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Development and validation of an epidermolysis bullosa family/parental burden score
Author(s) -
Dufresne H.,
HadjRabia S.,
Taieb C.,
Bodemer C.
Publication year - 2015
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1111/bjd.14072
Subject(s) - epidermolysis bullosa , medicine , dermatology , library science , computer science
Summary Background The notion of the individual burden associated with a disease has been introduced to determine ‘disability’ in the broadest sense: psychological, social, economic and physical. Subtypes of epidermolysis bullosa ( EB ) are rare, life‐threatening, untreatable chronic genodermatoses. Objectives To develop and validate a specific questionnaire assessing the burden on families of children with EB : Epidermolysis Bullosa Burden of Disease ( EB ‐BoD). Methods Items were generated by a verbatim report from parents of children with EB . Subsequently, a study was implemented for psychometric analysis. An epidermolysis bullosa burden‐of‐disease questionnaire was refined via item reduction according to inter‐question correlations, consensus among experts and exploratory factor analysis. Internal consistency was determined by calculating Cronbach's α. Concurrent validity was determined by calculating the correlation between EB ‐BoD and the Short‐Form 12 items ( SF ‐12) questionnaire. Results From a primary list of 30 items, EB ‐BoD was reduced to a 20‐item questionnaire, covering four disease aspects based on the exploratory factor analysis. Construct validity was demonstrated and the EB ‐BoD questionnaire showed good internal consistency (Cronbach's α = 0·9). The resulting EB ‐BoD score was significantly correlated to the mental dimension of SF ‐12 ( r = −0·61), but it was not correlated to it's physical dimension ( r = 0·04). EB ‐BoD scores were significantly discriminating between EB subtypes. Conclusions The EB ‐BoD questionnaire appears to be a useful assessment tool regarding medical and socioeconomic issues in patients with EB and their families. EB ‐BoD scores correlate well with the family/parental burden experienced by the families of patients with EB .