Background
Hereditary Angioedema (HAE) is a rare disease characterized by recurrent angioedema attacks involving larynx, abdomen, extremities and various body parts. The reactions are by and large self-limited, but potentially, could be fatal. Until recently, the only approved treatment in Canada is an intravenous C1-esterase inhibitor infusion. However, intravenous therapy can be challenging for those who have co-morbid disorders. Icatibant (Firazyr®) —which received approval in Canada in June 2014 — offers administration through subcutaneous delivery. Through a special access program, here we present self-administered icatibant treatment on a female subject with Charcot-Marie-Tooth disease, a rare genetic, neuromuscular disorder, which limits her ability to self-administer intravenous therapy.
Methods
During each icatibant self-administration event, a diary method was used to collect the following patient-reported outcomes: attack intensity, anatomical location & trigger, number of doses, onset of relief, time elapsed until complete resolution, and adverse reactions.
Anzeige
Results
From 2012- May 2014, the patient logged a total of 12 events, in which she treated each attack with a single self-administered 30 mg dose of icatibant via subcutaneous injection. She experienced moderate to severe abdominal and peripheral HAE attacks. Onset of relief occurred within 15 – 30 minutes and complete resolution occurred within 4-hours to 5-days. Adverse reactions were mild in severity, transient, and resolved without further intervention. They included local injection site reaction (100%), headache (58%), fatigue (25%), feeling “fuzzy-brained” (25%), and hot flush (8%).
Conclusion
This case report provides supporting evidence for icatibant as an effective, safe and viable subcutaneous therapeutic alternative to intravenous treatments for patients with HAE.
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.