CurrentViva Health PlanPolicy IC-0307

Haegarda (C1 Esterase Inhibitor Subcutaneous [Human]) Coverage Criteria

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This policy governs prior authorization, medical necessity, dosing, billing, and renewal requirements for Haegarda used to prevent hereditary angioedema attacks for Viva Health Plan members.

Policy Summary

PayerViva Health Plan

PolicyHaegarda coverage for hereditary angioedema prophylaxis

Policy CodePolicy IC-0307

Change TypeNo material change

Effective DateN/A

Last ReviewOct 1, 2026

Next Review DateN/A

Key ActionSubmit documentation confirming HAE manifestations, exclusion of other causes of angioedema, medical necessity for long-term prophylaxis, and diagnostic confirmation.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

≥6 yearsMinimum member age for initial approval

5,600Maximum billable units per 28 days

12 monthsInitial authorization validity

Twice weeklyDosing schedule

J0599

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CurrentViva Health PlanPolicy IC-0307

Haegarda (C1 Esterase Inhibitor Subcutaneous [Human]) Coverage Criteria

Customize your policy alerts

Sign up for vivahealthplan Policy IC-0307 alerts

Get alerted when Policy IC-0307 changes without checking for updates manually.

Monitor payer policy activity

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This policy governs prior authorization, medical necessity, dosing, billing, and renewal requirements for Haegarda used to prevent hereditary angioedema attacks for Viva Health Plan members.

Policy Summary

PayerViva Health Plan

PolicyHaegarda coverage for hereditary angioedema prophylaxis

Policy CodePolicy IC-0307

Change TypeNo material change

Effective DateN/A

Last ReviewOct 1, 2026

Next Review DateN/A

Key ActionSubmit documentation confirming HAE manifestations, exclusion of other causes of angioedema, medical necessity for long-term prophylaxis, and diagnostic confirmation.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

≥6 yearsMinimum member age for initial approval

5,600Maximum billable units per 28 days

12 monthsInitial authorization validity

Twice weeklyDosing schedule

J0599

Coverage Criteria

Initial Therapy — Universal Criteria

Covered for initial therapy when all of the following universal criteria are met:

Universal criteria

Minimum age: Member is at least 6 years of age.Age >= 6 years
Specialist oversight: Haegarda is prescribed by, or in consultation with, a specialist in allergy, immunology, hematology, pulmonology, or medical genetics.
No prohibited combination therapy: Haegarda will not be used in combination with another therapy indicated for prophylaxis of hereditary angioedema attacks.
Combination with Cinryze is allowable only when Cinryze is requested as short-term prophylaxis.
Trigger counseling: The provider confirms that the member has been counseled to avoid possible medication triggers for HAE attacks, including estrogen-containing medications and ACE inhibitors.

Initial Therapy — HAE Prophylaxis

For prophylaxis to prevent HAE attacks, all of the following medical-necessity criteria must be met, including one qualifying HAE history pathway and one diagnostic confirmation pathway:

HAE prophylaxis criteria

ONE qualifying HAE history
- Qualifying HAE manifestations: Recurrent swelling of the mucous membranes, skin surface, or subcutaneous tissue; recurrent abdominal attacks with severe abdominal pain, nausea, vomiting, or diarrhea; episodes of upper-airway edema, such as mouth, tongue, or laryngeal edema; prodromal signs or symptoms preceding angioedema attacks; or a positive family history of HAE.
Alternative causes excluded: Other causes of angioedema have been ruled out, including drug-induced angioedema, mast-cell-induced angioedema, and acquired angioedema due to C1-INH deficiency.
Long-term prophylaxis medically necessary: The provider determines that long-term prophylactic HAE treatment is necessary based on the member's disease activity, quality of life, availability of health care resources, and/or failure to achieve adequate control with appropriate acute HAE attack therapy.

Continuation/Renewal Therapy

Renewal requires all of the following criteria:

Renewal therapy

Member continues to meet the universal and other relevant indication-specific initial criteria.
Member has no unacceptable toxicity from Haegarda.
Examples include severe hypersensitivity reactions and thromboembolic events.
Member has experienced clinical benefit from therapy.
Examples include reduced attack severity or frequency and shortened attack duration.

Combination prophylaxis is not covered when Haegarda is used with another therapy indicated to prevent hereditary angioedema (HAE) attacks. An exception applies when Cinryze is requested for short-term prophylaxis only.

