Coverage is provided in the following conditions:
• Patient is at least 13 years of age; AND
Universal Criteria
• Must be prescribed by, or in consultation with, a specialist in: allergy, immunology, hematology, pulmonology, or medical genetics; AND
• Patient does not have a history of allergy to rabbits or rabbit-derived products; AND
• Confirmation the patient is avoiding the following possible triggers for HAE attacks:
o Estrogen-containing oral contraceptive agents AND hormone replacement therapy;
AND
o Antihypertensive agents containing ACE inhibitors; AND
o Dipeptidyl peptidase IV (DPP-IV) inhibitors (e.g., sitagliptin); AND
o Neprilysin inhibitors (e.g., sacubitril); AND
Treatment of acute abdominal, peripheral, or facial attacks of Hereditary Angioedema (HAE)
Patient has a history of moderate to severe cutaneous attacks (without concomitant hives)OR abdominal attacks OR mild to severe airway swelling attacks of HAE (i.e. debilitating cutaneous/gastrointestinal symptoms OR laryngeal/pharyngeal/tongue swelling); AND
Patient has one of the following clinical presentations consistent with a HAE subtype§,which must be confirmed by repeat blood testing (treatment for acute attack should not bedelayed for confirmatory testing):
HAE I (C1-Inhibitor deficiency)
ï Low C1 inhibitor (C1-INH) antigenic level (C1-INH antigenic level below the lower limit of
normal as defined by the laboratory performing the test); AND
ï Low C4 level (C4 below the lower limit of normal as defined by the laboratory performing
the test); AND
ï Low C1-INH functional level (C1-INH functional level below the lower limit of normal as
defined by the laboratory performing the test); AND
o Patient has a family history of HAE; OR
o Acquired angioedema has been ruled out (i.e., patient onset of symptoms occur prior
to 30 years old, normal C1q levels, patient does not have underlying disease such as
lymphoma or benign monoclonal gammopathy [MGUS], etc.)
HAE II (C1-Inhibitor dysfunction)
Normal to elevated C1-INH antigenic level; AND
• Low C4 level (C4 below the lower limit of normal as defined by the laboratory performing
the test); AND
• Low C1-INH functional level (C1-INH functional level below the lower limit of normal as
defined by the laboratory performing the test)
HAE with normal C1INH (formerly known as HAE III)
ï Normal C1-INH antigenic level; AND
ï Normal C4 level; AND
ï Normal C1-INH functional level; AND
ï Repeat blood testing during an attack has confirmed the patient does not have abnormal lab
values indicative of HAE I or HAE II; AND
ï Either of the following:
o Patient has a known HAE-causing mutation (e.g., mutation of coagulation factor XII gene [F12 mutation], mutation in the angiopoietin-1 gene, mutation in the plasminogen gene, mutation in the kininogen 1 gene, mutation in the myoferlin gene, mutation in the heparan sulfate 3-O-sulfotransferase 6 gene, etc.); OR
o Patient has a family history of HAE and documented evidence of lack of efficacy of chronic high-dose antihistamine therapy (e.g. cetirizine standard dosing at up to four times daily or an alternative equivalent, given for at least one month or an interval long enough to expect three or more angioedema attacks) AND corticosteroids with or without omalizumab
FDA Approved Indication(s); Ф Orphan Drug
