Alglucosidase alfa (injection)

Alglucosidase alfa is used to treat a glycogen storage disorder called Pompe disease, (also called GAA deficiency) in adults and children.

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

Alglucosidase alfa is given as an infusion into a vein. A healthcare provider will give you this injection.

Alglucosidase alfa must be given slowly, and the IV infusion can take up to 4 hours to complete.

alglucosidase alfa is usually given once every 2 weeks.

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

Before each injection, tell your doctor if you have recently been sick with a cold, flu, or other illness.

If you need surgery, tell the surgeon ahead of time that you are using alglucosidase alfa.

Alglucosidase alfa can have long lasting effects on your body. Your blood will need to be tested every 3 months for 2 years and then once every year after that.

Tell your medical caregivers or get emergency medical help right away if you have any signs of a severe allergic reaction, such as itching, rash, redness or tingling, severe headache, feeling light-headed, fast or slow heart rate, wheezing, trouble breathing, pale skin, and blue lips or fingernails.

Alglucosidase alfa causes your immune system to attack healthy organs and tissues in your body. This could lead to serious or life-threatening side effects on your lungs, skin, liver, pancreas, kidneys, intestines, thyroid, or adrenal glands.

Alglucosidase alfa may cause a serious heart or respiratory condition.

Tell your doctor if you have or have ever had:

• heart disease; or
• lung disease or breathing disorder.

It is not known if alglucosidase alfa will harm an unborn baby. Tell your doctor if you are pregnant or plan to become pregnant.

Do not breastfeed within 24 hours after using alglucosidase alfa. If you use a breast pump during this time, throw out the milk and do not feed it to your baby.

If you are pregnant, your name may be listed on a pregnancy registry to track the effects of alglucosidase alfa on the baby.

Get emergency medical help if you have signs of an allergic reaction: hives, difficult breathing; swelling of your face, lips, tongue, or throat.

Some allergic reactions may occur during the injection, or as long as 3 hours afterward. Tell your caregivers or get emergency medical help if you have:

• a feeling like you might pass out (even while lying down);
• slow heart rate, weak pulse, fainting, slow breathing (breathing may stop);
• bronchospasm (wheezing, tight feeling in your chest or throat, trouble breathing);
• pale skin, cold or clammy skin, blue lips or fingernails, cold hands or feet;
• warmth, redness, numbness, or tingly feeling under your skin;
• rash, itching, sweating, fever;
• severe headache, nausea, feeling restless or nervous;
• seizure (convulsions); or
• fast heart rate, pounding in your neck or ears, anxiety, confusion.

Alglucosidase alfa may cause serious side effects. Call your doctor at once if you have:

• skin changes (sores, lesions, ulcers, discoloring); or
• puffy eyes, swelling in your ankles or feet, weight gain, urine that looks foamy.

Common side effects of alglucosidase alfa may include:

• hives, difficult breathing, swelling of your face, lips, tongue, or throat;
• pale skin, blue lips, feeling hot or feverish;
• skin rash, itching, flushing (warmth, redness, or tingly feeling);
• headache, dizziness, agitation, muscle twitching;
• fast heart rate, rapid breathing, chest discomfort, cough;
• increased blood pressure;
• nausea, vomiting;
• tremor, shaking, increased sweating; or
• muscle pain, tiredness.

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.

Usual Adult Dose for Pompe disease:

20 mg/kg via IV infusion every 2 weeksComments:-Total volume of infusion is determined by patient's body weight and should be infused over about 4 hours.

Usual Pediatric Dose for Pompe disease:

20 mg/kg via IV infusion every 2 weeksComments:-Total volume of infusion is determined by body weight and should be infused over about 4 hours.

Call your doctor for instructions if you miss an appointment for your alglucosidase alfa injection.

Seek emergency medical attention or call the Poison Help line at 1-800-222-1222.

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

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