Coverage requires that other causes of angioedema be ruled out before Haegarda is authorized. This includes drug-induced angioedema, mast-cell-induced angioedema, and acquired angioedema due to C1-INH deficiency.

Coding and Quantity Parameters

Covered HCPCS CodesHCPCSCovered

J0599

Injection, c-1 esterase inhibitor (human), (haegarda), 10 units

Covered NDCsNDCCovered

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

Dosing Limit

Maximum quantity5,600 billable units

Time periodPer 28 days

Billing basisHCPCS billable units

Minimum Age

Minimum member ageAt least 6 years of age

ApplicabilityRequired for initial approval

Age threshold

Authorization, Documentation, and Denial Risks

Prior Authorization

Authorization Duration

The initial prior authorization is valid for 12 months (365 days). Renewal authorization may be granted for additional 12-month (365-day) periods when renewal criteria are met.

Initial authorization: 12 months (365 days).
Renewals: every 12 months (365 days).

Documentation Required

Specialist Prescribing and Counseling

Haegarda must be prescribed by, or used in consultation with, a specialist in allergy, immunology, hematology, pulmonology, or medical genetics. The provider must confirm that the member has been counseled to avoid possible medication triggers for HAE attacks.

Acceptable specialist areas: allergy, immunology, hematology, pulmonology, or medical genetics.
Counseling must address potential triggers, including estrogen-containing medications and ACE inhibitors.

Clinical Background

Hereditary angioedema (HAE) is characterized by recurrent episodes of swelling involving the skin, mucous membranes, subcutaneous tissues, abdomen, or upper airway. Haegarda is a subcutaneous C1 esterase inhibitor used for long-term prophylaxis to prevent HAE attacks, including HAE caused by C1 inhibitor deficiency and specified HAE with normal C1 inhibitor.

Utilization Management Definitions

Non-Quantitative Treatment Limitation (NQTL)

DefinitionMethods, guidelines, standards of evidence, or other conditions that can restrict how long or to what extent benefits are provided under a health plan.

ExamplesUtilization review and prior authorization

ApplicationThe utilization management NQTL applies comparably, and not more stringently, to mental health/substance use disorder and medical/surgical medical benefit prescription drugs.

Initial Therapy

Initial approval requires the member to be at least 6 years old, meet all universal criteria, and satisfy the HAE prophylaxis criteria.

Initial Haegarda approval

Minimum age: Member is at least 6 years of age.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 therapy indicated for prophylaxis of hereditary angioedema attacks.
  Combination with Cinryze is allowable only when Cinryze is requested as short-term prophylaxis.

Renewal Therapy

Renewal approval is available every 12 months when all of the following continuation criteria are met:

Continuation criteria

Member continues to meet applicable universal and indication-specific criteria.
Member has no unacceptable toxicity from Haegarda.
Examples include severe hypersensitivity reactions and thromboembolic events.
Member has experienced clinical benefit from Haegarda.
Examples include reduced attack severity or frequency and shortened attack duration.

Required Prior Treatment Pathways

Step	Required criteria
1	For the HAE with normal C1-INH pathway, recurrent angioedema attacks without hives must remain refractory to a 4-week trial of high-dose antihistamines (a second-generation H1 antagonist at four times the standard dose). The member must also have either a prior beneficial response to a therapy indicated for acute HAE attacks or inadequate response or intolerance to an adequate trial of another prophylactic HAE therapy.

Step

Required criteria

For the HAE with normal C1-INH pathway, recurrent angioedema attacks without hives must remain refractory to a 4-week trial of high-dose antihistamines (a second-generation H1 antagonist at four times the standard dose). The member must also have either a prior beneficial response to a therapy indicated for acute HAE attacks or inadequate response or intolerance to an adequate trial of another prophylactic HAE therapy.

Step	Alternative prophylactic therapy	Required outcome
2	Antifibrinolytic agent, such as tranexamic acid or aminocaproic acid; 17α-alkylated androgen, such as danazol; and/or progestin therapy specific to females.	The member must demonstrate an inadequate response or intolerance to an adequate trial of the alternative prophylactic HAE therapy.

