Pegzilarginase

Pegzilarginase is used in adults and children 2 years and older to treat high levels of arginine in people with a condition called arginase 1 deficiency (ARG1-D). Those taking pegzilarginase should also follow a special diet that limits protein.

Pegzilarginase may also be used for other purposes not mentioned in this medication guide.

Pegzilarginase is injected into a vein usually once every week. A healthcare provider will give you this injection.

You may be given other medications to help prevent serious side effects or allergic reactions. Keep taking these medicines for as long as your doctor has prescribed.

You will be monitored for a short time to make sure you do not have an allergic reaction.

After 8 weeks, your provider may switch you to injections under the skin at the same dose.

If you tolerate injections under the skin and your provider approves, you may be able to receive pegzilarginase injections at home, but only under the supervision of a healthcare provider.

You will need regular blood tests to check your arginine levels and to monitor for side effects.

Doses are based on weight. Your dose may change if you gain or lose weight.

You may need to follow a special diet. Follow all instructions from your doctor or dietitian. Learn about the foods you should eat or avoid.

Pegzilarginase can cause serious allergic reactions, including anaphylaxis. It must be given in a healthcare setting where severe allergic reactions can be treated quickly.

You should not use pegzilarginase if you are allergic to it.

Tell your doctor if you have or have ever had health problems, are using other medications, or have had any previous allergic reactions.

Tell your doctor if you are pregnant or breastfeeding.

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

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

Common side effects of pegzilarginase may include:

• vomiting;
• fever; or
• constipation.

This is not a complete list of side effects, and others may occur. Call your doctor for medical advice about side effects. You can report side effects to the FDA at 1-800-FDA-1088.

Usual Adult Dose for Arginase Deficiency:

Initial dose: 0.1 mg/kg IV infusion once a weekMaintenance dose: After four weeks of therapy, measure predose plasma arginine to determine the need for dosage adjustment.Maximum dose: 0.2 mg/kg IV infusion once a weekComments:-Obtain a baseline plasma arginine concentration.-Consider premedication with antihistamines.-Use actual body weight for dose calculation. Use: For the treatment of hyperargininemia in adult patients with arginase 1 deficiency, in conjunction with dietary protein restriction

Usual Pediatric Dose for Arginase Deficiency:

2 years and older:Initial dose: 0.1 mg/kg IV infusion once a weekMaintenance dose: After four weeks of therapy, measure predose plasma arginine to determine the need for dosage adjustment.Maximum dose: 0.2 mg/kg IV infusion once a weekComments:-Obtain a baseline plasma arginine concentration.-Consider premedication with antihistamines.-Use actual body weight for dose calculation. Use: For the treatment of hyperargininemia in pediatric patients 2 years and older with arginase 1 deficiency, in conjunction with dietary protein restriction

Call your doctor for instructions if you miss an appointment for your pegzilarginase injection.

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

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

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