What is hereditary angioedema (HAE)? HAE is a rare, inherited disease that results in a quantitative and/or qualitative deficiency in C1 esterase inhibitor (C1-INH), a key regulator of inflammation and integral in bradykinin mediated angioedema.1 HAE can be fatal if not properly diagnosed.1

HAE is characterized by acute, recurrent attacks of localized edema. This condition affects approximately 1:10,000 to 1:50,000 individuals.2, 3 As a result of C1-INH deficiency, people living with HAE develop recurring episodes of edema, which may occur in the extremities, abdomen, face, and throat.4

Diagnosing patients with HAE

HAE is often misdiagnosed because its symptoms mimic those of several common conditions. However, HAE can be confirmed with proper blood tests to evaluate C4 serum concentration and C1-INH function.5, 6 If no family history is available, misdiagnosis may occur. In addition, approximately 25% of HAE cases are due to de novo gene mutations.7, 8

HAE patients are often subjected to nonessential medical procedures, such as laparoscopy; ineffective treatments, such as administration of antihistamines; and/or unnecessary psychiatric referrals (due to unexplained or recurrent abdominal pain).5, 6

HAE should be suspected when a patient presents with a history of recurrent angioedema, especially if wheals (hives) are absent. This suspicion is further substantiated when patients report (1) a positive family history; (2) onset of symptoms in childhood/adolescence; (3) recurrent abdominal pain attacks; (4) occurrence of upper airway edema; (5) failure to respond to antihistamines, glucocorticoid, or epinephrine; and (6) presence of HAE attack signs or symptoms before swellings.

Learn 8 simple questions to help with your HAE diagnosis

Ask your patients the 8 questions below. Three or more positive answers may suggest a diagnosis of HAE, and testing is recommended.

YES NO
1. Unexplained edema?15
2. Asymmetric swelling attacks to the extremities?7
3. Unexplained abdominal pain?9,a
4. Recurrent attacks?15
5. Family history of similar episodes?8,b
6. Symptoms (eg, fatigue, tingling, or nausea) signaling the onset of an attack?16
7. Antihistamines, epinephrine, or corticosteroids provide little relief?7
8. Angioedema without urticaria?1

a The majority of patients with HAE will have abdominal attacks that cause pain, nausea, vomiting, and/or diarrhea.9
b Up to 75% of patients will have a positive family history for HAE; the remaining 25% of patients may develop de novo mutations.8

Attacks can also be migratory, starting in one location and spreading to another before resolving. Migratory attacks may spread from site to site over the course of a few days.2, 5

*Peer supporters are not healthcare professionals or medical experts. For medical questions, patients should contact their physicians.

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Signs and Symptoms

Learn about the different signs, symptoms and types of HAE, as well as the 8 simple questions to help with your HAE diagnosis.

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Important Safety Information

Berinert®, C1 Esterase Inhibitor (Human), 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.

Berinert 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 selfadministration 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.

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

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