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Severe combined immunodeficiency disease (SCID) associated with a deficiency of adenosine deaminase (ADA) is a rare, inherited, and often fatal disease. In the absence of the ADA enzyme, the purine substrates 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.
Pegademase bovine (Adagen®) 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 pegademase bovine (Adagen®) can eliminate the toxic metabolites of ADA deficiency and result in improved immune function. It is imperative that treatment with pegademase bovine (Adagen®) be carefully monitored by measurement of the level of ADA activity in plasma. Monitoring of the level is also helpful in determining that the dose is adequate.
Pegademase bovine (Adagen®) provides specific replacement of the deficient enzynme. The direct action is the correction of the metabolic abnormalities. Improvement in immune function and diminished frequency of opportunistic infections compared with the natural history of combined immunodeficiency due to ADA deficiency only occurs after metabolic abnormalities are corrected. There is a log between the correction of the metabolic abnormalities and improve immune function. This period of time is viable and has been reported to be from a few weeks to as long as six months.
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.
Prior authorization is required.
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.
The coverage guidelines outlined in the Medical Policy Manual should not be used in lieu of the Member's specific benefit plan language.
10/21/2003: Approved by Pharmacy & Therapeutics Committee (P & T)
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.
04/01/2014: Policy description revised. Policy statement unchanged.
02/05/2015: Added ICD-9 diagnosis code 279.2 to the Code Reference section. Removed deleted HCPCS code Q2012.
Clinical Pharmacology Online. Accessed 10-18-02.
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.
Adagen® Prescribing Information
This may not be a comprehensive list of procedure codes applicable to this policy.
The code(s) listed below are ONLY medically necessary if the procedure is performed according to the "Policy" section of this document.