Obtain prior authorization (12-week intervals; quantity tied to on-hand supply)

Prior authorization is required. Initial prior authorization is valid for 12 weeks and may be renewed every 12 weeks thereafter. Authorization quantity will consider the cumulative amount of HAE medication the patient has on-hand and will provide a sufficient quantity to treat up to 4 acute attacks per 4 weeks for the duration of the authorization. Maximum 1000 billable HCPCS units per 28 days apply.

• Initial authorization validity: 12 weeks; renewals every 12 weeks.
• Authorization quantity considers on-hand supply and supports up to 4 acute attacks per 4 weeks.
• Maximum 1000 billable units per 28 days (HCPCS units).

Verify and comply with applicable Medicare Part B coverage policies

For Medicare beneficiaries, coverage for outpatient drugs is governed by CMS manuals and any applicable National/Local Coverage Determinations or Analyses (NCDs/LCDs/LCAs). Providers must verify and comply with applicable Medicare coverage policies where relevant.

• Medicare Part B covered diagnosis codes: N/A per this policy, but applicable NCDs/LCDs/LCAs may exist.
• Verify and follow CMS manuals and any applicable NCD/LCD/LCA for the patient’s jurisdiction.

Document prior trial of generic icatibant (unless <18 years)

Document a trial of generic icatibant with inadequate response, intolerance, or contraindication prior to coverage, unless the patient is under 18 years of age; site-of-care specialty infusion program requirements must also be met.

• Acceptable documentation: prior trial notes showing inadequate response, intolerance, or contraindication to generic icatibant.
• If patient is <18 years old, the icatibant trial requirement does not apply.
• Site-of-care specialty infusion program requirements must be met.

Step therapy requirements

No step therapy requirements are specified in the provided document segments.

Submit repeat laboratory confirmation of HAE subtype

Provide repeat blood testing confirming HAE subtype: C1‑INH antigenic level, C1‑INH functional level, and C4 level. For HAE type I, also document family history or exclusion of acquired angioedema (e.g., onset <30 years, normal C1q, no underlying lymphoproliferative disease).

• HAE type I: low C1‑INH antigenic level AND low C4 AND low C1‑INH functional level, plus family history OR exclusion of acquired angioedema.
• HAE type II: normal/elevated C1‑INH antigenic level AND low C4 AND low C1‑INH functional level.
• Repeat testing must confirm subtype; acute treatment should not be delayed for confirmatory testing.

Report current on-hand medication inventory (used to set authorized quantity)

Document cumulative on-hand amounts of HAE medication(s); the on-hand inventory will be used to authorize a quantity sufficient to treat up to 4 acute attacks per 4 weeks for the authorization period.

• Provide current cumulative on-hand medication amounts as part of the prior authorization request.
• Authorization quantities will be adjusted so the patient has supply to treat up to 4 acute attacks per 4 weeks.

Document covered diagnosis code D84.1

Include the applicable ICD-10 diagnosis code D84.1 (Defects in the complement system) when documenting the covered diagnosis as listed in Appendix 1.

• Appendix 1 lists D84.1 = Defects in the complement system as a covered diagnosis code.

Provide documentation of icatibant trial or applicable exemption before initial approval

Ensure the patient has documentation of a trial with inadequate response, intolerance, or contraindication to generic icatibant unless the patient is under 18 years of age; also confirm site-of-care specialty infusion program requirements are met to avoid denial.

• Failure to document the icatibant trial (when applicable) or to meet site-of-care requirements may result in denial of coverage.

Confirm and follow applicable Medicare NCDs/LCDs/LCAs

Medicare coverage determinations (NCDs/LCDs/LCAs) may exist for outpatient drugs; where applicable, compliance with those CMS policies is required and failure to follow them could affect coverage determinations.

• Providers should verify applicable NCD/LCD/LCA for the beneficiary’s jurisdiction and comply with those policies.