FIRAZYR is indicated for the treatment of acute attacks of hereditary angioedema (HAE) in adults 18 years of age and older.

Diagnosing HAE

Hereditary angioedema (HAE) is so rare that many healthcare professionals have never seen a patient with HAE, which can lead to delayed diagnosis or misdiagnosis for some patients.1,2 In a 2017 survey of 445 patients with HAE, people with HAE on average experienced a delay of 8.4 years in getting an accurate diagnosis.3

Clinical presentation of HAE

HAE should be considered in patients with1:

  • History of recurrent angioedema without urticaria
  • Positive family history
  • Onset of symptoms in childhood or adolescence
  • Recurrent abdominal pain attacks
  • Occurrence of upper airway edema
  • Failure to respond to treatment with antihistamines, glucocorticoids, or epinephrine
  • Presence of prodromes

Confirming diagnosis

To confirm an HAE diagnosis, laboratory testing is necessary. Patients should have serum levels of C4 measured, as well as antigenic and functional C1 esterase inhibitor levels.2

Tests to Confirm HAE Types I and II5

Chart of tests to confirm HAE Types I and II.

Importance of diagnosis

Missed/delayed diagnosis can result in unnecessary treatments and surgery.2,6 The risk makes early, accurate diagnosis critical.2

Challenges associated with diagnosis

In a 2010 paper, patients saw an average of 4 different physicians before getting a diagnosis of HAE.