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Abstract

Introduction: In patients with sickle cell disease (SCD), sickle cell crisis (SCC) is a frequent cause of emergency department (ED) visit. Acute chest syndrome (ACS) is a known complication in patients with SCD and is a major cause of morbidity and mortality. Outcome data are limited to single center studies and there is a paucity of data regarding ED visits and resource utilization for patients with SCC and ACS. Methods: We performed a retrospective analysis of the Nationwide Emergency Department Sample (NEDS) for the years 2008 to 2010. The NEDS is a part of the Healthcare Cost and Utilization Project sponsored by the Agency for Healthcare Research and Quality. All children (aged <=21 years) visiting hospital based EDs with a diagnosis of sickle cell crisis (ICD-9-CM codes of 282.42, 282.62, 282.64, and 282.69) were selected. Occurrence of acute chest syndrome (ICD-9-CM code of 517.3) was estimated for these ED visits. Characteristics examined included age, gender, and type of sickle cell disease leading to crisis. Outcomes examined included disposition information following ED visit, hospital ED charges, and hospital mortality. Results: During the study period, there were a total of 149,472 ED visits for sickle cell crisis. The mean age of these patients was 14.6 years. Males comprised 49.3% of all ED visits. Close to 90.7% of all ED visits had Hb-SS disease with crisis, 3.3% had sickle-cell thalassemia with crisis, and 2.5% had sickle-cell/HB-C disease with crisis. About 3.6% had other sickle-cell disease with crisis. Close to 6.6% had acute chest syndrome. Medicaid was the major payer covering 62.2% of all ED visits. Twenty-six patients died in the ED while 116 died following admission as inpatients into the same hospital. Following ED visit, 42.3% were discharged routinely, 53.9% were admitted as inpatients into the same hospital, and 2.5% were transferred to another short-term hospital. The mean ED charge (inflation adjusted to year 2010) per visit was $2,162 and the total ED charges across the entire United States was $276.7 million. Conclusions: In this large retrospective nationwide emergency department study, sickle cell crisis was a frequent cause of emergency department visits in children with sickle cell disease. Acute chest syndrome is not an uncommon complication and is associated with significant utilization of hospital resources.