Trends in Sickle Cell Disease-related Priapism in U.S. Children's Hospitals

Abstract

To define rates of priapism diagnosis and inpatient admission among males with sickle cell disease (SCD). We retrospectively reviewed the Pediatric Health Information System database for males aged <21 years treated 2004-2012. We identified patients with SCD and priapism based on the International Classification of Diseases, Ninth Revision, Clinical Modification diagnosis codes. Logistic regression and generalized estimating equation models were used to control for confounding and to adjust for within-hospital clustering of similar patients. We identified 17,186 males who were admitted 137,710 times during the study period. Of these, 362 (2.1%) were diagnosed with priapism on 748 admissions. There was a significant decrease in the number of priapism admissions among patients with SCD over time (0.81% in 2004 to 0.44% in 2012, P < .001). The number of patients diagnosed with SCD-related priapism varied over time without a statistically significant trend (2.3% in 2004, 2.69% in 2008, 1.01% in 2012, P = .34). Rates of priapism admissions (0-4.4%) varied widely between hospitals. Older patient age was associated with an increased likelihood of a priapism admission in the multivariate logistic regression model after adjusting for treatment year, hospital region, and for hospital-level clustering of similar patients. From 2004 to 2012, the number of admissions for SCD-related priapism declined whereas the number of individual patients diagnosed with SCD-related priapism did not. Rates of priapism-related admissions in males with SCD vary widely among PHIS hospitals.