CurrentViva HealthPolicy IC-0307

Haegarda (C1 Esterase Inhibitor Subcutaneous) coverage

Defines prior authorization, eligibility, dosing, and renewal criteria for Haegarda (subcutaneous C1 esterase inhibitor) for prevention of hereditary angioedema (HAE) for Viva Health members.

Policy Summary

PayerViva Health

PolicyHaegarda (C1 Esterase Inhibitor Subcutaneous) coverage

Policy CodePolicy IC-0307

Change TypeNo material change

Effective Date

Next Review DateAug 5, 2025

Key ActionSubmit prior authorization with supporting documentation including indication, age, and prescriber specialty for J0599 (Haegarda); initial approvals are for 12 months and may be renewed every 12 months.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

12 moinitial prior authorization duration

5,600maximum billable units per 28 days

>=6minimum patient age for coverage

60 IU/kgrecommended prophylactic dosing

J0599HCPCS code

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inv-01: Initial Therapy Criteria

Covered when ALL of the following are met

Age: Patient is at least 6 years of age>=6 years

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

Concomitant therapies: Will not be used in combination with other prophylactic therapies targeting C1 inhibitor, activated Factor XII, or kallikrein

Examples: Cinryze; Andembry; Takhzyro; Orladeyo

Trigger avoidance: Confirmation patient is avoiding specified triggers for HAE attacks

Triggers include: estrogen-containing oral contraceptives and hormone replacement therapy; ACE inhibitors or ARBs; DPP‑IV inhibitors (e.g., sitagliptin); neprilysin inhibitors (e.g., sacubitril)

Indication - long-term prophylaxis: Patient requires long-term prophylactic treatment based on provider assessment of disease activity, quality of life, availability of health care resources, and/or failure to achieve adequate control with appropriate on‑demand therapy

On‑demand therapies include Kalbitor, Firazyr, Ruconest, or Berinert

HAE subtype diagnostic criteria

HAE I: Low C1‑INH antigenic level AND low C4 AND low C1‑INH functional level; PLUS family history of HAE OR acquired angioedema ruled out
HAE II: Normal to elevated C1‑INH antigenic level AND low C4 AND low C1‑INH functional level
HAE with normal C1‑INH: Prophylaxis considered case‑by‑case after inadequate response or intolerance to an adequate trial of antifibrinolytic agents and/or 17α‑alkylated androgens (unless contraindicated) AND demonstrated response to an agent indicated for acute attacks
Examples: tranexamic acid, aminocaproic acid, danazol; acute‑attack agents include C1 esterase inhibitor, icatibant, ecallantide

inv-02: Renewal Criteria

Renewal when ALL of the following are met

Continuation of criteria: Patient continues to meet the universal and indication‑specific criteria from initial approval

Toxicity: Absence of unacceptable toxicity from the drug (examples: severe hypersensitivity reactions, thromboembolic events)

Clinical benefit: Significant improvement in severity, frequency, and/or duration of attacks has been achieved and sustained

For members with HAE with normal C1‑INH (formerly HAE III), long‑term prophylaxis with subcutaneous C1 esterase inhibitor is not routinely recommended and will be considered on a case‑by‑case basis. Prior to approval for prophylaxis, documentation must show an inadequate response or intolerance to an adequate trial of prophylactic antifibrinolytic therapy (e.g., tranexamic acid or aminocaproic acid) and/or a 17α‑alkylated androgen (e.g., danazol), unless contraindicated, and evidence of response to an agent indicated for acute attacks (for example, C1 esterase inhibitor, icatibant, or ecallantide).

Provider Requirements and Prior Authorization

Prior Authorization

Prior Authorization Required

Prior authorization is required. Initial approvals will be provided for 12 months; renewals may be authorized every 12 months thereafter.

Initial authorization duration: 12 months
Renewal authorization: May be renewed every 12 months

Step Therapy

Step Therapy — HAE with Normal C1‑INH

Step therapy applies for patients with HAE with normal C1-INH. Long-term prophylaxis is not routinely recommended and will be considered only after required prior therapeutic trials and documented clinical response to on-demand therapy.

