Generic name: vestronidase alfa [ ves-tron-i-dase-al-fa ]
Drug class: Lysosomal enzymes
Dosage form: intravenous solution (2 mg/ml)
Availability: Prescription only
Pregnancy & Lactation: Risk data available
Brand names: Mepsevii
What is Vestronidase alfa?
Vestronidase alfa contains an enzyme that occurs naturally in the body in healthy people. Some people lack this enzyme because of a genetic disorder. Vestronidase alfa helps replace this missing enzyme in such people.
Vestronidase alfa is used to treat some of the symptoms of a genetic condition called mucopolysaccharidosis VII (MYOO-koe-pol-ee-SAK-a-rye-DOE-sis Seven) or MPS VII, also called Sly syndrome.
MPS VII is a metabolic disorder in which the body lacks the enzyme needed to break down certain natural substances. These substances can build up in the body, causing enlarged organs, abnormal bone structure, changes in facial features, breathing problems, heart problems, vision or hearing loss, and changes in mental or physical abilities.
Vestronidase alfa may improve walking ability in people with this condition. However, this medication is not a cure for MPS VII.
Vestronidase alfa may also be used for purposes not listed in this medication guide.
Warnings
An allergic reaction may occur during or shortly after infusion of this medicine. Tell your caregivers or get emergency medical help right away if you have any signs of a severe allergic reaction, such as sweating, trouble breathing, pale skin, blue lips or fingernails, and feeling like you might pass out.
How should I take Vestronidase alfa
Vestronidase alfa is given as an infusion into a vein. A healthcare provider will give you this injection.
vestronidase alfa is usually given once every 2 weeks. Vestronidase alfa must be given slowly, and the infusion can take about 4 hours to complete.
About 30 to 60 minutes before each injection, you will be given other medications to help prevent a serious allergic reaction.
Tell your caregivers if you feel any burning, pain, or swelling around the IV needle when vestronidase alfa is injected.
You will be watched closely for at least 60 minutes after receiving vestronidase alfa, to make sure you do not have an allergic reaction.
Vestronidase alfa doses are based on weight. Your dose needs may change if you gain or lose weight.
Dosing information
Usual Adult Dose for Mucopolysaccharidosis Type VII:
4 mg/kg IV infusion once every 2 weeks
Use: For the treatment of Mucopolysaccharidosis VII (MPS VII) also known as Sly syndrome
Usual Pediatric Dose for Mucopolysaccharidosis Type VII:
4 mg/kg IV infusion once every 2 weeks
Use: For the treatment of Mucopolysaccharidosis VII (MPS VII) also known as Sly syndrome
Before Taking
Tell your doctor if you have ever had:
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a lung disease or breathing problems.
Tell your doctor if you are pregnant or breastfeeding.
What happens if I miss a dose?
Call your doctor for instructions if you miss an appointment for your vestronidase alfa injection.
What happens if I overdose?
Seek emergency medical attention or call the Poison Help line at 1-800-222-1222.
What should I avoid while using Vestronidase alfa?
Follow your doctor's instructions about any restrictions on food, beverages, or activity.
Vestronidase alfa side effects
Get emergency medical help if you have signs of an allergic reaction: hives; difficult breathing; swelling of your face, lips, tongue, or throat.
An allergic reaction may occur during or shortly after infusion of vestronidase alfa. Tell your caregivers or get emergency medical help right away if you have any signs of a severe allergic reaction, such as:
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feeling like you might pass out, even while lying down;
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sweating, cold or clammy skin;
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feeling short of breath;
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pale skin; or
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blue lips or fingernails.
Common side effects of vestronidase alfa may include:
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diarrhea;
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swelling in your hands or feet;
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rash, itching; or
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swelling or irritation around the IV needle.
This is not a complete list of side effects and others may occur. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.
See more: Vestronidase alfa Side Effects