HAE: a rare and life-threatening disease1

About HAE

Hereditary angioedema (HAE) is a genetic disorder characterized by recurrent attacks of angioedema without urticaria or pruritus.2

  • The severity and frequency of HAE attacks vary greatly from patient to patient and can also change over time.2,3
  • When untreated, the swelling of an HAE attack typically increases over a 24-hour period and then gradually subsides during the next 48 to 72 hours.2
  • Attacks can often occur without warning, but some triggers may include stress, physical trauma, surgery, a dental procedure, infection, hormonal influence, or mechanical pressure.3-7

HAE attacks typically cause swelling in these main sites1,2,5:

  • Skin/subcutaneous tissues
  • Gastrointestinal tract
  • Upper airways

Warning Signs

Many patients may experience prodromal signs before an HAE attack, including2:

  • A tingling sensation2
  • Erythema marginatum, a mild, nonpruritic rash2,8
  • Fatigue8
  • Nausea8

Location of HAE Attacks

Skin/Subcutaneous Tissue

  • Typically affects the face, extremities, and genitals7
Example of a HAE attack affecting the Skin/Subcutaneous Tissue

Gastrointestinal Tract

  • May present with mild to severe abdominal pain accompanied by vomiting and/or diarrhea7
  • Untreated abdominal attacks may require, on average, between 24 and 50 hours of bed rest9,a

aBased on a survey of 23 patients.

Example of a HAE attack affecting the gastrointestinal tract
After a HAE abdominal attack

Upper Airway, Including Larynx and Oropharnyx

  • In the past, untreated laryngeal attacks resulted in mortality rates up to 30%2
  • 50% of patients experience at least 1 laryngeal episode during their lifetime7,10,b
  • Can cause death by asphyxiation2

bBased on a survey of 209 patients.

Example of a HAE attack affecting the upper airway

Images obtained from www.haeimages.com

HAE Treatment Guidelines

The WAO/EAACI* guidelines outline recommendations for the treatment of HAE attacks. Current guidelines recommend that all patients should have an acute therapy as a part of their treatment plan. Patients should also be evaluated for long-term preventive treatment at every doctor's visit, or at least once a year. It is important to discuss the frequency and severity of your patient's attacks when developing their treatment plan.11

*World Allergy Organization/European Academy of Allergy and Clinical Immunology

Important Safety Information

WARNING: Anaphylaxis

  • Anaphylaxis has been reported after administration of KALBITOR.
  • Because of the risk of anaphylaxis, KALBITOR should only be administered by a healthcare professional with appropriate medical support to manage anaphylaxis and HAE.
  • Healthcare professionals should be aware of similarity of symptoms between hyper sensitivity reactions and HAE patients should be monitored closely.
  • Do not administer KALBITOR to patients with known clinical hypersensitivity to KALBITOR.

Do not administer KALBITOR to a patient who has known clinical hypersensitivity to KALBITOR

WARNINGS AND PRECAUTIONS: Hypersensitivity Reactions, Including Anaphylaxis
Potentially serious hypersensitivity reactions, including anaphylaxis, have occurred in patients treated with KALBITOR. In 255 HAE patients treated with intravenous or subcutaneous KALBITOR in clinical trials, 10 patients (4%) experienced anaphylaxis. For the subgroup of 187 patients treated with subcutaneous KALBITOR, 5 patients (3%) experienced anaphylaxis. These reactions occurred within the first hour after dosing.

Symptoms associated with hypersensitivity reactions have included chest discomfort, flushing, pharyngeal edema, pruritus, rhinorrhea, sneezing, nasal congestion, throat irritation, urticaria, wheezing, and hypotension.

Other adverse reactions indicative of hypersensitivity reactions included the following: pruritus (5%), rash (3%), and urticaria (2%).

Patients should be observed for an appropriate period of time after administration of KALBITOR, taking into account the time to onset of anaphylaxis seen in clinical trials. Given the similarity in hypersensitivity symptoms and acute HAE symptoms, patients should be monitored closely in the event of a hypersensitivity reaction.

The most common adverse reactions (≥3% and greater than placebo) in HAE patients were headache (8%), nausea (5%), diarrhea (4%), pyrexia (4%), injection site reactions (3%), and nasopharyngitis (3%).

There is a potential for immunogenicity with the use of KALBITOR. Overall, 20.2% of patients seroconverted to anti-ecallantide antibodies. Patients who seroconvert may be at a higher risk of a hypersensitivity reaction. The long-term effects of antibodies to KALBITOR are not known.

Please see the Full Prescribing Information, including Boxed Warning.

  1. Cicardi M, Bork K, Caballero T, et al, on behalf of HAWK (Hereditary Angioedema International Working Group). Evidence-based recommendations for the therapeutic management of angioedema owing to hereditary C1 inhibitor deficiency: consensus report of an International Working Group. Allergy. 2012;67(2):147-157.
  2. Zuraw BL. Hereditary angioedema. N Engl J Med. 2008;359(10):1027-1036.
  3. Zuraw BL, Banerji A, Bernstein JA, et al. US Hereditary Angioedema Association Medical Advisory Board 2013 recommendations for the management of hereditary angioedema due to C1 inhibitor deficiency. J Allergy Clin Immunol Pract. 2013;1(5):458-467.
  4. Longhurst HJ, Bork K. Hereditary angioedema: causes, manifestations, and treatment. Br J Hosp Med. 2006;67(12):654-657.
  5. Banerji A. The burden of illness in patients with hereditary angioedema. Ann Allergy Asthma Immunol. 2013;111(5):329-336.
  6. Nielsen EW, Gran JT, Straume B, et al. Hereditary angio-oedema: new clinical observations and autoimmune screening, complement and kallikrein-kinin analyses. J Intern Med. 1996;239(2):119-130.
  7. Agostoni A, Aygören-Pürsün E, Binkley KE, et al. Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond. J Allergy Clin Immunol. 2004;114(3 supple):S51-S131.
  8. Lang DM, Alberer W, Bernstein JA, et al. International consensus on hereditary and acquired angioedema. Ann Allergy Asthma Immunol. 2012;109(6):395-402.
  9. Bork K, Staubach P, Eckardt AJ, et al. Symptoms, course, and complications of abdominal attacks in hereditary angioedema due to C1 inhibitor deficiency. Am J Gastroenterol. 2006;101(3):619-627.
  10. Bork K, Meng G, Staubach P, et al. Hereditary angioedema: new findings concerning symptoms, affected organs, and course. Am J Med. 2006;119(3):267-274.
  11. Maurer M, Magerl M, Ansotegui I, et al. The international WAO/EAACI guideline for the management of hereditary angioedema - the 2017 revision and update. Allergy. 2018;73(8):1575-1596.