Evaluation of sickle cell patients treated with hidroxyurea at the National Children´s Hospital

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Background: Since 1984 research proved that hydroxyrea increases fetal hemoglobin and the medium corpuscular volume. Consequently, as of 1998, it became the first drug approved for the specific treatment of sickle cell anemia. The purpose of this study was to describe the response and complications of sickle cell patients that underwent treatment with hydroxyrea at the National Children´s Hospital. Methods: a transversal and retrospective study, carried out on sickle cell patients treated at the Hematology Department of the National Children´s Hospital from January 2006 to December 2009; including patients whose clinical records complied with the inclusion criteria. Results: 30 patients were recruited, 60% male; the majority of patients came from Guanacaste, San José and Limón (26.7%, 23.3% and 20%, respectively). In 87% of the cases the patients maintained a normal growth rate. The comparison of fetal hemoglobin levels before and after the hydroxyrea treatment showed that the difference was not statistically significant. The comparison of annual transfusions before and after the hydroxyrea treatment showed a statistically significant difference. A decrease in the number of admissions was observed. The main causes of admission were vaso occlusive crisis, septic processes and stroke. Conclusion: the use of hydroxyrea in sickle cell patients was related to a decrease of hospital admissions due to vaso occlusive crises; additionally there was a reduction in the number of annual transfusions, with no side effects and without compromising the growth rate of patients.the use of hydroxyrea in sickle cell patients was related to a decrease of hospital admissions due to vaso occlusive crises; additionally there was a reduction in the number of annual transfusions, with no side effects and without compromising the growth rate of patients.

sickle cell disease; hidroxyurea; fetal hemoglobin; vaso occlusive crisis