L31508-LCD

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Local Coverage Determination (LCD) for Selective Treatment of HAE with Cinryze ™, Berinert ® and Ecallantide (L31508)

Contractor Information

Contractor Name

First Coast Service Options, Inc. opens in new window

Contractor Number 09101

Contractor Type MAC - Part A

LCD Information

Document Information

LCD ID Number L31508

LCD Title

Selective Treatment of HAE with Cinryze ™, Berinert ®

and Ecallantide

Contractor's Determination Number AHAE

Primary Geographic Jurisdiction opens in new window Florida

Oversight Region Region IV

AMA CPT/ADA CDT Copyright Statement

Applicable FARS/DFARS Apply to Government Use. Fee schedules, relative value units, conversion factors and/or related components are not assigned by the AMA, are not part of CPT, and the AMA is not recommending their use. The AMA does not directly or indirectly practice medicine or dispense medical services. The AMA assumes no liability for data contained or not contained herein. The Code on Dental Procedures and Nomenclature (Code) is published in Current Dental Terminology (CDT). Copyright © American Dental Association. All rights reserved. CDT and CDT-2010 are trademarks of the American Dental Association.

Original Determination Effective Date

For services performed on or after 01/23/2011 Original Determination Ending Date

Revision Effective Date

For services performed on or after 01/23/2011 Revision Ending Date

CMS National Coverage Policy

Language quoted from CMS National Coverage Determination (NCDs) and coverage provisions in interpretive manuals are italicized throughout the Local Coverage Determination (LCD). NCDs and coverage provisions in interpretive manuals are not subject to the LCD Review Process (42 CFR 405.860[b] and 42 CFR 426 [Subpart D]). In addition, an administrative law judge may not review an NCD. See §1869(f)(1)(A)(i) of the Social Security Act.

Unless otherwise specified, italicized text represent quotation from one or more CMS sources N/A

Indications and Limitations of Coverage and/or Medical Necessity

Indications of Coverage

Hereditary Angioedema (HAE) is a very rare inherited disease caused by low levels or improper function of a blood protein called C1 inhibitor (C1 – INH). HAE is caused by a genetic defect of chromosome 11. The incidence of HAE is estimated at 1 out of 50,000 individuals. There are three types of HAE. Approximately 85% of those diagnosed have Type I HAE, in which the individuals have low levels of normal C1-INH. Type II HAE patients

represent approximately 15% of those diagnosed with the disease and they have normal or elevated levels of C-1 INH, but it does not function correctly. Type III HAE is extremely rare and is an estrogen dependant form of angioedema and only occurs in women. HAE affects the blood vessels and can cause the affected individuals to develop rapid swelling and or pain of the hands, feet, limbs, face, intestinal tract, larynx or trachea. The internal swelling of the intestines may cause pain, nausea, and vomiting. The swelling of the airway may cause difficulty

in swallowing, change in voice pitch or difficulty breathing and can be potentially life-threatening.

Indications for testing include:

• recurrent angioedema, or

• laryngeal edema, or

• abdominal pain in the absence of urticaria, and

• a family history of HAE.

The initial laboratory test includes two specific blood tests to confirm the diagnosis of HAE by testing the level of C1-INH and whether it is functioning properly, also C2 and C4, which will usually reveal low levels even when an attack is not ongoing. C-1 esterase for type I will reveal low antigenic and functional levels; Type II will reveal normal antigenic level but low functional level of C1-INH; and Type III will reveal normal antigenic and functional levels. Genetic testing is not necessary.

Indications of Coverage for the following three Medication Treatments are as follows for the diagnosis of Hereditary Angioedema (HAE) (ICD-9-CM code 277.6, other deficiencies of circulating enzymes; Hereditary Angioedema):

Prophylaxis Treatment Against Reoccurring Acute HAE Attacks:

Cinryze ™ is a plasma derived C1 Inhibitor and it is indicated for routine prophylaxis against Hereditary Angioedema (HAE) attacks in adolescent and adult patients with HAE. FCSO Medicare considers Cinryze ™ treatment medically reasonable and necessary when all of the following indications are met:

• Diagnosis of HAE confirmed by proper laboratory testing serum levels; and

• History of at least 2 HAE attacks per month; and

In the Medicare beneficiary population, it is expected that the documentation supports that other therapies have unsuccessfully been attempted, such as 17 alpha-alkylated androgens (Danazol, Stanozolol), or anti-fibrinolytic agents such as epsilon amniocaproic acid and tranexamic acid for HAE prophylaxis.

Treatment for Acute Attacks of HAE:

Berinert ® is a plasma-derived C1 Esterase Inhibitor (Human) indicated for the treatment of acute abdominal or facial attacks in adult and adolescent patients diagnosed with Hereditary Angioedema (HAE). FCSO Medicare considers Berinert® treatment medically reasonable and necessary when all of the following indications are met:

• Diagnosis of HAE confirmed by proper laboratory testing serum levels; and

• Acute abdominal or facial attack

Kalbitor® (ecallantide) is a plasma kallkrein inhibitor for treatment of acute attacks of herediatary angioedema (HAE) in patients 16 years of age and older. Kalbitor® is a non-blood product and is approved by the Food and Drug Administration (FDA) for all HAE attack locations including hands, feet, abdomen, genitals and throat. Only a healthcare professional with appropriate medical support to manage anaphylaxis and hereditary angioedema (HAE) should administer this drug. Medicare considers Kalbitor® (ecallantide) treatment medically reasonable and necessary when all of the following indications are met:

• Diagnosis of HAE confirmed by proper laboratory testing serum levels; and

• Acute attacks of HAE are documented in the medical record.

