CurrentHawaii Medical Service Association (HMSA)Policy N/A

Prior authorization requirements for hereditary angioedema (HAE) medications

Form and criteria governing prior authorization requests to CVS Caremark for medications used to treat hereditary angioedema (HAE), including acute attack treatment and short-term preprocedural prophylaxis; applies to HMSA members whose prescription benefit is administered by CVS Caremark.

Policy Summary

PayerHawaii Medical Service Association (HMSA)

PolicyPrior authorization requirements for hereditary angioedema (HAE) medications

Policy CodePolicy N/A

Change TypeNo material change

Effective DateN/A

Next Review DateN/A

Key ActionComplete and fax the CVS Caremark prior authorization form and attach required laboratory or genetic documentation as specified for the HAE subtype.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

2settings explicitly queried

lab/geneticdocumentation required

100 kgweight branching

Coverage Criteria for HAE Medications

Prior authorization criteria for HAE medications

Coverage decisions require that the following branching criteria are addressed on the prior authorization form according to clinical setting.

Policy Summary

PayerHawaii Medical Service Association (HMSA)

PolicyPrior authorization requirements for hereditary angioedema (HAE) medications

Policy CodePolicy N/A

Change TypeNo material change

Effective DateN/A

Next Review DateN/A

Key ActionComplete and fax the CVS Caremark prior authorization form and attach required laboratory or genetic documentation as specified for the HAE subtype.

SourceLink

On This Page

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Diagnosis selection

Rule out other causes: Document whether other causes of angioedema (e.g., ACE‑I induced, estrogen‑related, allergic) have been ruled out
Refer to question 2 on the prior authorization form
Specialty prescriber: Indicate whether the requested drug will be prescribed by or in consultation with a prescriber who specializes in HAE management
Refer to question 3 on the prior authorization form
Clinical setting: Select intended use: short‑term preprocedural prophylaxis OR acute HAE attacks OR Other
If 'Other' is selected the form directs 'No Further Questions' (no authorization pathway via this form)

Preprocedural prophylaxis - C1‑INH deficiency/dysfunction: If HAE with C1‑INH deficiency or dysfunction: attach laboratory documentation confirming C1‑INH antigenic level below the laboratory lower limit OR C1‑INH functional level <50% (or below lab lower limit)

Action required: attach lab test or medical record documentation (see question 11)

Preprocedural prophylaxis - normal C1‑INH: If HAE with normal C1‑INH: attach genetic testing documentation confirming a pathogenic variant (F12, angiopoietin‑1, plasminogen, KNG1, HS3ST6, or MYOF) OR chart notes documenting family history and angioedema refractory to high‑dose antihistamine therapy

Action required: attach genetic test or medical record documentation (see question 12); alternatively, for refractory cases attach documentation per question 4/10

Preprocedural prophylaxis - refractory/high‑dose antihistamine and family history option: For preprocedural prophylaxis an alternative pathway requires BOTH: 1) angioedema refractory to a trial of high‑dose antihistamine therapy (cetirizine 40 mg/day or equivalent for ≥1 month) AND 2) family history of angioedema — attach documentation

See question 10 and question 4 for details

Acute attacks - diagnostic documentation: For acute attack requests: attach laboratory documentation for C1‑INH antigenic and/or functional levels if C1‑INH deficiency is indicated, or attach genetic testing/chart documentation for normal C1‑INH per the documented subtype

Action required: attach documentation as specified in questions 31–32

Acute attacks - prior exposure and response: If the patient previously received the requested medication, provide documentation whether there was a reduction in severity and/or duration of attacks; if 'Yes' attach supporting chart notes

Refer to questions 34–35

Acute attacks - prophylaxis consideration: Document assessment of whether the patient's attack frequency, severity, comorbid conditions, and quality of life warrant prophylactic therapy; indicate whether prophylactic treatment has been considered and provide rationale if it has not

Refer to questions 36–38

Quantity limits and weight threshold: Provide patient body weight. Quantity/dosing considerations branch at 100 kg (220.5 lbs): indicate whether the patient is ≤100 kg or >100 kg as directed on the form100 kg (220.5 lbs)

Refer to questions 50–51 for weight‑based branching

Provider Submission Requirements and Actions

Prior Authorization

Prior Authorization Required

Prior authorization is required for the medications governed by this policy. Failure to obtain prior authorization may result in non-coverage under the member's benefit plan. Approvals may be subject to dosing limits in accordance with FDA‑approved labeling, accepted compendia, and/or evidence‑based practice guidelines.

