Obtain prior authorization for 12 months and meet site-of-care requirements

Prior authorization is required. Prior authorization validity will be provided initially for 12 months and may be renewed every 12 months thereafter. Site-of-care specialty infusion program requirements must be met (refer to Moda Site of Care Policy).

• Initial PA duration: 12 months; renewals: every 12 months.
• Site-of-care: meet site-of-care specialty infusion program requirements per Moda Site of Care Policy.

Link prior authorization to a listed covered diagnosis (e.g., D84.1)

Obtain prior authorization tied to a covered diagnosis; coverage is provided only for listed diagnosis codes (e.g., D84.1) and PA should be consistent with Moda Health policy and applicable CMS determinations where relevant.

• Document the covered ICD-10 diagnosis (Appendix 1 lists D84.1).
• When Medicare Part B may apply, follow applicable CMS NCDs/LCDs/LCAs in addition to Moda PA requirements.

Consider long‑term prophylaxis when on‑demand therapy is inadequate or for procedural prophylaxis

Consider long‑term prophylaxis when the patient requires ongoing prevention because short‑term measures or on‑demand therapies fail to provide adequate control or when prophylaxis is needed for procedures.

• Long‑term prophylaxis may be warranted based on disease activity, quality of life, health‑care resource availability, or failure of appropriate on‑demand therapy (e.g., Kalbitor, Firazyr, Ruconest, Berinert).
• Short‑term prophylaxis is appropriate prior to dental or medical procedures.

Include specialty prescriber, site‑of‑care, and clinical rationale in documentation

Provide complete clinical documentation to support medical necessity, including diagnostic confirmation, treatment intent, and clinical rationale for prophylaxis or continuation of therapy.

• Document prescriber specialty (allergy/immunology, hematology, pulmonology, or medical genetics) or consultation with such a specialist.
• Document site‑of‑care specialty infusion program compliance and the clinical reason for prophylaxis (short‑term or long‑term).

Provide repeat blood testing confirming HAE subtype (C1‑INH antigenic, C1‑INH functional, and C4)

Repeat blood testing confirming HAE subtype must be provided: for HAE I show low C1‑INH antigenic level, low C4 level, and low C1‑INH functional level; for HAE II show normal to elevated C1‑INH antigenic level with low C4 level and low C1‑INH functional level.

• HAE I: C1‑INH antigenic below lab lower limit of normal AND C4 below lab lower limit AND C1‑INH functional below lab lower limit; plus family history or acquired angioedema ruled out.
• HAE II: Normal/elevated C1‑INH antigenic level AND low C4 AND low C1‑INH functional level.

Follow Medicare Benefit Policy Manual Chapter 15 §50 and applicable NCDs/LCDs/LCAs when Medicare applies

When Medicare Part B is implicated, follow the Medicare Benefit Policy Manual (Pub. 100‑2), Chapter 15 §50 and comply with any applicable NCDs, LCDs, or LCAs for documentation and coverage rules.

• Search the CMS Coverage Database for relevant NCDs/LCDs/LCAs: https://www.cms.gov/medicare-coveragedatabase/search.aspx.
• Medicare policies take precedence where applicable and may impose additional documentation or coverage requirements.

Do not combine with other prophylactic C1‑INH/FXII/kallikrein‑targeting therapies

Do not use Cinryze in combination with other prophylactic therapies targeting C1 inhibitor, activated Factor XII, or kallikrein; concurrent use with agents such as Haegarda, Andembry, Takhzyro, or Orladeyo is not permitted and may lead to denial.

• Explicit prohibition: concurrent prophylactic therapies targeting C1‑INH, activated Factor XII, or kallikrein are disallowed.
• Verify medication list for Haegarda, Andembry, Takhzyro, Orladeyo, and similar agents before approving.

Verify and comply with applicable CMS Medicare determinations when relevant

Be aware that Medicare determinations may affect coverage: the policy describes non‑Medicare determinations but compliance with Medicare NCDs, LCDs, and LCAs is required where applicable and failure to follow them may result in denial.

• When a claim involves Medicare Part B, verify applicable NCDs/LCDs/LCAs and document compliance.
• Non‑Medicare coverage guidance in this policy does not replace CMS requirements for Medicare beneficiaries.