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Self-administration of intravenous C1-inhibitor therapy for hereditary angioedema and associated quality of life benefits


European Journal of Dermatology. Volume 19, Number 2, 147-51, March-April 2009, Therapy

Free Article  

Author(s) : Anette Bygum, Klaus Ejner Andersen, Carsten Sauer Mikkelsen

Summary : Hereditary angioedema (HAE) is often debilitating with a serious effect on quality of life (QOL). Treatment of acute HAE attacks is usually with C1 esterase inhibitor (C1-INH) concentrates\; however, treatment can be delayed by patients’ travel time for attending emergency units. We assessed the impact of self-administered home therapy with intravenous C1-INH concentrate on QOL in patients with HAE. Nine patients experiencing frequent or severe debilitating HAE attacks were offered self-administration of C1-INH concentrate. QOL was assessed prior to and following home therapy using the Dermatology Life Quality Index (DLQI) and 36-Item Short Form Survey (SF-36) questionnaires. Seven patients were recruited into the study. QOL was assessed at baseline and after 3 to 48 months of home therapy. The mean DLQI score fell from 12.6 ± 4.65 to 2.7 ± 1.38 (P <\; 0.001). Mean SF-36 scores for the individual and combined components also improved significantly. No serious complications were documented during a follow-up period of 27 to 72 months. Self-administration of C1-INH improved QOL on both physical and psychological parameters. Patients were able to resume a normal life without restrictions caused by the condition.

Keywords : hereditary angioedema, C1-INH concentrate, Berinert P, home therapy, quality of life, self-administration

 

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