Haegarda (C1 esterase inhibitor, human) Medication Precertification Request — Coverage Criteria
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This document is Aetna's precertification request form and clinical checklist for Haegarda used for prevention of hereditary angioedema (HAE); it governs information and documentation providers must submit to request authorization or continuation of therapy for Aetna members.
No material clinical or coverage changes in this revision.
Coverage Criteria for Haegarda (C1 Esterase Inhibitor, Human)
Initial and continuation criteria
Coverage is considered when required documentation and diagnostic criteria for hereditary angioedema (HAE) are provided and when continuation demonstrates clinical benefit.
Initial request requirements
- Indication: Documentation that the requested medication is being used for prevention (prophylaxis) of hereditary angioedema (HAE) attacks.
- Concomitant therapy: Whether the requested medication will be used in combination with any other medication used for prophylaxis of HAE.
- Attack frequency: Number of HAE attacks per month (provide current attack frequency).
- Prescriber specialty: Prescribed by or in consultation with a prescriber who specializes in management of HAE (or documentation of specialist consultation).
Diagnostic confirmation of HAE with C1‑INH deficiency/dysfunction (ONE of)
- C1‑INH antigenic level below the lower limit of normal as defined by the performing laboratory.
- Normal C1‑INH antigenic level AND low C1‑INH functional level (functional C1‑INH < 50% or below the laboratory lower limit of normal).
- Other documentation supporting HAE with C1‑INH deficiency/dysfunction (as indicated on the form).
Diagnostic confirmation of HAE with normal C1‑INH (ONE of)
- Genetic testing confirming a pathogenic mutation (e.g., F12, angiopoietin‑1, plasminogen, KNG1, HS3ST6, or MYOF).
- Both of the following: 1) Angioedema refractory to a trial of high‑dose antihistamine therapy (e.g., cetirizine 40 mg/day or equivalent) for at least 1 month; AND 2) Family history of angioedema.
Continuation of therapy requirements
- Evidence of significant clinical benefit defined as a reduction in frequency of acute attacks since starting treatment (e.g., >= 50%).>=50% attack reduction
- Reduced use of medications to treat acute attacks since initiating the requested medication (document change in acute‑treatment use).
This precertification form distinguishes between HAE with C1‑INH deficiency or dysfunction and HAE with normal C1‑INH and requires laboratory or genetic confirmation appropriate to the subtype. For HAE with C1‑INH deficiency/dysfunction the form requires either an antigenic C1‑INH level below the laboratory lower limit of normal or a normal antigenic level with a low functional C1‑INH (defined as functional C1‑INH less than 50% or below the laboratory lower limit of normal). For HAE with normal C1‑INH the form requires genetic confirmation of a relevant mutation or documentation that both (1) angioedema is refractory to a trial of high‑dose antihistamine (e.g., cetirizine 40 mg/day or equivalent for ≥1 month) and a family history of angioedema.
The form does not include a standalone list of conditions labeled as "not medically necessary." Instead, it relies on explicit diagnostic confirmation fields to establish medical necessity for Haegarda. Providers must complete the clinical diagnostic sections that specify the C1‑INH antigenic and/or functional laboratory results or the genetic testing results for HAE with normal C1‑INH to support a coverage determination.
Coding and Clinical Metrics
| Administration code(s) (CPT) | Form requests administration CPT code(s) to be provided by the provider |
| Primary ICD Code | Primary diagnosis ICD code field for HAE or other diagnosis |
| Secondary ICD Code | Secondary diagnosis ICD code field |
Provider Actions and Submission Requirements
Precertification Required
Precertification required — providers must submit the Aetna precertification request form to Aetna Precertification Notification via phone or fax prior to initiation.
- Phone: 1-866-752-7021
- FAX: 1-888-267-3277
- For Medicare Advantage Part B: Use Medicare Request Form
Prior Therapy Documentation
Prior therapy documentation — the precertification form asks whether Haegarda will be used with other prophylactic HAE medications and requests documentation of prior trials and attack frequency. For continuation requests, the form requires documentation of clinical benefit (e.g., >= 50% reduction in attack frequency) and reduced use of medications to treat acute attacks.
- Indicate if Haegarda will be used in combination with other prophylactic HAE medications
- Document number of HAE attacks per month
- For continuation: document significant reduction in attack frequency (e.g., >= 50%) and reduced use of acute-attack medications
Required Documentation
Required documentation — complete all required form fields including patient, insurance, prescriber, dispensing/provider, product (dose and frequency), primary and other clinical details. Ensure prescriber specialty and diagnostic laboratory/genetic test results (C1-INH antigenic and/or functional levels, or relevant genetic mutation) and trials of alternative therapies or high‑dose antihistamine therapy (when applicable) are included.
- Patient demographics and contact information
- Prescriber name, credentials, specialty, NPI, license, and contact information
- Product: Haegarda dose and frequency
- Primary and secondary ICD codes
- Clinical details: diagnosis confirmation, laboratory or genetic test results, prior therapy trials, attack frequency
- For continuation: evidence of clinical response since initiating therapy
Fraud Warning
Fraud warning — providing materially false information or concealing material information may be considered fraud and could lead to denial of the request and criminal or civil penalties.
- Request must be signed and dated by the person completing the form
- Plan may request additional information or clarification to evaluate requests
Background
Hereditary angioedema (HAE) is a disorder characterized by recurrent angioedema due to C1 inhibitor deficiency or dysfunction or other genetic causes. Haegarda is a C1 esterase inhibitor (human) product used for prophylaxis of HAE attacks; the Aetna precertification form collects the specific diagnostic data (antigenic and functional C1‑INH levels or genetic testing) and attack frequency to document the indication and support authorization decisions.
Definitions and Laboratory Criteria
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