Hereditary angioedema symptoms and diagnosis

Recognizing hereditary angioedema symptoms

Hereditary angioedema (HAE) attacks can cause swelling, or edema (EH deem uh), in specific parts of the body, including the abdomen, face, and throat. Berinert, a safe and effective on-demand therapy, can help treat acute abdominal, facial, and laryngeal attacks. The safety and efficacy of Berinert for prophylactic therapy have not been established. Berinert is approved for patient self-administration after proper training by a healthcare professional.

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 to 24 hours, and resolves within 72 hours. However, symptoms can last for up to five days. 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 dizziness and vomiting.

Importance of treating at the first sign of an attack

HAE episodes are usually asymmetrical and localized to a single area of the body, but simultaneous or closely spaced attacks can also occur. Attacks can also be migratory – starting in one location and then spreading to another before resolving. Migratory attacks tend to be erratic. They can begin abruptly and move from site to site within hours, or they can spread slowly, taking days to move from one site to the next.

Sometimes, early HAE symptoms appear that herald the onset of an HAE attack. These prodromal (pro DROAM ul) symptoms may include sudden mood changes, rash, irritability, aggressiveness, anxiety, extreme fatigue, or a tingling sensation of the skin where the swelling will begin. It is important to treat at the first sign of an attack.

How doctors diagnose hereditary angioedema

A hereditary angioedema diagnosis is often delayed. This is partly due to the fact that the condition is fairly rare and many doctors don’t have experience with it. In addition, HAE symptoms may resemble other conditions, such as allergies or stomach problems. To help avoid a delay in diagnosis, learn what to communicate to your doctor.

A series of blood tests, measuring levels of serum C4 and C1-INH proteins, are needed to confirm the disease. The tests measure for both type I and type II HAE. Not all laboratories are equipped to handle the necessary blood specimens, so be sure to have your tests done at a qualified lab. Contact the US Hereditary Angioedema Association (HAEA) to locate a qualified lab.

Important Safety Information

Berinert®, C1 Esterase Inhibitor (Human), is for the treatment of ongoing, acute attacks of hereditary angioedema (HAE) affecting the abdomen, face or throat in adults and adolescents. 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. Immediately report to your physician or an emergency department any signs or symptoms of allergic reactions to Berinert, including hives, chest tightness, wheezing, turning blue, fast heartbeat, and shock. Also report signs and symptoms of thrombosis that occur after infusing: including swelling and pain in the limbs or abdomen, chest pain, shortness of breath, loss of sensation or motor power, or altered consciousness or speech.

Berinert can be self-administered if you have been trained and advised to do so by your healthcare provider. Seek immediate medical attention if an HAE attack has progressed to a point where you will be unable to prepare or administer Berinert.

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

Because Berinert is made from human blood, the risk that it may transmit infectious agents, including viruses and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent, cannot be completely eliminated.

In clinical studies, the most serious adverse reaction reported in subjects who received Berinert was an increased severity of 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).

Berinert has not been evaluated in pregnant women or nursing mothers; inform your doctor if you are pregnant or are planning to become pregnant, or if you are breastfeeding or plan to breastfeed. The safety and effectiveness of Berinert have not been established in children under 12 or adults over 65 years of age.

Please see full prescribing information for Berinert, including 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.