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Hereditary angioedema (HAE)

HAE causes swellings (angioedema) affecting any part of the body, but typically hands and feet, face, stomach and occasionally the throat. It is called hereditary because it runs in families, passed in the genes from one generation to the next.

What are the common causes of HAE?

A common cause of HAE is a defect in the gene for C1 inhibitor. This is an enzyme which helps to control swellings, so when it is missing swellings are more likely to happen. Other genes have also been found that cause HAE.

A much more common cause of such swellings is spontaneous angioedema. This can happen to anyone, and is often mistaken for an allergic reaction. Spontaneous angioedema usually responds well to antihistamines, steroids and sometimes adrenaline; HAE swellings do not. The level of Complement C4 in the blood is often low in an attack of HAE, which is useful clue.

What treatments are available for HAE?

The treatment of Hereditary Angioedema (HAE) has transformed in recent years. Previously patients often suffered disabling attacks of swelling or abdominal pain without seeking medical help. When they did go to the Emergency Department they often experienced difficulty getting the right  treatment.

Self-treatment of HAE attacks

A range of treatment options is available for treating attacks of HAE, and these can all be given at home. We discuss the treatment options with the patient and family. Once the most suitable treatment is agreed, we can train patients and carers to administer

  • C1 inhibitor injections
  • icatibant injections
  • Oral treatments

After training a patient (or carer) how to manage HAE attacks we monitor how these treatments are going, and provide support and advice, and further training if needed. We also prescribe the medications and can arrange for their delivery to the home.

Prevention of HAE attacks

An increasing number of medications is available for HAE patients who suffer from frequent attacks. These include:

  • C1 inhibitor injections
  • Danazol tablets
  • Tranexamic acid tablets
  • Berotralstat capsules
  • Lanadelumab injections

Contact the specialist nursing team for further advice about this service, and see the external website listed below for more information about treatments.

Last reviewed: 19 May 2023