CurrentViva HealthPolicy IC-0169

Icatibant (Firazyr) for acute hereditary angioedema (HAE)

Defines prior authorization, eligibility, dosing, and renewal criteria for icatibant (Firazyr) to treat acute HAE attacks for Viva Health members. Applies to prescribers and prior authorization reviewers.

Policy Summary

PayerViva Health

PolicyIcatibant (Firazyr) for acute hereditary angioedema (HAE)

Policy CodePolicy IC-0169

Change TypeNo material changes

Effective Date

Next Review DateAug 5, 2025

Key ActionObtain prior authorization (initially for 12 weeks; may be renewed every 12 weeks) and document confirmatory repeat blood testing consistent with the HAE subtype.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

12 weeksinitial prior authorization duration

360maximum billable units per 28 days

30 mgstandard subcutaneous dose

J1744HCPCS billing code

D84.1covered diagnosis ICD-10 code

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Initial therapy for acute HAE attacks

Covered when ALL of the following are met

Age: Patient is at least 18 years of age>=18 years

Prescriber: Must be prescribed by, or in consultation with, a specialist in allergy, immunology, hematology, pulmonology, or medical genetics

No duplicate acute therapy: Will not be used in combination with another agent indicated for the treatment of acute attacks (Berinert, Kalbitor, Ruconest) unless case-by-case justification provided

Requests for combination duplicate therapy may be reviewed case-by-case

Trigger avoidance: Confirmation patient is avoiding estrogen-containing OCPs/HRT, ACE inhibitors or ARBs, DPP-IV inhibitors, and neprilysin inhibitors

Clinical presentation: History of moderate to severe cutaneous attacks (without urticaria) OR abdominal attacks OR mild to severe airway swelling attacks (debilitating cutaneous/GI symptoms OR laryngeal/pharyngeal/tongue swelling)

HAE subtype confirmation

HAE I labs: Low C1-INH antigenic level AND low C4 AND low C1-INH functional level
Family history of HAE or exclusion of acquired angioedema
HAE II labs: Normal to elevated C1-INH antigenic level AND low C4 AND low C1-INH functional level
HAE with normal C1-INH: Normal C1-INH antigenic, C4, and functional levels AND repeat testing during an attack excludes I/II AND (known HAE-causing mutation OR family history plus documented lack of efficacy of chronic high-dose antihistamines)

Dose: Administer 30 mg subcutaneously in the abdominal area; may repeat every 6 hours up to 3 doses (90 mg) in 24 hours; patient may self-administer after instruction30 mg; up to 90 mg/24h

Renewal criteria

Renewal covered when ALL of the following are met

Continued criteria: Patient continues to meet universal and indication-specific criteria

Clinical benefit: Significant improvement in severity and duration of attacks has been achieved and sustained

Tolerability: Absence of unacceptable toxicity from the drug (examples include laryngeal HAE attacks)

On-hand medication consideration: Authorization will account for cumulative amount of HAE medications on-hand to provide sufficient quantity to treat up to 4 acute attacks per 4 weeks for the duration of the authorizationup to 4 attacks per 4 weeks

Medicare Part B Coverage Reference

Medicare Part B outpatient drug coverage follows CMS manuals and applicable NCD/LCD/LCA determinations:

Medicare applicability: Coverage for outpatient (Part B) drugs is determined by the Medicare Benefit Policy Manual Chapter 15 §50 and any applicable National or Local Coverage Determinations or Local Coverage Articles

Providers must comply with NCDs/LCDs/LCAs where applicable

Combination use of icatibant (Firazyr) with another agent indicated for acute HAE attacks (for example, Berinert, Kalbitor, or Ruconest) is not authorized as routine therapy. Such requests will only be considered when a clear, case‑specific justification is provided and will be reviewed on a case‑by‑case basis.

This appendix is intended to support non‑Medicare coverage determinations. Medicare Part B outpatient drug coverage is governed by the Medicare Benefit Policy Manual (Pub. 100‑2), Chapter 15, §50 and by any applicable National Coverage Determinations (NCDs), Local Coverage Determinations (LCDs), or Local Coverage Articles (LCAs). Where Medicare rules apply, providers and reviewers must comply with those CMS documents; use the CMS coverage database search (https://www.cms.gov/medicare-coveragedatabase/search.aspx) for applicable NCD/LCD/LCA guidance.

Provider Actions and Prior Authorization

Prior Authorization

Prior Authorization Required

Prior authorization is required. Initial authorizations are provided for 12 weeks and may be renewed every 12 weeks. Authorizations will account for the cumulative amount of on‑hand medication for acute HAE treatment and will generally provide sufficient quantity to treat up to 4 acute attacks per 4 weeks during the authorization period.

