Cipaglucosidase alfa

Generic name: cipaglucosidase alfa [ SIP-a-gloo-KOE-si-dase ]
Brand name: Pombiliti
Dosage form: intravenous powder for injection (atga 105 mg)
Drug class: Lysosomal enzymes

Medically reviewed by Drugs.com on Feb 8, 2024. Written by Cerner Multum.

What is cipaglucosidase alfa?

Cipaglucosidase alfa is used in combination with Opfolda (miglustat) to treat late-onset Pompe disease in adults who weigh at least 88 pounds (40 kg), and when enzyme replacement therapy did not work or stopped working.

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

Cipaglucosidase 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 people have a severe reaction to cipaglucosidase alfa. These side effects may occur during or within a few hours after the injection. Tell your medical caregiver if you feel dizzy, nauseated, itchy, sweaty, light-headed, have a headache, chest pain or tightness, back pain, chills, swelling, or trouble breathing.

Also tell your medical caregivers right away if you have:

• slow heart rate, weak pulse, fainting, slow breathing (breathing may stop).

Common side effects of cipaglucosidase alfa may include:

• headache, weak or tired feeling;

• diarrhea, nausea, stomach pain; or

• fever.

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.

Related/similar drugs

Warnings

Cipaglucosidase alfa must be administered together with Opfolda (miglustat).

Some people have a severe reaction to cipaglucosidase alfa. Tell your medical caregiver if you have a skin rash or hives, chills, swelling, feel like you might pass out, chest pain, trouble breathing, or severe dizziness.

People with severe heart or respiratory problems or prone to fluid retention may have a higher risk of their condition worsening. You will be watched closely to make sure you do not have serious side effects.

Before taking this medicine

Tell your doctor if you have or have ever had:

• fluid retention;

• heart problems; or

• lung disease or breathing problems.

People with severe heart or respiratory problems or prone to fluid retention may have a higher risk of their condition worsening. Talk to your doctor about your risk.

May harm an unborn baby. Do not use if you are pregnant. You will need to have a negative pregnancy test before starting this treatment. Use effective birth control while receiving this treatment and for at least 60 days after your last dose. Tell your doctor if you become pregnant.

Do not breastfeed while receiving cipaglucosidase alfa.

When used together with Opfolda (miglustat), cipaglucosidase alfa may affect fertility in men or women. Pregnancy could be harder to achieve while either parent is using this medicine.

How should I receive cipaglucosidase alfa?

Follow all directions on your prescription label and read all medication guides or instruction sheets. Cipaglucosidase alfa must be administered together with Opfolda (miglustat).

Your doctor will stop your other treatments for Pompe disease before your first dose of cipaglucosidase alfa. Follow your doctor's dosing instructions very carefully.

Make sure you take Opfolda (miglustat) 1 hour before receiving cipaglucosidase alfa.

You will receive cipaglucosidase alfa as an infusion into a vein about 1 hour after you take Opfolda (miglustat). A healthcare provider will give you this injection every other week.

Your breathing, blood pressure, oxygen levels, and other vital signs will be watched closely to make sure you do not have serious side effects.

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

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

Cipaglucosidase alfa dosing information

Usual Adult Dose for Pompe disease:

At least 40 kg: 20 mg/kg IV every other week, infused over approximately 4 hours
-This drug must be given in combination with miglustat (Opfolda), an enzyme stabilizer.

Comments:
-Therapy with this drug may be started 2 weeks after the last dose of enzyme replacement therapy (ERT).
-Calculate the dose of this drug using the patient's actual body weight.
-Miglustat should be orally administered 1 hour before initiating infusion of this drug; refer to the manufacturer product information for miglustat for dosage and administration timeline instructions.

Use: In combination with miglustat, for the treatment of late-onset Pompe disease (lysosomal acid alpha-glucosidase [GAA] deficiency) in patients weighing 40 kg or greater and who are not improving on their current ERT.

What happens if I miss a dose?

You must take Opfolda (miglustat) before receiving cipaglucosidase alfa injection. Call your doctor for instructions if you skip an Opfolda dose or miss an appointment for the injection.

What happens if I overdose?

In a medical setting an overdose would be treated quickly.

What should I avoid while receiving cipaglucosidase alfa?

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

What other drugs will affect cipaglucosidase alfa?

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

Cipaglucosidase 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 cipaglucosidase 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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