BlueCross BlueShield of Tennessee Medical Policy Manual

Pegademase Bovine

NDC CODE(S)

57665-0001-xx Adagen® 250 Units/mL SOLN SIGMA-TAU

DESCRIPTION

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.

REFER TO DECISION SUPPORT TREE

POLICY

MEDICAL APPROPRIATENESS

INITIAL APPROVAL

RENEWAL CRITERIA

INDICATION(S) DOSAGE & ADMINISTRATION
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.

Recommended dosing schedule- 10 U/kg for the first dose, 15 U/kg for the second dose, 20 U/kg for the third dose 

Usual maintenance dose, 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

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.

IMPORTANT REMINDER

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.

ADDITIONAL INFORMATION 

For appropriate dosage information, contraindications, precautions, warnings, and monitoring information, please refer to one of the standard reference compendia (e.g., The American Hospital Formulary Service Drug Information).

A review of the published literature found no controlled studies to validate the use of pegademase bovine for the treatment or prevention of any other conditions or diseases.

SOURCES

MICROMEDEX Healthcare Series. Drugdex Drug Evaluations. (2016, February). Pegademase bovine. Retrieved June 28, 2016 from MICROMEDEX Healthcare Series.

U.S. Food and Drug Administration. (2014, June). Center for Drug Evaluation and Research. Adagen® (pegademase bovine) injection. Retrieved June 28, 2016 from http://www.accessdata.fda.gov/drugsatfda_docs/label/2014/019818s053lbl.pdf.

ORIGINAL EFFECTIVE DATE:  12/1/2016

MOST RECENT REVIEW DATE:  2/27/2018

ID_MRx

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.

Pharmaceutical Decision Support Tree

Pegademase Bovine (Adagen®)

  1. Is this the initial request for this agent for this individual?

If yes, go to question #2

If no, go to question #5

  1. Does the individual have a diagnosis of adenosine deaminase deficiency (ADA) with ALL of the following?

If yes, go to question #3

If no, this does not meet medical necessity and/or medical appropriateness criteria

  1. Are there 150 total billable units (1 IU = 25 IU) or less requested per 7 days?

If yes, go to question #4

If no, this does not meet medical necessity and/or medical appropriateness criteria

  1. Is the request for a maximum of 6 months for a total dosage maximum of 25 U/kg per week based on the following?

If yes, this meets medical necessity and/or medical appropriateness criteria

If no, this does not meet medical necessity and/or medical appropriateness criteria

  1. Does the individual continue to meet the initial approval criteria in questions 2 through 4?

If yes, go to question #6

If no, this does not meet medical necessity and/or medical appropriateness criteria

  1. Is there an absence of toxicity or hypersensitivity reaction with the intramuscular injections?

If yes, go to question #7

If no, this does not meet medical necessity and/or medical appropriateness criteria

  1. Are current laboratory values provided for ADA for dosage calculation of ANY ONE of the following?

If yes, this meets medical necessity and/or medical appropriateness criteria

If no, this does not meet medical necessity and/or medical appropriateness criteria

This document has been classified as public information.