Disease Knowledge and Readiness for Transition in Adolescents with Sickle Cell Disease in Jamaica: A Mixed-Methods Study

Abstract

Introduction: Sickle cell disease (SCD) is a genetically inherited recessive blood disorder that affects millions of people worldwide. The management of SCD should and can be considered a collaborative team effort, and requires the comprehensive and coordinated support of several medical professionals. The rising number of adults living with SCD creates a need for long term therapeutic and management strategies as well as a better understanding of the transition from pediatric to adult care. The research goal for this project is to compare the two systems that exist for treatment of adolescents with SCD in Jamaica and the United States by assessing differences and similarities in patients’ readiness for adult treatment and their understanding of SCD and its management. Methods: This study was conducted in the Sickle Cell Unit at the University of West Indies (UWI) hospital in Kingston, Jamaica. Eligibility for this study was defined as patients with SCD, between the ages of 13-19, seeking treatment at the health facility in the University of the West Indies, who have no acute illness at the time of study. After a verbal and written consent process during check-in, each participant completed a demographic survey, disease knowledge questionnaire, the ASH Transition Readiness Assessment Questionnaire, and had the opportunity to participate in in-depth interviews. Following data collection, results subsequently with similar previously completed surveys from patients at the Duke University Sickle Cell Center. Results: Gender and socioeconomic factors were not associated with differences in assessment scores in Jamaica. Total scores for disease knowledge questionnaires increased with age, however mean scores for the 17-19 age group were 62.17% lower than Duke University patients of the same age. Self-evaluation with the ASH Transition Readiness Assessment also showed an increase in scores with age, and significant increases in disease knowledge and appointments sections in both the 13-14 and 17-19 age groups, estimated by a p-value of 0.023 and 0.006, respectively. The results, however, were also generally lower than similar Transition Readiness Assessment measures at Duke. In-depth interviews revealed patient insight into disease knowledge, treatment involvement and experiences with doctors, family, and in the clinic. Answers align with both questionnaires used in this study.