Double disadvantage: a case control study on health-related quality of life in children with sickle cell disease

Health and Quality of Life Outcomes

Research

Jaap Oosterlaan¹  Karin Fijnvandraat²  Marjolein Peters²  Harriët Heijboer²  Martha A Grootenhuis³  Channa T Hijmans⁴ 

[1] Department of Clinical Neuropsychology, VU University Amsterdam, Van der Boechorststraat 1, 1081 BT, Amsterdam, The Netherlands;Department of Pediatric Hematology, Emma Children's Hospital, Academic Medical Center, P.O. Box 304, 1100 VC, Amsterdam, The Netherlands;Psychosocial Department, Emma Children's Hospital, Academic Medical Center, P.O. Box 304, 1100 VC, Amsterdam, The Netherlands;Psychosocial Department, Emma Children's Hospital, Academic Medical Center, P.O. Box 304, 1100 VC, Amsterdam, The Netherlands;Department of Pediatric Hematology, Emma Children's Hospital, Academic Medical Center, P.O. Box 304, 1100 VC, Amsterdam, The Netherlands;

关键词: Sickle Cell Disease;    HRQoL Score;    Sickle Cell Disease Patient;    Lower HRQoL;    Cognitive Debriefing;   

DOI  :  10.1186/1477-7525-8-121

 received in 2010-05-31, accepted in 2010-10-26,  发布年份 2010

来源: Springer

【 摘 要 】

BackgroundLow health-related quality of life (HRQoL) of children with sickle cell disease (SCD) may be associated with consequences of the disease, or with the low socio-economic status (SES) of this patient population. The aim of this study was to investigate the HRQoL of children with SCD, controlling for SES by comparing them to healthy siblings (matched for age and gender), and to a Dutch norm population.MethodsThe HRQoL of 40 children with homozygous SCD and 36 healthy siblings was evaluated by the KIDSCREEN-52. This self-report questionnaire assesses ten domains of HRQoL. Differences between children with SCD and healthy siblings were analyzed using linear mixed models. One-sample t-tests were used to analyze differences with the Dutch norm population. Furthermore, the proportion of children with SCD with impaired HRQoL was evaluated.ResultsIn general, the HRQoL of children with SCD appeared comparable to the HRQoL of healthy siblings, while children with SCD had worse HRQoL than the Dutch norm population on five domains (Physical Well-being, Moods & Emotions, Autonomy, Parent Relation, and Financial Resources). Healthy siblings had worse HRQoL than the Dutch norm population on three domains (Moods & Emotions, Parent Relation, and Financial Resources). More than one in three children with SCD and healthy siblings had impaired HRQoL on several domains.ConclusionThese findings imply that reduced HRQoL in children with SCD is mainly related to the low SES of this patient population, with the exception of disease specific effects on the physical and autonomy domain. We conclude that children with SCD are especially vulnerable compared to other patient populations, and have special health care needs.

【 授权许可】

CC BY   
© Hijmans et al; licensee BioMed Central Ltd. 2010