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Am J Hematol. 2011 Feb;86(2):203-5. doi: 10.1002/ajh.21905.

Health-related quality of life in adults with sickle cell disease (SCD): a report from the comprehensive sickle cell centers clinical trial consortium.

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  • 1Department of Pediatrics, Emory University School of Medicine, Atlanta, Georgia 30322, USA.


Adults with Sickle Cell Disease (SCD) experience multiple disease-related complications, but few studies have examined relationships between these events and health-related quality of life (HRQOL). We determined the number and type of previous or co-occurring SCD-related complications and their reported HRQOL in a cohort of 1,046 adults from the Comprehensive Sickle Cell Centers (CSCC). Participants had a median age of 28.0 years (48% male, 73% SS or Sβ⁰ thalassemia) and had experienced several SCD-related complications (mean 3.8 ± 2.0), which were influenced by age, gender, and hemoglobinopathy type (P < 0.0001). In multivariate models, increasing age reduced all SF-36 scales scores (P < 0.05) except mental health, while female gender additionally diminished physical function and vitality scale scores (P < 0.01). Of possible complications, only vaso-occlusive crisis, asthma, or avascular necrosis diminished SF-36 scale scores. Chronic antidepressants usage predominantly diminished scores on bodily pain, vitality, social functioning, emotional role, and mental health scales, whereas chronic opioid usage diminished all scale scores (P < 0.01). Our study documents substantial impairment of HRQOL in adults with SCD that was influenced by only a few of many possible medical complications. It suggests that more effective treatments of persistent pain and depression would provide the largest HRQOL benefit.

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