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

Wed, 2014-07-23

Dyax Corp. Announces Publication of Scientific Data for DX-2930 in Peer-Reviewed Journals

Wed, 2014-07-23

Recommended combination of Shire and AbbVie

Fri, 2014-07-18

Recommended combination of Shire and AbbVie

Fri, 2014-07-18

First Subcutaneous Treatment Now Available in Canada

Thu, 2014-07-17

FDA approves Ruconest for HAE treatment in the USA

Thu, 2014-07-10

July 2014 newsletter out now

Newsletter Teaser ( In News Section on Homepage )

Newsletter

Make sure to stay updated. HAEi will keep you informed when you sign up for our newsletter.

Sign up for HAEi Newsletter and register at the same time.


Join our Facebook group - and follow HAEi on Twitter

Facebook logo SFacebook logo STwitter logo STwitter logo S

  

Events

Thu, 2014-11-06 09:00 - Mon, 2014-11-10 16:00

ACAAI - 2014 Annual Scientific Meeting

Venue: Georgia World Congress Convention Center, Atlanta, GA, United States of America
Language: English

Plan to join your colleagues at the 71st ACAAI Annual Scientific Meeting in Atlanta, Nov. 6-10. Broaden your expertise, enhance your practice management skills and improve patient care.

For more information please visit: ACAAI Annual Meeting 2014

..continue
Fri, 2014-10-24 13:00 - Sun, 2014-10-26 12:30

International scientific conference on Expanding Boundaries of our HAE Knowledge

Venue: Bethesda Marriott, 5151 Pools Hill Road, Bethesda, MD 20814, USA
Language: English

This first international scientific meeting on HAE, sponsored by National Institutes of Health in conjunction with the US HAEA Angioedema Center at UCSD, will bring together experts in all types of angioedema, as well as clinical medicine to discuss the emerging basic science.

Read more about the conference here

..continue
Wed, 2014-10-15 12:00 - Thu, 2014-10-16 13:00

Bradykinin Symposium

Venue: Kaiserin Friedrich-Haus, Lecture Hall, Robert-Koch-Platz 7, 10115 Berlin, Germany
Language: English

Welcome to this year's Bradykinin Symposium hosted by the Charité and icare on 15-16 October 2014 in Berlin. International experts in the field of bradykinin have been invited to discuss the role of bradykinin in present and in the future.

The symposium will be complemented by the Charité/UNEV/icare/GA²LEN Angioedema School on 16-17 October 2014.

For more information please visit: Bradykinin Symposium 2014

..continue

Homepage Teaser 6, 2nd row right

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


Experiencing browser problems?

Our website is working properly on most web browsers. However, we know that the site will not perform correctly on Microsoft Internet Explorer version 6 and earlier! So if you are using Internet Explorer from Microsoft please ensure that you have version 7 or higher!


See the video from the 2012 HAE Global Conference in Copenhagen

Click here


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


www.haeday.org