CurrentSecurity Health Plan of Wisconsin, Inc.Policy IC-0307

Haegarda (C1 Esterase Inhibitor Subcutaneous [Human]) for Hereditary Angioedema Prophylaxis

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This policy governs prior authorization, dosing, coverage criteria, renewal, administration, and billing for Haegarda used to prevent hereditary angioedema attacks in patients seeking coverage under Security Health Plan of Wisconsin, Inc.

Policy Summary

PayerSecurity Health Plan of Wisconsin, Inc.

PolicyHaegarda for hereditary angioedema prophylaxis

Policy CodePolicy IC-0307

Change TypeNo material change

Effective DateN/A

Last ReviewAug 5, 2025

Next Review DateN/A

Key ActionSubmit prior authorization initially for 12 months; renewal may be requested every 12 months thereafter.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

12 monthsInitial authorization

5,600/28 daysMaximum units

≥6 yearsMinimum age

60 IU/kg twice weeklyProphylactic dose

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CurrentSecurity Health Plan of Wisconsin, Inc.Policy IC-0307

Haegarda (C1 Esterase Inhibitor Subcutaneous [Human]) for Hereditary Angioedema Prophylaxis

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Policy Summary

PayerSecurity Health Plan of Wisconsin, Inc.

PolicyHaegarda for hereditary angioedema prophylaxis

Policy CodePolicy IC-0307

Change TypeNo material change

Effective DateN/A

Last ReviewAug 5, 2025

Next Review DateN/A

Key ActionSubmit prior authorization initially for 12 months; renewal may be requested every 12 months thereafter.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

12 monthsInitial authorization

5,600/28 daysMaximum units

≥6 yearsMinimum age

60 IU/kg twice weeklyProphylactic dose

Medical Necessity and Coverage Criteria

Initial approval and universal criteria

Initial coverage is provided when ALL of the following are met:

Initial approval

Age requirement
- The patient is at least 6 years of age.≥6 years
Universal criteria
- Haegarda is prescribed by or in consultation with a specialist in allergy, immunology, hematology, pulmonology, or medical genetics.
- Haegarda will not be used in combination with another prophylactic therapy targeting C1 inhibitor, activated Factor XII, or kallikrein.
  Examples include Cinryze, Andembry, Takhzyro, and Orladeyo.
- The patient is avoiding potential hereditary angioedema attack triggers.
  Triggers include estrogen-containing oral contraceptives and hormone replacement therapy; ACE inhibitors or angiotensin II receptor blockers; DPP-IV inhibitors; and neprilysin inhibitors.
Qualifying indication
- The patient requires long-term prophylactic treatment based on the provider's assessment of disease activity, quality of life, availability of health care resources, and/or failure to achieve adequate control with appropriate on-demand therapy.
  Appropriate on-demand therapies include Kalbitor, Firazyr, Ruconest, or Berinert.
- The patient has a qualifying hereditary angioedema subtype confirmed by repeat blood testing.
  Treatment for an acute attack should not be delayed for confirmatory testing.
One qualifying HAE presentation
- HAE subtype
  - HAE type I (C1-inhibitor deficiency) criteria are met.
  - HAE type II (C1-inhibitor dysfunction) criteria are met.
  - HAE with normal C1-INH criteria are met on a case-by-case basis.

HAE I (C1-inhibitor deficiency)

Coverage for HAE type I requires ALL laboratory findings plus ONE of the following etiologic or exclusion criteria:

C1-INH antigenic level is low.Below the lower limit of normal as defined by the performing laboratory
C4 level is low.Below the lower limit of normal as defined by the performing laboratory
C1-INH functional level is low.Below the lower limit of normal as defined by the performing laboratory
Family history or exclusion of acquired angioedema

HAE II (C1-inhibitor dysfunction)

Coverage for HAE type II requires ALL of the following laboratory findings:

C1-INH antigenic level is normal to elevated.
C4 level is low.Below the lower limit of normal as defined by the performing laboratory
C1-INH functional level is low.Below the lower limit of normal as defined by the performing laboratory

HAE with normal C1-INH

Long-term prophylaxis for HAE with normal C1-INH is not routinely recommended and is evaluated case by case. ALL of the following prerequisites must be met:

The patient has demonstrated an inadequate response or intolerance to an adequate trial of prophylactic therapy with an antifibrinolytic agent and/or a 17α-alkylated androgen, unless contraindicated.
Examples include tranexamic acid or aminocaproic acid and danazol. Female patients may derive additional benefit from progestins.
The patient has demonstrated a response to an agent indicated for treatment of acute attacks.
Examples include C1 esterase inhibitor, icatibant, or ecallantide.

