Welcome to HAE UK on line support for all UK patients with HAE – Hereditary Angioedema
Hereditary Angioedema is a rare but potentially life-threatening inherited condition. HAE symptoms include episodes of oedema (swelling) in various body parts including the hands, feet, face and airway.
In addition, patients often have bouts of excruciating abdominal pain, nausea and vomiting that is caused by swelling in the intestinal wall. Airway swelling is particularly dangerous and can lead to death by asphyxiation.
HAE patients frequently spend a long time before they are correctly diagnosed. They are often referred to allergy, yet their swellings will not respond to antihistamine or steroids. Some of them with abdominal swelling are subjected to unnecessary exploratory procedures even going so far as to have appendectomies or bowel surgery; some are even considered to have psychiatric problems (it is no coincidence that Hereditary Angioedema used to be called ‘Angio-Neurotic Oedema’). One of the diagnostic features is family history; prior to the condition being identified, about 30% of patients died from lack of treatment.
And yet treatment is now available, is easily administered and is relatively inexpensive. Patients with Hereditary Angioedema either lack a regulatory protein called ‘C1-Inhibitor’ or their C1-Inhibitor has very low activity. Most patients have a family history, but some are spontaneous mutations. The problem is, these mutations can be passed on to offspring.
C1-inhibitor can either be replaced directly, by using a C1-Inhibitor concentrate; or the process that it controls can be interrupted by use of a bradykinin antagonist.
There are some more complicated conditions that are very similar to typical HAE, but are classed as ‘HAE Type III’; identification of this subtype is increasing exponentially. There is also Acquired Angioedema – AAE – who are people who have angioedema as a side effect from treatment for other illnesses.
Welcome to our HAE Family
HAE UK wishes to expand the Trustees Board by making further appointments to it. Anyone who is interested in becoming a Trustee should apply to The Trustees of HAE UK c/o Rachel Annals: email@example.com with a brief description of their experience (e.g. diagnosed with HAE, family member) and what skills and experience they feel they can bring to the role of Trustee, noting that experience in healthcare, business and/or work with other charities would be really helpful. All applications to be received by 25th February.
A new clinical trial for HAE will begin soon. Please see the news page for more information.
Some of you may have seen and been alarmed by postings on Facebook concerning the care of HAE patients at the Royal London Hospital. We hope this statement from Professor Finbarr Cotter, Clinical Director, will address your concerns and reassure you about patient care at the Royal London Hospital.
Dr Stephen Hughes is running a study for Shire, please see the news page for more details.