour.
Following administration of last syringe, replace dosage syringe with 20 mL syringe filled with NS; continue infusion of 10 mL NS to infuse remaining dose in infusion set.
Missed dose: If one or more doses are missed and the next scheduled dose is ≥3 days away, restart treatment as soon as possible. If one or more doses are missed and the next scheduled dose is within 3 days, administer next dose as scheduled.
IV: Do not shake syringe(s). Consider premedication with antihistamines, antipyretics, and/or corticosteroids. Administer via infusion set equipped with pump and low protein binding, 0.2 micron in-line filter. To control protein load, rate of administration is dependent on patient weight:
Actual body weight <50 kg : Administer over at least 60 minutes.
Actual body weight ≥50 kg : Maximum infusion rate: 25 mL/hour.
Following administration of last syringe, replace dosage syringe with 20 mL syringe filled with NS; continue infusion of 10 mL NS to infuse remaining dose in infusion set.
Store intact vials refrigerated at 2°C to 8°C (36°F to 46°F) in the original carton to protect from light; do not freeze. If not administered immediately, may store reconstituted vials at 2°C to 8°C (36°F to 46°F) for up to 24 hours (including infusion time). Do not freeze; protect from light. Infuse reconstituted solution within 10 hours after removal from refrigeration (including infusion time); discard if not used within 10 hours. Reconstituted solution must be completely infused within 24 hours from time of preparation.
Treatment of noncentral nervous system manifestations of alpha-mannosidosis (FDA approved in pediatric patients [age not specified] and adults).
Velmanase alfa may be confused with velaglucerase alfa.
None known.
There are no known significant interactions.
Evaluate pregnancy status prior to use; verify the patient is not pregnant prior to treatment initiation in patients who could become pregnant.
Patients who may become pregnant should use effective contraception during therapy and for 14 days after the last dose of velmanase alfa.
Based on data from animal reproduction studies, in utero exposure to velmanase alfa may cause fetal harm.
Pregnancy test in patients who can become pregnant (prior to the initiation of therapy and as deemed clinically necessary during treatment and up to 14 days after stopping therapy), signs and symptoms of hypersensitivity reactions or infusion-associated reactions.
Velmanase alfa binds to extracellular mannose-6-phosphate receptors and is transported to lysosomes, where it provides an exogenous source of alpha mannosidase. Alpha mannosidase degrades mannose-containing oligosaccharides; lack of this enzyme leads to the accumulation of mannose-rich oligosaccharides in tissue, which causes clinical symptoms associated with alpha-mannosidosis lysosomal disease (Borgwardt 2018; manufacturer’s labeling).
Distribution: Vd: 276 mL/kg.
Metabolism: Metabolized into small peptides via catabolic pathways.
Half-life elimination: 33.6 hours.
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