Treatment of an Acute Attack

​Available options for treatment of acute attack are:

• C1 inhibitor concentrate (plasma derived C1INH)
• Recombinant human C1 inhibitor
• Ecallantide, a drug that prevents the production of bradykynin
• Icatibant, which blocks bradykinin’s receptor to prevent swelling
• Human plasma, either solvent/detergent-treated plasma or fresh frozen plasma

​It is important to have a plan to ensure appropriate care and to make sure you are prepared in case of emergency:

• The patient should wear a medical information bracelet that identifies their condition.
• The patient should carry a form or ID card that gives some information about their condition and treatment options in case of acute attack.
• The patient should communicate with the health care providers to discuss the condition and ensure the availability of needed therapy.
• The patient should share their emergency plan with family, friends and colleagues.

​Laryngeal Attack

This is the most serious type and requires early recognition and treatment to prevent the progression to full airway obstruction and death.

Early intubation to maintain the patency of airways is very important and should be done immediately when any sign of laryngeal swelling is detected. This includes any change in voice or noisy breathing.

After intubation, the first line drugs are C1 inhibitor concentrate, ecallantide or icatibant.

Personal Emergency Plan

It is important to have a plan to ensure appropriate care and to make sure you are prepared in case of emergency:

• The patient should wear a medical information bracelet that identifies their condition.
• The patient should carry a form or ID card that gives some information about their condition and treatment options in case of acute attack.
• The patient should communicate with the health care providers to discuss the condition and ensure the availability of needed therapy.
• The patient should share their emergency plan with family, friends and colleagues.
  

Some of the drugs required in an emergency can be self-administered at home. The patient should discuss this with their physician as the decision to prescribe therapies for acute treatment at home must be individualized. The clinician will consider each patient's situation, history of attacks, proximity to care, ability to self-administer medications, and preferences before recommended the medication that can be taken home.