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About HAE	Educational Materials	Conferences and Events	Key Publications	Additional Resources

About HAE

Hereditary Angioedema (HAE) is a rare hereditary disease that can cause attacks of swelling, and often pain, in specific parts of the body including the stomach, hands, feet, arms, legs, genitals, throat, and face. Depending on the severity of the disease, some people will have many attacks each month, while others will go months without an attack.

People with HAE are missing or have low levels of a protein called C1 esterase inhibitor (C1-INH); in some cases, the C1-INH levels are sufficient, but the protein does not function properly. The defect with C1-INH lies within a person's genetic code, which is why HAE runs in families.

Hereditary Angioedema is classified into three types based on what problem the genetic defect causes.

Type I - Low levels of C1-INH in the body; this is the most common form of HAE and accounts for ~85% of people with HAE.
Type II – Normal or elevated levels of C1-INH, but C1-INH is dysfunctional; this accounts for ~15% of people with HAE.
HAE with Normal C1-INH (formerly known as HAE Type III) - Normal and functioning levels of C1-INH; this type of HAE is extremely rare and not well understood.

References:

Zuraw, B. Hereditary Angioedema. N Engl J Med. 2008; 359:1027-36.

Important Safety Information for HAEGARDA

HAEGARDA^®, C1 Esterase Inhibitor Subcutaneous (Human), is a plasma-derived concentrate of C1 Esterase Inhibitor (C1-INH) indicated for routine prophylaxis to prevent Hereditary Angioedema (HAE) attacks in patients 6 years of age and older. HAEGARDA is for subcutaneous use after reconstitution only.

HAEGARDA is contraindicated in patients with a history of life-threatening hypersensitivity reactions, including anaphylaxis, to C1-INH preparations or their excipients.

Severe hypersensitivity reactions to HAEGARDA could occur. In such cases, discontinue administration and institute appropriate treatment. Epinephrine should be immediately available to treat hypersensitivity reactions.

At the recommended subcutaneous dose of HAEGARDA, no causal relationship to thromboembolic events (TEs) has been established. However, TEs have been reported with intravenous administration of C1-INH products, usually at high doses.

In clinical trials, adverse reactions observed in more than 4% of subjects treated with HAEGARDA were injection-site reactions, hypersensitivity, nasopharyngitis, and dizziness.

HAEGARDA is derived from human plasma. The risk of transmission of infectious agents, including viruses and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent and its variant (vCJD), cannot be completely eliminated.

Please see full prescribing information for HAEGARDA.

To report SUSPECTED ADVERSE REACTIONS, contact the CSL Behring Pharmacovigilance Department at 1-866-915-6958 or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.