CurrentNeighborhood Health Plan of Rhode IslandPolicy N/A

Ekterly (sebetralstat) — coverage criteria for acute hereditary angioedema (HAE) attacks

Covers authorization criteria, continuation, and quantity limits for Ekterly (sebetralstat) tablets to treat acute HAE attacks in patients aged 12 and older when clinical and safety criteria are met.

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyEkterly (sebetralstat) — coverage criteria for acute HAE attacks

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateFeb 1, 2026

Next Review DateN/A

Key ActionObtain prior authorization for up to 6 months when all clinical criteria for initial approval are met; document diagnosis, prior treatments, and treatment benefit for renewals.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

12+minimum covered patient age

6 moauthorization duration

300 mgtablet strength

4 / 30 daysquantity limit

FDA-approvedindication coverage

Trek Health ingests and normalizes Transparency in Coverage data and payer policy updates to give provider organizations a clear view of how commercial reimbursement behaves across markets, payers, and services. Our platform transforms raw payer disclosures into structured intelligence that supports contract evaluation, payer negotiations, and service line strategy. By combining market benchmarks with ongoing policy visibility, Trek helps teams identify variability, risk, and opportunity in commercial reimbursement. The result is faster insight, stronger negotiating positions, and more informed financial decisions.

Coverage Criteria for Ekterly (sebetralstat)

Initial Therapy

Authorization of 6 months may be granted when ALL of the following are met:

Initial Approval Criteria: A. Medication is prescribed by, or in consultation with an allergist/immunologist or a physician who specializes in the management of HAE.

Initial Approval Criteria: B. Member is 12 years of age or older.

Initial Approval Criteria: C. Documentation of history of moderate to severe cutaneous attacks (without concomitant hives) OR abdominal attacks OR mild to severe airway swelling attacks of HAE (e.g., debilitating cutaneous/gastrointestinal symptoms OR laryngeal/pharyngeal/tongue swelling).

Initial Approval Criteria: D. Documentation that the member does not have a history of severe laryngeal attacks, as Ekterly (sebetralstat) tablets need to be swallowed whole.

Initial Approval Criteria

(a) C1-INH deficiency/dysfunction: a. Documentation that the member has C1 inhibitor deficiency or dysfunction as confirmed by laboratory testing; and meets one of: (i) C1-INH antigenic level below the laboratory lower limit of normal, or (ii) normal C1-INH antigenic level with low C1-INH functional level (functional C1-INH <50% or below the laboratory lower limit).
(b) Normal C1-INH with supporting evidence: b. Documentation that the member has normal C1 inhibitor by lab testing and meets one of: (i) pathogenic variant confirmed by genetic testing (F12, angiopoietin-1, plasminogen, KNG1, HS3ST6, or MYOF), or (ii) documented family history of angioedema and angioedema refractory to a trial of high‑dose antihistamine therapy (e.g., cetirizine 40 mg/day or equivalent) for at least one month.

Initial Approval Criteria: F. Member will not use Ekterly concomitantly with other medications used for treatment of acute HAE attacks (e.g., icatibant, ecallantide, C1 esterase inhibitor products).

Initial Approval Criteria: G. Other causes of angioedema have been ruled out (e.g., ACE inhibitor–induced angioedema, estrogen‑related angioedema, allergic angioedema).

Initial Approval Criteria: H. Documentation that the member does not require sustained use of strong CYP3A4 inhibitors or moderate/strong CYP3A4 inducers.

Initial Approval Criteria: I. Documentation that the member does not have severe hepatic impairment (Child‑Pugh Class C).

Initial Approval Criteria: J. For members 18 years of age and older: documentation of inadequate response, intolerance, or contraindication to icatibant.

Continuation Therapy

Authorization of 6 months may be granted for continuation when ALL of the following are met:

Continuation Criteria: A. Documentation that the member continues to meet the criteria for initial approval.

Continuation Criteria: B. Documentation that the member has experienced a reduction in severity and/or duration of attacks when the requested medication is used to treat an acute attack.

Continuation Criteria: C. Consideration of prophylaxis based on attack frequency, attack severity, comorbid conditions, and the member's quality of life.

All uses of Ekterly (sebetralstat) outside the FDA‑approved indication are excluded. Specifically, treatment for any indication other than acute hereditary angioedema (HAE) attacks in patients aged 12 years and older is considered experimental/investigational and not medically necessary.