Renewal therapy

Coverage may be renewed when ALL of the following criteria are met:

Renewal criteria

The patient continues to meet the universal and applicable indication-specific initial criteria.
The patient has no unacceptable toxicity from Haegarda.
Examples of unacceptable toxicity include severe hypersensitivity reactions and thromboembolic events.
Significant improvement in attack severity, frequency, and/or duration has been achieved and sustained.

Coverage is not provided when Haegarda is used in combination with another prophylactic therapy targeting C1 inhibitor, activated Factor XII, or kallikrein.

Long-term prophylaxis for HAE with normal C1-INH is not routinely recommended and is evaluated on a case-by-case basis. Before consideration, the patient must have demonstrated an inadequate response or intolerance to an adequate trial of an antifibrinolytic agent, such as tranexamic acid or aminocaproic acid, and/or a 17α-alkylated androgen such as danazol, unless contraindicated, as well as a response to an agent indicated for acute attacks.

Coding, Dosing, and Quantity Limits

HCPCS CodeHCPCSCovered

J0599

Injection, c-1 esterase inhibitor (human), (haegarda), 10 units; 1 billable unit = 10 IU

NDC(s)NDCCovered

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

Covered Diagnosis CodesICD-10Covered

D84.1

Defects in the complement system

Covered Diagnosis CodesICD-10Covered

D84.1

Defects in the complement system

Maximum HCPCS units

Maximum quantity5,600 billable units

Billing unitHCPCS unit

Measurement periodPer 28 days

Minimum age

Minimum ageAt least 6 years of age

Coverage applicationInitial approval criteria

Requirement type

Authorization, Documentation, and Billing Actions

Prior Authorization

12-Month Authorization Period

Obtain prior authorization for an initial 12-month period. Renewal authorization may be requested every 12 months thereafter.

Initial authorization: 12 months
Renewal authorization: every 12 months

Documentation Required

Inadequate Control With On-Demand Therapy

Document that the patient requires long-term prophylaxis based on the provider’s assessment and has not achieved adequate control with appropriate on-demand therapy.

Address disease activity, quality-of-life impact, availability of health care resources, and/or inadequate control with Kalbitor, Firazyr, Ruconest, or Berinert

Policy Background

Haegarda is a subcutaneous C1 esterase inhibitor used for long-term prophylaxis to prevent hereditary angioedema attacks. The policy recognizes hereditary angioedema types I and II based on abnormalities in C1-INH antigenic, C4, and functional C1-INH testing; HAE with normal C1-INH is evaluated case by case.

Hereditary Angioedema Definitions

HAE I (C1-inhibitor deficiency)

C1-INH antigenic levelLow; below the lower limit of normal as defined by the performing laboratory

C4 levelLow; below the lower limit of normal as defined by the performing laboratory

C1-INH functional levelLow; below the lower limit of normal as defined by the performing laboratory

Additional characteristicFamily history of HAE or acquired angioedema has been ruled out

HAE II (C1-inhibitor dysfunction)

C1-INH antigenic levelNormal to elevated

C4 level

Policy Summary

PayerSecurity Health Plan of Wisconsin, Inc.

PolicyHaegarda for hereditary angioedema prophylaxis

Policy CodePolicy IC-0307

Change TypeNo material change

Effective DateN/A

Last ReviewAug 5, 2025

Next Review DateN/A

Key ActionSubmit prior authorization initially for 12 months; renewal may be requested every 12 months thereafter.

SourceLink

On This Page

The patient has a family history of hereditary angioedema.

Acquired angioedema has been ruled out.

Examples supporting exclusion include symptom onset before age 30, normal C1q levels, and no underlying disease such as lymphoma or benign monoclonal gammopathy (MGUS).

Patient age threshold

Haegarda prophylaxis dose

Dose60 IU/kg body weight

RouteInjected subcutaneously

FrequencyTwice weekly

IntervalEvery 3 or 4 days

Step Therapy

Alternative Prophylaxis Trial

Before requesting Haegarda for HAE with normal C1-INH, document an inadequate response or intolerance to an adequate trial of required alternative prophylaxis, unless contraindicated.

Trial an antifibrinolytic agent such as tranexamic acid or aminocaproic acid and/or a 17α-alkylated androgen such as danazol
Female patients may derive additional benefit from progestins
Document response to an acute-attack treatment agent such as C1 esterase inhibitor, icatibant, or ecallantide

Documentation Required

Initial Clinical Documentation

Submit initial clinical documentation supporting medical necessity for long-term prophylaxis, including specialist involvement, disease burden, quality-of-life impact, and the rationale for prophylaxis.

