Cipaglucosidase alfa-atga (Intravenous)

Cipaglucosidase alfa-atga injection is used along with miglustat to treat late-onset Pompe disease in patients who are not getting better with their current enzyme replacement therapy (ERT). It has a human enzyme called acid alpha-glucosidase, which helps break down and absorb glycogen. People with Pompe disease cannot produce enough of this enzyme.

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

It is very important that your doctor check your progress closely to ensure this medicine is working properly and to decide if you should continue to receive it. Blood tests may be needed to check for unwanted effects.

Using this medicine while you are pregnant can harm your unborn baby. If you are a woman who can get pregnant, your doctor may do tests to make sure you are not pregnant before receiving this medicine. Use an effective form of birth control during treatment and for at least 60 days after the last dose. If you think you have become pregnant while using this medicine, tell your doctor right away.

This medicine may cause serious allergic reactions, including anaphylaxis, which can be life-threatening and require immediate medical attention. Tell 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 after receiving this medicine.

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 chest discomfort, fever, chills or shaking, confusion, difficulty swallowing, dizziness, fast heartbeat, flushing, redness of the skin, increased or decreased blood pressure, trouble breathing, itching or rash, unusually warm skin, or lightheadedness, or fainting after receiving this medicine.

Talk with your doctor before using this medicine if you plan to have children. Some men and women who use this medicine have become infertile (unable to have children).

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

Allergies

Let your doctor know if you have ever had any unusual or allergic reaction to this medicine or any other medicines. Also, inform your healthcare professional 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

Studies have not been done to understand how age affects the effects of cipaglucosidase alfa-atga injection in children. Safety and effectiveness have not been established.

Geriatric

Studies done so far have not shown any specific problems in older adults that would limit the usefulness of cipaglucosidase alfa-atga injection in the elderly.

Breast Feeding

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

Interactions with Medicines

Some medicines should never be used together, while others can be used together even if there might be an interaction. In such cases, your doctor may need to adjust the dose or take other precautions. 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 around the time of eating or with certain types of food due to possible interactions. Using alcohol or tobacco with some medicines may also cause interactions. Talk to your healthcare professional about using your medicine with food, alcohol, or tobacco.

Other Medical Problems

Having other medical problems may affect the use of this medicine. Be sure to tell your doctor if you have any other medical issues, especially:

• Heart disease or
• Lung or breathing problems—Use with caution. May worsen these conditions.

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 need medical attention.

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

Less common

• Blurred vision
• chest tightness
• chills
• confusion
• cough
• difficulty swallowing
• dizziness, faintness, or lightheadedness when getting up suddenly from lying or sitting
• fast, pounding, or irregular heartbeat or pulse
• feeling of warmth
• fever
• headache
• hives, itching, skin rash
• increased blood pressure
• lower back or side pain
• nausea and vomiting
• puffiness or swelling of the eyelids or around the eyes, face, lips, or tongue
• redness of the face, neck, arms, and occasionally, upper chest
• shakiness in the legs, arms, hands, or feet
• sweating
• trembling or shaking of the hands or feet
• trouble breathing
• unusual tiredness or weakness

Some side effects may happen 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 provide ways to prevent or lessen 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

Less common

• Acid or sour stomach
• belching
• burning, crawling, itching, numbness, prickling, "pins and needles," or tingling feelings
• change in taste
• constipation
• difficulty in moving
• full or bloated feeling
• general feeling of discomfort or illness
• heartburn
• indigestion
• joint or muscle pain
• lack or loss of strength
• loss of taste
• muscle spasms
• pressure in the stomach
• stomach discomfort, upset, or pain
• swelling of the stomach area

Other side effects not listed may also happen 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.

• Pombiliti

Available Dosage Forms:

• Powder for Solution

Therapeutic Class: Enzyme Replacement

Pharmacologic Class: Cipaglucosidase Alfa

Find detailed information on biosimilars for this medication.

A nurse or other trained health professional will give you or your child this medicine in a hospital. This medicine is given through a needle placed in one of your veins. The medicine must be injected slowly, so your IV tube will need to stay in place for 4 hours every other week.

Your doctor will give you other medicines (eg, allergy, fever, or steroid medicine) before you receive this medicine to help prevent unwanted effects.

Missed Dose

Call your doctor or pharmacist for instructions.