CurrentHawaii Medical Service Association (HMSA)Policy N/A

Prior authorization for hereditary angioedema (HAE) medications

Form and criteria for requesting prior authorization of medications used to treat hereditary angioedema for members whose prescription benefit is administered by CVS Caremark on behalf of HMSA.

Policy Summary

PayerHawaii Medical Service Association (HMSA)

PolicyPrior authorization for hereditary angioedema (HAE) medications

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateN/A

Next Review DateN/A

Key ActionSubmit the completed prior authorization form and supporting documentation by fax to CVS Caremark at 1-866-237-5512.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

12criteria questions on form

1-866-237-5512fax to submit PA

CaremarkConnectspecialty customer care

Coverage Criteria for HAE Medications

Form-based coverage criteria and documentation required

Covered when ALL of the following are met (form-based logical flow):

Policy Summary

PayerHawaii Medical Service Association (HMSA)

PolicyPrior authorization for hereditary angioedema (HAE) medications

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateN/A

Next Review DateN/A

Key ActionSubmit the completed prior authorization form and supporting documentation by fax to CVS Caremark at 1-866-237-5512.

SourceLink

On This Page

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.

Confirmation for C1-INH deficiency/dysfunction: If HAE with C1-INH deficiency/dysfunction selected, attach laboratory confirmation of either: a C1-INH antigenic level below the laboratory lower limit of normal OR a normal C1-INH antigenic level with low C1-INH functional level (functional C1-INH <50% or below the laboratory lower limit of normal)C1-INH functional <50% or below lab lower limit

Action required: attach laboratory test or medical record documentation confirming C1-INH antigenic and/or functional levels (per form #2)

Confirmation for HAE with normal C1-INH: If HAE with normal C1-INH selected, attach genetic testing confirming mutation in F12, angiopoietin-1, plasminogen, KNG1, HS3ST6, or MYOF OR attach chart notes documenting family history of angioedema AND angioedema refractory to a trial of high-dose antihistamine therapy (e.g., cetirizine 40 mg/day for ≥1 month)trial of high-dose antihistamine (e.g., cetirizine 40 mg/day) for ≥1 month

Action required: attach genetic test results or documentation of family history and antihistamine-refractory disease (per form #3 and #- in chunk 3)

Requests must document that other causes of angioedema have been considered and ruled out before approval. The form explicitly asks whether angiotensin‑converting enzyme inhibitor (ACE‑I) induced angioedema, angioedema related to an estrogen‑containing drug, or allergic angioedema have been excluded. If these alternative etiologies are not addressed in the submitted record, the request lacks required confirmation and may be denied.

Providers should attach chart notes or other medical record documentation demonstrating evaluation for these alternative causes. A completed prior‑authorization form should indicate “Yes” to the question about ruling out other causes and include the supporting documentation with the faxed submission.

Provider Actions and Submission Requirements

Prior Authorization

Prior Authorization Required

Prior authorization is required for certain hereditary angioedema (HAE) medications administered through CVS Caremark. Submit the completed prior authorization form and required documentation to CVS Caremark for review.

Fax: CVS Caremark toll-free 1-866-237-5512
Questions: CVS Caremark 1-808-254-4414
Specialty Customer Care: CaremarkConnect 1-800-237-2767

Documentation Required

Action Required: Laboratory Documentation for C1‑INH‑related HAE

Attach laboratory results or medical record documentation confirming C1‑INH antigenic and functional protein levels when HAE with C1‑INH deficiency or dysfunction is indicated. Failure to attach these laboratory tests or medical record documentation may result in denial of the request.

Required tests: C1‑INH antigenic level and C1‑INH functional level (report lab reference ranges)
Attach: lab reports or chart notes demonstrating the values

Denial Risk

Combination Therapy, Prophylaxis, and Continuation Documentation

Indicate whether the requested medication will be used in combination with other acute HAE therapies and document prior trials of prophylactic treatments. For continuation requests, attach chart notes demonstrating a reduction in severity and/or duration of attacks. Failure to provide documentation of prior or concurrent therapies or evidence of clinical benefit for continuation requests may affect approval.

Document if used with other acute HAE therapies (e.g., Berinert, Firazyr, Ruconest)
For continuation: attach chart notes showing reduction in attack severity and/or duration
For HAE with normal C1‑INH: attach genetic testing or documentation of family history and trial of high‑dose antihistamines

HAE Subtypes and Diagnostic Definitions

HAE with C1 inhibitor deficiency or dysfunction — diagnostic criteria

Laboratory confirmation requiredAttach laboratory test or medical record documentation confirming C1‑INH antigenic and functional protein levels at time of diagnosis.

Low antigenic C1‑INHC1‑INH antigenic level below the lower limit of normal as defined by the laboratory performing the test.

Normal antigenic with low functionNormal C1‑INH antigenic level with low C1‑INH functional level — functional C1‑INH less than 50% or below the laboratory's lower limit of normal.

Action on selectionIf HAE with C1‑INH deficiency or dysfunction is selected on the form, continue through subsequent form questions (proceed to #4).

HAE with normal C1 inhibitor — diagnostic support

Genetic testing supported diagnosisAttach genetic test confirming mutation in F12, angiopoietin‑1, plasminogen, KNG1, HS3ST6, or MYOF to support HAE with normal C1‑INH.

Alternative documentation pathChart notes documenting family history of angioedema and angioedema refractory to a trial of high‑dose antihistamine therapy may be attached if genetic testing is not provided.

Definition of antihistamine trialRefractory defined as lack of response after a trial of high‑dose antihistamine (e.g., cetirizine 40 mg per day or equivalent) for at least one month.

Form routingIf HAE with normal C1‑INH is selected on the form, attach genetic testing or relevant chart notes and continue to #4 on the form.

Background on Hereditary Angioedema (HAE)

Hereditary angioedema (HAE) is characterized by recurrent episodes of angioedema and can result from C1 inhibitor (C1‑INH) deficiency or dysfunction or from genetic variants with normal C1‑INH. Diagnosis and management decisions rely on clinical history, family history, and targeted testing.

The policy form gathers information on presentation and etiology: it asks whether angioedema is refractory to a trial of high‑dose antihistamine (for example, cetirizine 40 mg per day for ≥1 month), whether there is a family history, and whether the requested medication is for acute attacks or continued therapy. The form also asks about specialty involvement and whether other causes (e.g., ACE‑I, estrogen‑related, allergic) have been excluded, reflecting the need to confirm HAE subtype and context before authorizing HAE‑specific therapies.