Velmanase alfa

What is Velmanase alfa?

• Velmanase alfa (Lamzede) is lysosomal alpha-mannosidase produced by recombinant DNA technology in Chinese Hamster Ovary (CHO) cells.
• The amino acid sequence of the monomeric protein is identical to the naturally occurring human enzyme, alpha-mannosidase.

What are the uses of this medicine?

• Velmanase alfa (Lamzede) is used for the treatment of non-central nervous system manifestations of alpha-mannosidosis in adult and pediatric patients.

How does this medicine work?

• Alpha-mannosidosis is a lysosomal storage disease that results from reduced activity of the enzyme alpha-mannosidase, caused by gene variants in Mannosidase Alpha Class 2B Member 1.
• Alpha-mannosidase catalyzes the degradation of accumulated mannose-containing oligosaccharides.
• The deficiency of alpha-mannosidase causes an intra-lysosomal accumulation of mannose-rich oligosaccharides in various tissues.
• Velmanase alfa-tycv provides an exogenous source of alpha-mannosidase.
• Velmanase alfa-tycv is internalized via binding to the mannose-6-phosphate receptor on the cell surface and transported into lysosomes where it is thought to exert enzyme activity.

Who Should Not Use this medicine ?

• This medicine have no usage limitations.

What drug interactions can this medicine cause?

• Tell your healthcare provider about all the medicines you take, including prescription and over-the-counter medicines, vitamins, and herbal supplements.
• Your doctor may need to change the doses of your medications or monitor you carefully for side effects.

Is this medicine FDA approved?

• Velmanase alfa was approved for medical use in the United States in February 2023.
• Velmanase alfa is the first enzyme replacement therapy approved in the US for the treatment of the non-central nervous system manifestations of alpha-mannosidosis.

How should this medicine be used?

• For females of reproductive potential, verify that the patient is not pregnant prior to initiating treatment.
• Consider pretreating with antihistamines, antipyretics, and/or corticosteroids prior to Lamzede administration.

Recommended dosage:

• Recommended Lamzede dosage is 1 mg/kg (actual body weight) administered once every week as an intravenous infusion.
• If one or more doses are missed, restart the treatment as soon as possible, as long as it is at least 3 days from the next scheduled dose. If it is within 3 days from the next scheduled dose, give only the next dose per schedule.
• In the event of a severe hypersensitivity reaction (including anaphylaxis) or severe infusion-associated reaction (IAR), immediately discontinue Lamzede administration and initiate appropriate medical treatment.

Administration:

• Lamzede (velmanase alfa-tycv) comes as injection in a sterile, preservative-free, white to off-white lyophilized powder with a cake-like appearance for intravenous infusion after reconstitution.
• It is usually given every week.
• Velmanase alfa controls symptoms associated with alpha-mannosidosis but does not cure it.
• Continue to show up for your scheduled infusion appointments unless your doctor tells you not to.

What are the dosage forms and brand names of this medicine?

This medicine is available in fallowing doasage form:

• As injection: 10 mg of velmanase alfa-tycv as a lyophilized powder in a single-dose vial for reconstitution.

This medicine is available in fallowing brand namesː

• Lamzede

What side effects can this medication cause?

The most common side effects of this medicine include:

• Nasopharyngitis
• Pyrexia
• Headache
• Arthralgia
• Acute tonsillitis
• Urinary tract infection
• Eye pruritus
• Gastroenteritis
• Hypersensitivity
• Influenza
• Syncope
• Toothache
• Back pain
• Ear infection

Less common, but serious side effects may include:

• Hypersensitivity Reactions Including Anaphylaxis
• Infusion-Associated Reactions (IARs)

What special precautions should I follow?

• Hypersensitivity reactions including anaphylaxis have been reported in Lamzede-treated patients. Advise the patient and caregiver that reactions related to the infusion may occur during and after Lamzede treatment, including anaphylactic reactions, other serious or severe hypersensitivity reactions, and IARs. Inform the patient and caregiver of the signs and symptoms of hypersensitivity reactions and IARs and to seek medical care should signs and symptoms occur.
• Infusion-associated reactions (IARs) have been reported in Lamzede-treated patients. If severe IARs occur, discontinue Lamzede and initiate appropriate medical treatment.
• Based on findings from animal reproduction studies, Lamzede may cause embryo-fetal harm when administered to a pregnant female. Advise the pregnant female of the potential risk to the fetus. Advise a female patient and caregiver to inform their healthcare provider of a known or suspected pregnancy. Advise a female of reproductive potential to use effective contraception during treatment and for 14 days after the last dose if Lamzede is discontinued.
• There are no data on the presence of velmanase alfa-tycv or its metabolite in either human or animal milk, the effects on the breastfed infant, or the effects on milk production.

What to do in case of emergency/overdose?

• In case of overdose, call the poison control helpline of your country. In the United States, call 1-800-222-1222.

• Overdose related information is also available online at poisonhelp.org/help.
• In the event that the victim has collapsed, had a seizure, has trouble breathing, or can't be awakened, immediately call emergency services. In the United States, call 911.

Can this medicine be used in pregnancy?

• Based on findings from animal reproduction studies, Lamzede may cause embryo-fetal harm when administered to a pregnant female.
• Advise females of reproductive potential to use effective contraception during treatment and for 14 days after the last dose if Lamzede is discontinued.

Can this medicine be used in children?

• The safety and effectiveness of Lamzede for the treatment of alpha-mannosidosis have been established in pediatric patients.

What are the active and inactive ingredients in this medicine?

Active ingredient:

• velmanase alfa-tycv

Inactive ingredients:

• dibasic sodium phosphate, glycine, mannitol and monobasic sodium phosphate.

Who manufactures and distributes this medicine?

Manufactured by:

• Chiesi Farmaceutici S.p.A.
• Via Palermo 26/A, 43122 Parma, Italy

Manufactured at:

• Patheon Italia S.p.A.,
• Ferentino FR, Italy

Manufactured for:

• Chiesi USA, Inc.,
• Cary NC, USA.
• Product of Germany.

• Lamzede is a registered trademark of Chiesi Farmaceutici S.p.A.

What should I know about storage and disposal of this medication?

• Store refrigerated at 2°C to 8°C (36°F to 46°F) in the original carton to protect from light.
• Do not freeze.

• Dailymed label info on Velmanase alfa
• FDA Velmanase alfa

Velmanase alfa Resources
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