Health-related quality of life, cognitive functioning and behaviour problems in children with Langerhans cell histiocytosis

Abstract

Background. This study was designed to evaluate generic and disease-specific health-related quality of life (HRQoL), cognitive functioning and behaviour problems of children with Langerhans Cell Histiocytosis (LCH). Furthermore, we investigated which medical determinants and social demographic factors were predictive for HRQoL, cognitive functioning and behavioural problems. Procedure. In this cross-sectional case-control study 24 children ranging from 7 to 17 years of age were administered a HRQoL questionnaire, cognitive tests and behaviour ratings. In addition, a disease-specific HRQoL measure was developed and tested. Results were compared to a reference group consisting of healthy peers and to proxy-ratings by parents and teachers. Results. Children with LCH reported a lower physical HRQoL than the reference group (P ≤ 0.05). Children older than 12 reported lower HRQoL scores. Scores on the disease-specific HRQoL questionnaire were lower than on the generic measure used. Performances on cognitive tests varied widely, short term visual memory was most affected. Twenty-five percent of the children follow special education. According to parents and teachers, children with LCH had more internalising behaviour problems (i.e., anxiety and depression), compared to the instrument norms. Children with Diabetes Insipidus, other CNS involvement and children who have had chemotherapy had more cognitive and behaviour problems than the other children with LCH. Conclusions. HRQoL is affected in children with LCH, especially in older children. Children with LCH show more internalising problem behaviour than their peers. Teachers are important additional informants about behaviour problems. © 2008 Wiley-Liss, Inc.