title: Self-administration of intravenous C1-inhibitor therapy for hereditary angioedema and associated quality of life benefits.
abstract: 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.
authors.affiliation: Department of Dermatology, Odense University Hospital, 5000 Odense C, Denmark. anette.bygum@ouh.regionsyddanmark.dk
authors.forename: Anette
authors.initials: A
authors.lastname: Bygum
authors.forename: Klaus Ejner
authors.initials: KE
authors.lastname: Andersen
authors.forename: Carsten Sauer
authors.initials: CS
authors.lastname: Mikkelsen
mesh_terms: D000328:Adult; D054179:Angioedemas, Hereditary / Q000188:drug therapy*; D050718:Complement C1 Inhibitor Protein / Q000008:administration & dosage*; D051056:Complement Inactivating Agents / Q000008:administration & dosage*; D016001:Confidence Intervals; D005260:Female; D006801:Humans; D007275:Injections, Intravenous; D008297:Male; D011788:Quality of Life; D012646:Self Administration; D011795:Surveys and Questionnaires; D016896:Treatment Outcome
chemical_list: D050718:Complement C1 Inhibitor Protein; D051056:Complement Inactivating Agents