CurrentNeighborhood Health Plan of Rhode IslandPolicy N/A

Haegarda (C1 esterase inhibitor) prophylaxis for Hereditary Angioedema

Defines coverage and authorization criteria for Haegarda as routine prophylaxis to prevent hereditary angioedema (HAE) attacks for members (medical products across Medicaid, Commercial, Medicare) when approval criteria are met.

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyHaegarda (C1 esterase inhibitor) prophylaxis for Hereditary Angioedema

Policy CodePolicy N/A

Change TypeNo material clinical or coverage changes

Effective Date/1/2019

Next Review Date07/2025

Key ActionSubmit prior authorization with documentation of age ≥6, specialist prescribing/consultation, laboratory confirmation of C1‑INH deficiency/dysfunction or qualifying genetic mutation, and clinical justification for prophylaxis.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

≥6 yrsMinimum age

6 monthsInitial/continuation approval

20 vials/30 daysQuantity limit

J0599HCPCS code

65,600/28 daysMax billable units

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Coverage and Medical Necessity Criteria

Initial Approval Criteria

Covered when ALL of the following are met

Age and indication: Patient is >= 6 years of age AND therapy is routine prophylaxis to prevent hereditary angioedema (HAE) attacks (FDA‑approved/compendial use)age >=6

Prescriber: Medication is prescribed by, or in consultation with, an allergist/immunologist or a physician who specializes in the treatment of HAE or related disorders

Diagnostic confirmation: Documentation of C1 inhibitor deficiency or dysfunction OR documentation of a qualifying genetic mutation associated with HAE

Acceptable laboratory evidence includes low C1‑INH antigenic level (below lab lower limit of normal) OR normal antigenic level with low functional C1‑INH (functional C1‑INH <50% or below lab normal). Acceptable genetic mutations include F12, ANGPT1, PLG, KNG1, HS3ST6, MYOF.

Clinical necessity for prophylaxis: Patient meets one of the following: history of at least one severe HAE attack per month (e.g., airway swelling, debilitating cutaneous or gastrointestinal episodes); disability >5 days/month from HAE; history of at least one laryngeal attack; need for short‑term prophylaxis for a procedure (e.g., dental or medical procedure); OR Berinert did not provide satisfactory control or access to on‑demand therapy is limited.

Other requirements: Other causes of angioedema have been ruled out (e.g., ACE‑inhibitor induced angioedema, estrogen‑related angioedema, allergic angioedema); patient has relevant family history or refractory course as applicable; patient will not use Haegarda concomitantly with Andemry, Orladeyo, Takhzyro, or Cinryze; and dose does not exceed FDA‑approved labeling.

Continuation Therapy

Continuation criteria — Covered when ALL of the following are met

Prior approval criteria: Patient continues to meet all criteria for initial approval

Clinical response: Documentation of favorable clinical response since initiating Haegarda (e.g., decreased HAE attack frequency, decreased attack severity, or decreased duration of attacks) and documentation of reduced use of medications to treat acute attacks

Requests for uses of Haegarda other than routine prophylaxis to prevent hereditary angioedema (HAE) attacks in patients ≥6 years are experimental/investigational and not covered. The policy limits coverage to the FDA‑approved/compendial indication of routine prophylaxis for HAE in patients 6 years and older; any non‑FDA or non‑compendial indications should be referred to the payer’s experimental/investigational review process and will generally be denied.

Requests that do not include laboratory confirmation of C1‑INH deficiency or dysfunction, or that lack documentation demonstrating adequate clinical severity/frequency to justify prophylaxis, may be considered not medically necessary. Acceptable diagnostic documentation includes laboratory evidence of low C1‑INH antigenic level, or a normal antigenic level with low functional C1‑INH (<50% or below the laboratory lower limit of normal), or a qualifying genetic mutation. Other causes of angioedema (e.g., ACE inhibitor–induced, estrogen‑related, allergic) must be ruled out and a trial of high‑dose antihistamine (when applicable) documented prior to approval considerations.

