Firazyr — Medical Mutual

Initial criteria

• Patient age ≥ 18 years; AND
• Must be prescribed by, or in consultation with, a specialist in allergy, immunology, hematology, pulmonology, or medical genetics; AND
• Confirmation the patient is avoiding the following possible triggers for HAE attacks: Estrogen-containing oral contraceptive agents AND hormone replacement therapy; AND antihypertensive agents containing ACE inhibitors or angiotensin II receptor blockers (ARBs); AND dipeptidyl peptidase IV (DPP-IV) inhibitors (e.g., sitagliptin); AND neprilysin inhibitors (e.g., sacubitril); AND
• Patient has a history of moderate to severe cutaneous attacks (without concomitant urticaria) OR abdominal attacks OR mild to severe airway swelling attacks of HAE (i.e. debilitating cutaneous/gastrointestinal symptoms OR laryngeal/pharyngeal/tongue swelling); AND
• Laboratory confirmation of HAE subtype:
• HAE I (C1-Inhibitor deficiency): Low C1-INH antigenic level AND Low C4 level AND Low C1-INH functional level; AND patient has a family history of HAE OR acquired angioedema has been ruled out (symptom onset < 30 years old, normal C1q, no underlying disease such as lymphoma or MGUS); OR
• HAE II (C1-Inhibitor dysfunction): Normal to elevated C1-INH antigenic level AND Low C4 level AND Low C1-INH functional level; OR
• HAE with normal C1-INH (formerly HAE III): Normal to near normal C1-INH antigenic, C4, and functional levels; AND repeat blood testing during attack confirms no abnormal values consistent with HAE I or II; AND either patient has a known HAE-causing mutation (e.g., F12, angiopoietin-1, plasminogen, kininogen 1, myoferlin, HS3ST6) OR patient has family history of HAE AND documented lack of efficacy of chronic high-dose antihistamine therapy and corticosteroids ± omalizumab

Reauthorization criteria

• Patient continues to meet the universal and indication-specific criteria; AND
• Significant improvement in severity and duration of attacks has been achieved and sustained; AND
• Absence of unacceptable toxicity from the drug (e.g., laryngeal HAE attacks); AND
• The cumulative amount of medication on-hand will be considered; authorization allows sufficient quantity for up to 4 acute attacks per 4 weeks for the duration of the approval

Approval duration

12 weeks