Priapism is a common urologic complication of sickle cell anemia, and is frequently cited as one of the more difficult clinical problems encountered by pediatric urologists. Hydroxyurea is associated with significant reductions in both morbidity and mortality from sickle cell anemia; however, there is significant variability in hydroxyurea usage rates around the country. Our objective was thus to compare rates of priapism in boys and young men with sickle cell anemia at children’s hospitals across the United States.
We retrospectively reviewed all inpatient, emergency room, or short-stay visits captured by the PHIS database for boys and young men (aged <21 years) treated for a diagnosis of sickle cell disease between 2003 and 2011. We then identified those patients with sickle cell who were admitted with a diagnosis of priapism. Rates of inpatient hydroxyurea use were determined based on pharmacy and billing codes. Logistic regression and generalized estimating equation models were used to control for confounding and to adjust for within-hospital clustering of similar patients.
The rate of priapism-related admissions decreased during the study period (1.4% in 2003 vs. 0.6% in 2011, p<0.001), while hydroxyurea use remained stable (16.7% in 2003 vs. 12.0% in 2011, p=0.06) among the 17,810 sickle cell patients in PHIS (125,923 hospital admissions during the study period, mean 13.3 admissions per patient, range 1-178). Of these, 315 patients (1.8%) were diagnosed with priapism on 705 admissions (median 6, range 1-120 admissions per patient), and 2,728 (15.3%) patients received hydroxyurea during 14,382 admissions (median 8, range 1-178 admissions per patient). Rates of both priapism diagnosis (0.2 -1.8%, p<0.001) and hydroxyurea use (0-47.5%, p<0.001) varied significantly between hospitals. After adjusting for clustering of similar patients at each hospital, older patient age (teen vs. infant OR 11.7, 95% CI 4.4-31.4, p=0.02) and more frequent hydroxyurea use (p=0.006) were associated with an increased likelihood of priapism diagnosis. The rate of hydroxyurea use at each hospital was not associated with the likelihood of priapism diagnosis (p=0.39).
The rate of priapism has decreased over the last decade at U.S. children’s hospitals. Although inpatient hydroxyurea use varies widely among these hospitals, this clinical variation does not appear to influence the likelihood of patients being treated for priapism. Because PHIS only captures emergency room and inpatient medication use, it is unclear whether outpatient hydroxyurea use has played a role in the decreased rate of sickle-cell-related priapism. n:yes5 /��P�r adjusting for clustering of similar patients at each hospital, older patient age (teen vs. infant OR 11.7, 95% CI 4.4-31.4, p=0.02) and more frequent hydroxyurea use (p=0.006) were associated with an increased likelihood of priapism diagnosis. The rate of hydroxyurea use at each hospital was not associated with the likelihood of priapism diagnosis (p=0.39).