Initial authorization: 12 weeks
Renewal: may be renewed every 12 weeks
Billing code: HCPCS J1744 (icatibant), 1 mg per unit

Documentation Required

Documentation Required — Diagnostic Testing

Providers must document confirmatory laboratory testing consistent with the applicable HAE subtype prior to and/or at time of request. Documentation should include relevant antigenic and functional C1‑INH testing and C4 levels; for HAE with normal C1‑INH, repeat blood testing during an attack to exclude HAE I/II and genetic testing or family history and treatment response where applicable.

HAE I: antigenic C1‑INH low, C4 low, C1‑INH functional low
HAE II: C1‑INH antigenic normal/elevated, C4 low, C1‑INH functional low
HAE with normal C1‑INH: repeat testing during attack to exclude I/II; genetic mutation or family history and lack of response to high‑dose antihistamines

Prior Authorization

Trigger Avoidance and Combination Therapy

Providers must confirm avoidance of known potential HAE triggers prior to authorization. Examples include estrogen‑containing therapies, ACE inhibitors or ARBs, DPP‑IV inhibitors, and neprilysin inhibitors. Requests that propose combination use with another agent indicated for acute HAE attacks (e.g., C1 esterase inhibitors, ecallantide, recombinant C1‑INH) will be reviewed and require case‑by‑case justification.

Avoid estrogen‑containing oral contraceptives and hormone replacement therapy
Avoid ACE inhibitors and ARBs
Avoid DPP‑IV inhibitors (e.g., sitagliptin)
Avoid neprilysin inhibitors (e.g., sacubitril)
Combination therapy (e.g., Berinert, Kalbitor, Ruconest) requires justification and will be reviewed case‑by‑case

Denial Risk

Medicare Requirements and Noncompliance Risk

Providers should reference the Medicare Benefit Policy Manual Chapter 15 §50 and review any applicable NCDs, LCDs, or LCAs when Medicare Part B coverage may apply. Noncompliance with existing NCDs or LCDs may result in denial of coverage where Medicare policies govern; providers must consult CMS resources and include relevant documentation to demonstrate compliance.

Follow Medicare Benefit Policy Manual Chapter 15 §50 when Medicare Part B applies
Search for applicable NCDs/LCDs/LCAs via the CMS Coverage Database
Noncompliance with NCDs/LCDs may lead to denial — include documentation showing compliance when applicable

Prior Authorization

Requests for Combination Use Reviewed Case‑by‑Case

Requests for use in combination with another agent indicated for acute HAE attacks are not routinely approved and will be evaluated on a case‑by‑case basis. The provider must supply clinical justification, supporting documentation of prior therapies and responses, and rationale why combination therapy is clinically necessary for the individual patient.

Combination use with C1 esterase inhibitors (human or recombinant), ecallantide, or other acute HAE agents requires detailed justification
Provide prior therapy history, response documentation, and clinical rationale for combination use

Definitions and Subtypes of HAE

HAE type I

DefinitionLow C1-INH antigenic level, low C4, low C1-INH functional level

Supporting featuresFamily history of HAE or onset before age 30

Exclusion criteriaAcquired angioedema must be ruled out (e.g., normal C1q, no underlying lymphoma or MGUS)

HAE type II

DefinitionNormal to elevated C1-INH antigenic level with low C4 and low C1-INH functional level

Laboratory patternC1-INH antigenic: normal/elevated; C4: below LLN; C1-INH function: below LLN

Use in criteriaMeets subtype confirmation for HAE II when repeat testing supports this pattern

HAE with normal C1‑INH (formerly HAE III)

DefinitionNormal C1-INH antigenic, C4, and functional levels

Confirmatory testingRepeat blood testing during an attack must exclude abnormal values indicative of HAE I or II

Additional requirementAnd either a known HAE-causing mutation OR family history plus documented lack of efficacy of chronic high‑dose antihistamines

NCD/LCD/LCA

DefinitionCMS documents (NCD/LCD/LCA) that define Medicare coverage and billing policy for drugs and biologics under Part B

Primary referenceMedicare Benefit Policy Manual (Pub. 100-02), Chapter 15, §50 Drugs and Biologicals

Search resourceCMS Coverage Database: https://www.cms.gov/medicare-coveragedatabase/search.aspx

OpenPayer

Icatibant (Firazyr) for acute hereditary angioedema (HAE)

Coverage Criteria for Icatibant (Firazyr)

Coding and Billing

Provider Actions and Prior Authorization

Background

Definitions and Subtypes of HAE