Pegunigalsidase alfa-iwxj (Intravenous)

Pegunigalsidase alfa-iwxj injection is used to treat Fabry disease, an inherited condition caused by not having enough of an enzyme called alpha-galactosidase A in the body. This enzyme is important for your body. Pegunigalsidase alfa-iwxj helps to replace this enzyme.

This medicine should only be given by your doctor or under their supervision.

It is very important that your doctor check your progress closely while you are receiving this medicine to make sure that it is working properly. Blood and urine tests may be needed to check for unwanted effects.

This medicine may cause serious allergic reactions, including anaphylaxis, which can be life-threatening and require immediate medical attention. Check with your doctor right away if you have chest tightness, cough, difficulty swallowing, dizziness, fast heartbeat, hives, itching, puffiness or swelling of the eyelids or around the eyes, face, lips, or tongue, skin rash, trouble breathing, or unusual tiredness or weakness.

This medicine may cause infusion-related reactions, which can be life-threatening and require immediate medical attention. Tell your doctor right away if you start to have a fever, chills or shaking, chest pain, dizziness, flushing, headache, trouble breathing, hives, itching, or skin rash, lightheadedness, fainting, fast, pounding, or uneven heartbeat, or trouble breathing after receiving this medicine.

Check with your doctor right away if you have cloudy or bloody urine, high blood pressure, or swelling of the face, feet, or lower legs. These may be symptoms of a serious kidney problem.

In deciding to use a medicine, you and your doctor need to consider the risks of taking the medicine against the benefits it may provide. For this medicine, keep the following in mind:

Allergies

Inform your doctor if you have ever had any unusual or allergic reactions to this medicine or any other medicines. Also, let your health care professional know if you have any other types of allergies, such as to foods, dyes, preservatives, or animals. For non-prescription products, read the label or package ingredients carefully.

Pediatric

There have not been appropriate studies to determine how age affects the use of pegunigalsidase alfa-iwxj injection in children. Safety and effectiveness have not been established.

Geriatric

There have not been appropriate studies to determine how age affects the use of pegunigalsidase alfa-iwxj injection in older patients.

Breast Feeding

There are no adequate studies in women to assess the risk to infants when using this medication during breastfeeding. Consider the potential benefits and risks before taking this medication while breastfeeding.

Interactions with Medicines

Some medicines should not be used together at all, while in other cases, two different medicines may be used together even if there is a possibility of interaction. In such cases, your doctor may want to adjust the dose, or other precautions may be necessary. Inform your healthcare professional if you are taking any other prescription or nonprescription (over-the-counter [OTC]) medicine.

Interactions with Food/Tobacco/Alcohol

Some medicines should not be taken at or around mealtime or with certain types of food due to possible interactions. Using alcohol or tobacco with certain medicines may also lead to interactions. Discuss with your healthcare professional how to use your medicine with food, alcohol, or tobacco.

Other Medical Problems

Having other medical problems may affect how this medicine works. Make sure to tell your doctor if you have any other medical issues, especially:

• Heart problems—Use with caution. You may be at a higher risk of severe complications from infusion reactions.

• Kidney disease—Use with caution. This condition may worsen with this medicine.

Along with its necessary effects, a medicine may cause some unwanted effects. Not all of these side effects may happen, but if they do, they may require medical attention.

Check with your doctor or nurse right away if any of the following side effects occur:

More common

• Back pain
• Bloody or cloudy urine
• Burning, numbness, tingling, or painful sensations
• Chest tightness
• Chills
• Cough
• Ear congestion
• Fast heartbeat
• Fever
• Flushing
• Headache
• Loss of voice
• Muscle aches
• Nausea and vomiting
• Nerve pain
• Pain in the arms, legs, lower back, bottom, or hips
• Skin itching, rash, or redness
• Sneezing
• Sore throat
• Stuffy or runny nose
• Swelling of the face, throat, or tongue
• Trouble breathing
• Trouble swallowing
• Unsteadiness or awkwardness
• Unusual tiredness or weakness
• Weakness in the arms, hands, legs, or feet

Incidence unknown

• Blurred vision
• Chest pain or discomfort
• Confusion
• Dizziness, faintness, or lightheadedness when getting up suddenly from a lying or sitting position
• Nervousness
• Pounding in the ears
• Puffiness or swelling of the eyelids or around the eyes, face, lips, or tongue
• Shakiness in the legs, arms, hands, or feet
• Slow or irregular heartbeat
• Sweating
• Swelling of the face, feet, or lower legs
• Trembling or shaking of the hands or feet
• Vomiting

Some side effects may occur that usually do not need medical attention. These side effects may go away during treatment as your body adjusts to the medicine. Your health care professional may also be able to tell you about ways to prevent or reduce some of these side effects. Check with your health care professional if any of the following side effects continue, are bothersome, or if you have any questions about them:

More Common

• Diarrhea
• Pain or tenderness around the eyes and cheekbones
• Stomach pain

Incidence not known

• Burning, crawling, itching, numbness, prickling, "pins and needles," or tingling feelings
• Difficulty in moving
• Joint pain
• Lack or loss of strength
• Muscle cramps, pains, or stiffness
• Sneezing
• Swollen joints
• Throat irritation

Other side effects not listed may also occur in some patients. If you notice any other effects, check with your healthcare professional.

Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088.

In the U.S.

• Elfabrio

Available Dosage Forms:

• Solution

Therapeutic Class: Enzyme Replacement

Pharmacologic Class: Pegunigalsidase Alfa

Find detailed information on biosimilars for this medication.

A nurse or other trained health professional will give you this medicine in a medical facility. This medicine is given through a needle placed in one of your veins. The medicine must be injected slowly, so your IV will need to stay in place for at least 1½ hours every 2 weeks.

Your doctor will give you other medicines (eg, allergy medicine, fever medicine, steroid) before starting treatment with this medicine.

Missed Dose

Call your doctor or pharmacist for instructions.