Mepsevii

You should not receive Mepsevii if you are allergic to it.

To make sure Mepsevii is safe for you, tell your doctor if you have ever had:

• a lung disease or breathing problems.

It is not known whether this medicine will harm an unborn baby. Tell your doctor if you are pregnant or plan to become pregnant.

It is not known whether Mepsevii passes into breast milk or if it could affect the nursing baby. Tell your doctor if you are breast-feeding.

Mepsevii is injected into a vein through an IV. A healthcare provider will give you this injection.

This medicine is usually given once every 2 weeks.

Mepsevii must be given slowly, and the IV infusion can take up to 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 Mepsevii is injected.

You will be watched closely for at least 60 minutes after receiving Mepsevii, to make sure you do not have an allergic reaction.

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 this medicine. Tell your caregivers or get emergency medical help right away if you have any signs of a severe allergic reaction, such as:

• feeling like you might pass out, even while lying down;

• sweating, cold or clammy skin;

• feeling short of breath;

• pale skin; or

• blue lips or fingernails.

Common side effects may include:

• diarrhea;

• swelling in your hands or feet;

• rash, itching; or

• 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.

Other drugs may interact with Mepsevii, including prescription and over-the-counter medicines, vitamins, and herbal products. Tell each of your health care providers about all medicines you use now and any medicine you start or stop using.

• Your doctor or pharmacist can provide more information about Mepsevii.

Copyright 1996-2012 Cerner Multum, Inc. Version: 1.01.

Last reviewed: December 05, 2017
Date modified: January 03, 2018

• If you have an allergy to this medicine or any part of Mepsevii.
• If you are allergic to any drugs like this one, any other drugs, foods, or other substances. Tell your doctor about the allergy and what signs you had, like rash; hives; itching; shortness of breath; wheezing; cough; swelling of face, lips, tongue, or throat; or any other signs.

This medicine may interact with other drugs or health problems.

Tell your doctor and pharmacist about all of your drugs (prescription or OTC, natural products, vitamins) and health problems. You must check to make sure that it is safe for you to take this medicine with all of your drugs and health problems. Do not start, stop, or change the dose of any drug without checking with your doctor.

Applies to vestronidase alfa: intravenous solution

General

The most frequently reported side effects were infusion site extravasation, diarrhea, rash, anaphylaxis, infusion site swelling, peripheral swelling, and pruritus.[Ref]

Immunologic

Very common (10% or more): Anti-drug antibodies developed (78%), neutralizing antibodies developed (55.6%), anaphylaxis (16.67%)[Ref]

Nervous system

Frequency not reported: Febrile convulsion[Ref]

A week 66 of treatment, 1 patient experiences a febrile convulsion during infusion of a 4 mg/kg dose. The infusion was stopped and the patient was given anticonvulsants, antipyretics, and antibiotics and the adverse reaction resolved. The patient was subsequently rechallenged without recurrence and continued on treatment with this drug.[Ref]

Dermatologic

Very common (10% or more): Rash (25%)
Common (1% to 10%): Pruritus[Ref]

Gastrointestinal

Very common (10% or more): Diarrhea (25%)[Ref]

Other

Common (1% to 10%): Peripheral swelling[Ref]

Local

Very common (10% or more): Infusion site extravasation (33.3%)
Common (1% to 10%): Infusion site swelling[Ref]

Some side effects of vestronidase alfa may not be reported. Always consult your doctor or healthcare specialist for medical advice. You may also report side effects to the FDA.

4 mg/kg IV infusion once every 2 weeks

Use: For the treatment of Mucopolysaccharidosis VII (MPS VII) also known as Sly syndrome

Data not available

US BOXED WARNING:
-ANAPHYLAXIS: Anaphylaxis has occurred with administration of this drug, as early as the first dose, therefore, appropriate medical support should be readily available when this drug is administered. Closely observe patients during and for 60 minutes after infusion. Immediately discontinue the infusion if the patient experiences anaphylaxis.

Consult WARNINGS section for additional precautions.

Contraindications

None.1

Warnings/Precautions

Anaphylaxis

Anaphylaxis to vestronidase alfa-vjbk was reported in 2 of 20 patients in the clinical program. These reactions occurred during vestronidase alfa-vjbk infusion and were observed as early as the first dose of vestronidase alfa-vjbk for one patient. Manifestations included respiratory distress, cyanosis, decreased oxygen saturation, and hypotension. The two patients with anaphylaxis to vestronidase alfa-vjbk during the clinical trials had one occurrence each and tolerated subsequent infusions of vestronidase alfa-vjbk, without recurrence.1

Anaphylaxis can be life-threatening. Vestronidase alfa-vjbk should be administered under the supervision of a healthcare professional with the capability to manage anaphylaxis. Patients should be observed for 60 minutes after vestronidase alfa-vjbk administration. If severe systemic reactions occur, including anaphylaxis, immediately discontinue the vestronidase alfa-vjbk infusion and provide appropriate medical treatment. Prior to discharge, inform patients of the signs and symptoms of anaphylaxis and instruct them to seek immediate medical care if symptoms occur. Consider the risks and benefits of re-administering vestronidase alfa-vjbk following anaphylaxis.1

Specific Populations

Risk Summary: There are no available data on vestronidase alfa-vjbk use in pregnant women to determine a drug-associated risk of adverse developmental outcomes. In animal reproduction studies, vestronidase alfa-vjbk administered intravenously to pregnant rats and rabbits during the period of organogenesis showed no maternal toxicity or adverse developmental outcomes at doses causing maternal serum exposures (AUC) up to 1.6 and 10 times, respectively for rats and rabbits, the exposure at the recommended human dose. 1

The estimated background risk of major birth defects and miscarriage for the indicated population is unknown. All pregnancies have a background risk of birth defect, loss, or other adverse outcomes. In the U.S. general population, the estimated background risk of major birth defects and miscarriage in clinically recognized pregnancies is 2 to 4% and 15 to 20%, respectively.1

Animal Data: In animal reproduction studies, vestronidase alfa-vjbk administered intravenously to pregnant rats (once a week) and rabbits (once every 3 days) during the period of organogenesis showed no adverse developmental outcomes at doses up to 20 mg/kg. The 20 mg/kg dose in rats and rabbits provides approximately 1.6 and 10 times the human exposure (AUC) of 57.9 hr•mcg/mL at the 4 mg/kg dose administered once every other week, respectively. 1

There are no data on the presence of vestronidase alfa-vjbk in either human or animal milk, the effects on the breastfed infant, or the effects on milk production. The developmental and health benefits of breastfeeding should be considered along with the mother’s clinical need for vestronidase alfa-vjbk and any potential adverse effects on the breastfed infant from vestronidase alfa-vjbk or from the underlying maternal condition.1

The safety and effectiveness of vestronidase alfa-vjbk have been established in pediatric patients less than 18 years of age.1

Clinical trials of vestronidase alfa-vjbk did not include any patients aged 65 and over. It is not known whether elderly patients respond differently from younger patients.1

Common Adverse Effects

Most common adverse reactions (≥1 patient) are: infusion site extravasation, diarrhea, rash, anaphylaxis, infusion site swelling, peripheral swelling, and pruritus.)1

Specific Drugs

It is essential that the manufacturer's labeling be consulted for more detailed information on interactions with this drug, including possible dosage adjustments. Interaction highlights:

Please see product labeling for drug interaction information.

Follow your doctor's instructions about any restrictions on food, beverages, or activity.

Other drugs may interact with vestronidase alfa, including prescription and over-the-counter medicines, vitamins, and herbal products. Tell each of your health care providers about all medicines you use now and any medicine you start or stop using.