Hereditary Angioedema - BMC HealthNet Plan

important regulator in bradykinin production via the Factor XII/kallikrein proteolyticcascade. Low levels of C1-INH causes excess bradykinin that leads to leaky bloodvessels and fluid accumulation in body tissues resulting in HAE symptoms. Clinicalimplications of HAE include recurrent angioedema affecting the upper gastrointestinaland respiratory tracts. Laryngeal edema can be fatal if symptoms are not recognized andtreated promptly.Approaches to treatment of HAE include acute treatment of angioedema attacks, shorttermprophylaxis (i.e. dental procedures, surgery, intubation), and long term prophylaxisfor patients that experience more than one severe event per month or are disabled morethan 5 days per month. All patients with HAE should receive education on triggeravoidance (e.g. facial trauma, avoidance of certain medications) and a written plan fortreatment of acute attacks that can be used in emergency department care.There are currently three medications FDA approved for the treatment of HAE:Berinert ® , Firazyr ® and Kalbitor ® . Cinryze TM is indicated for the prevention of HAEattacks. Berinert ® and Cinryze TM are both C1-esterase inhibitors. Firazyr ® and Kalbitor ®both modulate bradykinin activity; Firazyr ® is a selective bradykinin B2 receptorantagonist and Kalbitor ® inhibits plasma kallikrein activity. Cinryze TM and Berinert ® areadministered by intravenous infusion, while Kalbitor ® and Firazyr ® are administeredsubcutaneously. Berinert ® , Cinryze TM and Firazyr ® can be self-administered; Kalbitor ®requires administration by a healthcare provider.Specifically, product labeling for Kalbitor ® contains a black box warning stating that dueto the risk of anaphylaxis, Kalbitor ® should only be administered by a healthcareprofessional with appropriate medical support to manage anaphylaxis and hereditaryangioedema. The manufacturer of Kalbitor ® (Dyax) is required by the FDA toparticipate in the Risk Evaluation and Mitigation Strategy Program (REMS).Other medications used for treatment and prophylaxis of HAE include attenuatedandrogens (danazol, oxandrolone, methyltestosterone), antifibrinolytic therapy (oraltranexamic acid, oral aminocaproic acid), and fresh frozen plasma (only useful in shortterm prophylaxis). Oral tranexamic acid, the preferred antifibrinolytic therapy inEurope, was approved for use in the United States under the brand name Lysteda ® inNovember 2009 for the treatment of heavy menstrual bleeding.This guideline provides information on BMC HealthNet Plan clinical criteria and claims adjudication processing guidelines. Theuse of this guideline is not a guarantee of payment and will not determine how a specific claim(s) will be paid. Reimbursement isbased on member benefits and eligibility, medical necessity review, where applicable, coordination of benefits, adherence to Planpolicies, clinical coding criteria, and the BMC HealthNet Plan agreement with the rendering or dispensing provider.Reimbursement policies may be amended at BMC HealthNet Plan’s discretion. BMC HealthNet Plan will always use the mostrecent CPT and HCPCS coding guidelines. All Plan policies are developed in accordance with state, federal and accreditingorganization guidelines and requirements, including NCQA.This document is subject to further revision in response to additional terms and requirements imposed under the Integrated CareProgram, including the ICP contract.BMCHP refers to Boston Medical Center HealthNet Plan in Massachusetts and Well Sense Health Plan in New Hampshire.Boston Medical Center HealthNet Plan and Well Sense Health Plan are trade names used by Boston Medical Center Health Plan,Inc.2 of 7BMC HealthNet Plan – Hereditary Angioedema