CurrentAvMedPolicy N/A

Hereditary Angioedema Drugs - Acute Treatment

Pharmacy prior authorization and step-edit policy for icatibant (sajazir / Firazyr) for acute treatment of hereditary angioedema (HAE), including initial and renewal clinical criteria, quantity limits, billing units, and documentation requirements.

Policy Summary

PayerAvMed

PolicyHereditary Angioedema Drugs - Acute Treatment

Policy CodePolicy N/A

Change TypeAdministrative/formatting updates

Effective Date

Next Review Date

Key ActionSubmit a completed pharmacy prior authorization/step-edit request with prescriber signature and supporting documentation (labs, chart notes, diagnosis, dosing) via fax to 1-305-671-0200; approval requires all clinical criteria and supporting documentation.

SourceLink

POLICY UPDATE CHANGES

Reformatted and updated multiple dates of review and approval through 4/1/2021.

1Drug covered (icatibant)

3/28dPharmacy quantity limit

90/28dMedical benefit max units

>=18Minimum age (years)

Coverage Summary

Coverage stance: icatibant (Firazyr) is covered with clinical criteria for the acute treatment of hereditary angioedema (HAE). Scope: this is a pharmacy prior authorization/step-edit policy that defines initial and renewal criteria, required documentation, quantity limits, and billing units for icatibant (Firazyr). Initial approval is for 12 months and eligible for renewal subject to meeting renewal criteria. Quantity limits: Pharmacy benefit: 3 subcutaneous pens per 28 days. Medical billing: up to 90 billable units per 28 days where 1 mg = 1 billable unit (J1744). Required documentation includes prescriber signature, member and prescriber information, diagnosis, weight, dosing schedule, labs, chart notes, and repeat blood testing to confirm HAE subtype lab criteria.

Age>= 18 years

Pharmacy quantity limit3 subcutaneous pens per 28 days

Medical benefit max90 billable units per 28 days

Billable unit definition1 mg = 1 billable unit

Initial Approval Criteria — Acute HAE Treatment

Initial Approval Criteria - Treatment of acute attacks of HAE

All criteria must be met for approval. Provide documentation (lab results, diagnostics, chart notes) to support each checked line.

ALL of the following

ALL of the following
- Member must be at least 18 years of age
- Must be prescribed by or in consultation with a specialist in allergy, immunology, hematology, pulmonology or medical genetics
  Provider attestation required
- Provider attests the patient is avoiding ALL listed possible triggers for HAE attacks
  - Helicobacter pylori infections (confirmed by lab test)
  - Estrogen-containing oral contraceptive agents AND hormone replacement therapy
  - Antihypertensive agents containing ACE inhibitors
- Member history requirement
  - Three (3) or more severe HAE attacks per month (i.e., airway swelling, debilitating cutaneous or gastrointestinal episodes)
  - Disablement for more than 5 days per month by HAE
  - Recurrent laryngeal attacks caused by HAE
- HAE subtype (one of I, II, or III must apply with their respective requirements)
  - II.A. HAE I
    Low C1 inhibitor (C1-INH) antigenic level (C1-INH antigenic level below the lower limit of normal as defined by the laboratory performing the test)
    Low C4 level (C4 below the lower limit of normal as defined by the laboratory performing the test)
    Low C1-INH functional level (C1-INH functional level below the lower limit of normal as defined by the laboratory performing the test)
    ANY of the following
    Member has a family history of HAE
    Acquired angioedema has been ruled out (i.e., patient onset of symptoms occur prior to 30 years old, normal C1q levels, patient does not have underlying disease such as lymphoma or benign monoclonal gammopathy [MGUS])

Renewal / Continuation Criteria

Renewal Criteria

All criteria must be met for renewal approval; provide supporting documentation including lab results, diagnostics, and chart notes.

ALL of the following

Member must continue to meet initial criteria
Significant improvement in severity and duration of attacks have been achieved and sustained
Absence of unacceptable toxicity from the drug (e.g., hypersensitivity reactions)

Prior Authorization

Coverage duration

Coverage is provided for 12 months and will be eligible for renewal contingent on meeting renewal criteria.

