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. Ĭhildren and adolescents with SCD report poor HRQOL in qualitative studies using focus groups, and fare worse in their HRQOL compared to controls on health surveys or on assessments of general physical, motor and independent daily functioning. Frequently, patients surviving until adulthood experience significant organ system damage that may include stroke, pulmonary failure and pulmonary hypertension, renal failure, congestive heart failure, leg ulcers, and osteonecrosis of the femoral or humeral heads. Treatment advances have now transformed SCD into a chronic disease suffered by children and adults. The Cooperative Study of Sickle Cell Disease (CSSD) found that morbid events such as strokes that impaired function often preceded death in childhood Until recent decades, SCD was associated with chronic childhood pain, organ failure and death in very early adulthood. Interventions in SCD should consider improvements in health related quality of life as important outcomes.įunctional 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. Practitioners should regard their HRQOL as severely compromised. SCD patients experience health related quality of life worse than the general population, and in general, their scores were most similar to patients undergoing hemodialysis. However, scores significantly decreased as pain levels increased. Surprisingly, genotype did not influence HRQOL except for vitality. 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. 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.
Patients with SCD had lower HRQOL than cystic fibrosis patients except for mental health.
Patients with SCD scored significantly worse than national norms on all subscales except mental health. 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. 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. Scales included physical function, physical and emotional role function, bodily pain, vitality, social function, mental health, and general health. We administered the Medical Outcomes Study 36-item Short-Form to 308 patients in the Pain in Sickle Cell Epidemiology Study (PiSCES) to assess HRQOL. Health-related quality of life (HRQOL) among adults with sickle cell disease has not been widely reported. Sickle cell disease (SCD) is a chronic disease associated with high degrees of morbidity and increased mortality.
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