Medical Policy

Effective Date:03/01/2017 Title:Hereditary Angioedema Treatment (HAE)
Revision Date:10/01/2019 Document:BI532:00
CPT Code(s):C9015, J0593, J0596-J0598, J1290
Public Statement

Effective Date:

a)    This policy will apply to all services performed on or after the above revision date which will become the new effective date.

b)    For all services referred to in this policy that were performed before the revision date, contact customer service for the rules that would apply.

1)    Cinryze, Takhzyro, and Haegarda require prior authorization for use as prophylaxis therapy in hereditary angioedema (HAE).

2)    Berinert, Firazyr, Kalbitor, and Ruconest are covered to treat acute attacks of hereditary angioedema (HAE) based on the criteria in this policy.

Medical Statement

Coverage of all products in this policy requires a diagnosis of hereditary angioedema (HAE) based on the following criteria demonstrated on two separate occasions:

·         Low C4 level (C4 < 14mg/dl; normal range 14-40mg/dl or C4 below the lower limit of normal as defined by the laboratory performing the test, plus:

a.    A low C1 inhibitor (C1INH) antigenic level (C1INH < 19mg/dl; normal range 19-37mg/dl, or C1INH antigenic level below the lower limit of normal as defined by the laboratory performing the test, OR

b.    A normal C1INH antigenic level (C1INH > or = to 19mg/dl) and a low C1INH functional level (functional C1INH < 50%) or below the lower limit of normal as defined by the laboratory performing the test.

A.   Cinryze (J0598), Takhzyro (J0593), and Haegarda (J0599) is considered medically necessary for prophylaxis against angioedema attacks in adolescents and adults when the following criteria are met. Haegarda is preferred over Cinryze and Takhzyro. Haegarda must be tried first before Cinryze or Takhzyro will be approved.

1)    Member has no signs of current acute angioedema; AND

2)    Member has a history of at least 1 HAE attack per month; AND

3)    Diagnosis of HAE (see above); AND

4)    Medications known to cause angioedema (i.e. ACE-inhibitors, estrogens, angiotensin II receptor antagonists) have been evaluated and discontinued when appropriate; AND

5)    Member has tried and failed or is intolerant to or has a contraindication to 17 alpha-alkylated androgens (e.g. Danazol, Stanozolol) or anti-fibrinolytic agents (e.g. aminocaproic acid or tranexamic acid) for HAE prophylaxis.

 

B.   The following products are covered for the treatment of acute moderate to severe attacks of hereditary angioedema (HAE) based on the diagnosis criteria above, and subject to the additional criteria below for each drug.

1)    Berinert (J0597) for the treatment of a HAE acute attack of abdominal, facial, or laryngeal areas in adolescents and adults.

2)    Kalbitor (J1290) for the treatment of acute attacks of HAE in patients 16 years of age and older.

3)    Firazyr (J1744) for the treatment of acute attacks of HAE in patients 18 years of age or older.

4)    Ruconest (J0596) for the treatment of acute attacks of HAE in adolescents and adults.

Codes Used In This BI:

ACTIVE

J0593

J0596

Injection, lanadelumab-flyo, 1mg

Injection, C-1 esterase inhibitor (recombinant), Ruconest, 10 units

J0597

Injection, C-1 esterase inhibitor (human), Berinert, 10 units

J0598 

Injection, C-1 esterase inhibitor (human), Cinryze, 10 units

J0599

Injection, C-1 esterase inhibitor (human), Haegarda, 10 units (new code 1/1/19)

J1290

Injection, ecallantide, 1 mg

J1744

Injection, icatibant, 1 mg

DELETED

C9015

Injection, C-1 esterase inhibitor (human), Haegarda, 10 units (code deleted 1/1/19)

Limits
Intentially left empty
Reference

Addendum:

Effective 01/01/2018: added Haegarda to coverage policy.

Application to Products
This policy applies to all health plans administered by QualChoice, both those insured by QualChoice and those that are self-funded by the sponsoring employer, unless there is indication in this policy otherwise or a stated exclusion in your medical plan booklet. Consult the individual plan sponsor Summary Plan Description (SPD) for self-insured plans or the specific Evidence of Coverage (EOC) for those plans insured by QualChoice. In the event of a discrepancy between this policy and a self-insured customer’s SPD or the specific QualChoice EOC, the SPD or EOC, as applicable, will prevail. State and federal mandates will be followed as they apply.

Changes: QualChoice reserves the right to alter, amend, change or supplement benefit interpretations as needed.