Pegademase bovine

What is Pegademase bovine?

• Pegademase bovine (Adagen) is a modified enzyme used for enzyme replacement therapy for the treatment of severe combined immunodeficiency disease (SCID) associated with a deficiency of adenosine deaminase.

What are the uses of this medicine?

Pegademase bovine (Adagen) 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.

Limitations of use:

• 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.

How does this medicine work?

• Severe combined immunodeficiency disease (SCID) associated with a deficiency of ADA is a rare, inherited, and often fatal disease.
• In the absence of the ADA enzyme, the purine substrates adenosine and 2´-deoxyadenosine accumulate, causing metabolic abnormalities that are directly toxic to lymphocytes.
• The immune deficiency can be cured by bone marrow transplantation. When a suitable bone marrow donor is unavailable or when bone marrow transplantation fails, non-selective replacement of the ADA enzyme has been provided by periodic irradiated red blood cell transfusions. However, transmission of viral infections and iron overload are serious risks associated with irradiated red blood cell transfusions, and relatively few ADA deficient patients have benefitted from chronic transfusion therapy.
• Adagen® (pegademase bovine) Injection provides specific and direct replacement of the deficient enzyme, but will not benefit patients with immunodeficiency due to other causes.
• In patients with ADA deficiency, rigorous adherence to a schedule of Adagen® (pegademase bovine) Injection administration can eliminate the toxic metabolites of ADA deficiency and result in improved immune function.

Who Should Not Use this medicine ?

This medicine cannot be used in patients:

• with thrombocytopenia and should not be used if thrombocytopenia is severe.
• There is no evidence to support the safety and efficacy of Adagen® (pegademase bovine) Injection as preparatory or support therapy for bone marrow transplantation.

What drug interactions can this medicine cause?

• Tell your doctor and pharmacist what other prescription and nonprescription medications, vitamins, nutritional supplements, and herbal products you are taking or plan to take.

Be sure to mention any of the following:

• Vidarabine

Is this medicine FDA approved?

• Pegademase bovine is designated as an orphan drug by the US FDA on 5/29/1984, used in For enzyme replacement therapy for ADA deficiency in patients with severe combined immunodeficiency.

How should this medicine be used?

Recommended dosage:

• The dosage of Adagen® (pegademase bovine) Injection should be individualized.
• The recommended dosing schedule is 10 U/kg for the first dose, 15 U/kg for the second dose, and 20 U/kg for the third dose.
• The usual maintenance dose is 20 U/kg per week. Further increases of 5 U/kg/week may be necessary, but a maximum single dose of 30 U/kg should not be exceeded.

Administration: Adagen® (pegademase bovine) Injection should be administered every 7 days as an intramuscular injection.

What are the dosage forms and brand names of this medicine?

This medicine is available in fallowing doasage form:

• As Adagen® Injection.
• It is supplied in 1.5 mL single-dose vials.

This medicine is available in fallowing brand namesː

• Adagen

What side effects can this medication cause?

The most common side effects of this medicine include:

• headache
• pain at the injection site
• hemolytic anemia
• auto-immune hemolytic anemia
• thrombocythemia
• injection site erythema
• urticaria

What special precautions should I follow?

• There have been no reports of hypersensitivity reactions in patients who have been treated with Adagen®(pegademase bovine) Injection.
• Appropriate care to protect immune deficient patients should be maintained until improvement in immune function has been documented. The degree of immune function improvement may vary from patient to patient and, therefore, each patient will require appropriate care consistent with immunologic status.
• The treatment of SCID associated with ADA deficiency with Adagen® (pegademase bovine) Injection should be monitored by measuring plasma ADA activity and red blood cell dATP levels. Between 3 and 9 months, plasma ADA should be determined twice a month, then monthly until after 18-24 months of treatment with Adagen® (pegademase bovine) Injection.
• In patients undergoing treatment with Adagen® (pegademase bovine) Injection, a decline in immune function, with increased risk of opportunistic infections and complications of infection.

What to do in case of emergency/overdose?

Symptoms of overdosage may include:

• There is no documented experience with Adagen® (pegademase bovine) Injection overdosage.

Management of overdosage:

• In case of overdose, call the poison control helpline of your country. In the United States, call 1-800-222-1222.

• Overdose related information is also available online at poisonhelp.org/help.
• In the event that the victim has collapsed, had a seizure, has trouble breathing, or can't be awakened, immediately call emergency services. In the United States, call 911.

Can this medicine be used in pregnancy?

• Pregnancy Category C.
• It is also not known whether Adagen® (pegademase bovine) Injection can cause fetal harm when administered to a pregnant woman or can affect reproduction capacity.
• Adagen® (pegademase bovine) Injection should be given to a pregnant woman only if clearly needed.

Can this medicine be used in children?

• Adagen® (pegademase bovine) Injection is recommended for use in infants from birth or in children of any age at the time of diagnosis.

What are the active and inactive ingredients in this medicine?

• Adagen® (pegademase bovine) Injection is a clear, colorless, preservative free solution for intramuscular injection.
• Each vial contains 250 units/mL and is supplied as a 1.5 mL single-use vial, in boxes of 4 vials.

Who manufactures and distributes this medicine?

• ENZON Pharmaceuticals, Inc.
• Bridgewater, NJ

What should I know about storage and disposal of this medication?

• Refrigerate.
• Store between +2°C and +8°C (36°F and 46°F).
• DO NOT FREEZE. Adagen® (pegademase bovine) Injection should not be stored at room temperature.
• This product should not be used if there are any indications that it may have been frozen.
• Use only one dose per vial; do not re-enter the vial.
• Discard unused portions.
• Do not save unused drug for later administration.

• Dailymed label info on Pegademase bovine
• FDA Pegademase bovine

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