Studies Examine Quality of Care for Hospitalized Sickle Cell Disease Patients and Effective Methods for Detecting Risk of Stroke in Children With Thi...(is disease. The beneficial effects of hy...),Health

Hydroxyurea is commonly used for preventing painful sickle cell crises, reducing the frequency and length of hospital stays, and improving the quality of life of patients with sickle cell disease. However, determining whether hydroxyurea could also prevent severe chronic complications of the disease or improve survival in these patients had not yet been confirmed.

The purpose of this study was to evaluate the effects of hydroxyurea in a large number of patients who received the therapy over a long period of time. The medical records of 330 patients with sickle cell disease treated at the Thalassemia Center of Laikon Hospital in Athens, Greece, over the course of 20 years were retrospectively analyzed. Patients were divided into two groups: a total of 131 patients who received hydroxyurea treatment and 199 patients whose symptoms were addressed with conventional therapies. The typical dose of hydroxyurea given was 20 mg/kg/day, except for some patients where toxicity or lack of effectiveness required dose modifications of 15 to 35 mg/kg/day. All patients, except for rare exceptions, reported to the center every four to eight weeks for thorough laboratory work. The median follow-up was eight years for patients receiving hydroxyurea and five years for those who did not receive hydroxyurea.

The study found that the probability of 10-year survival was 91 percent for the patients treated with hydroxyurea compared with 70 percent for the conventionally treated patients. Additionally, patients receiving hydroxyurea showed a dramatic reduction of the frequency of severe painful crises (from 7.4±6.5 episodes per year pre-hydroxuyrea to 0.2±0.4 episodes post-hydroxyurea). A significant reduction in the number of red blood cell transfusions was also seen
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