CurrentNeighborhood Health Plan of Rhode IslandPolicy 2100-A

Haegarda (C1 esterase inhibitor) — Prophylaxis for Hereditary Angioedema

Covers prior authorization and medical necessity criteria for Haegarda used for routine prophylaxis to prevent hereditary angioedema (HAE) attacks in patients aged 6 years and older; applies to members of Neighborhood Health Plan of Rhode Island subject to benefit coverage.

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyHaegarda (C1 esterase inhibitor) — Prophylaxis for Hereditary Angioedema

Policy CodePolicy 2100-A

Change TypeNo material changes

Effective Date

Next Review Date

Key ActionObtain prior authorization with documentation of C1‑INH functional and antigenic levels (and genetic testing or family history when applicable).

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

6 monthsauthorization period granted for initial and continuation approvals

>=6minimum patient age for FDA-approved indication

≥50%example reduction in attack frequency required for continuation

<50%functional C1 inhibitor threshold cited for deficiency

Coverage Criteria for Haegarda (C1 esterase inhibitor)

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Initial Therapy

Covered when ALL of the following are met

Age and indication: Patient is 6 years of age or older and the request is for routine prophylaxis to prevent hereditary angioedema (HAE) attacks (FDA‑approved indication) or a compendial useage >= 6 years

FDA‑approved indication

Prescriber: Medication is prescribed by or in consultation with a prescriber who specializes in the management of HAE

specialty requirement

Diagnostic criteria (one required): Either A OR B must be met: A) C1 inhibitor deficiency or dysfunction confirmed by laboratory testing: either C1‑INH antigenic level below the laboratory lower limit of normal OR normal antigenic level with C1‑INH functional level <50% (or below lab lower limit); OR B) Normal C1‑INH by laboratory testing with either a qualifying genetic mutation (F12, angiopoietin‑1, plasminogen, KNG1, HS3ST6, MYOF) confirmed by genetic testing OR documented family history of angioedema with angioedema refractory to a trial of high‑dose antihistamine therapy (e.g., cetirizine 40 mg/day or equivalent for at least one month)functional C1‑INH <50% qualifies

diagnostic branching

Concomitant prophylaxis: Requested medication will not be used in combination with any other medication used for prophylaxis of HAE attacks

monotherapy for prophylaxis required

Continuation Therapy

Covered when ALL of the following are met

Meets initial criteria: Member continues to meet the criteria for initial approval

restates baseline eligibility

Clinical response: Member has experienced a significant reduction in frequency of attacks since starting treatment (example threshold: ≥ 50%)>= 50% reduction

example provided in policy

Reduced acute medication use: Member has reduced the use of medications to treat acute HAE attacks since starting treatment, as documented in chart notes

documented in chart notes per continuation requirements

Not Medically Necessary

Non‑approved indications: All indications beyond the FDA‑approved routine prophylaxis to prevent HAE attacks and compendial uses are considered experimental/investigational and not medically necessary

policy explicitly states non‑approved uses are not medically necessary

All indications for Haegarda other than routine prophylaxis to prevent hereditary angioedema (HAE) attacks and recognized compendial uses are considered experimental/investigational and not medically necessary.

Use of Haegarda for indications other than routine prophylaxis to prevent HAE attacks and compendial uses is not medically necessary.

Coding and Clinical Thresholds

Codes - none listedmixed

not specified

No explicit CPT/HCPCS/ICD-10/NDC codes listed in the document.

Attack reduction required for continuation

Attack reduction thresholdMember has experienced a significant reduction in frequency of attacks (example: ≥ 50%) since starting treatment.

Authorization period for continuationAuthorization of 6 months may be granted for continuation of therapy when continuation criteria are met.

Evidence required for continuationChart notes documenting reduction in attack frequency and decreased use of medications for acute attacks are required.

C1-INH functional threshold

Functional C1-INH thresholdC1-INH functional level less than 50% or below the laboratory lower limit of normal.

