Symptoms and Diagnosis Hereditary angioedema can take a long time to diagnose, because HAE symptoms may resemble other conditions, such as allergies or stomach problems.

How doctors diagnose hereditary angioedema

A series of blood tests that measure levels of serum C4 and C1-INH proteins are needed to confirm the disease. The tests determine HAE type.

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

Because delay of diagnosis is so common, it’s important to talk to your doctor about HAE as a possible cause of your symptoms.

Be prepared to discuss these topics with your doctor:

  1. Any family history of HAE.
  2. Your personal health history, as well as any medications you may be taking.
  3. Symptoms that may point to the disease.
  4. Specifics about an attack, such as trigger, location, frequency, and duration. It may be helpful to record this information in an interactive treatment journal.
  5. The impact HAE attacks are having on your life. Consider using the HAE Treatment Plan designed to help manage future HAE attacks.
  6. Your comfort level with learning to self-administer Berinert via intravenous infusion.

The more information you can give to your doctor, the better.

If your doctor suspects you may have hereditary angioedema, he or she will order a series of blood tests, which is the only way HAE can be confirmed. Your physician may then refer you to a specialist who knows about HAE, such as an allergist or immunologist. If your doctor has trouble locating a specialist, you may want to contact the US Hereditary Angioedema Association. This organization maintains a robust list of doctors across the country who treat people living with hereditary angioedema.

Recognizing HAE symptoms

HAE attacks can cause swelling, or edema, in specific parts of the body, including the abdomen, face, and throat. Attacks often begin in childhood, becoming more severe over time. The number of episodes an individual may experience is unpredictable. Some people experience weekly attacks, while others may go years without one.

During an attack, abdominal, facial, and laryngeal swelling often gets worse over a period of 12 hours to 24 hours, and resolves within 72 hours. Unlike allergic reactions, there is no itching or redness with HAE. In addition to abdominal swelling and pain, patients often experience nausea, which can be accompanied by vomiting.

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

Be prepared with Berinert

Self-administration at the first sign of an attack with fast and reliable Berinert can help put you in control of your HAE and increase your independence.

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Living With HAE

Effectively managing your HAE requires knowing and avoiding your triggers. Learn how to work with your doctor to be prepared for a potential attack.

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Important Safety Information

Berinert®, C1 Esterase Inhibitor (Human), is used in adults and children to treat swelling and/or painful attacks of hereditary angioedema (HAE) affecting the abdomen, face or throat. The safety and efficacy of Berinert in preventing HAE attacks have not been established.

Do not use Berinert if you have experienced life-threatening allergic reactions or severe hypersensitivity to the product. Inform your healthcare provider of all medications you are taking and of any medical conditions, especially any history of blood-clotting problems.

Blood clots have occurred in patients receiving Berinert. Tell your healthcare provider if you have a history of heart or blood vessel disease, stroke, or blood clots, or if you have thick blood, an indwelling catheter/access device in a vein, or have been immobile for some time. Certain medications, such as birth control pills, may also increase your risk of clotting problems.

Report to your physician or an emergency room any signs and symptoms of a blood clot, including pain and/or swelling or discoloration of an arm or leg, with warmth over affected area; unexplained shortness of breath; chest pain or discomfort that worsens on deep breathing; rapid pulse; and numbness or weakness on one side of the body.

In addition, report immediately any signs or symptoms of allergic reactions to Berinert, including hives, chest tightness, wheezing, difficulty breathing, turning blue, faintness, facial swelling and fast heartbeat.

If you have been trained to self-administer Berinert, immediately prepare the prescribed dose at the first symptoms of an attack. Seek immediate medical attention and do not begin to self-adminster if an HAE attack has progressed to a point where you will be unable to prepare or administer a dose of Berinert.

If you self-administer to treat a laryngeal attack, immediately seek medical attention afterward. If you self-administer for an abdominal attack, inform your physician so that other possible causes can be ruled out.

Call your doctor right away if swelling is not controlled after use of Berinert.

In clinical studies, the most serious adverse reaction reported in subjects who received Berinert was an increased severity of the pain associated with HAE. In the placebo-controlled clinical trial, the most common adverse reaction reported more often among subjects who received Berinert than those receiving placebo was dysgeusia (a bad taste in mouth). Tell your healthcare provider about any side effect that bothers you or does not go away.

Because Berinert is made from human blood, the risk that it may transmit infectious agents, including viruses and theoretically, the agents of Creutzfeldt-Jakob Disease (CJD) and its variant form (vCJD), cannot be completely eliminated.

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

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch or call 1-800-FDA-1088.

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