BlueCross BlueShield of Tennessee Medical Policy Manual

C1 Esterase Inhibitor (Berinert®)

NDC CODE(S)

Berinert 500 unit vial – 63833-0825-xx (CSL Behring LLC)

DESCRIPTION

C1 esterase inhibitor (C1-INH) is a normal constituent of human blood and is a serine proteinase inhibitor or serpin. C1-INH’s primary function is to regulate the activation of the complement and intrinsic coagulation pathway. It also has a role in regulation of the fibrinolytic system. 

Individuals with an inherited deficiency of C1-INH suffer from sudden, recurrent edematous swellings of the subcutaneous or submucosal tissues.  This condition is known as hereditary angioedema (HAE).

Commercially, C1-INH is available in two forms, those derived from purified human plasma which has undergone multiple viral reduction steps and a recombinant analogue purified from the milk of transgenic rabbits.  Berinert® is a human plasma-derived product formulated for treatment of attacks.

REFER TO DECISION SUPPORT TREE

POLICY

MEDICAL APPROPRIATENESS

INITIAL APPROVAL

RENEWAL CRITERIA

INDICATION(S) DOSAGE & ADMINISTRATION
Hereditary Angioedema (HAE) 20 units per kg body weight by intravenous injection

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 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).

No controlled studies were found in the published literature that validate the use of C1 Esterase Inhibitor - Berinert® for the treatment of other conditions or diseases.

SOURCES

Bowen, T., Cicardi, M., Farkas, H., Bork, K., Longhurst, H. J.. Zuraw, B. et al. 2010 International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema. Allergy Asthma & Clinical  Immunology 2010. AACI Journal;6(1):24.

Lexi-Comp Online. (2016). AHFS DI. C1 Esterase Inhibitor. Retrieved October 5, 2016 from Lexi-Comp Online with AHFS.

MICROMEDEX Healthcare Series. Drugdex Evaluations. (2016, August). C1 Esterase Inhibitor. Retrieved October 5, 2016 from MICROMEDEX Healthcare Series.

U. S. Food and Drug Administration. (2015, April). Center for Biologics Evaluation and Research. Berinert® C1 Esterase Inhibitor (Human) label. Retrieved October 7, 2016 from http://www.fda.gov/downloads/biologicsbloodvaccines/bloodbloodproducts/approvedproducts/licensedproductsblas/fractionatedplasmaproducts/ucm186268.pdf.

ORIGINAL EFFECTIVE DATE:  6/13/2009

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

C1 Esterase Inhibitor (Berinert®)

  1. Is this the initial request for treatment?

If yes, go to question #2

If no, go to question #8

  1. Is the request for treatment of hereditary angioedema 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. Is the request for treatment of HAE Type I (HAE-C1INH-Type I) with ALL of the following?

If yes, go to question #6

If no, go to question #4

  1. Is the request for treatment of HAE Type II (HAE-C1INH-Type II) with ALL of the following?

If yes, go to question #6

If no, go to question #5

  1. Is the request for treatment of Type III HAE (HAE-FXII, HAE-unknown) with ALL of the following?

If yes, go to question #6

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

  1. Is there confirmation of avoidance of the following triggers of HAE attacks?

If yes, go to question #7

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

  1. Is the request for 1500 billable units or less per 28 days (500 unit vial, 30 vials or less every 28 days) at a dosage of 20 units per kg of body weight by intravenous injection for a period of 12 months?

If yes, this satisfies medical necessity and 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 criteria in questions 2 through 7 as appropriate?

If yes, go to question #9

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

  1. Has significant improvement of attacks in severity and duration have been achieved and sustained over the past 12 months?

If yes, go to question #10

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

  1. Is there absence of unacceptable toxicity from the drug, e.g., hypersensitivity reactions, thrombotic events, laryngeal attacks?

If yes, this satisfies medical necessity and medical appropriateness criteria for an additional 12 months

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

This document has been classified as public information.