Objectives: Research-derived evidence about the impact of sickle cell disease (SCD) on the lives of affected adults is lacking. We conducted formative research to provide the basis for a comprehensive description of how SCD affects the lives of adults, with the goal of developing a SCD-specific quality-of-life measurement system.
Methods: We conducted a comprehensive literature review of patient-reported outcomes, followed by a series of focus groups and structured individual interviews with adults with SCD (n=122) and their health care providers (n=15).
Results: We reviewed 473 abstracts and included 86 articles in the final review. The literature revealed broad categories of the impact of SCD and its treatment on the lives of adults-pain; emotional distress; social-role functioning; overall quality-of-life; and quality of care. We classified 1213 incidents from the focus groups and interviews into a taxonomy (16 domains) that met the criterion for saturation and was demonstrated to be reliable for the classification of incidents. The final conceptual model was built upon the taxonomy.
Discussion: Our conceptual model was similar to previous models with the effects of pain predominating, interwoven with emotional distress, quality of care, and stigmatization. We found a broad range of emotions reflected, including positive effects of SCD. Items for the quality-of-life measure were derived from the taxonomy and the conceptual model may be of use in generating hypotheses for clinical research and improving understanding for clinicians of the lived experience of adults with SCD.