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Studies Examine Quality of Care for Hospitalized Sickle Cell Disease Patients and Effective Methods for Detecting Risk of Stroke in Children With This Disorder
Date:12/6/2008

in patients treated with hydroxyurea (mean number of administered packed red cell units: 1.5±5.9 per year pre-hydroxyurea diminishing to almost zero during hydroxyurea treatment) as well as a significant reduction in hospital admissions (from 2.1±2.9 per year pre-hydroxyurea to 0.06±0.02 per year post-hydroxyurea).

Sickle cell disease results in many, often severe, complications affecting all organs due to vaso-occlusion (the blockage of small vessels by sickled cells). One of these severe complications is chest syndrome, which is often fatal. Hydroxyurea produced a significant reduction in the frequency of chest syndrome (from 6.1 percent pre-hydroxyurea to 0.8 percent during the hydroxyurea period). No significant difference was observed between the two treatments for the prevention or reduction of the frequency of avascular necrosis (blockage of small vessels in the bone area causing destruction to joints) or cerebrovascular episodes (blockage of small blood vessels in the brain).

Another characteristic feature of the disease is the presence of hemolytic crises (rapidly evolving anemia, jaundice, and fever), which further reduce hemoglobin levels and produces severe dysfunction in several organs. Patients treated with hydroxyurea had reduced hemolysis and improved total hemoglobin in comparison to the group of patients not treated with hydroxyurea. The difference observed in overall survival in patients who were given hydroxyurea is further magnified by the fact that that, prior to starting hydroxyurea, these patients had significantly higher transfusion requirements, frequency of painful crises, yearly number of admissions to the hospital, and increased intensity of hemolysis in comparison to patients who did not receive hydroxyurea.

Side effects of hydroxyurea therapy were minimal, predictable, and easily manageable. However, all patients were under close follow-up, with visits including thoroug
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SOURCE American Society of Hematology
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