Pegademase Bovine

Avoid being near people who are sick or have infections. Tell your doctor at once if you develop signs of infection.

Other drugs may interact with pegademase bovine, including prescription and over-the-counter medicines, vitamins, and herbal products. Tell each of your health care providers about all medicines you use now and any medicine you start or stop using.

ADAGEN® (pegademase bovine) Injection is indicated for enzyme replacement therapy for adenosine deaminase (ADA) deficiency in patients with severe combined immunodeficiency disease (SCID) who are not suitable candidates for – or who have failed – bone marrow transplantation. ADAGEN® (pegademase bovine) Injection is recommended for use in infants from birth or in children of any age at the time of diagnosis. ADAGEN® (pegademase bovine) Injection is not intended as a replacement for HLA identical bone marrow transplant therapy. ADAGEN® (pegademase bovine) Injection is also not intended to replace continued close medical supervision and the initiation of appropriate diagnostic tests and therapy (e.g., antibiotics, nutrition, oxygen, gammaglobulin) as indicated for intercurrent illnesses.

There are no restrictions regarding foods, beverages, or activities during treatment with pegademase bovine unless otherwise directed by your doctor.

Pegademase bovine is a man-made form of an enzyme called adenosine deaminase (ADA). ADA is important in the body for preventing the buildup of certain proteins harmful to the white blood cells that help your body fight infections.

Pegademase bovine is used to replace ADA in people with severe combined immunodeficiency disease (SCID).

Pegademase bovine may also be used for purposes not listed in this medication guide.

You should not use pegademase bovine if you are allergic to it, or if you have:

• severe thrombocytopenia (low levels of platelets in blood).

To make sure pegademase bovine is safe for you, tell your doctor if you have:

• easy bruising or bleeding.

FDA pregnancy category C. It is not known whether pegademase bovine will harm an unborn baby. Tell your doctor if you are pregnant or plan to become pregnant while using this medicine.

It is not known whether pegademase bovine passes into breast milk or if it could harm a nursing baby. Tell your doctor if you are breast-feeding a baby.

Follow all directions on your prescription label. Your doctor may occasionally change your dose to make sure you get the best results. Do not use this medicine in larger or smaller amounts or for longer than recommended.

Pegademase bovine is injected into a muscle. You may be shown how to use injections at home. Do not self-inject this medicine if you do not understand how to give the injection and properly dispose of used needles and syringes.

Pegademase bovine is usually given once every 7 days. Follow your doctor's dosing instructions very carefully.

Use a disposable needle and syringe only once. Follow any state or local laws about throwing away used needles and syringes. Use a puncture-proof "sharps" disposal container (ask your pharmacist where to get one and how to throw it away). Keep this container out of the reach of children and pets.

Each single-use vial (bottle) of this medicine is for one use only. Throw away after one use, even if there is still some medicine left in it after injecting your dose.

Do not use pegademase bovine if it has changed colors or has particles in it. Call your pharmacist for new medication.

While using pegademase bovine, your blood will need to be tested often.

It may take up to 6 months before your immune system improves and you have fewer infections. Keep using the medication as directed and tell your doctor if your symptoms do not improve.

Call your doctor if your symptoms do not improve after the first few weeks of treatment.

Store in the refrigerator, do not freeze. Throw away the medicine if it has become frozen.

Usual Pediatric Dose for Adenosine Deaminase Deficiency:

-First dose: 10 units/kg intramuscularly
-Second dose: 15 units/kg intramuscularly
-Third dose: 20 units/kg intramuscularly

-Maintenance dose: 20 units/kg/week intramuscularly
-Maximum dose: 30 units/kg intramuscularly

Comments:
-This drug should be administered every 7 days.
-Dose should be individualized based on monitoring of plasma adenosine deaminase (ADA) activity levels and red blood cell deoxyadenosine triphosphate (dATP) content.
-Dose increases of 5 units/kg/week may be necessary.
-Dose proportionality has not been established and patients should be closely monitored when the dosage is increased.

Use: Enzyme replacement therapy for ADA deficiency in infants from birth and children of any age with severe combined immunodeficiency disease (SCID) who are not suitable candidates for, or have failed, bone marrow transplantation.

Applies to pegademase bovine: intramuscular solution

Along with its needed effects, pegademase bovine may cause some unwanted effects. Although not all of these side effects may occur, if they do occur they may need medical attention.

Check with your doctor immediately if any of the following side effects occur while taking pegademase bovine:

• Back, leg, or stomach pains
• bleeding gums
• chills
• dark urine
• difficulty breathing
• dizziness
• fever
• general body swelling
• headache
• loss of appetite
• nausea or vomiting
• nosebleeds
• pain, warmth, or burning in the fingers, toes, and legs
• pale skin
• problems with vision or hearing
• sore throat
• unusual tiredness or weakness
• yellowing of the eyes or skin

Some side effects of pegademase bovine may occur that usually do not need medical attention. These side effects may go away during treatment as your body adjusts to the medicine. Also, your health care professional may be able to tell you about ways to prevent or reduce some of these side effects. Check with your health care professional if any of the following side effects continue or are bothersome or if you have any questions about them:

• Hives or welts
• itching
• redness of skin
• redness or pain at the site of injection
• skin rash