Hereditary angioedema (HAE) is a hereditary disorder that causes severe swelling, usually in the limbs, face, airway, and intestines. This can lead to abdominal pain, nausea, vomiting, and life-threatening airway obstruction. Swelling can be triggered by stress but often has no clear cause. A rash called erythema marginatum occurs during attacks in about a third of patients. Episodes involving the intestinal tract cause severe abdominal pain, nausea, and vomiting. Swelling in the airway can restrict breathing and lead to life-threatening obstruction of the airway.
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About 1 in 50,000 people are affected by HAE. A patient’s specific type of hereditary angioedema can be determined by the level of a protein, called C1 inhibitor, in their bloodstream. About 85 percent have Type 1, while Type 2 makes up about 15 percent of cases and Type 3 is extremely rare. All three types have similar signs and symptoms.
Although HAE is usually inherited, it can occur from new gene mutations with no family history. Symptoms usually begin in childhood, getting more intense during puberty. The frequency and duration of attacks varies, but in general, they last 3–4 days and happen about once every 1–2 weeks.
There are multiple treatments on the market today to treat or prevent HAE attacks, most of which can be self-administered. Diplomat Specialty Infusion Group offers multiple HAE treatments, including Berinert® (C1 esterase inhibitor [human]) and Firazyr® (icatibant injection).
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