what is hae - the clinical picture

Hereditary angioedema (HAE) is a rare genetic disorder characterized by episodes of swelling (edema attacks) in different areas of the skin or the internal organs. In particular, it occurs in the upper respiratory tract and intestine.(2,4)

Edema of the skin occurs mainly in the face (e.g. lips, eyelids), although the extremities and genitals are also often affected. In many cases, patients experience swelling and tightness of the skin or a tingling sensation, and in some cases pain. Although it is not life-threatening, people with this kind of edema often withdraw from their social lives because of the disfiguration caused.(4)

Edema of the mucous membranes of the larynx, nose or tongue is potentially life-threatening, as it can cause death by suffocation. Symptoms that are felt just before an attack in the throat may include difficulty swallowing, a hoarse voice, whistling or wheezing when breathing, a swollen tongue, cough, or shortness of breath. Patients who experience these symptoms and suspect the start of such an attack should seek emergency attention immediately. More than 50% of HAE patients will have at least one laryngeal edema in their lifetime.(4,8-10)

Abdominal pain is reported by 70-80% of patients with HAE.(2) This manifests itself primarily as loss of appetite, nausea, vomiting, diarrhea and abdominal cramps.(7) If HAE has not been diagnosed in these patients yet, suspicion of colic or appendicitis can lead to unnecessary surgical procedures or even to removal of healthy organs.(12)

It is estimated that about 1 in 10,000 to 1 in 50,000 people worldwide have HAE.(2,11) It occurs with roughly the same frequency in men and women.(14) Most patients experience their first attack during childhood or adolescence.(4)

The development of edema does not follow a typical pattern and therefore the site of the next episode of swelling cannot be predicted. The frequency, duration and severity of the edema vary considerably. Approximately a third of patients report more than one 1 attack per month; about 40% of patients experience on average 6-11 attacks per year and the remaining 22% only suffer attacks from time to time.(2) In most cases, the symptoms of HAE increase over 12 to 36 h and last for 2-5 days before resolving. However, some patients may experience attacks that last over a week.(2,7)

Most HAE attacks occur spontaneously, but patients can often link specific situations that occur in their lives, such as stress, infections or even the consumption of certain foods, to the development of edema.

The disorder places extraordinary strain on patients, often restricting their ability to lead normal lives. Untreated patients with HAE can lose 100 to 150 work days per year - if not more!

News

Thu, 2014-04-03

Dyax Receives FDA Approval to Expand Use of Kalbitor(R)

Thu, 2014-03-27

From the Shire 2013 Annual Report

Fri, 2014-03-14

Dyax Announces Pricing of $74 Million Public Offering of Common Stock

Tue, 2014-03-04

Patient Satisfaction and Convenience Increased with Current HAE Treatment Options, According to Study Findings

Mon, 2014-03-03

BioCryst Presents BCX4161 Phase 1 Trial and Kallikrein Inhibition Assay Results

Fri, 2014-02-28

More travel grants for HAE Global Conference 2014 now available

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Events

Fri, 2014-05-16 (All day)

2014 hae day :-)

On 16 May 2014 patient organizations throughout the globe will hold events, meetings, and fundraisers to support the global awareness day hae day :-)

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Thu, 2014-05-15 12:00 - Sun, 2014-05-18 12:00

2014 HAE Global Conference

Venue: Hyatt Regency Reston, 1800 Presidents St., Reston, VA 20190, United States of America
Language: English

HAEi - International Patient Organization for C1 Inhibitor Deficiencies cordially invites you to attend the 2014 HAE Global Conference which will take place in Washington DC, 15 - 18 May 2014.

Registration for the conference is now open! Registration will take place on a first come, first serve basis – and since we again offer some extremely attractive registration rates and conditions, please do not wait too long registering for this exciting conference.

Please register here

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HAE Global Community

Be part of the HAEi Community

HAEi is here to help you. Therefore we have created the HAEi Community that gives you all the bells and whistles you know from other social media (such as Facebook) and even a very sophisticated Forum, where you can discuss with fellow HAE patients and care givers. The only difference is - that here your information stays safe - and will only be shared with other HAE patients and care givers. If you are a HAE patient or directly related to a HAE patient we urge you to register today - and become an important member of our community

Help yourself and stay updated by registering already today - then you will be the first to know.....

Do you have Questions?
You can find answers on our FAQ-Page or
send us an e-mail to: info@haei.org
or go to the contact form page


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See the video from the 2012 HAE Global Conference in Copenhagen

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HAEDAYHAEDAYLook out for next hae day :-) on 16 May 2014


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