Sickle cell and the burden of disease in England

Abstract

Background Sickle cell disorders (SCD) are the most common inherited blood disorders in England. Without prompt diagnosis and proper treatment, they can be a serious source of morbidity and mortality. Sickle cell diseases affect mainly black minority and ethnic populations, and have so far received relatively low priority from a health policy perspective. Antenatal and newborn screening, the development of minimum standards, antibiotic prophylaxis, comprehensive immunisations, and preventive diagnostic tests have positively influenced SCD management. There remains an unclear picture as to the trends and health care utilization of patients with SCD in England. Aims This study looks at the burden of disease in England by assessing hospital admissions, readmissions and related costs. It also aims to identify gaps in care and prevention which may identify possible contributors to avoidable admissions. Findings Using Hospital Episode Statistics (HES) data, trends for SCD hospital admissions in England showed a rise in 50% of hospital admissions over a 10 year period. The most deprived areas had a higher rate of readmission and in-patient mortality among those with SCD. Adolescents had a higher rate of readmission possibly identifying a gap in health care access. Local findings in a high prevalence area showed that the majority of admissions were for a short length of stay and 74% of patients accounted for multiple admissions. A patient focus group and questionnaire both identified potential gaps in care and prevention. Conclusion Through the use of 6 studies which showed the SCD admission rates in England, the readmission rates, local admissions, costs associated with admission and patient perspectives in both care and prevention, there is a clearer picture as to the trends and health care utilization of patients with SCD in England. The studies suggest that ascertaining the prevalence of at-risk groups in England as well as addressing inequalities in health care access among minority groups and areas of high disease prevalence can further aid in disease management. Shifting diagnostic and follow-up care from acute care facilities to primary care facilities and promoting preventive care measures and adherence to standards and guidelines may possibly decrease the cost burden, reduce avoidable hospital admissions and increase the timeliness and effectiveness of disease management. Investing in training and education of primary care physicians for sickle cell diseases may also improve quality of care.