Signs, Symptoms and Types of HAE HAE can produce episodes of nonpitting, nonerythematous swelling in the abdomen, face, and throat. The number of episodes an individual may experience is unpredictable. Some people experience weekly attacks, while others may go years between attacks.7 Laryngeal edema poses the greatest risk for patients with HAE, due to the chance of asphyxiation.7

The swelling associated with an attack often gets worse over a period of 12 hours to 24 hours, then typically resolves within 72 hours. However, symptoms can last up to five days.4, 7 Unlike allergic reactions, there is no itching or redness with HAE.2, 4 In addition, patients with abdominal swelling often experience severe abdominal pain and nausea, which can be accompanied by vomiting and diarrhea.7, 9

HAE episodes are usually asymmetrical and localized to a single area of the body, but simultaneous or closely spaced attacks can also occur.4, 5

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

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.

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

Types of HAE

Type I: The body does not produce enough C1-1NH. Approximately 85% of people living with the disease have type I.2

Type II: The body produces C1-INH that does not function correctly. Approximately 15% of people living with HAE have Type II.2

Another, very rare form of HAE is HAE with normal C1-INH (aka Type III). Berinert is not indicated for this type of HAE. Berinert treats the two most common types of HAE, I and II.

Test for HAE by screening for C4 Deficiency10

Type I Low Low Low Normala Normal
Type II Low Normal or High Low Normal Normal
Type III Normal Normal Normal Normal Normal
Acquired C1-INH Deficiency Low Low Low Low Normal or Low
ACE-1-Induced Angioedema Normal Normal Normal Normal Normal
Allergic or Idiopathic Angioedema Normal Normal Normal Normal Normal

aLow C1q concentrations occur in some patients with HAE. ACE-1 = angiotensin-converting enzyme 1; C1-INH = C1 esterase inhibitor. Chart adapted with permission from Weiler CR et al. Mayo Clinic Proceedings. 2006;81(7):958-972.

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About Berinert

Berinert is the only on-demand C1 esterase inhibitor (C1-INH) approved for treatment of acute abdominal, facial, or laryngeal attacks of HAE in adult and pediatric patients.

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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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