Haegarda — CareFirst (Caremark)

Initial criteria

• Requested medication will not be used in combination with any other medication used for prophylaxis of HAE attacks
• Member has C1 inhibitor deficiency or dysfunction as confirmed by laboratory testing AND one of the following: (a) C1 inhibitor (C1-INH) antigenic level below the lower limit of normal as defined by the laboratory, OR (b) Normal C1-INH antigenic level and a low C1-INH functional level (functional C1-INH less than 50% or below the lower limit of normal)
• OR Member has normal C1 inhibitor as confirmed by laboratory testing AND one of the following: (a) F12, angiopoietin-1, plasminogen, kininogen-1 (KNG1), heparan sulfate-glucosamine 3-O-sulfotransferase 6 (HS3ST6), or myoferlin (MYOF) pathogenic variant as confirmed by genetic testing, OR (b) Documented family history of angioedema and angioedema refractory to a trial of high-dose antihistamine therapy (e.g., cetirizine 40 mg per day or equivalent) for at least one month
• Other causes of angioedema have been ruled out (e.g., angiotensin-converting enzyme inhibitor induced angioedema, estrogen-containing drug related angioedema, allergic angioedema)
• Medication must be prescribed by or in consultation with a prescriber who specializes in management of HAE

Reauthorization criteria

• Member meets all initial coverage criteria
• Member has experienced a significant reduction in frequency of attacks (e.g., ≥ 50%) since starting prophylactic treatment
• Member has reduced the use of medications to treat acute attacks since starting prophylactic treatment

Approval duration

12 months