Cipaglucosidase alfa-atga

Cipaglucosidase alfa-atga may cause severe hypersensitivity reactions or infusion-related reactions or cardiorespiratory failure. Tell your doctor if you currently feel ill or have an infection of any type or if you have or had any cardiovascular or respiratory disease. If you experience any of the following symptoms, seek medical treatment immediately: hives, itching, flushing, weak and rapid pulse (heart rate), swelling of tongue or throat, difficulty breathing, dizziness or fainting, loss of consciousness. Your doctor may give you medicines prior to cipaglucosidase alfa-atga infusion and will monitor you closely during and after treatment. Cipaglucosidase alfa-atga must be given by a healthcare provider in a monitored setting.

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.

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.

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

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.

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

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

Some side effects can be serious. If you experience any of these symptoms, call your doctor immediately or get emergency medical treatment:

• 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).

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.

• Pombiliti^®