Use of Ekterly for indications other than the FDA‑approved indication — treatment of acute HAE attacks in adults and pediatric patients aged 12 years and older — is not medically necessary and will not be covered.

Provider Requirements and Prior Authorization

Prior Authorization

Prior Authorization Required

Prior authorization for up to 6 months is required and may be granted when all clinical criteria for initial approval are met. Authorization of 6 months may also be granted for continuation of therapy when continuation criteria are satisfied.

Prior authorization duration: up to 6 months
Prescriber requirement: prescribed by or in consultation with an allergist/immunologist or physician specializing in HAE management

Step Therapy

Step Therapy: Icatibant Trial for Adults

For members 18 years of age and older, documentation of inadequate response, intolerance, or contraindication to icatibant is required prior to approval.

Applies only to adults (≥ 18 years)

Denial Risk

Indication Not Covered / Experimental

Requests for indications other than treatment of acute hereditary angioedema (HAE) attacks in patients aged 12 years and older are considered experimental/investigational and not medically necessary.

Not covered: prophylactic use or any off-label indications outside acute HAE attacks for ages <12

Documentation Required

Required Clinical Documentation

Documentation required with the prior authorization request must include diagnostic and supporting clinical information confirming HAE type I or II and relevant laboratory and clinical history.

Diagnosis of HAE type I or II with confirmatory laboratory testing: C1-INH antigenic level below lab lower limit of normal, or normal antigenic level with low C1-INH functional level (<50% or below lab lower limit).
If C1-INH is normal, genetic testing confirming pathogenic variant (e.g., F12, ANGPT1, PLG, KNG1, HS3ST6, MYOF) OR documented family history with angioedema refractory to high-dose antihistamine trial (e.g., cetirizine 40 mg/day for ≥1 month).
Clinical history documenting moderate to severe cutaneous attacks (without concomitant hives), abdominal attacks, or airway swelling attacks; and ruling out other causes of angioedema (e.g., ACE inhibitor–induced, estrogen-related, allergic).
Confirmation that patient is ≥12 years of age and that concomitant use with other acute HAE treatments will not occur.
Medication prescribed by or in consultation with an allergist/immunologist or HAE specialist, and documentation regarding CYP3A4 inhibitor/inducer use and hepatic function (no severe hepatic impairment Child-Pugh C).

Background

Hereditary angioedema (HAE) is a genetic disorder that causes recurrent episodes of localized swelling that can affect the skin, gastrointestinal tract, and airway and may be life‑threatening. Acute therapies are intended to reduce the severity and duration of attacks; appropriate patient selection for Ekterly requires confirmation of HAE (type I/II or compatible genetic/family history) and assessment of attack phenotype and prior treatment responses. Documentation should demonstrate that other causes of angioedema have been excluded and that patient‑specific safety considerations (for example, absence of severe hepatic impairment and avoidance of contraindicated concomitant medications such as sustained strong CYP3A4 inhibitors or moderate/strong CYP3A4 inducers) are addressed prior to therapy.

Definitions

HAE type I/II — diagnostic criteria

DefinitionHAE type I or II: diagnosis confirmed by laboratory testing showing C1 inhibitor (C1-INH) deficiency or dysfunction.

Diagnostic criteria (antigenic)C1-INH antigenic level below the lower limit of normal as defined by the performing laboratory.

Diagnostic criteria (functional)Normal C1-INH antigenic level with low C1-INH functional level (<50% or below the laboratory lower limit of normal).

Required documentationLaboratory confirmation of C1-INH antigenic and/or functional levels as evidence of type I or II HAE.

Related clinical noteThis diagnosis is required for initial authorization of Ekterly for acute HAE attacks in patients aged 12 years and older.

HAE with normal C1‑INH — supportive diagnostic findings

DefinitionHAE with normal C1-INH: HAE phenotype with normal C1 inhibitor levels on laboratory testing.

Genetic supportDiagnosis may be supported by a documented pathogenic variant in F12, ANGPT1 (angiopoietin-1), PLG (plasminogen), KNG1 (kininogen-1), HS3ST6, or MYOF as confirmed by genetic testing.

Familial and treatment historyAlternatively, diagnosis may be supported by documented family history of angioedema plus angioedema refractory to a trial of high‑dose antihistamine therapy (e.g., cetirizine 40 mg/day or equivalent for ≥1 month).

Contextual useUsed to clarify non‑type I/II HAE when C1‑INH testing is normal but clinical and genetic/family history indicate HAE.