Quantity Limits

Haegarda Quantity Limit

DrugHaegarda

Quantity limit5,600 billable units

Limit periodPer 28 days

Administration Setting

Note

Instruct on home administration

Haegarda is injected subcutaneously twice weekly, every 3 or 4 days. Members may self-administer after receiving instruction from their healthcare provider.

Dose for HAE prophylaxis: 60 IU/kg body weight injected subcutaneously twice weekly.

Policy Summary

PayerViva Health Plan

PolicyHaegarda coverage for hereditary angioedema prophylaxis

Policy CodePolicy IC-0307

Change TypeNo material change

Effective DateN/A

Last ReviewOct 1, 2026

Next Review DateN/A

Key ActionSubmit documentation confirming HAE manifestations, exclusion of other causes of angioedema, medical necessity for long-term prophylaxis, and diagnostic confirmation.

SourceLink

On This Page

ONE diagnostic confirmation pathway

HAE-C1-INH Type 1 or 2
- Current or historical C1-INH antigenic level is below the lower limit of normal as defined by the laboratory performing the test.
- Current or historical C1-INH functional level is below the lower limit of normal as defined by the laboratory performing the test.
- Genetic testing confirms pathogenic mutation(s) in SERPING1.
HAE with normal C1-INH
- Current or historical C1-INH level is normal as defined by the laboratory performing the test.
- ONE qualifying HAE-nC1-INH pathway
  - Confirmed presence of a known HAE-causing mutation in F12, ANGPT1, PLG, KNG1, MYOF, and/or HS3ST6.
  - Recurrent angioedema attacks without hives remain refractory to a 4-week trial of high-dose antihistamines.Second-generation H1 antagonist at 4 times the standard dose for 4 weeks
    For this clinical pathway, the member must also demonstrate either a prior beneficial response to acute HAE therapy or inadequate response or intolerance to an adequate trial of another prophylactic HAE therapy.

Documentation Required

Specialist and Trigger-Counseling Documentation

Document the required specialist involvement and the provider's counseling regarding avoidance of potential HAE mediators and triggers.

Record the specialist prescriber or consultation.
Record counseling to avoid possible triggers such as estrogen-containing medications and ACE inhibitors.

Prior Authorization

Prior Authorization Factors

Prior authorization for this drug was considered based on the indication and drug cost. Safety and efficacy, and potential for misuse or abuse, were not prioritized as prior-authorization factors.

Indication: considered for prior authorization.
Drug cost: considered for prior authorization.
Safety and efficacy: prior authorization not a priority.
Potential for misuse/abuse: prior authorization not a priority.

Billing Rule

Medicare Part B Requirements

For Medicare coverage determinations, the policy's preceding information applies to non-Medicare coverage. Outpatient Medicare Part B drug coverage must follow the Medicare Benefit Policy Manual, Pub. 100-2, Chapter 15, §50, along with any applicable NCD, LCD, and LCA requirements.

Search applicable NCDs, LCDs, and LCAs in the CMS Medicare Coverage Database: https://www.cms.gov/medicare-coveragedatabase/search.aspx.
Additional indications may be applied at the health plan's discretion.
Medicare Part B covered diagnosis codes for applicable existing NCD/LCD/LCA: N/A.
Applicable MAC jurisdiction and contractor: E (CA, HI, NV, AS, GU, CNMI)—Noridian Healthcare Solutions, LLC; F (AK, WA, OR, ID, ND, SD, MT, WY, UT, AZ)—Noridian Healthcare Solutions, LLC/Wisconsin Physicians Service Insurance Corp. (WPS); 5 (MN, WI, IL)—Wellpoint Federal; H (LA, AR, MS, TX, OK, CO, NM)—Novitas Solutions, Inc.; 8 (MI, IN)—Wisconsin Physicians Service Insurance Corp. (WPS); N (FL, PR, VI)—First Coast Service Options, Inc.; J (TN, GA, AL)—Palmetto GBA; M (NC, SC, WV, VA, excluding specified areas)—Palmetto GBA; L (DE, MD, PA, NJ, DC, including Arlington and Fairfax)—Novitas Solutions, Inc.; K (NY, CT, MA, RI, VT, ME, NH)—Wellpoint Federal; 15 (KY, OH)—CGS Administrators, LLC.

Step Therapy

Prior HAE Treatment Response or Trial

For the applicable recurrent angioedema pathway, documentation must show a prior beneficial response to acute HAE therapy or an inadequate response or intolerance to an adequate trial of another prophylactic HAE therapy.

