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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 BERINERT

BERINERT^®, C1 Esterase Inhibitor (Human), is contraindicated in individuals with a history of life-threatening systemic reactions to C1 esterase inhibitor preparations (including anaphylaxis).

Monitor patients for early signs of allergic or hypersensitivity reactions (including hives, generalized urticaria, chest tightness, wheezing, hypotension, and anaphylaxis). If hypersensitivity is suspected, immediately discontinue administration of BERINERT and initiate appropriate treatment. Epinephrine should be immediately available for treatment of acute severe hypersensitivity reactions.

Serious arterial and venous thromboembolic (TE) events have been reported following administration of recommended doses of C1 Esterase Inhibitor (Human) products to patients with HAE. Risk factors may include presence of an indwelling venous catheter/access device; prior history of thrombosis; underlying atherosclerosis; use of oral contraceptives or certain androgens; morbid obesity; and immobility. Weigh benefits/risks before administering to patients with known risk factors for TE events and closely monitor such patients during and after BERINERT administration. TE events also have been reported with C1 Esterase Inhibitor (Human) products when used for unapproved indications at higher than recommended doses.

Appropriately trained patients may self-administer BERINERT upon recognition of an HAE attack. Advise patients to seek medical attention immediately following self-administration for laryngeal attacks, and to seek medical attention if progress of any attack makes them unable to properly prepare or administer dose of BERINERT.

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

The most serious adverse reaction reported in subjects who received BERINERT in clinical studies was an increase in severity of pain associated with HAE. Dysgeusia was the most common adverse reaction reported in over 4% of subjects and more frequently than in the placebo group.

BERINERT has not been evaluated in pregnant women or nursing mothers, and should be used only if clearly needed. In clinical trials, the half-life of BERINERT was shorter and clearance was faster in children than in adults; the clinical implication of this difference is not known.

Indications for BERINERT

BERINERT is a plasma-derived concentrate of C1 Esterase Inhibitor (Human), indicated for the treatment of acute abdominal, facial or laryngeal attacks of hereditary angioedema (HAE) in adult and pediatric patients. The safety and efficacy of BERINERT for prophylactic therapy have not been established.

Please see full prescribing information for BERINERT, including the patient product information.

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.