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Treating hereditary angioedema (HAE)

Treating hereditary angioedema (HAE)

Treating acute attacks

Although HAE does not currently have a cure, there are three treatment options for attacks (also known as ‘acute attacks’) of HAE:

C1 esterase inhibitor

C1 esterase inhibitor

C1 esterase inhibitor (C1-INH) therapy works by replacing the missing or malfunctioning C1-INH protein in patients with a C1-INH deficiency. C1-INH for acute attacks is given when an attack occurs, ideally at the first sign of the attack (when prodromal symptoms occur – see Recognising the signs of an HAE attack).

Bradykinin inhibitor

Bradykinin inhibitor

Bradykinin inhibitor directly blocks bradykinin, a protein thought to be responsible for HAE symptoms. Bradykinin inhibitor for acute attacks is given when an attack occurs, ideally at the first sign of the attack.

Recombinant C1 esterase inhibitor

Recombinant C1 esterase inhibitor

Recombinant C1 esterase inhibitor (rhC1INH) is a recombinant (i.e. not made from human plasma) version of the C1 esterase inhibitor. rhC1INH for acute attacks is given when an attack occurs, ideally at the first sign of the attack.

You should work with your doctor to choose the best treatment plan for you.

Acute laryngeal oedema

An acute laryngeal oedema attack is a swelling attack of the larynx, or voice box. These can be particularly concerning as they can sometimes restrict the ability to breathe.

If this happens, intensive support may be necessary to control the attack and protect the airways. C1-INH, bradykinin inhibitor and rhC1INH may be administered for this type of attack, but if the laryngeal oedema has progressed rapidly, a tracheotomy may have to be performed to allow oxygen to flow into the lungs. A tracheotomy is an incision through the neck into the windpipe that is made when the upper airway is blocked.

Emergencies that require immediate medical treatment

Some HAE attacks can be extremely serious.

The following symptoms can indicate a situation that may require immediate medical attention:

Throat swelling that blocks the airway and prevents normal breathing is potentially life-threatening, and therefore requires immediate emergency care. Some of the symptoms of throat swelling may include a hoarse voice or laryngitis, difficulty in swallowing, throat tightness, and other voice changes.

This type of attack should be treated at the nearest A&E department.

Intestinal swelling which can cause intense abdominal pain that can progress to vomiting and diarrhoea; intestinal swelling can be mistaken for an abdominal disorder, such as appendicitis, as the symptoms can be similar, which makes diagnosis difficult.

Call 999 immediately if you notice any of the following symptoms:

  • Hoarse voice or laryngitis
  • Whistling or wheezing when breathing
  • Shortness of breath
  • Swollen tongue
  • Lip or facial swelling
  • Throat swelling

Reporting side effects of medication

If you get any side effects, talk to your doctor, pharmacist or nurse. This includes any possible side effects not listed in the package leaflet. You can also report side effects directly via the Yellow Card Scheme at yellowcard.mhra.gov.uk.

By reporting side effects you can help provide more information on the safety of your medicine.

Other useful resources