Treatment options

Unfortunately, in many countries there are currently no acute attack treatments available for HAE patients. In those countries physicians are therefore limited to providing patients with a short and long term prophylactic treatment with attenuated androgens (such as Danazol and Oxandrolone) and in some cases tranexamic acid (such as Cyklokapron).

An overview of treatment options

Until now, there is neither a cure for patients who suffer from HAE attacks nor a therapeutic concept to prevent these attacks completely.

Unlike allergic angioedema, HAE attacks do not respond to treatment with anti-histamines, corticosteroids or epinephrine.24 Current HAE treatment options focus on providing rapid relief during attacks or on the prevention of symptoms in patients who experience a high frequency of attacks or who undergo dental or surgical procedures which may trigger an attack.8

Acute treatment

The aim of acute treatment is to halt the progression of the edema and alleviate the symptoms. This applies particularly to episodes affecting the larynx, which can cause death by suffocation if left untreated.

The recommended options for acute treatment vary from country to country due to the fact that drugs for specific treatment are not licensed in all countries. In these cases acute treatment may be limited to more unspecific drugs such as tranexamic acid or even just pain killers.

In countries where it is available, C1-INH concentrate, Icatibant or Ecallantide can be used for the treatment of acute attacks. Icatibant and Ecallantide must be administered by subcutaneous injection by a healthcare professional, C1-INH concentrate must be administered intravenously.

Long-term prophylaxis

Long-term prophylaxis is given to patients whose quality of life is clearly reduced by the disease. These are usually patients who have either very frequent or very painful attacks or are at high risk of developing laryngeal edema.

Long-term prophylaxis consists mainly of attenuated androgens, synthetically produced derivatives of the male sex hormone testosterone. They can reduce the number of attacks. Because these medications are associated with a range of severe side-effects, attenuated androgens are generally reserved for patients suffering from frequent and/or severe symptoms. In the US, long-term prophylaxis with C1-INH concentrate has recently been approved.

In some countries, antifibrinolytic drugs such as tranexamic acid or aminocaproic acid are used as alternative to androgens.

Short-term prophylaxis

Short-term preventative therapy is recommended for patients undergoing dental procedures or surgery, which have been known to trigger an attack. One option for short-term prophylaxis consists of high dose androgen therapy for at least 5 days prior to surgery and 4 days afterwards. Where available, another option is to administer C1-INH concentrate approx. 1-2 hours prior to surgery.

Treatment of Acute Attacks

As might be expected from ist different pathogenesis, angioedema seen in HAE does not respond to the drugs employed in treating other forms of urticaria/angioedema such as antihistamines, epinephrine and corticosteroids. While epinephrine, in particular, may have a transient effect on swelling, it does not alter the course of an attack.

Maintaining airway patency is the primary concern for patients with laryngeal edema. If the airway is threatened, the patient should be intubated by an experienced physician. In addition, the capability for emergency tracheotomy should be readily available. Because gastrointestinal edema usually involves excruciating pain, frequent vomiting and the potential for hypotension, therapy should include aggressive fluid replacement and pain management. Clinicians report that Zofran, Compazine and Phenergan are effective in reducing nausea and vomiting, while morphine or other narcotics are routinely used to relieve attack related abdominal pain. Some physicians use fresh frozen plasma in the acute attack setting, but this therapy is considered controversial because in addition to C1-inhibitor, fresh frozen plasma contains substrates of the complement and kinin systems that could produce a vasoactive peptide and cause an attack exacerbation.

HAE Clinical Trials Offer Hope for Better Therapeutic Alternatives

Six pharmaceutical companies are currently conducting or have recently concluded their HAE clinical trials. There will be continuously activities with each of the below mentioned pharmaceutical companies. Both with regards to clinical trials and to registration and licensing in new countries.

Shire Human Genetic Therapies (HGT) - B2 Bradykinin Receptor Antagonist - Icatibant (Trade name: Firazyr)

CSL Behring - C1-Inhibitor Concentrate (Trade name: Berinert)

Viropharma - C1-Inhibitor Concentrate (Trade name: Cinryze)

Sanquin - C1-Inhibitor Concentrate (Trade name: Cetor)

Dyax Corp. - Kallikrein Inhibitor - Ecallantide (DX-88) (Trade name: Kalbitor)

Pharming NV - Recombinant C1-Inhibítor Concentrate (Trade name: Ruconest/Rhucin)


Tue, 2015-03-31

Dyax Announces Positive Results from Phase 1b Clinical Trial of DX-2930

Fri, 2015-03-27

New hae day :-) website

Thu, 2015-03-19

Nominations to Pharming's Board Of Supervisory Directors

Fri, 2015-03-06

OPuS-2 study now enrolling patients

Thu, 2015-03-05

Pharming Reports On Preliminary Financial Results 2014

Thu, 2015-02-26

ISIS Pharmaceuticals Reports Positive Phase 1 Data

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Thu, 2015-05-28 13:00 - Sun, 2015-05-31 14:00

9th C1 Inhibitor Deficiency Workshop

Venue: Danubius Thermal Hotel Margitsziget, Margitsziget, 1133 Budapest, Hungary
Language: English

It is our great pleasure to invite you to the 9th C1-INH Deficiency Workshop. This event has a history of many years, as well as its nature and objectives are familiar to many of us. Nevertheless, given the continued interest from first-time attendees, we would like to introduce the Workshop for them in brief, as follows.

This conference focuses on bradykinin-mediated angioedemas, and particularly on the types resulting from C1-inhibitor deficiency. The topics of this four-day long event cover a wide range of subjects.

Read more about the workshop here.

Fri, 2015-02-20 10:00 - Tue, 2015-02-24 16:00

AAAAI - Annual Meeting 2015

Venue: Houston, TX, United States of America
Language: English

Mark your calendars and head to Houston, Texas for the premier event in allergy/immunology. You will be able to earn CME/CE, learn about cutting-edge research and enrich your networking connections at the 2015 AAAAI Annual Meeting, February 20-24.

For more information please visit: AAAAI 2015

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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.....

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