57665-0001-XX Adagen 250 UNIT/ML SOLN (LEADIANT BIOSCIENCES)
Pegademase bovine is an enzyme derived from bovine intestine modified for human enzyme replacement therapy for the treatment of severe combined immune deficiency disease (SCID) associated with a deficiency of adenosine deaminase (ADA). SCID associated with ADA deficiency is a rare, inherited and often fatal disease. Without the ADA enzyme, purine substrates accumulate and cause metabolic abnormalities that are directly toxic to lymphocytes.
While this immune deficiency can be cured by bone marrow transplantation, if a suitable marrow donor is not available or if the transplantation fails, prior to the development of an enzyme replacement, periodic transfusions of irradiated red blood cells have provided some relief, but with serious risks of iron overload and viral transmission. Direct replacement of the deficient enzyme eliminates toxic metabolites of the deficiency and improved immune function.
Pegademase bovine for the treatment of adenosine deaminase (ADA) deficiency is considered medically necessary if the medical appropriateness criteria are met. (See Medical Appropriateness below.)
Pegademase bovine for the treatment of other conditions/diseases is considered investigational.
Pegademase bovine is considered medically appropriate if ALL of the following criteria are met:
Diagnosis of adenosine deaminase deficiency (ADA) in individuals with ALL of the following:
Definitive diagnosis of severe combined immunodeficiency disease (SCID) as determined by one of the following:
Deficient ADA catalytic activity (<1% of normal) in hemolysates (in untransfused individuals) or in extracts of other cells (e.g., blood mononuclear cells, fibroblasts)
Detection of pathogenic mutations in the ADA gene by molecular genetic testing
Marked elevation of deoxyadenosine (dAdo) triphosphate (dATP) OR total dAdo nucleotides (dAXP, measured as the sum of dAMP + dADP + dATP) in erythrocytes
Failed or is not a candidate for bone marrow transplantation (BMT)
Absence of severe thrombocytopenia, considered to be platelets<50,000/microliter
Baseline values for plasma ADA activity and red blood cell deoxyadenosine triphosphate (dATP) levels have been obtained
Pegademase bovine is considered medically appropriate for renewal/maintenance therapy if ALL of the following criteria are met:
Individual continues to meet initial approval criteria
Absence of toxicity or hypersensitivity reaction, e.g., severe injection site reactions, hemolytic anemia, severe thrombocytopenia, lymphoma, etc.
Adequate documentation of disease stability and/or improvement as indicated by ANY ONE of the following:
Increase in plasma ADA activity (target trough level 15-35 μmol/hr/mL)
Red blood cell dATP level decreased (target ≤ 0.005 to 0.015 μmol/mL)
Improvement in immune function with diminished frequency/complications of infection
|INDICATION(S)||DOSAGE & ADMINISTRATION|
|Adenosine deaminase deficiency||
Adagen is administered every 7 days by IM (intramuscular) injection.
First dose: 10 units/kg
Second dose: 15 units/kg
Third dose: 20 units/kg
The weekly dose may be increased by increments of 5 units/kg/week based on laboratory monitoring up to a maximum of 30 units/kg/week.
LENGTH OF AUTHORIZATION
Coverage will be provided per 12 months and may be renewed.
Refer to DOSAGE LIMITS below
APPLICABLE TENNESSEE STATE MANDATE REQUIREMENTS
BlueCross BlueShield of Tennessee’s Medical Policy complies with Tennessee Code Annotated Section 56-7-2352 regarding coverage of off-label indications of Food and Drug Administration (FDA) approved drugs when the off-label use is recognized in one of the statutorily recognized standard reference compendia or in the published peer-reviewed medical literature.
We develop Medical Policies to provide guidance to Members and Providers. This Medical Policy relates only to the services or supplies described in it. The existence of a Medical Policy is not an authorization, certification, explanation of benefits or a contract for the service (or supply) that is referenced in the Medical Policy. For a determination of the benefits that a Member is entitled to receive under his or her health plan, the Member's health plan must be reviewed. If there is a conflict between the Medical Policy and a health plan, the express terms of the health plan will govern.
For appropriate chemotherapy regimens, dosage information, contraindications, precautions, warnings, and monitoring information, please refer to one of the standard reference compendia (e.g., the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) published by the National Comprehensive Cancer Network®, Drugdex Evaluations of Micromedex Solutions at Truven Health, or The American Hospital Formulary Service Drug Information).
Hirschfield, M. (2006, October, Updated 2017, March). Adenosine Deaminase Deficiency. In: Adam, M. P., Ardinger, H. H., Pagon, R. A., et al., eds., GeneReviews®. Seattle (WA): University of Washington, Seattle; 1993-2018. Retrieved October 31, 2018 from https://www.ncbi.nlm.nih.gov/books/NBK1483/.
MICROMEDEX Healthcare Series. Drugdex Drug Evaluations. (2017, August). Pegademase bovine. Retrieved October 31, 2018 from MICROMEDEX Healthcare Series.
National Heart, Lung and Blood Institute Health Topics. (2018) Thrombocytopenia. National Institutes of Health; U.S. Department of Health and Human Services. Retrieved October 31, 2018 from https://www.nhlbi.nih.gov/health-topics/thrombocytopenia.
U.S. Food and Drug Administration. (2014, June). Center for Drug Evaluation and Research. Adagen® (pegademase bovine) injection. Retrieved October 31, 2018 from https://www.accessdata.fda.gov/drugsatfda_docs/label/2014/019818s053lbl.pdf.
ORIGINAL EFFECTIVE DATE: 12/1/2016
MOST RECENT REVIEW DATE: 3/2/2019
Policies included in the Medical Policy Manual are not intended to certify coverage availability. They are medical determinations about a particular technology, service, drug, etc. While a policy or technology may be medically necessary, it could be excluded in a member's benefit plan. Please check with the appropriate claims department to determine if the service in question is a covered service under a particular benefit plan. Use of the Medical Policy Manual is not intended to replace independent medical judgment for treatment of individuals. The content on this Web site is not intended to be a substitute for professional medical advice in any way. Always seek the advice of your physician or other qualified health care provider if you have questions regarding a medical condition or treatment.
This document has been classified as public information.
Maximum billable units per dose and over time by indication as a Medical Benefit