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Adenosine deaminase deficiency is a hereditary disorder. Lack of adenosine deaminase activity results in severe combined immunodeficiency disease, a fatal disorder of infancy that causes profound defects of both humoral and cellular immunity. In most patients, clinical disease is observed within 1 to 6 months of birth, although disease onset has occurred later than 6 months in up to 15% of patients. Most children present with failure to thrive, diarrhea, persistent oral and perianal candidiasis, and recurring bacterial, viral, and other opportunistic infections. In addition to the immunodeficient state, a distinctive bone dysplasia has been observed in many children which has a common feature of flaring and cupping of the costochondral rib junctions.

Death usually intervenes before the child reaches 2 years of age unless children are kept in protective isolation or receive bone marrow transplantation to rebuild the immune system. The primary problem is accumulation of toxic intracellular and extracellular metabolites, which results in failure of immune system development. Lymphopenia is present at birth in adenosine deaminase deficiency; whereas absence of thymic lymphocytes, T lymphopenia, and accumulation of deoxyadenosine triphosphate in red blood cells have been observed at 16 to 17 weeks gestation. The treatment of choice for severe combined immunodeficiency secondary to adenosine deaminase deficiency is bone marrow transplantation. However, histocompatible donors of bone marrow are frequently unavailable. Also, adenosine deaminase is present in normal erythrocytes, and transfusion of red cells, usually biweekly or monthly, has been effective in treating adenosine deaminase deficiency in some children as well as reducing levels of toxic metabolites.

Higher levels of circulating adenosine deaminase are achieved with pegademase bovine (Adagen®) as compared to erythrocyte transfusion, and pegademase bovine (Adagen®) appears to be more effective in reversing biochemical abnormalities in patients with adenosine deaminase deficiency.

FDA APPROVED INDICATIONS

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. It is recommended for use in infants from birth or in children of any age at the time of diagnosis (added 09-06-2006).

Pegademase bovine (Adagen®) is considered medically necessary 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 (revised 09-06-2006).

3/9/2006:  Coding updated.  CPT4/HCPCS 2006 revisions added to policy.

09/06/2006: FDA Approved Indications section added. Policy section and Accredo information revised.

01/01/2009: Accredo preferred provider information removed. BCBSMS information added.

American Hospital Formulary Service. Accessed 10-18-02.

Levy Y, Hershfield MS, Fernandez-Mejia C et al: Adenosine deaminase deficiency with late onset of recurrent infections: response to treatment with polyethylene glycol-modified adenosine deaminase. J Pediatr 1988; 113:312-317.

Lexi-Comp Online (Added 09-06-2006)

Adagen® Prescribing Information (Added 09-06-2006)

Covered Codes

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