Pegunigalsidase alfa

Pegunigalsidase alfa is used in adults to treat Fabry disease.

Pegunigalsidase alfa may also be used for reasons not mentioned in this medication guide.

Pegunigalsidase alfa is given as an injection into a vein, usually once every 2 weeks. A healthcare provider will administer this injection.

You may receive other medications to help prevent serious side effects or an allergic reaction.

After the injection, you will be monitored closely to ensure you do not experience serious side effects.

Doses are determined by your weight. Your dose may change if you gain or lose weight.

You will need to have frequent medical tests.

Many people have a severe reaction to pegunigalsidase alfa. Tell your caregiver if you have a skin rash or hives, fever, chills, headache, muscle pain, numbness, swelling, vomiting, diarrhea, chest pain, trouble swallowing or breathing, fast or slow heart rate, or severe dizziness.

Tell your doctor if you have ever had:

• any reaction from enzyme replacement therapy (called IgG antidrug antibodies); or
• kidney problems.

Tell your doctor if you are pregnant or breastfeeding.

If you are pregnant, your name may be added to a pregnancy registry to monitor the effects of pegunigalsidase alfa on the baby.

Get emergency medical help if you have signs of an allergic reaction: hives, difficulty breathing, swelling of your face, lips, tongue, or throat.

Some side effects may occur during the injection. Tell your medical caregiver if you feel dizzy, nauseous, light-headed, itchy, sweaty, or have a headache, chest tightness, back pain, trouble breathing, or swelling in your face.

Pegunigalsidase alfa may cause serious side effects. Call your doctor right away if you have:

• kidney problems--swelling, urinating less, feeling tired or short of breath.

Your treatments may be delayed or stopped permanently if you have certain side effects.

Common side effects of pegunigalsidase alfa may include:

• cold symptoms such as stuffy nose, sneezing, sore throat;
• headache;
• feeling tired;
• nausea or diarrhea;
• back pain; or
• pain in your hands or feet.

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.

Usual Adult Dose for Fabry Disease:

1 mg/kg (actual body weight) via IV infusion every 2 weeksInitial Infusion Rates (first 4 to 6 doses):Enzyme replacement therapy (ERT)-experienced patients: -Weight less than 70 kg: 0.83 mL/min (50 mL/hr) for 150 mL -Weight 70 to 100 kg: 1.39 mL/min (83 mL/hr) for 250 mL -Weight greater than 100 kg: 2.78 mL/min (167 mL/hr) for 500 mL -For patients on prior ERT infused over more than 3 hours, maintain the same rate with this drug. ERT-naive patients:-Weight less than 70 kg: 0.63 mL/min (37.5 mL/hr) for 150 mL -Weight 70 to 100 kg: 1 mL/min (60 mL/hr) for 250 mL -Weight greater than 100 kg: 1.38 mL/min (83 mL/hr) for 500 mL Comments:-Infusion rates may be increased based on tolerance (after 4 to 6 doses) or slowed based on reactions to therapy; the minimum maintenance infusion duration is 1.5 hours. -Pretreatment with antihistamines, antipyretics, and/or corticosteroids may be recommended. -Consult the manufacturer product information. Use: For the treatment of patients with confirmed Fabry disease

Call your doctor for instructions if you miss a dose of your pegunigalsidase alfa injection.

In a medical setting, an overdose will be treated quickly.

Follow your doctor's instructions about any limits on food, drinks, or activities.

Other drugs may affect pegunigalsidase alfa, including prescription and over-the-counter medicines, vitamins, and herbal products. Tell your doctor about all other medicines you use.