Treating HAE

ACE Inhibitor: Class of medicines used to treat high blood pressure and other diseases.
Anabolic steroid: Also referred to as attenuated androgen, a class of steroid hormones used to prevent HAE attacks. Although their mechanism of action in HAE patients is unknown, these agents are believed to partially reverse the biochemical defect by increasing levels of C1-esterase inhibitor (C1-INH), helping to prevent angioedema attacks in patients with inherited C1-INH deficiency.
Antifibrolytic agent: Medicines used to prevent HAE attacks. These agents inhibit the activation of plasmin, a chemical in the blood that can make the C1-INH less effective.
Attenuated androgen: Also referred to as anabolic steroid, a class of steroid hormones used to prevent HAE attacks. Although their mechanism of action in HAE patients is unknown, these agents are believed to partially reverse the biochemical defect by increasing levels of C1-esterase inhibitor (C1-INH), helping to prevent angioedema attacks in patients with inherited C1-INH deficiency.
Bradykinin: A protein that causes blood vessels to expand (dilate) and leak serum and other fluids.
C1 inhibitor: Also referred to as C1-INH, a protein found in the fluid part of your blood that controls C1, a component of your body's complement system. Abnormal swelling can result when C1-INH is insufficient or defective.
C1-INH: Also referred to as C1 inhibitor, a protein found in the fluid part of your blood that controls C1, a component of your body's complement system. Abnormal swelling can result when C1-INH is insufficient or defective.
Complement system: The complement system is made up of proteins like C1 that float in your bloodstream, which work with the immune system to fight organisms like bacteria, viruses and parasites.
Drug formulary: A list of prescription medications selected for coverage under a health insurance plan.
HAE: HAE, or Hereditary Angioedema, is a rare, hereditary disease of the immune system that affects an estimated 1 in 10,000 to 1 in 50,000 adults and children globally. HAE causes attacks of spontaneous swelling that are often painful and severe, and can be life-threatening.
Hereditary Angioedema: A rare, hereditary disease of the immune system that affects an estimated 1 in 10,000 to 1 in 50,000 adults and children globally. Hereditary Angioedema (HAE) causes attacks of spontaneous swelling that are often painful and severe, and can be life-threatening.
Laryngeal attack: An HAE attack that affects the larynx (throat); this can block or close the airway, leading to difficulty breathing and, potentially, death.
Plasma kallikrein: A chemical in the bloodstream that is involved in blood clotting; it plays an important role in causing the swelling of HAE attacks.
Preventive therapy: In HAE, medicine taken to prevent attacks.
Prodrome: One or more physical warnings of an HAE attack; can appear as a rash or a feeling of skin tightness.
Serum: The clear, watery, fluid-component of blood that resembles fluid in a blister.
Subcutaneous: Beneath the skin.
Trigger: In HAE, something that causes an attack. Stress and trauma are two common HAE attack triggers.

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Treating HAE

Although there is no cure for HAE, there are treatments available to help prevent attacks and treat acute attack symptoms, with more new therapies being developed.

Types of Treatment
Descriptions of the treatment options currently available.

On the Horizon
New options for HAE treatment in development.

Insurance Issues
Potential insurance coverage implications of the 1983 Orphan Drug Act.

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