Once diagnosed, a treatment plan should be agreed with your consultant and care team.

Depending on the severity and frequency of your attacks, treatment could be:

  • On demand or rescue treatment which is only taken once a patient ‘feels’ an attack happening or has happened. Usually in the form of Icatibant which can also be used for children aged 2 years plus
  • C1-INH concentrate (Berinert, Cinryze, Ruconest) – The first two are human plasma derived, the third is a human plasma-free therapy. All three are powder which has to be reconstituted in order to be administered as a slow intravenous injection (into the vein). These medications act to replace the missing or non-functioning C1-INH and therefore controls not only the bradykinin production but also controls the entire Hereditary Angioedema cascade. Administration as early as possible in an attack will prevent swellings getting too large.
  • Prophylaxis – medication that is taken daily, weekly or sometimes monthly depending on attack frequency to prevent attacks happening. These are either plasma derived like C1-INH concentrate and administered by IV, or sub cutaneous injections or oral medications. Prophylaxis is the use of treatment to prevent attacks

Remember – Icatibant and C1-INH both act to stop the swelling, but they do not make swellings reduce, this will be done by the body’s own circulation. Therefore it is important to inject sooner rather than later!

There are NHS guidelines for management of severe attacks of Hereditary Angioedema and also for use of C1-INH treatments.

In addition to the above, there are currently several more medications in trial phase at the time of this leaflet being produced (March 2024). Gene therapy treatments are also in final stages of clinical trial though unlikely to be available in the UK for several years and may not be suitable for all patients.

Whilst there is currently no cure for HAE there are many medications now available which enable patients to live a normal life with attacks managed, reduced or even prevented altogether. The future is looking bright for achieving the ultimate goal for all patients to have zero attacks.

Treatment in children

Some medications are currently suitable for children aged over 2 but most become useable from 12 years old onwards. The ‘old’ treatments of Androgens such as Danazol may still be used in Under 12s but due to some possible side effects they are being discouraged.

A diagnosis of HAE does not mean you cannot lead a good quality of life. Good planning and sensible precautions will enable you to live life to the full.

On demand or acute treatment

Info to follow
Ruconest, Icatibant, Berinert

Long term prophylactic treatment

Info to follow
Cinryze, Berinert, Lanadelumab, Berotralstat