Fax completed CVS Caremark prior authorization form and supporting documentation to CVS Caremark toll-free at 1-866-237-5512.
For questions regarding prior authorization determinations, contact CVS Caremark at 1-808-254-4414.
For eligibility, copay, or medication delivery inquiries, contact CaremarkConnect Specialty Customer Care at 1-800-237-2767.
Provide the most accurate diagnosis for the use of the prescribed medication on the PA form; include relevant chart notes.

Documentation Required

Prior Authorization and Form Completion

Complete the CVS Caremark Prior Authorization Request form in full and sign any required prescriber attestation. The prescriber must attest that the information provided is accurate and that supporting documentation (e.g., chart notes, laboratory or genetic test results) is attached as required by the clinical questions on the form.

Include patient identifiers (name, DOB, patient ID) and prescriber information (name, NPI, office phone/fax) on the faxed form.
Attach chart notes or test results that corroborate the diagnosis and the indication for therapy.
Opt-out/fax-advertising information: recipients may opt out by calling 877-265-2711 or emailing do_not_call@cvscaremark.com.

Billing Rule

Quantity and Dosing Considerations

Quantity limits and dosing are weight-based where specified. Approvals may be limited to dosing consistent with FDA‑approved labeling, accepted compendia, and evidence‑based guidelines. When the form asks for weight-based branching, document patient weight and select the appropriate branch (≤100 kg or >100 kg) to determine quantity limits.

Preprocedural prophylaxis: indicate patient body weight and select whether ≤100 kg (220.5 lbs) or >100 kg; quantity limits apply accordingly.
Acute attacks: indicate patient body weight where requested; follow the weight-based branch at 100 kg (220.5 lbs).
Note that some approvals may impose maximum dosing or quantity limits per FDA label or medical policy even if higher doses are requested.

Background

Hereditary angioedema (HAE) is characterized by recurrent episodes of angioedema and can result from C1‑inhibitor (C1‑INH) deficiency or dysfunction or from forms with normal C1‑INH related to other genetic causes. Diagnostic evaluation on the prior authorization form requires identifying the diagnosis and ensuring other causes of angioedema (for example, ACE‑inhibitor induced, estrogen‑related, or allergic angioedema) are considered and documented. The form also queries prescriber specialty and directs the clinical setting for treatment—either short‑term preprocedural prophylaxis or acute HAE attacks—to determine which subtype‑specific documentation (laboratory or genetic testing) will be required.

Definitions and Subtypes of HAE

HAE with C1‑INH deficiency/dysfunction — laboratory criteria

Diagnostic requirementHAE with C1-INH deficiency/dysfunction confirmed by laboratory testing

Antigenic level criterionC1-INH antigenic level below the laboratory lower limit of normal (attach documentation)

Functional level criterionNormal antigenic level with low C1-INH functional level (functional <50% or below lab lower limit of normal) (attach documentation)

Documentation requiredAttach laboratory test or medical record documentation confirming C1-INH functional and antigenic protein levels

HAE with normal C1‑INH — genetic testing requirement

Diagnostic requirementHAE with normal C1‑INH requires confirmation of normal C1‑INH antigenic and functional levels and additional evidence for HAE with normal C1‑INH

Genetic testingAttach genetic test documentation confirming a pathogenic variant (e.g., F12, angiopoietin‑1, plasminogen, KNG1, HS3ST6, MYOF)

Alternate documentationIf genetic testing not available, attach chart notes confirming family history of angioedema and refractory response to high‑dose antihistamine therapy

OpenPayer

Prior authorization requirements for hereditary angioedema (HAE) medications

Coverage Criteria for HAE Medications

Coding and Dosing Thresholds

Provider Submission Requirements and Actions

Background

Definitions and Subtypes of HAE