Cipaglucosidase alfa-atga

Brand Name(s): Pombiliti®

WHY is this medicine prescribed?

Cipaglucosidase alfa-atga is used in combination with another medication called miglustat (Opfolda) to treat late onset Pompe disease (a genetic condition that causes deficiency in lysosomal acid alpha-glucosidase which can lead to muscle weakness and wasting) in adult patients weighing 92 pounds (40 kg) or more and who have not been improving on their enzyme therapy. Cipaglucosidase alfa-atga is in a class of medications called recombinant lysosomal enzymes. It works by helping break glycogen down to glucose to keep glycogen from building up in tissues.

HOW should this medicine be used?

Cipaglucosidase alfa-atga comes as a powder to be mixed with water to be given intravenously (into the vein) by a doctor or a nurse at hospital or clinic. It is usually given over 4 hours every other week. Cipaglucosidase alfa-atga infusion must be started 1 hour, and no later than 3 days, after you receive a dose of miglustat by mouth. If you do not take your miglustat, then you cannot receive your cipaglucosidase alfa-atga infusion. Your doctor may ask you to take or give you other medications before each dose of cipaglucosidase alfa-atga to help make sure you tolerate your treatment.

Ask your pharmacist or doctor for a copy of the manufacturer's information for the patient.

Are there OTHER USES for this medicine?

This medication may be prescribed for other uses; ask your doctor or pharmacist for more information.

What SPECIAL PRECAUTIONS should I follow?

Before receiving cipaglucosidase alfa-atga,

• tell your doctor and pharmacist if you are allergic to cipaglucosidase alfa-atga, any other medications, or any of the ingredients in cipaglucosidase alfa-atga for injection. Ask your pharmacist for a list of the ingredients.
• tell your doctor and pharmacist what prescription and nonprescription medications, vitamins, nutritional supplements, and herbal products you are taking or plan to take.
• tell your doctor if you have or have ever had heart or lung disease.
• tell your doctor if you are pregnant or plan to become pregnant. You should not become pregnant while you are receiving cipaglucosidase alfa-atga and for 60 days after your last dose. Talk to your doctor about birth control methods that you can use during your treatment. If you become pregnant while receiving cipaglucosidase alfa-atga, call your doctor immediately. Cipaglucosidase alfa-atga may harm the fetus. If you are a woman capable of become pregnant, your doctor will do a pregnancy test before you start cipaglucosidase alfa-atga.

What SPECIAL DIETARY instructions should I follow?

Unless your doctor tells you otherwise, continue your normal diet.

What SIDE EFFECTS can this medicine cause?

Cipaglucosidase alfa-atga may cause side effects. Tell your doctor if any of these symptoms are severe or do not go away:

• headache
• diarrhea
• fever
• bloating

• difficulty breathing, rash, swelling of legs or arms, swelling or feeling of tightness of throat, tongue swelling
• itching, flushing or chills

Cipaglucosidase alfa-atga may cause other side effects. Call your doctor if you have any unusual problems while taking this medication.

If you experience a serious side effect, you or your doctor may send a report to the Food and Drug Administration's (FDA) MedWatch Adverse Event Reporting program online (http://www.fda.gov/Safety/MedWatch) or by phone (1-800-332-1088).

What OTHER INFORMATION should I know?

Keep all appointments with your doctor. If you missed an appointment for an infusion, call your doctor immediately.

It is important for you to keep a written list of all of the prescription and nonprescription (over-the-counter) medicines you are taking, as well as any products such as vitamins, minerals, or other dietary supplements. You should bring this list with you each time you visit a doctor or if you are admitted to a hospital. It is also important information to carry with you in case of emergencies.

This report on medications is for your information only, and is not considered individual patient advice. Because of the changing nature of drug information, please consult your physician or pharmacist about specific clinical use.

The American Society of Health-System Pharmacists, Inc. represents that the information provided hereunder was formulated with a reasonable standard of care, and in conformity with professional standards in the field. The American Society of Health-System Pharmacists, Inc. makes no representations or warranties, express or implied, including, but not limited to, any implied warranty of merchantability and/or fitness for a particular purpose, with respect to such information and specifically disclaims all such warranties. Users are advised that decisions regarding drug therapy are complex medical decisions requiring the independent, informed decision of an appropriate health care professional, and the information is provided for informational purposes only. The entire monograph for a drug should be reviewed for a thorough understanding of the drug's actions, uses and side effects. The American Society of Health-System Pharmacists, Inc. does not endorse or recommend the use of any drug. The information is not a substitute for medical care.

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