LCD/NCD Portal

L31508

 

SELECTIVE TREATMENT OF HAE WITH CINRYZE ™, BERINERT ® AND ECALLANTIDE

 

01/23/2011

 

Indications and Limitations of Coverage and/or Medical Necessity

 

Indications of Coverage

 

• Indications for testing include:

o Recurrent angioedema.

o Laryngeal edema.

o Abdominal pain in the absence of urticaria.

o 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.

o C-1 esterase for type I will reveal low antigenic and functional levels.

o Type II will reveal normal antigenic level but low functional level of C1-INH.

o 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:

o 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.

o 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.

 History of at least 2 HAE attacks per month.

• In the Medicare beneficiary population, it is expected that the documentation supports that other therapies have unsuccessfully been attempted, such as:

o 17 alpha-alkylated androgens (Danazol, Stanozolol),

o Anti-fibrinolytic agents such as:

 Epsilon amniocaproic acid.

 Tranexamic acid for HAE prophylaxis.

• Treatment for Acute Attacks of HAE:

o 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).

o 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.

 Acute abdominal or facial attack

o Kalbitor® (ecallantide) is a plasma kallkrein inhibitor for treatment of acute attacks of herediatary angioedema (HAE) in patients 16 years of age and older.

o 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.

o Only a healthcare professional with appropriate medical support to manage anaphylaxis and hereditary angioedema (HAE) should administer this drug.

o 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 attacks.

o Only a healthcare professional with appropriate medical support to manage anaphylaxis and hereditary angioedema (HAE) should administer this drug.

 

 

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

 

 

Documentation 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 or in the office/progress notes of the medical record.

 

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.

 

Treatment Logic

• 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 dependent 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.

 

Sources of Information and Basis for Decision

 

Ecallantide (Kalbitor) Advisory Committee Briefing Document: Kalbitor® (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

 

FCSO LCD 31475, Selective Treatment of HAE with Cinryze ™, Berinert ® and Ecallantide, 01/23/2011. The official local coverage determination (LCD) is the version on the Medicare coverage database at www.cms.gov/medicare-coverage-database/.

 

FDA Full Prescribing Information Bernerte® 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® 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 Laboratory 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

 

 

AMA CPT / ADA CDT Copyright Statement

CPT codes, descriptions and other data only are copyright 2012 American Medical Association (or such other date of publication of CPT). All Rights Reserved. Applicable FARS/DFARS Clauses Apply. Current Dental Terminology, (CDT) (including procedure codes, nomenclature, descriptors and other data contained therein) is copyright by the American Dental Association. © 2002, 2004 American Dental Association. All rights reserved. Applicable FARS/DFARS apply.

 

CMS LCD L31508 Selective Treatment of HAE with Cinryze ™, Berinert ® and Ecallantide