Bygum et al outlined instructions for self-administration of C1 esterase inhibitor (C1-INH) for a patient with hereditary angioedema (HAE). The authors are from Odense University Hospital in Denmark.


Indications for self-administration:

(1) angioedema of the face, mouth, pharynx, larynx and/or upper airway

(2) painful abdominal attack

(3) progressive limb swelling


Information given to the patient and family members:

(1) about hereditary angioedema

(2) about how to store and reconstitute C1-INH concentrate

(3) about how to work with needles and syringes safely

(4) about infection control and how to avoid infections

(5) about how to record each episode including signs and symptoms

(6) about potential allergic reactions



(1) The reconstituted solution is infused intravenously through a butterfly.

(2) The usual dose is 500 to 1,500 units.

(3) The person can self-administer the dose but it is usually done by a family member.

(4) The episode is recorded in a diary with indication, dose, and response.


Problem handling:

(1) when to call the physician (atypical attack, fever, etc)

(2) when to go to the Emergency Department (ED)

(3) when to administer epinephrine for an allergic reaction


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