Normal antigenic with low function qualifiesNormal C1-INH antigenic level with low functional C1-INH (functional C1-INH <50% or below lab lower limit) meets diagnostic criteria for deficiency/dysfunction.

Laboratory referenceThresholds referenced to the lower limit of normal as defined by the laboratory performing the test.

Provider Requirements and Authorization Instructions

Prior Authorization

Prior Authorization Required

Authorization of up to 6 months may be granted for prevention of hereditary angioedema (HAE) attacks when the requested medication will not be used in combination with any other prophylactic HAE medication and the member meets applicable diagnostic criteria at time of request.

Authorization duration: up to 6 months when not used with other prophylactic HAE medications.

Documentation Required

Required Clinical Documentation (Initial Authorization)

For initial authorization, submit the following clinical documentation: C1 inhibitor functional and antigenic protein levels; genetic testing results for F12, angiopoietin-1, plasminogen, KNG1, HS3ST6, or MYOF when applicable; and chart notes documenting family history of angioedema and that symptoms were refractory to a trial of high‑dose antihistamine therapy when member has normal C1‑INH.

Required labs: C1‑INH antigenic level and C1‑INH functional level.
Genetic testing: F12, angiopoietin‑1, plasminogen, KNG1, HS3ST6, or MYOF, if applicable.
Clinical notes: family history of angioedema and documentation of trial and failure of high‑dose antihistamine therapy when C1‑INH is normal.

Documentation Required

Continuation Documentation

For continuation of therapy, provide chart notes demonstrating a clinical benefit such as a reduction in frequency of HAE attacks.

Continuation documentation: chart notes showing reduced attack frequency.

Documentation Required

Antihistamine Trial When Applicable

When the member has normal C1‑INH, a trial of high‑dose antihistamine therapy (for example, cetirizine 40 mg per day or equivalent for at least one month) must be documented showing lack of adequate response prior to approval for prophylaxis, unless genetic testing confirms an applicable HAE‑related mutation or other criteria are met.

High‑dose antihistamine example: cetirizine 40 mg/day (or equivalent) for ≥1 month.
If genetic testing confirms an F12, angiopoietin‑1, plasminogen, KNG1, HS3ST6, or MYOF mutation, antihistamine trial documentation may not be required.

Note

Prescriber Specialty Requirement

This medication must be prescribed by or in consultation with a prescriber who specializes in the management of hereditary angioedema.

Prescriber requirement: HAE specialist or consultation with HAE specialist.

Definitions

C1-INH deficiency or dysfunction

Antigenic level criterionC1-INH antigenic level below the lower limit of normal as defined by the laboratory performing the test.

Functional deficiency criterionNormal antigenic C1-INH with low functional C1-INH (functional C1-INH <50% or below lab lower limit) qualifies as deficiency or dysfunction.

Role in authorizationC1-INH deficiency or dysfunction is one of the diagnostic criteria that may support authorization for prophylaxis of HAE attacks.

HAE with normal C1-INH

DefinitionHAE with normal C1-INH is a phenotype where C1-INH levels are normal but disease is associated with specific genetic mutations (e.g., F12, angiopoietin-1, plasminogen, KNG1, HS3ST6, MYOF).

Genetic confirmationMember with normal C1-INH must have a qualifying gene mutation confirmed by genetic testing (F12, angiopoietin-1, plasminogen, KNG1, HS3ST6, MYOF) to meet diagnostic criteria.

Alternate pathwayOr documented family history of angioedema plus angioedema refractory to a trial of high-dose antihistamine (e.g., cetirizine 40 mg/day for at least one month) may qualify when genetic testing is not present.

OpenPayer

Haegarda (C1 esterase inhibitor) — Prophylaxis for Hereditary Angioedema

Coverage Criteria for Haegarda (C1 esterase inhibitor)

Coding and Clinical Thresholds

Provider Requirements and Authorization Instructions

Background

Definitions