Stigma, Disease Self-Management, and Quality of Life in Adults with Sickle Cell Disease

Abstract

Sickle cell disease (SCD) is the most common genetic blood disorder in the United States (US) and Jamaica and primarily affects individuals of African descent. SCD can result in severe and debilitating complications, including vaso-occlusive crises and organ damage. To prevent these complications SCD requires complex self-management. SCD is associated with significantly shortened lifespans in both countries. Many personal and background factors including, perceived stigma, demographics (country, age, race, sex, socioeconomic status) and clinical (disease severity, hydroxyurea use, genotype) characteristics may influence self-management strategies and health-related quality of life (QoL) in SCD. These characteristics have not been explored fully in either country and there are many differences between countries in how SCD is managed and stigma is perceived. This dissertation aims to develop knowledge related to the relationships between SCD self-management, stigma of SCD, and health-related QoL. A systematic literature review was conducted to appraise the current state of knowledge surrounding stigma of SCD. Conclusions from this review revealed that 1) sources of stigma were varied including institutions, healthcare systems and providers, and interpersonal relationships; 2) stigma had negative impacts on participants’ social, psychological, and physiological well-being; 3) stigma had resulted in poor patient-provider relationships and altered care-seeking behaviors in individuals with SCD, and 4) there are gaps in the literature regarding the influence of sources of stigma on self-management and QoL. The primary study of this dissertation utilized a cross sectional, convergent parallel mixed methods design (individual interviews and self-report surveys). Participants were interviewed about disease self-management strategies and how sources of stigma influence these strategies. Demographic and clinical characteristics were assessed using questionnaires. Quantitative measures were used to assess perceived stigma [SCD Health-Related Stigma Scale (SCD-HRSS) and Measure of Sickle Cell Stigma (MoSCS)] and health-related QoL [Adult Sickle Cell Quality of Life Measures (ASCQ-Me)]. Because there were no publication using the ASCQ-Me to assess health-related QoL in adults with SCD, aside from psychometric studies, a pilot study was conducted evaluating the feasibility of using the ASCQ-Me prior to the primary dissertation study. Use of ASCQ-Me was determined to be feasible. There were several important findings in the primary dissertation study. Employment and low disease severity were significant predictors of health-related QoL. Nonetheless, participants reported experiencing stigma from family, friends, and people in the workplace and school that impeded their access to the social support and financial resources needed to effectively self-manage. Self-management strategies were similar between the two countries with the exception of there being less opioid use and a greater focus on nutrition in Jamaica. Lastly, participants in both countries, regardless of demographic and clinical characteristics, reported perceiving stigma from healthcare settings. As a result, they altered their self-management in many ways including avoiding or delaying seeking care and pain management. Findings from this study will be used to generate hypotheses for future studies seeking to improve self-management and QoL of individuals with SCD in the presence of stigma.