Andembry — Blue Cross Blue Shield of Kansas

Initial criteria

• 1. The patient has a diagnosis of hereditary angioedema (HAE) due to C1-INH deficiency [HAE-C1INH (Type 1 or Type 2)] evidenced by ONE of the following:
• A. The patient’s diagnosis has been confirmed with measurements of C1-INH protein level, C1-INH function level, and C4 level as follows:
• 1. Type 1 HAE: Decreased quantities of C4 level, C1-INH protein level, and C1-INH function level OR
• 2. Type 2 HAE: Decreased quantities of C4 level and C1-INH function level (C1-INH protein level may be normal or elevated) OR
• B. The patient’s diagnosis has been confirmed by mutation in the C1-INH gene altering protein synthesis and/or function AND
• 2. The requested agent is being prescribed for HAE prophylaxis AND
• 3. The patient has a history of at least three moderate to severe acute HAE attacks per month (e.g., airway swelling, severe abdominal pain, painful facial swelling) AND
• 4. If the patient has an FDA labeled indication, then ONE of the following:
• A. The patient’s age is within FDA labeling for the requested indication for the requested agent OR
• B. There is support for using the requested agent for the patient’s age for the requested indication