1/10,000 may be the small prevalence of HAE, but it was the big thinking at this year’s AAAAI

Hereditary Angioedema (HAE), a very rare and potentially life threatening genetic condition, stole the thunder at the AAAAI this year.  One of our brand engagement managers, Layne Shapiro, attended the conference to represent the Stinson Brand Innovation team.  She shares these observations.

HAE is a rare disease that occurs in only about 1/10,000 to 1/50,000 people. Symptoms include episodes of swelling (edema) in various body parts including the hands, feet, face, and airway.  Until this past year, there were no approved treatments of HAE in the US.


The buzz at AAAAI was regarding the 3 recently approved treatment options for HAE:

• CINRYZE™ (C1 esterase inhibitor (human): Approved for patient self administration, CINRYZE™ is a C1 esterase inhibitor indicated for the routine prophylaxis against angioedema attacks in adolescent and adult patients with HAE.
• KALBITOR^® (ecallantide): The first approved for subcutaneous treatment for all acute HAE attack locations, Kalbitor must be given by a doctor or nurse in a healthcare setting. KALBITOR^® is an inhibitor of the protein kallikrein used for HAE. 
• BERINERT^® (C1 Esterase Inhibitor, Human): Approved for treatment of acute abdominal or facial attacks of HAD in adult and adolescent patients, BERINERT^® is a plasma-derived concentrate of C1 esterase inhibitor (human).

 The US Hereditary Angioedema Association (HAEA) had a table in the exhibit hall, as well as a postcard insert in the registrants’ bag which described the condition, the association, and the US HAEA Scientific Registry.

Through educational grants, CSL Behring, Dyax Corp, and ViroPharma funded a dinner symposium entitled "Hereditary Angioedema: Diagnosis and Management Options for the 21st Century." Speakers from around the world included Bruce L. Zuraw, MD, Marco Cicardi, MD, and Konrad Bork, MD. They discussed topics such as identifying appropriate diagnostic tests, proper interpretation and limitation for each assay, acute treatement options, and the selection, dosage and monitoring of therapeutic agents used in controlling HAE.

Typically it takes about 10 years before a person will be diagnosed with HAE. Marc Riedl, MD, MS, has put together a CME course with ReachMD for allergists and healthcare professionals on overcoming the many obstacles in HAE with regard to diagnosis and treatment.

With new treatment options available, diagnosis has become increasingly important.