HAE

What is Hereditary Angioedema?

Hereditary angioedema is a genetic disorder that causes sufferers to experience skin swelling, along with lung and gut linings becoming swollen too. The swelling may also happen elsewhere in the body, and if that is in the throat it creates a potentially more dangerous situation with airway constriction. For most people, angioedema will occur in bouts, and after them the swelling subsides for prolonged periods of time. These bouts of hereditary angioedema can be as often as every one or two weeks, or as infrequently as less than five times a year.

Signs of the disorder usually first appear for people when they are in their teenage years, and then these symptoms become more pronounced as they move into adulthood. Hereditary angioedema cannot be cured, but it is a manageable disorder.

What Causes Hereditary Angioedema?

Hereditary angioedema is the result of a faulty C1 inhibitor gene and a blood protein in the body being imbalanced because of it. Insufficient blood protein is what causes hereditary angioedema in most instances. It may also be possible that there is enough of the protein, but it isn’t working as it would for people with normal blood composition.

There is often a genetic predisposition for having hereditary angioedema. If one of your parents has had this disorder then it is 50% more likely you’ll develop it too. This is why it is referred to as hereditary angioedema. It is uncommon for people who don’t have it running in their families.

Hereditary Angioedema Symptoms

Excessive swelling of skin or tissue is what is going to be most problematic for people with hereditary angioedema, and usually occurring in the mouth or throat, feet, hands, face, genitals, or belly. Belly swelling can then promote pain, nausea, vomiting, or diarrhea.

Certain symptoms may present themselves before this swelling occurs. Sufferers may have fatigue, muscle ache, headache, tingling sensations in the skin, hoarseness, or be unable to control mood swings. Triggers for hereditary angioedema include stress and anxiety, surgery, illnesses, and physical exertion. Use of certain medications can worsen hereditary angioedema.

Hereditary Angioedema Treatment

The most conventional approach to hereditary angioedema treatment is to have the person on a medication like Orladeyo or Firazyr. Supplementary oxygen or IV fluids may be used to make hereditary angioedema less severe for people afflicted with it. Sufferers can also benefit from identifying triggers likely to cause bouts of the disorder and then avoiding them to the greatest extent possible.