Abstract
BackgroundSickle cell disease (SCD) is a chronic disease associated with high degrees of morbidity and increased mortality. Health-related quality of life (HRQOL) among adults with sickle cell disease has not been widely reported.MethodsWe administered the Medical Outcomes Study 36-item Short-Form to 308 patients in the Pain in Sickle Cell Epidemiology Study (PiSCES) to assess HRQOL. Scales included physical function, physical and emotional role function, bodily pain, vitality, social function, mental health, and general health. We compared scores with national norms using t-tests, and with three chronic disease cohorts: asthma, cystic fibrosis and hemodialysis patients using analysis of variance and Dunnett's test for comparison with a control. We also assessed whether SCD specific variables (genotype, pain, crisis and utilization) were independently predictive of SF-36 subscales, controlling for socio-demographic variables using regression.ResultsPatients with SCD scored significantly worse than national norms on all subscales except mental health. Patients with SCD had lower HRQOL than cystic fibrosis patients except for mental health. Scores were similar for physical function, role function and mental health as compared to asthma patients, but worse for bodily pain, vitality, social function and general health subscales. Compared to dialysis patients, sickle cell disease patients scored similarly on physical role and emotional role function, social functioning and mental health, worse on bodily pain, general health and vitality and better on physical functioning. Surprisingly, genotype did not influence HRQOL except for vitality. However, scores significantly decreased as pain levels increased.ConclusionSCD patients experience health related quality of life worse than the general population, and in general, their scores were most similar to patients undergoing hemodialysis. Practitioners should regard their HRQOL as severely compromised. Interventions in SCD should consider improvements in health related quality of life as important outcomes.
Highlights
Sickle cell disease (SCD) is a chronic disease associated with high degrees of morbidity and increased mortality
Despite the considerable evidence in children for reduced Health-related quality of life (HRQOL) in SCD, few studies have evaluated the impact of this disease on health related quality of life in adults [8,9,10,11]
When the gender stratified Pain in Sickle Cell Epidemiology Study (PiSCES) cohort was compared with national norms, values were significantly lower for all subscales (P < 0.0001) with one exception – the mental health scale was not significantly different from the national norm
Summary
Sickle cell disease (SCD) is a chronic disease associated with high degrees of morbidity and increased mortality. Health-related quality of life (HRQOL) among adults with sickle cell disease has not been widely reported. Functional status and health-related quality of life (HRQOL) may be impaired in sickle cell disease (SCD) due to morbid events, such as stroke, or other organ system failures. The Cooperative Study of Sickle Cell Disease (CSSD) found that morbid events such as strokes that impaired function often preceded death in childhood [13] Until recent decades, SCD was associated with chronic childhood pain, organ failure and death in very early adulthood. Despite the considerable evidence in children for reduced HRQOL in SCD, few studies have evaluated the impact of this disease on health related quality of life in adults [8,9,10,11]
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