CurrentHawaii Medical Service Association (HMSA)Policy N/A

Prior authorization form for medications to treat hereditary angioedema (HAE)

A CVS Caremark prior authorization request form used by HMSA to collect clinical information and documentation to determine coverage for medications used to treat hereditary angioedema (HAE), including acute and prophylactic therapy. The form specifies required diagnostic confirmation, laboratory/genetic documentation, specialist involvement, prior use/authorization history, and outcomes evidence.

Policy Summary

PayerHawaii Medical Service Association (HMSA)

PolicyPrior authorization form for medications to treat hereditary angioedema (HAE)

Policy CodePolicy N/A

Change TypeNo material change

Effective Date

Next Review Date

Key ActionFax the completed form to CVS Caremark toll-free at 1-866-237-5512 to request prior authorization; contact CVS Caremark at 1-808-254-4414 for PA questions.

POLICY UPDATE CHANGES

No material clinical/coverage changes

FormDocument Type

HAETarget Condition

Lab/GeneticDocumentation Requirement

Coverage Summary

This is a CVS Caremark prior authorization request form used by HMSA to collect clinical information to determine coverage for medications to treat hereditary angioedema (HAE). The form is used to document diagnosis, required laboratory/genetic confirmation, specialist involvement, prior use/authorization history, and treatment intent to support coverage decisions.

Medical Necessity / Form Criteria

Primary diagnostic branches

Select the diagnosis and follow the indicated question path:

ANY of the following

Hereditary angioedema (HAE) with C1 inhibitor deficiency or dysfunction confirmed by laboratory testing
If selected, continue to #2
HAE with normal C1 inhibitor confirmed by laboratory testing
If selected, continue to #3
Other
If selected, No Further Questions

C1-INH deficiency/dysfunction diagnostic criteria (Q2)

For C1-INH deficiency/dysfunction, attach laboratory test or medical record documentation confirming C1 inhibitor functional and antigenic protein levels. Continue to #4.

ANY of the following

A C1 inhibitor (C1-INH) antigenic level below the lower limit of normal as defined by the laboratory performing the test
A normal C1-INH antigenic level and a low C1-INH functional level (functional C1-INH less than 50% or C1-INH functional level below the lower limit of normal as defined by the laboratory performing the test)less than 50% or below the laboratory lower limit of normal
Other

HAE with normal C1-INH diagnostic criteria (Q3)

For HAE with normal C1 inhibitor, attach genetic test or documentation confirming listed gene mutations or family history and refractory to high-dose antihistamine therapy. Continue to #4.

F12, angiopoietin-1, plasminogen, kininogen-1 (KNG1), HS3ST6, or MYOF gene mutation as confirmed by genetic testing

Alternative pathway

Angioedema refractory to a trial of high-dose antihistamine therapy (i.e., cetirizine at 40 mg per day or the equivalent) for at least one monthcetirizine 40 mg/day or equivalent for ≥1 month
Family history of angioedema

Other

Treatment intent and concomitant therapy (Q4-Q6)

Determine use-case and treatment context. Continue through listed questions.

Is the requested medication being used for the treatment of acute HAE attacks? (Yes/No)

Will the requested medication be used in combination with any other medication used for treatment of acute HAE attacks (e.g., Berinert, Firazyr, Kalbitor)? (Yes/No)

Will the requested medication be prescribed by or in consultation with a prescriber who specializes in the management of hereditary angioedema (HAE)? (Yes/No)

Have other causes of angioedema been ruled out (e.g., ACE-I induced angioedema, estrogen-related, allergic)? (Yes/No)

Prior use and authorization history (Q8-Q10)

Capture prior exposure and prior authorizations to inform continuation/renewal decisions.

Has the patient previously received treatment with the requested medication? (Yes/No)

Was the requested drug previously authorized by HMSA/CVS for this member? (Yes/No/Unknown)

If previously received and authorized, has the patient experienced a reduction in severity and/or duration of acute attacks? (Yes/No) - attach supporting chart notes if 'Yes'

Attach supporting chart notes if Yes

Consideration of prophylactic therapy (Q11-Q13)

Determine need for prophylaxis and whether it has been considered.

Does the patient's attack frequency, attack severity, comorbid conditions and patient's quality of life warrant prophylactic therapy? (Yes/No)

Has prophylactic treatment been considered?

Has prophylactic treatment been considered? (Yes -> No Further Questions)
Has prophylactic treatment NOT been considered? (No -> Provide brief rationale as to why prophylactic treatment has not been considered)
Provide brief rationale

Provider Actions & Documentation Requirements

Prior Authorization

Submit completed prior authorization form to CVS Caremark

Fax the completed form to CVS Caremark toll-free at 1-866-237-5512 to request prior authorization. For prior authorization questions, contact CVS Caremark at 1-808-254-4414.

Fax: 1-866-237-5512
Phone (PA questions): 1-808-254-4414

Documentation Required

Attach laboratory or genetic documentation

Attach laboratory test or medical record documentation confirming C1-INH functional and antigenic protein levels for patients with C1-INH deficiency/dysfunction. For HAE with normal C1-INH, attach genetic testing confirming mutations (F12, angiopoietin-1, plasminogen, KNG1, HS3ST6, or MYOF) or documentation of family history plus a trial of high-dose antihistamine therapy.

C1-INH functional and antigenic protein levels
Genetic testing or family history plus high-dose antihistamine trial

Documentation Required

Attach evidence of clinical benefit for continuation

If the patient has previously received the requested medication, attach supporting chart notes demonstrating a reduction in severity and/or duration of acute attacks to support continued authorization.

Documentation Required

Specialist involvement

The requested medication should be prescribed by or in consultation with a prescriber who specializes in the management of hereditary angioedema (HAE). Indicate the specialist's name and NPI on the form.

Documentation Required

Rule out alternative causes

Document that other causes of angioedema have been ruled out (for example, ACE‑I induced angioedema, estrogen-related angioedema, or allergic angioedema).

Background

Background: This prior authorization form is administered by CVS Caremark on behalf of HMSA to collect clinical details required for making coverage determinations for HAE therapies, including acute and prophylactic use. The form instructs providers to fax the completed form to CVS Caremark and provides contact numbers for PA questions.

The form requires attachment of laboratory documentation for C1 inhibitor antigenic and functional levels to confirm HAE due to C1-INH deficiency or dysfunction, or genetic testing/family history plus a trial of high-dose antihistamines for HAE with normal C1-INH. Approvals may be subject to dosing limits in accordance with FDA-approved labeling, accepted compendia, and/or evidence-based practice guidelines.

Definitions

C1-INHC1 inhibitor antigenic and functional protein levels used to confirm HAE due to C1-INH deficiency or dysfunction; antigenic below lab lower limit or functional <50% (or below lab lower limit)

HAE with normal C1-INHRequires genetic confirmation (F12, angiopoietin-1, plasminogen, KNG1, HS3ST6, MYOF) or family history plus angioedema refractory to high‑dose antihistamines

OpenPayer

Prior authorization form for medications to treat hereditary angioedema (HAE)

Coverage Summary

Medical Necessity / Form Criteria

Coding

Provider Actions & Documentation Requirements

Background

Revision History