Hereditary Angioedema is "a rare inherited form that differs from other types of angiodema. It is not triggered by an allergic reaction and involves deep tissue swelling and is never associated with hives. Swelling in the gastrointestinal tract can led to severe pain in the abdomen and can be mistaken for appendicitis. Hereditary angiodema can be detected through blood tests and can be effectively treated with special hormone medications." Reference here.
This rare disorder results from genetic deficiency of the blood-based protein C1 inhibitor. Check out the table to determine the kinds of angioedema.
There are a number of associations for people who suffer from this disorder. Check out the United States Hereditary Angioedema Association and the Canadian Hereditary Angioedema Society
The FDA is once again in the crosshairs. Dr Kondrad Bork addresses a letter to Journal of the American Medical Association calling on the FDA to "expedite approval of the promising therapies being tested in the United States. It is high time for the Food and Drug Administration to adopt a more balanced and flexible approach to licensing pharmaceuticals targeted for rare diseases. How many more patients will die before FDA adopts regulatory practices that recognize the inherent risk of death faced by patients with rare, potentially fatal diseases like HAE"
Read the whole thing here.
One response to “Hereditary Angioedema”
This is a very interesting article. I am part of the HAE Association and want to invite you, if you are not alredy a member, to join the association. Membership is free. Just go to http://www.haea.org. Please let me know if I can be of an assistance.
Den