Limitations of Coverage

Cinryze ™ is not FDA approved for the treatment of acute HAE attacks. Cinryze ™ is not considered medically reasonable and necessary and is not covered by Medicare for the treatment of acute HAE attacks.

Berinert ® is not FDA approved for prophylaxis of HAE attacks. Berinert ® is not considered medically reasonable and necessary and is not covered by Medicare for prophylaxis of HAE attacks.

Kalbitor ® (ecallantide) is not FDA approved for prophylaxis of HAE attacks. Kalbitor ® is not considered medically reasonable and necessary and is not covered by Medicare for prophylaxis of HAE attacks. Only a healthcare professional with appropriate medical support to manage anaphylaxis and hereditary angioedema (HAE) should administer this drug.

Coding Information

Bill Type Codes:

Contractors may specify Bill Types to help providers identify those Bill Types typically used to report this service. Absence of a Bill Type does not guarantee that the policy does not apply to that Bill Type. Complete absence of all Bill Types indicates that coverage is not influenced by Bill Type and the policy should be assumed to apply equally to all claims.

011x Hospital Inpatient (Including Medicare Part A) 012x Hospital Inpatient (Medicare Part B only) 013x Hospital Outpatient

014x Hospital - Laboratory Services Provided to Non-patients 022x Skilled Nursing - Inpatient (Medicare Part B only) 023x Skilled Nursing - Outpatient

071x Clinic - Rural Health 083x Ambulatory Surgery Center 085x Critical Access Hospital

Revenue Codes:

Contractors may specify Revenue Codes to help providers identify those Revenue Codes typically used to report this service. In most instances Revenue Codes are purely advisory; unless specified in the policy services reported under other Revenue Codes are equally subject to this coverage determination. Complete absence of all Revenue Codes indicates that coverage is not influenced by Revenue Code and the policy should be assumed to apply equally to all Revenue Codes.

0636 Pharmacy - Drugs Requiring Detailed Coding

CPT/HCPCS Codes

J0597 INJECTION, C-1 ESTERASE INHIBITOR (HUMAN), BERINERT, 10 UNITS J0598 INJECTION, C-1 ESTERASE INHIBITOR (HUMAN), CINRYZE, 10 UNITS J1290 INJECTION, ECALLANTIDE, 1 MG

ICD-9 Codes that Support Medical Necessity

277.6 OTHER DEFICIENCIES OF CIRCULATING ENZYMES

Diagnoses that Support Medical Necessity N/A

ICD-9 Codes that DO NOT Support Medical Necessity

ICD-9 Codes that DO NOT Support Medical Necessity Asterisk Explanation

Diagnoses that DO NOT Support Medical Necessity N/A

General Information

Documentations Requirements

Medical record documentation maintained by the ordering/referring physician or the nonphysician practitioner must substantiate the medical need for the use of these drugs by clearly indicating the condition for which the drug is being used. The medical record must indicate that the treatment is based on diagnostic information and lab work completed to confirm the diagnosis of HAE, and the specific signs and symptoms must be documented to substantiate the FDA indications for the drug usage are followed. This documentation is usually found in the

history and physical and or in the office/progress notes of the medical record.

Appendices

Utilization Guidelines It is expected that these services would be performed as indicated by current literature and/or standards of practice and should follow the guidelines for administration and safety found in the FDA approved labels for these drugs. When services are performed in excess of established parameters, they may be

subject to medical review for medical necessity.

Sources of Information and Basis for Decision

Ecallantide (Kalbitor) Advisory Committee Briefing Document: Kalbitor&REG; (ecallantide), For Acute Attacks of Hereditary Angioedema. Pulmonary-Allergy Drugs Advisory Committee January 25, 2009. Retrieved from the Internet: www.fda.gov/downloads/AdvisoryCommittee/.../UCM170334.pdf

FDA Full Prescribing Information Bernerte&REG; C1 Esterase Inhibitor (Human) Freeze Dried Powder for Intravenous Use Only.

FDA Full Prescribing Information Cinryze™ C1 Inhibitor (Human) Freeze Dried Powder for Intravenous Use Only. FDA Full Prescribing Information Kalbitor&REG; Plasma Kallikrein Inhibitor

FDA News Release: (October 10, 2008). FDA Licenses for Marketing New Therapy for Rare Genetic Disease. Retrieved from the Internet: http://www.fda.gov/NewsEvents/Newsroom/Press Announcements/ucm161477.htm

Medical News Today: March 17, 2009: Cinryze Infusion Reverses Plasma Activation in Hereditary Angioedema

The Physician’s Guide to Labaroatory Test Selection and Interpretation ARUP consult: Hereditary Angioedema: C1

-INH Deficiency Testing Algorhythm

ViroPharma Inc., Cinryze; Hereditary Angioedema (HAE): Overview and Impact of Disease (ViroPharma, Inc., is the sponsor of the clinical trial) June 3, 2009

Advisory Committee Meeting Notes This Local Coverage Determination (LCD) does not reflect the sole opinion of the contractor or Contractor Medical Director. Although the final decision rests with the contractor, this LCD was

developed in cooperation with advisory groups, which includes representatives from numerous societies.

Start Date of Comment Period 09/30/2010

End Date of Comment Period 11/13/2010

Start Date of Notice Period 12/09/2010

Revision History Number Original

Revision History Explanation Revision Number: Original Start Date of Comment Period:09/30/2010

Start Date of Notice Period:12/09/2010 Original Effective Date Original:01/23/2011

LCR A2010-064

December 2010 Bulletin

11/21/2010 - For the following CPT/HCPCS codes either the short description and/or the long description was changed. Depending on which description is used in this LCD, there may not be any change in how the code displays in the document:

J0598 descriptor was changed in Group 1

11/21/2010 - The following CPT/HCPCS codes were deleted: C9263 was deleted from Group 1

Reason for Change