Billing and Coding

Listed HCPCS/CPT CodeHCPCS

J0599

Injection, C1 esterase

Quantity / Billing thresholds

Haegarda vial limit20 vials per 30 days for Haegarda 2000 or 3000 units

Daily dose noteDaily dose reference in policy: 0.667 (as listed with vial limit)

Maximum billable unitsMaximum billable 65,600 units per 28 days

Billing period specificsVial limit applies to a 30‑day period; maximum billable units calculated over a 28‑day period

Provider Requirements, Documentation & Authorization

Prior Authorization

Prior Authorization Required

Prior authorization required — Prior authorization required; initial and continuation authorizations may be granted for 6 months when all listed clinical criteria are met.

Authorization duration: up to 6 months for initial and continuation.
Affected therapy: routine prophylaxis to prevent hereditary angioedema (HAE) attacks in patients ≥6 years.

Prior Authorization

Prior Acute Therapy Considerations

Prior acute therapy considerations — Consideration given to prior use of Berinert or access to on‑demand therapy; documentation that Berinert did not provide satisfactory control or access to on‑demand therapy is limited may be required.

Document prior acute on‑demand therapy history, including trial of Berinert and reason for inadequate control or access.
If on‑demand therapy access is limited, provide documentation describing the limitation.

Clinical Background

Hereditary angioedema (HAE) is caused by C1 inhibitor (C1‑INH) deficiency or dysfunction and leads to recurrent episodic swelling. Routine prophylaxis with C1 esterase inhibitor concentrate (Haegarda) is intended to reduce the frequency, severity, and duration of HAE attacks in appropriately selected patients. Coverage is limited to patients age ≥ 6 years with documented C1‑INH deficiency/dysfunction (laboratory confirmation or qualifying genetic mutation) and clinical need for prophylaxis, and treatment should be prescribed by or in consultation with an allergist/immunologist or a physician who specializes in HAE.

Key Definitions

C1‑INH deficiency/dysfunction

DefinitionLaboratory-confirmed low C1 inhibitor antigenic level OR normal antigenic level with low functional C1‑INH (functional C1‑INH <50% or below the laboratory lower limit of normal)

Genetic confirmationAlternatively, documentation of a qualifying genetic mutation associated with HAE (examples listed in policy: F12, ANGPT1, PLG, KNG1, HS3ST6, MYOF)

Rule out other causesOther causes of angioedema must be ruled out (e.g., ACE‑I induced angioedema, estrogen‑related, allergic angioedema)

Severe HAE attack

Severe HAE attack examplesEpisodes such as airway swelling, debilitating cutaneous or gastrointestinal episodes, or laryngeal attacks

Disability threshold

Documentation Required

Required Clinical Documentation

Required Clinical Documentation — Provider must document age, specialty involvement, diagnostic confirmation of C1‑INH deficiency/dysfunction, exclusion of other causes of angioedema, HAE subtype, and clinical history supporting prophylactic therapy.

Patient age ≥ 6 years.
Medication prescribed by or in consultation with an allergist/immunologist or a physician who specializes in HAE or related disorders.
Laboratory confirmation of C1‑INH deficiency or dysfunction (one of): C1‑INH antigenic level below laboratory lower limit of normal; or normal antigenic level with low C1‑INH functional level (<50% or below lab lower limit).
If applicable, genetic testing confirming pathogenic mutation (e.g., F12, ANGPT1, PLG, KNG1, HS3ST6, MYOF) when indicated.
Documentation that other causes of angioedema have been ruled out (e.g., ACE‑inhibitor induced, estrogen‑related, allergic angioedema).
Clinical history supportive of prophylaxis: history of frequent or severe HAE attacks (eg, ≥1 severe attack/month, ≥1 laryngeal attack, disability >5 days/month), or need for short‑term prophylaxis for procedures.
Documentation of prior and current therapies and clinical response (for continuation: decreased attack frequency, severity, duration, and reduced use of acute medications).