Background: Sickle cell disease (SCD) is an autosomal recessive, multisystem disorder, characterized by chronic hemolytic anemia, painful episodes of vaso-occlusion, progressive organ failure and a reduced life expectancy. SCD is the most common monogenetic disease, with millions affected worldwide. It is estimated that there are 100,000 people living with SCD in the United States (US). Patients with SCD often use emergency department (ED) services to obtain medical care. Limited nationwide information in the recent years is available about ED use among patients with SCD.
Methods: Data from the National Hospital Ambulatory Medical Care Survey (NHAMCS) for the years 2010-2015 were analyzed, to examine the prevalence and care of SCD patients in the EDs across the US. The NHAMCS is a survey of hospital ED and outpatient visits. Cases were identified using ICD 9 codes and all analyses accounted for complex survey structure of the data.
Results: On average, approximately 1.4 million ED visits were estimated to have occurred between the years 2010 and 2015 with SCD as one of the diagnoses listed, where 860,000 were attributed to sickle cell crises. 37.29% were of female sex while 62.71% were males. 11.62% were under 15 years of age, 28.22% were between 15 and 24 years of age, 51.15% were between the ages of 25 and 44 years and 9.02% were older than 45 years. The chief complaint for the majority of cases was pain. The expected source of payment were Medicare and Medicaid at 30.45% and 41.98% respectively, while private insurance coverage and self-pay were only at 11.71% and 5.81% respectively. Of all SCD ED visits, 68.5% were considered as initial presentations and up to 23.37% were considered as follow-up. 33.31% of the visits ended up needing a hospital admission while 66.69% got discharged from the ED.In 43.39% of the visits, a form of imaging was ordered, compared to 56.61% where it was not. Upon getting triaged in the ED, 67.62% of all SCD visits needed either emergent or urgent medical attention, while the rest were either none-urgent, or a regular check-up.
Conclusion: In well-resourced countries, like the US, comprehensive care programs have increased life expectancy of sickle cell disease patients, with almost all infants surviving into adulthood. However unfortunately with poor control of the disease due to limited therapeutic options and with growing morbidity for the aging SCD patients, the burden on the medical system becomes extremely high. Our study highlights the significant burden SCD creates in the EDs across the US based on data collected from a national database.
No relevant conflicts of interest to declare.
Author notes
Asterisk with author names denotes non-ASH members.
