Improving Health Outcomes in Persons with Sickle Cell Disease

Purpose: The purpose of this compendium is to identify strategies that influence health outcomes of persons with sickle cell disease (SCD) by investigating available pain instruments, current use of genetic testing to personalize therapies, and the associations of psychosocial characteristics with quality of life. Problem: Suboptimal care for patients with SCD is associated with non-comprehensive pain assessment instruments, limited use of available genetic tests to prescribe the potentially most effective pharmaceuticals in a personalized plan of care, and the absence of national evidence-based guidelines for the psychosocial care of this population. Approach: The research designs used were an integrative review with a biopsychosocial-religiosity/spirituality (BPS-R/S) framework, a literature review using Diffusion of Innovation as a framework, and a quantitative study guided by the Theory of Self-Care Management for Sickle Cell Disease using secondary analysis. Findings: The integrative review suggested that pain control outcomes might be improved by incorporating biopsychosocial-religiosity/spirituality measures to the pain instruments. The literature review indicated that the use of available genetic tests to determine the efficacy of pharmaceutical treatment options might lead to personalized care in the person with SCD. The secondary analysis found that the association between psychological factors and quality of life in the SCD sample was significant and suggested that randomized controlled trials be conducted to confirm findings prospectively. The overall findings identified that persons with SCD have complex needs inherent in a disease process that affects all body systems. Pain is the hallmark characteristic of the disease and is a dynamic biopsychosocial phenomenon. Conclusion: BPS-R/S and genetic domains contribute to humanistic care and may benefit persons with SCD. Persons with SCD may experience improved health outcomes when the biopsychosocial- religiosity/spirituality dimensions are supported by services in the healthcare environment. The implementation of these findings through researcher, provider, patient, and family education may improve health outcomes and quality of life