Provider Actions & Billing Rules

Prior Authorization

Prior authorization required

Submit completed pharmacy prior authorization/step-edit request with prescriber signature and required member and prescriber information. Include diagnosis, weight, dosing schedule, laboratory results, and chart notes. Fax the completed form to 1-305-671-0200. Approval requires that all clinical criteria are met and all supporting documentation is provided.

Prescriber signature
Member and prescriber information (contact, DEA/NPI)
Diagnosis and ICD code if applicable
Weight
Dosing schedule and length of therapy
Laboratory results
Chart notes
Fax number: 1-305-671-0200

Documentation Required

Laboratory confirmation required

Submit repeat blood testing results confirming the HAE subtype laboratory criteria and include supporting diagnostics and chart notes. Specifically provide C1‑INH antigenic level, C4 level, and C1‑INH functional level results (repeat testing during an attack where applicable).

C1‑INH antigenic level
C4 level
C1‑INH functional level
Repeat blood testing during an attack when indicated
Supporting diagnostics and chart notes

Billing Rule

Quantity and units billing

Follow the specified pharmacy and medical billing limits. Pharmacy benefit: limit of 3 subcutaneous pens per 28 days. Medical benefit: bill up to 90 billable units per 28 days (1 mg = 1 billable unit). Use HCPCS J1744 and the product NDC 54092-0702-xx when billing.

Pharmacy benefit limit: 3 pens per 28 days
Medical benefit: up to 90 billable units per 28 days
Billing codes: J1744
NDC: 54092-0702-xx
Note: 1 mg = 1 billable unit

Step Therapy

Prior therapies verified

Previous therapies will be verified through pharmacy paid claims or submitted chart notes. Use of samples to initiate therapy does not meet step-edit/preauthorization criteria.

Verification via pharmacy paid claims or chart notes
Samples do not meet step-edit/preauthorization criteria

Prior Authorization

Coverage duration

Coverage is provided for 12 months and will be eligible for renewal contingent on meeting renewal criteria.

Clinical Evidence & Definitions

Background: This form is a pharmacy prior authorization/step-edit request used by AvMed to authorize icatibant (sajazir / Firazyr) for acute HAE. It specifies clinical and laboratory criteria for HAE subtypes I, II, and III, and requires submission of supporting documentation (lab results, diagnostics, chart notes). The brief contained no formal evidence entries (evidence[] is empty).

Definitions: Billable unit — Per policy, 1 mg = 1 billable unit for J1744.

Policy Summary

PayerAvMed

PolicyHereditary Angioedema Drugs - Acute Treatment

Policy CodePolicy N/A

Change TypeAdministrative/formatting updates

Effective Date

Next Review Date

SourceLink

On This Page

II.B. HAE II (C1-Inhibitor dysfunction)

Normal to elevated C1-INH antigenic level
Low C4 level (C4 below the lower limit of normal as defined by the laboratory performing the test)
Low C1-INH functional level (C1-INH functional level below the lower limit of normal as defined by the laboratory performing the test)
ANY of the following
- Member has a family history of HAE
- Acquired angioedema has been ruled out (i.e., patient onset of symptoms occur prior to 30 years old, normal C1q, patient does not have underlying disease such as lymphoma or benign monoclonal gammopathy [MGUS])

II.C. HAE III with normal C1-INH

Normal C1-INH antigenic level
Normal C4 level
Normal C1-INH functional level
Repeat blood testing during an attack has confirmed the patient does not have abnormal lab values indicative of HAE I or HAE II
ANY of the following
- antifibrinolytic agent (e.g., tranexamic acid (TXA) or aminocaproic acid)
- 17α-alkylated androgen: danazol
- progestins (female patients only)
ANY of the following
- Patient has a known HAE-causing mutation (e.g., F12, angiopoietin-1, plasminogen, kininogen-1)
- Patient has a family history of HAE and documented lack of efficacy of chronic high-dose antihistamine therapy (e.g. cetirizine up to four times daily or equivalent for at least one month or interval long enough to expect three or more angioedema attacks) AND corticosteroids

OpenPayer

Hereditary Angioedema Drugs - Acute Treatment

Coverage Summary

Initial Approval Criteria — Acute HAE Treatment

Renewal / Continuation Criteria

Provider Actions & Billing Rules

Applicable Codes

Clinical Evidence & Definitions

Revision History