This medication is used to treat a certain inherited disorder (Fabry disease) that causes buildup of a certain fat substance (Gb3) in some parts of your body. If this condition gets worse, it can lead to kidney disease, heart attack, or stroke. Pegunigalsidase alfa works by replacing an enzyme that your body lacks, which helps break down the fat substance Gb3 in your body. This can help to lessen the buildup of Gb3 in the kidneys and blood.

This medication is given by slow injection into a vein as directed by your doctor, usually every 2 weeks. The injection is given by a health care professional. The dosage is based on your medical condition, weight, and response to treatment.

Infusion reactions may happen during the infusion of this drug and for up to 24 hours afterward. Your doctor may prescribe other medications (such as acetaminophen, antihistamine, corticosteroids) before each treatment to help prevent these side effects. Tell your doctor right away if you have any symptoms of infusion reactions such as chills, flushing, shaking, cold symptoms (such as sneezing, runny/stuffy nose), nausea, vomiting, diarrhea, dizziness, numb/tingling skin, shooting/stabbing pain, muscle pain, shortness of breath, slow heartbeat, headache, or chest/abdominal pain. Your doctor may give you other medications to help with these side effects or may slow down or stop your treatment for some time.

If you are using this medication at home, learn all preparation and usage instructions in the product package and from your health care professional. If any of the information is unclear, consult your doctor or pharmacist. Remove this medication from the refrigerator and allow the medication and mixing solution to warm to room temperature before mixing. Do not shake the mixed drug. Doing so can make it work less well.

Use this medication regularly to get the most benefit from it. To help you remember, mark your calendar with a reminder.

Tell your doctor if your condition does not get better or if it gets worse.

See also Warning and How to Use sections.

Tiredness or back pain may occur. If either of these effects lasts or gets worse, tell your doctor or pharmacist promptly.

Remember that this medication has been prescribed because your doctor has judged that the benefit to you is greater than the risk of side effects. Many people using this medication do not have serious side effects.

Tell your doctor right away if you have any serious side effects, including:

• pain/redness/swelling at injection site

This is not a complete list of possible side effects. If you notice other effects not listed above, contact your doctor or pharmacist.

In the US - Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088 or at www.fda.gov/medwatch.

In Canada - Call your doctor for medical advice about side effects. You may report side effects to Health Canada at 1-866-234-2345.

Before using pegunigalsidase alfa, tell your doctor or pharmacist if you are allergic to it; or if you have any other allergies. This product may contain inactive ingredients, which can cause allergic reactions or other problems. Talk to your pharmacist for more details.

Before using this medication, tell your doctor or pharmacist your medical history, especially of:

• previous enzyme replacement therapy

Before having surgery, tell your doctor or dentist about all the products you use (including prescription drugs, nonprescription drugs, and herbal products).

During pregnancy, this medication should be used only when clearly needed. Discuss the risks and benefits with your doctor.

It is unknown if this medication passes into breast milk. Consult your doctor before breastfeeding.

Drug interactions may change how your medications work or increase your risk for serious side effects. This document does not contain all possible drug interactions. Keep a list of all the products you use (including prescription/nonprescription drugs and herbal products) and share it with your doctor and pharmacist. Do not start, stop, or change the dosage of any medicines without your doctor's approval.

If someone has overdosed and has serious symptoms such as passing out or trouble breathing, call 911. Otherwise, call a poison control center right away. US residents can call 1-800-222-1222. Canada residents can call 1-844-764-7669.

Lab and/or medical tests (such as kidney function) may be done while you are using this medication. Keep all medical and lab appointments.

It is important to get each dose of this medication as scheduled. Do not use an extra dose to make up for a missed dose. If you miss a dose, ask your doctor or pharmacist right away for a new dosing schedule.

Store in the refrigerator. Do not freeze. After removing from the refrigerator, use the mixed drug within 8 hours. Discard any unused portion. Keep all medications away from children and pets.

Do not flush medications down the toilet or pour them into a drain unless instructed to do so. Properly discard this product when it is expired or no longer needed. Consult your pharmacist or local waste disposal company.