HAE with normal C1-INH: Evaluate prophylaxis case-by-case
Required trial: Inadequate response or intolerance to an adequate trial of an antifibrinolytic (e.g., tranexamic acid, aminocaproic acid) and/or a 17α-alkylated androgen (e.g., danazol) unless contraindicated
Female patients: may derive additional benefit from progestins
Required demonstration of response to an agent indicated for acute attack treatment (e.g., C1 esterase inhibitor, icatibant, ecallantide)

Documentation Required

Provider Documentation and Denial Triggers

Provider documentation must support medical necessity and confirm subtype with laboratory testing; denials may be triggered if specialist involvement or required documentation is absent or if excluded treatment combinations are prescribed.

Prescriber requirement: Must be prescribed by, or in consultation with, a specialist in allergy, immunology, hematology, pulmonology, or medical genetics — absence of specialist involvement may trigger denial
Required documentation: Specialist prescription or consultation notes, laboratory confirmation of HAE subtype (repeat blood testing showing C1‑INH antigenic level, C1‑INH functional level, and C4 level as appropriate), documentation of clinical presentation and family history or exclusion of acquired angioedema
Prophylaxis criteria documentation: Evidence the patient requires long‑term prophylaxis (disease activity, QoL impact, failure of adequate on‑demand therapy), and for HAE with normal C1‑INH provide documentation of prior trials (antifibrinolytic and/or androgen) or contraindications
Therapy exclusions: Will not be used in combination with other prophylactic therapies targeting C1 inhibitor, activated Factor XII, or kallikrein (e.g., Cinryze, Andembry, Takhzyro, Orladeyo) — concurrent use may trigger denial
Medication/treatment avoidance confirmation: Documentation that the patient is avoiding potential HAE triggers (estrogen-containing OCPs/HRT, ACE inhibitors or ARBs, DPP‑IV inhibitors, neprilysin inhibitors)

Definitions of HAE Subtypes

inv-13: HAE type I

DefinitionC1-INH deficiency characterized by low C1-INH antigenic level, low C4, and low C1-INH functional level

Supporting criteriaDiagnosis supported by family history of HAE or exclusion of acquired angioedema (e.g., symptom onset before age 30, normal C1q, no underlying lymphoma or MGUS)

Laboratory thresholds’Low’ defined as below the lower limit of normal for the laboratory performing the test

inv-14: HAE type II

DefinitionC1-INH dysfunction with normal or elevated C1-INH antigenic level and low C4 and low C1-INH functional level

Laboratory requirementsNormal/elevated antigenic level AND low C4 AND low functional level, each per laboratory lower limits of normal

Distinguishing featureAntigenic C1-INH level is not reduced (normal to elevated) compared with HAE type I

inv-15: HAE with normal C1-INH

Definition/alternative namePreviously known as HAE III (HAE with normal C1-INH)

Prophylaxis stanceProphylaxis for HAE with normal C1-INH is not routinely recommended and will be evaluated on a case-by-case basis

Prerequisites before prophylaxisRequires inadequate response or intolerance to adequate trial of antifibrinolytic agents and/or 17α-alkylated androgens (unless contraindicated) and demonstrated response to an acute‑attack agent

Example agents referencedAntifibrinolytics (e.g., tranexamic acid, aminocaproic acid); androgens (e.g., danazol); acute‑attack agents include C1 esterase inhibitor, icatibant, ecallantide

63833-0828-xx	Haegarda 2000 IU single-dose vial kit NDC
63833-0829-xx	Haegarda 3000 IU single-dose vial kit NDC

OpenPayer

Haegarda (C1 Esterase Inhibitor Subcutaneous) coverage

Coverage Criteria for Haegarda (C1-INH Subcutaneous)

Coding and Quantity Limits

Provider Requirements and Prior Authorization

Background

Definitions of HAE Subtypes