Hereditary Angioedema (HAE) is a disease characterized by low levels of the plasma protein C1 inhibitor (C1-INH).  There are three types of HAE, and it is considered a genetic disease that is hereditary.  It is a rare condition that effects 1 in 10,000 to 50,000 individuals and can be life threatening.  There are three blood tests used to confirm HAE: a C1 inhibitory quantitative, C1- inhibitor functional, and a C4 serum level.

Symptoms and Diagnosis

Symptoms of HAE include recurrent episodes of swelling in the hands, feet, gastrointestinal tract, genitals and throat.  These episodes can last anywhere from two to five days and range in severity.  These episodes are different from allergic reactions and cannot be treated the same way with typical corticosteroids, antihistamines, or epinephrine.  Episodes involving the throat are the most life threatening, as they can cause closure of the airway.  Abdominal attacks can cause severe pain, nausea, vomiting, and diarrhea.

Treatment of Hereditary Angioedema

Treatment of HAE includes prophylaxis, management of acute attacks, and prophylactic therapy in situations where attacks may occur.  There are currently six treatment options available for HAE, each with different routes of administration, age restrictions, and places in therapy.  Since each individual patient is different you should speak with your healthcare provider to determine the most appropriate therapy for you.  Therapies currently available include: Berinert©, Cinryze©, Firazyr ©, Haegarda©, Kalbitor©, and Ruconest©.

There are many advocacy groups and support out there for patients diagnosed with HAE.  Talk with your healthcare provider about action plans in case of an attack and learn as much as you can about your disease.

For more information, please visit:

US Hereditary Angioedema Association

References:

“Hereditary Angioedema Background” Michael M. Frank, MD, et al.  Medscape Reference: Drugs and Diseases 

“HAE Symptoms” US Hereditary Angioedema Association

“HAE Treatment Options” US Hereditary Angioedema Association

Services provided by Long’s Drugs are not intended to replace the services of a physician. This information is provided for informational purposes only and is not a substitute for professional medical advice. You should consult a physician in all matters relating to your health, and particularly in respect to any symptoms that may require diagnosis or medical attention.