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DESCRIPTIONHereditary angioedema is a disorder characterized by recurrent episodes of severe swelling (angioedema). The most common areas of the body to develop swelling are the limbs, face, intestinal tract, and airway. Minor trauma or stress may trigger an attack, but swelling often occurs without a known trigger. Episodes involving the intestinal tract cause severe abdominal pain, nausea, and vomiting. Swelling in the airway can restrict breathing and lead to life-threatening obstruction of the airway.
Symptoms of hereditary angioedema typically begin in childhood and worsen during puberty. On average, untreated individuals have an attack every 1 to 2 weeks, and most episodes last for about 3 to 4 days. The frequency and duration of attacks vary greatly among people with hereditary angioedema, even among people in the same family.
HAE is estimated to affect 1 in 50,000 people.
Icatibant (Firazyr®) is a bradykinin B2 receptor antagonist. Herediatary angoiedema is caused by an absence or dysfunction of C1-esterase-inhibitor, a key regulator of the cascade that leads to bradykinin production. Firazyr® inhibits bradykinin from binding the B2 receptor and thereby treats the clinical symptoms of an acute, episodic attack of HAE.
FDA APPROVED INDICATIONS
Icatibant (Firazyr®) is indicated for the treatment of acute attacks of hereditary angioedema in adults 18 years of age and older. Firazyr® is administered by subcutaneous injection.
C1 Esterase Inhibitor Human (Berinert®) is indicated for the treatment of acute abdominal, facial, or laryngeal attacks of hereditary angioedema in adult and adolescent patients. Berinert® is administered by IV injection.
C1 Esterase Inhibitor Human (Cinryze®) is indicated for routine prophylaxis against angioedema attacks in adolescent and adult patients with hereditary angioedema. Cinryze® is administered IV every three or four days.
Ecallantide (Kalbitor®) is indicated for treatment of acute attacks of hereditary angioedema in patients 16 years of age and older. Kalbitor® is administered subcutaneously by a healthcare professional with appropriate medical support.
Generic Name: Icatibant
Brand Name: Firazyr®
Generic Name: C1 Esterase Inhibitor Human
Brand Name: Berinert®
Generic Name: C1 Esterase Inhibitor Human
Brand Name: Cinryze®
Generic Name: Ecallantide
Brand Name: Kalbitor®
POLICYPrior authorization is required for Firazyr.
Icatibant (Firazyr®) is considered medically necessary for the treatment of acute attacks of hereditary angioedema in adults 18 years of age and older. Only three doses of Firazyr® are allowed per fill.
Berinert®, Cinryze®, and Kalbitor® should only be administered under the supervision of a healthcare professional; therefore, these medications are not appropriate for use in the home care setting and are not prior authorized.
POLICY GUIDELINESInvestigative service is defined as the use of any treatment procedure, facility, equipment, drug, device, or supply not yet recognized by certifying boards and/or approving or licensing agencies or published peer review criteria as standard, effective medical practice for the treatment of the condition being treated and as such therefore is not considered medically necessary.
The coverage guidelines outlined in the Medical Policy Manual should not be used in lieu of the Member's specific benefit plan language.
POLICY HISTORY04/01/2012: New policy added.
01/07/2013: Added the following new 2013 CPT code to the Code Reference section: J1744.
SOURCE(S)US National Library of Medicine
Firazyr® Prescribing Information
Kalbitor® Prescribing Information
Cinryze® Prescribing Information
Berinert® Prescribing Information
CODE REFERENCEThis is not intended to be a comprehensive list of codes. Some covered procedure codes have multiple descriptions.
The code(s) listed below are ONLY covered if the procedure is performed according to the "Policy" section of this document.
This is not an all-inclusive list of non-covered procedure codes.
The code(s) listed below and ANY code not listed in the previous section are considered non-covered for this procedure.