Prescriber or consultant must specialize in allergy, immunology, hematology, pulmonology, or medical genetics
Document disease activity, quality-of-life impact, available health care resources, and/or inadequate control with appropriate on-demand therapy

Documentation Required

Laboratory Confirmation of HAE

Confirm the qualifying HAE subtype with repeat blood testing; acute-attack treatment should not be delayed while confirmatory testing is obtained.

HAE type I: low C1-INH antigenic level, low C4, and low C1-INH functional level, plus family history or documentation that acquired angioedema has been ruled out
HAE type II: normal-to-elevated C1-INH antigenic level, low C4, and low C1-INH functional level

Step Therapy

Normal C1-INH Documentation

For HAE with normal C1-INH, coverage is evaluated case by case and requires documentation of inadequate response or intolerance to the required alternative prophylaxis trial, unless contraindicated.

Normal C1-INH HAE is not routinely recommended for prophylaxis
Document the required antifibrinolytic and/or androgen trial and the patient’s inadequate response or intolerance

Documentation Required

Renewal Documentation

For renewal, document continued eligibility under the universal and indication-specific criteria, absence of unacceptable toxicity, and sustained clinical benefit.

Document no unacceptable toxicity, including severe hypersensitivity reactions or thromboembolic events
Document significant and sustained improvement in attack severity, frequency, and/or duration

Denial Risk

Age, Specialist, and Combination-Therapy Requirements

Verify that the patient is at least 6 years old, that the prescription is from or coordinated with an appropriate specialist, and that Haegarda is not combined with other prohibited prophylactic therapies.

Minimum age: 6 years
Specialist: allergy, immunology, hematology, pulmonology, or medical genetics
Do not combine with prophylactic therapies targeting C1 inhibitor, activated Factor XII, or kallikrein, including Cinryze, Andembry, Takhzyro, or Orladeyo

Denial Risk

Trigger Medication Avoidance

Document that the patient is avoiding medications and hormonal therapies that may trigger HAE attacks.

Estrogen-containing oral contraceptives and hormone replacement therapy
ACE inhibitors or ARBs
DPP-IV inhibitors such as sitagliptin
Neprilysin inhibitors such as sacubitril

Prior Authorization

Qualifying HAE Diagnosis and Prophylaxis Need

Establish a qualifying HAE diagnosis and a documented need for long-term prophylaxis before requesting coverage.

Use repeat blood testing to confirm the applicable HAE subtype
Document the provider-assessed need for long-term prophylaxis based on disease activity, quality of life, health care resources, and/or inadequate control with appropriate on-demand therapy
For normal C1-INH HAE, document case-by-case justification and required prior prophylaxis trials

Denial Risk

Renewal Response and Safety Requirements

At renewal, coverage may be denied when eligibility criteria are no longer met, unacceptable toxicity is present, or meaningful sustained improvement has not been demonstrated.

Continued universal and indication-specific eligibility is required
Unacceptable toxicity includes severe hypersensitivity reactions and thromboembolic events
Significant improvement in attack severity, frequency, and/or duration must be sustained

Billing Rule

Dose and Quantity Limits

Prescribe and bill within the policy’s dosing and quantity limits for prophylaxis to prevent HAE attacks.

Dose: 60 IU/kg subcutaneously twice weekly, every 3 or 4 days
Maximum billing quantity: 5,600 HCPCS units per 28 days
Patients may self-administer after instruction by their health care provider

Billing Rule

Medicare CMS Requirements

For Medicare claims, apply the Medicare Benefit Policy Manual requirements and any applicable NCD, LCD, or LCA; these requirements govern Medicare outpatient Part B drug coverage and payment.

Reference Medicare Benefit Policy Manual (Pub. 100-2), Chapter 15, §50, Drugs and Biologicals
Check applicable NCDs and LCDs for coverage requirements
Check applicable LCAs for claims payment or Part B benefit-eligibility requirements
Search CMS coverage documents at https://www.cms.gov/medicare-coverage-database/search.aspx

Low; below the lower limit of normal as defined by the performing laboratory

C1-INH functional levelLow; below the lower limit of normal as defined by the performing laboratory

HAE with normal C1-INH

Former terminologyFormerly known as HAE III

Policy stanceLong-term prophylaxis is not routinely recommended

Coverage evaluationEvaluated on a case-by-case basis

PrerequisiteInadequate response or intolerance to an adequate trial of an antifibrinolytic agent and/or a 17α-alkylated androgen, unless contraindicated

Acute-attack therapyResponse to an agent indicated for treatment of acute attacks