Co-morbidities and mortality in patients with sickle cell disease in England: A 10-year cohort analysis using hospital episodes statistics (HES) data.

Patients with a primary diagnosis of sickle cell disease (SCD) with or without crisis during the 10-year period January 2009 to December 2018 were identified in the HES Admitted Patient Care (APC) dataset and matched with the Office for National Statistics (ONS) mortality dataset. Three sub-cohorts were defined: 'crises', 'transfusions' and 'other SCD'. APC records were examined for co-morbidities commonly associated with SCD and 10-year mortality rates compared with the general population. After data cleaning and exclusions, 9503 patients remained (entire cohort), with 1171, 201, and 8131 in crises, transfusions, and other SCD sub-cohorts, respectively. Median numbers of co-morbidities per patient were 2 (Interquartile range (IQR): 1-4), 2 (IQR: 1-3), and 1 (IQR: 0-2) in the crises, transfusions, and other SCD sub-cohorts, respectively. The majority of patients in the crises (63.2%) and transfusions (56.3%) cohorts had ≥2 co-morbidities, compared with 25.3% in the other SCD sub-cohort. Crude 10-year mortality rate was 5.3% (entire cohort), compared with 8.0% (crises) and 11.4% (transfusions) sub-cohorts; all rates were substantially higher than in age-sex matched general population. Our study adds further evidence that morbidity and mortality associated with SCD in England is high.

Co-morbidities and mortality associated with transfusion-dependent beta-thalassaemia in patients in England: a 10-year retrospective cohort analysis.

A retrospective cohort analysis to explore 10-year mortality and prevalence of transfusion-dependent ß-thalassaemia (TDT)-associated co-morbidities in patients with TDT was undertaken using Hospital Episode Statistics (HES) data from the National Health Service (NHS) in England. A 10-year forward-looking cohort analysis for the period 2009-2018 was completed using HES admitted patient care (APC), outpatient data, and linked HES/Office of National Statistics mortality data for patients with ß-thalassaemia (ICD-10 diagnosis code D56.1). TDT-associated co-morbidity rates were high in the 612 patients with TDT, with 76% having at least one co-morbidity, 54% suffering from two of more, and 37% three or more. The three most common TDT-associated co-morbidities, occurring in more than one third of patients were: endocrine disorders (excluding diabetes) 40%, osteoporosis 40%, and diabetes 34%. Cardiac disease was observed in 18% of patients overall, with atrial fibrillation and heart failure being the most common with a prevalence of 11% and 9%, respectively. The crude 10-year mortality rate in the TDT cohort was 6·2% (38/612), significantly greater than the 1·2% age/sex-adjusted mortality rate of the general population (P < 0·001). These data support the notion that the unmet need in TDT remains significant, with high rates of co-morbidity and mortality.