Pegunigalsidase alfa

Generic name: pegunigalsidase alfa [ peg-UE-ni-GAL-si-dase-AL-fa ]
Brand name: Elfabrio
Dosage form: intravenous solution (iwxj 2 mg/mL)
Drug class: Lysosomal enzymes

Medically reviewed by Drugs.com on Nov 9, 2023. Written by Cerner Multum.

What is pegunigalsidase alfa?

Pegunigalsidase alfa is used in adults to treat Fabry disease.

Pegunigalsidase alfa may also be used for purposes not listed in this medication guide.

Pegunigalsidase alfa side effects

Get emergency medical help if you have signs of an allergic reaction: hives, difficult 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, nauseated, 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 at once if you have:

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

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

Common side effects of pegunigalsidase alfa may include:

• cold symptoms such as stuffy nose, sneezing, sore throat;

• headache;

• tired feeling;

• 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.

Warnings

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.

Before taking this medicine

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 listed on a pregnancy registry to track the effects of pegunigalsidase alfa on the baby.

How is pegunigalsidase alfa given?

Pegunigalsidase alfa is injected into a vein usually once every 2 weeks. A healthcare provider will give you this injection.

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

After receiving an injection, you will be watched closely to make sure you do not have serious side effects.

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

You will need frequent medical tests.

Pegunigalsidase alfa dosing information

Usual Adult Dose for Fabry Disease:

RECOMMENDED DOSING: 1 mg/kg (based on actual body weight) via IV infusion every 2 weeks

PRE-TREATMENT RECOMMENDATIONS:
-In enzyme replacement therapy (ERT)-experienced patients who pre-treated with antihistamines, antipyretics, and/or corticosteroids during prior ERT, consider a similar pre-treatment strategy before the first several infusions of this drug.
-After 4 to 6 well-tolerated infusions in ERT-experienced patients, consider a stepwise decrease in pre-treatment medication dose(s) and/or discontinuation.
-In ERT-naive patients, consider pre-treating with antihistamines, antipyretics, and/or corticosteroids prior to infusing this drug.

RECOMMENDED INFUSION RATES FOR INITIAL 4 TO 6 INFUSIONS:
ERT-Experienced Patients:
-Actual body weight less than 70 kg: 0.83 mL/min (50 mL/hr); total infusion volume: 150 mL
-Actual body weight 70 to 100 kg: 1.39 mL/min (83 mL/hr); total infusion volume: 250 mL
-Actual body weight greater than 100 kg: 2.78 mL/min (167 mL/hr); total infusion volume: 500 mL
-If infusion rate during prior ERT was greater than 3 hours: Maintain same rate of infusion with this drug.
-Consider decreasing duration of every third infusion by 30 minute decrements after first 4 to 6 doses.
-The minimum maintenance infusion duration is 1.5 hours.

ERT-Naive Patients:
-Actual body weight less than 70 kg: 0.63 mL/min (37.5 mL/hr); total infusion volume: 150 mL
-Actual body weight 70 to 100 kg: 1 mL/min (60 mL/hr); total infusion volume: 250 mL
-Actual body weight greater than 100 kg: 1.38 mL/min (83 mL/hr); total infusion volume: 500 mL

Comments:
-Appropriate medical support measures, including cardiopulmonary resuscitation equipment, should be readily available during administration of this drug.
-Home infusion under the supervision of a healthcare provider may be considered for patients who have reached an infusion duration that is well tolerated and constant.
-Infusion rate decreases should only be made in a healthcare facility setting.

Use: For the treatment of adults with confirmed Fabry disease

Related/similar drugs

Fabrazyme, migalastat, Elfabrio, Galafold, agalsidase beta

What happens if I miss a dose?

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

What happens if I overdose?

In a medical setting an overdose would be treated quickly.

What should I avoid while receiving pegunigalsidase alfa?

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

What other drugs will affect pegunigalsidase alfa?

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.

Pegunigalsidase alfa Biosimilars

Biosimilar and interchangeable products are biological products that are highly similar to and have no clinically meaningful differences from the reference product.

Reference products

These are biological products that have already been approved by the FDA, against which biosimilar products are compared. There is 1 for pegunigalsidase alfa.

Further information

Remember, keep this and all other medicines out of the reach of children, never share your medicines with others, and use this medication only for the indication prescribed.

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.

Medical Disclaimer

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