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FAQ: Disease pattern
Answers on frequently asked questions about the clinical picture of HAE. Definitions can be looked up in the glossary.
What is hereditary Angioedema?
Hereditary angioedema (HAE) is a rare inherited disease characterised by recurrent episodes of swelling (edema). Episodes of HAE can affect different areas of the skin and internal organs. Episodes affecting the larynx or tongue are particularly dangerous and can cause death by suffocation if left untreated. About every third patient has at least one life-threatening episode of this type in his lifetime.How many people are affected by HAE?
Exact figures regarding the incidence of HAE are not available. It is estimated that about 1 in 10,000 to 1 in 50,000 people are affected worldwide. Experts assume that only 1 in 2 people with HAE are given the correct diagnosis.When do the symptoms of HAE first occur?
The age at which the symptoms of HAE first appear varies widely, but about 75% of those affected have their first episode as early as their first or second decade of life. The frequency of the episodes is particularly high during puberty and early adulthood. However, the disease does not always start early in life.How do the symptoms of HAE manifest themselves?
The oedema is often preceded ba y feeling of tension in the affected area of skin and/or a circular reddening (so called erythema marginatum). An episode of HAE involves an acute swelling of the skin or mucous membranes, primarily affecting the limbs, face and neck as well as the buttocks and genitalia. Although the swelling of the skin is almost never associated with itching, it causes a feeling of tension, the severity of which ranges from unpleasant to painful. The facial swelling can be disfiguring. Swelling can also occur in the wall of the intestine. This swelling is associated with colicky pain and can cause diarrhoea, vomiting and low blood pressure due to fluid loss. In extreme cases, even intestinal paralysis and intestinal obstruction are possible.The most feared complication of HAE is swelling in the larynx (laryngeal edema) or upper respiratory tract, which can cause death by suffocation if left untreated.
How often do episodes of HAE occur?
The occurrence of episodes of HAE does not follow a typical pattern. As a result, it is impossible to predict when the next episode of edema will occur. The number and severity of episodes vary considerably.Most people with HAE have an average of one episode per month. However, there are also patients who have weekly episodes of angioedema and patients who only have two episodes per year.
It is also possible to have symptom-free periods lasting several years.
How long does an episode of HAE last?
The edema is often preceded by a feeling of tension in the affected area of skin and/or a circular reddening (so called erythema marginatum). The swelling starts 30 minutes to several hours later and continues to develop over the course of 12-36 hours. After around 2-5 days, the swelling subsides spontaneously.Just one episode per month prevents the patient from having a social and professional life for 20-100 days of the year, as even painless swelling of the skin is often disfiguring or can considerably reduce mobility.
Why can certain episodes of HAE be life-threatening?
If an episode of HAE involves swelling of the mucous membranes of the larynx or tongue, this can cause narrowing of the upper respiratory tract. It is potentially life-threatening and if appropriate treatment is not administered, it is estimated that a third of these patients die from suffociation. If the mucous membranes in the larynx starts to swell, immediate medical treatment is required. It may be necessary to perform an emergency incision in the windpipe (cricothyreotomy).| Frequently inquired |
Author: Jerini AG
Source: HAE-NAetwork Status: 3/08 This FAQ is frequently updated. Please send us your comments and suggestions by e-mail. |