Prior beneficial response to a therapy indicated for acute HAE attacks; or
Inadequate response or intolerance to an adequate trial of another prophylactic HAE therapy, such as tranexamic acid, aminocaproic acid, danazol, or progestin therapy when specific to females.

Unsupported block type: documentation

Denial Risk

Age or Specialist Requirement Not Met

A request is at risk of denial when the member is younger than 6 years or when Haegarda is not prescribed by, and is not used in consultation with, an appropriate specialist.

Member must be at least 6 years of age.
Required specialist prescribing or consultation: allergy, immunology, hematology, pulmonology, or medical genetics.

Denial Risk

Prohibited Combination Prophylaxis

Haegarda must not be used in combination with another therapy indicated for prophylaxis of HAE attacks. Combination therapy with Cinryze is allowed only when Cinryze is requested for short-term prophylaxis.

Denial risk: concurrent use with another HAE prophylaxis therapy.
Exception: Cinryze may be combined only for short-term prophylaxis.

Denial Risk

HAE Diagnosis or Medical Necessity Not Established

Coverage is at risk of denial when the HAE diagnosis or medical necessity for long-term prophylaxis is not established in the clinical record.

No qualifying HAE manifestations or family history are documented.
Alternative causes of angioedema have not been ruled out.
The record does not support the need for long-term prophylaxis based on disease activity, quality of life, health care resources, or inadequate control with acute therapy.
Required laboratory or genetic confirmation of HAE subtype is absent.
For the normal-C1-INH pathway, recurrent attacks without hives and failure of a 4-week high-dose antihistamine trial are not established.

Denial Risk

Renewal Criteria Not Met

Renewal is at risk of denial when continued eligibility, tolerability, or clinical benefit is not documented.

The member no longer meets universal or applicable indication-specific criteria.
Unacceptable toxicity is present or has not been assessed.
Ongoing clinical benefit, such as reduced attack severity or frequency or shorter attack duration, is not demonstrated.

Denial Risk

Medicare Part B Policy Compliance

For Medicare Part B claims or coverage determinations, failure to follow applicable Medicare requirements may result in denial or require handling under the relevant Medicare policy. Apply the Medicare Benefit Policy Manual, Pub. 100-2, Chapter 15, §50, and any applicable NCD, LCD, or LCA.

The policy's non-Medicare criteria do not replace applicable Medicare Part B determinations.
Check the CMS Medicare Coverage Database for current NCD, LCD, and LCA requirements.
Existing applicable NCD/LCD/LCA Medicare Part B covered diagnosis codes are listed as N/A.
Follow the applicable Medicare Administrative Contractor jurisdiction and contractor requirements for the member's state or territory.

The provider confirms that the member has been counseled to avoid possible medication triggers for HAE attacks, including estrogen-containing medications and ACE inhibitors.

HAE prophylaxis medical necessity

ONE qualifying HAE history
- Recurrent swelling of the mucous membranes, skin surface, or subcutaneous tissue; recurrent abdominal attacks with severe abdominal pain, nausea, vomiting, or diarrhea; episodes of upper-airway edema; prodromal signs or symptoms preceding angioedema attacks; or a positive family history of HAE.
Other causes of angioedema have been ruled out.
The provider determines that long-term prophylactic HAE treatment is necessary based on disease activity, quality of life, availability of health care resources, and/or failure to achieve adequate control with appropriate acute HAE attack therapy.
ONE diagnostic confirmation pathway
- HAE-C1-INH Type 1 or 2
  - C1-INH antigenic level or functional level is currently or historically below the laboratory lower limit of normal, or genetic testing confirms pathogenic mutation(s) in SERPING1.
- HAE with normal C1-INH
  - Current or historical C1-INH level is normal as defined by the laboratory.
  - ANY of the following
    Known HAE-causing mutation(s) are confirmed in F12, ANGPT1, PLG, KNG1, MYOF, and/or HS3ST6.
    Recurrent angioedema attacks without hives are refractory to a 4-week trial of high-dose antihistamines.Second-generation H1 antagonist at 4 times the standard dose for 4 weeks
    The member must also demonstrate either prior beneficial response to acute HAE therapy or inadequate response or intolerance to an adequate trial of another prophylactic HAE therapy.