Treatment of acute attacks

Painless swelling affecting the arms, legs and trunk may not require treatment if they do not prevent work or school activities.

Swelling of the abdomen, face or airway should be treated promptly with intravenous injections of C1 Inhibitor concentrate or a subcutaneous injection of Icatibant.

Acute attack medications

C1 Inhibitor Concentrate – trade name: Berinert

Berinert is a concentrate of C1 Inhibitor. Berinert is a blood product which raises the level of C1 Inhibitor in the blood and halts the progress of an acute attack of oedema.

Berinert is given by slow intravenous injection, either in hospital by a medical professional, or at home if the patient has been trained in home therapy.

Berinert is licensed in the UK for use in adults and children.

Information about Berinert

C1 Inhibitor Concentrate – trade name: Cinryze

Cinryze is another plasma product that is licensed for use in the UK.

Information about Cinryze

Recombinant C1 Inhibitor (conestat alpha) – trade name: Ruconest

Ruconest is a recombinant C1 inhibitor which is derived from the milk of female rabbits that have been genetically altered to produce the human C1 Inhibitor protein.

Ruconest is given by slow intravenous injection. It is not suitable for patients who have a rabbit allergy.

Information about Ruconest

Icatibant – trade name: Firazyr

Icatibant is produced as a synthetic protein (a non blood product), and it is effective in halting an attack of oedema.

Icatibant is given by subcutaneous injection (an injection into the tissues just below the skin).

Icatibant comes in a pre-filled 3 ml syringe ready for injection. More than one syringe may be required to fully resolve attacks.

Icatibant is licensed for adults for administration by a medical professional or at home if the patient has been trained in home therapy.

Information about Firazyr

Download our patient information booklet

HAE patients should carry a letter from their Specialist stating the nature of their disease, the recommended treatment and a telephone number where the Specialist can be contacted.

All HAE patients should be offered home possession of a therapeutic dose of C1 Inhibitor concentrate or Icatibant to take to hospital in the case of a severe abdominal or laryngeal attack.

Short term prevention of acute attacks

It is important to discuss management of possible precipitating factors with your specialist.

Danazol or Oxandrolone or C1 Inhibitor concentrate may be used to prevent an attack.

If a person with HAE or AAE requires surgical procedures (including dental treatment) actions should be taken to prevent a possible attack.

The person should receive an infusion of concentrate prior to surgery. There will be a slight increase in the risk of swelling in the 3 days following surgery. This should be treated with C1 Inhibitor or Icatibant in the usual way.

Long term preventative treatment

The frequency and severity of attacks of angioedema can be reduced by attenuated androgens such as Danazol and Oxandrolone, or by Tranexamic Acid. Your doctor will discuss these treatments with you.

Preventative Medications – drugs used to reduce the incidence and severity of an attack

Danazol

Danazol may be used to reduce the level of HAE attacks. However, Danazol is an attenuated androgen which may cause unacceptable side effects, particularly in females. It is important to keep the doses of the medication as low as possible, and side effects should be reported as they are usually reversible if danazol is stopped promptly.

Possible side effects of danazol

Oxandrolone

Oxandrolone is also an attenuated androgen that is used to reduce the level of HAE attacks. Oxandrolone may be helpful even if Danazol has not been effective, or if Danazol has not been well tolerated.

Attenuated androgens, if used wisely, can be well tolerated even in women, and can improve quality of life for HAE patients.

Attenuated androgens should not be used in pregnancy or for children.

Tranexamic Acid

Tranexamic Acid can be used in children and adults in an effort to reduce the number of attacks of angioedema.

Even if prophylactic medications are used, patients may still get a severe breakthrough attack, so it is important to always have an emergency plan in place.

Maintenance therapy using C1 Inhibitor concentrate

Occasionally regular injections of C1 Inhibitor may be needed as a preventative measure.

The use of regular injections of C1 Inhibitor may be recommended in:

Cases of frequent severe attacks of angioedema where attenuated androgens are insufficient or unacceptable
In frequent severe attacks in pregnancy
In severe HAE in children

Home therapy for HAE patients

Suitable patients who get frequent severe attacks of HAE will be offered training to give their acute attack medications at home.

See Clinical Commissioning Policy: Treatment of Acute Attacks in Hereditary Angioedema. This provision for home therapy applies to both adults and children.

2013 Clinical Commissioning Policy: Treatment of Acute Attacks in Hereditary Angioedema

2014 UK Revised Consensus Guidelines for the Management of Hereditary Angioedema