CT Hijmans et al. Double disadvantage: a case control study on health-related quality of life in children with sickle cell disease. Health Quality of Live Outcomes, 2010;8:121.

Low 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 a study by Hijmans and associates 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. The 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. The proportion of children with SCD with impaired HRQoL was also evaluated. In 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.

WHAT THE STUDY MAY MEAN TO YOU AS A HEALTH CARE PROVIDER: These 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.

WHAT THE STUDY MAY MEAN TO YOU AS A HEALTH CARE CONSUMER:
If there was ever a population that needs self-management training, it is patients with sickle cell disease. As shown in the study, however, this is a disease that requires a program that should involve all family members and not just patients.