Effectiveness of Hydroxyurea in the Management of the Painful Crisis in Sickle Cell Anemia

Abstract

Background: Sickle cell syndromes are inbred disorders distinguished by the presence of blade (Sickle) hemoglobin in red blood corpuscles. The sickling of red cells in patients with SCA is caused by the polymerization of molecules of non-oxygenated hemoglobin S (α2ß2s) into rigid rod-like polymers. In the open-label study of hydroxyurea therapy, the synthesis of embryo hemoglobin increased in most patients with SCA resulting in a humane myelotoxicity and rise in painful crises. By suppressing the sickling process, increased levels of embryo hemoglobin sinew the less frequency of painful crises.

Methods: In prospective observational learning, we assessed the efficiency of hydroxyurea in decreasing the recurrence of painful episodes associated with the reduction in the prevalence of blood transfusion in children and adults with a history of complications. The trial was aborted after a mean follow-up of 4 months.

Results: Among 80 patients investigated, 41 patients were men and 39 patients were women. The prospective observational study in the department of pediatrics, Prathima Institute of Medical Sciences, Nagunoor, Karimnagar, T.S. The 80 patients designate to hydroxyurea treatment, out of which 64 patients were taking hydroxyurea in the duration of 0-5 yrs; 12 patients were taking hydroxyurea for a span of 6-10 yrs old; 4 patients were taking hydroxyurea for the period of 11-15 yrs. No patients were managed with hydroxyurea with the greater of 16 yrs. The dose is administered in the range of 15-35mg/kg per day. The serum ferritin levels, hemoglobin, direct, and total bilirubin levels were investigated for three follow up. It was found that F – statistical value -0.41467, P-value -0.66104 & right- tailed p-value (Z>z) -0.6554217 for Serum ferritin; F- statistical value – 0.9209, P-value - 0.3996 & right-tailed p-value (Z>z)-0.3594236 for haemoglobin ; F-statistical value - 0.92093P-value-0.39957 & right-tailed p-value (Z>z)-0.3993615 for total bilirubin ; FStatistical value -1.11703,P-value – 0.32897 &   right-tailed p-value ; P (Z>z) – 0.3993615 for direct bilirubin respectively. During the treatment with hydroxyurea, it was observed that none of the patients were reported the unwanted effects with hydroxyurea.

Conclusion: Hydroxyurea therapy can ameliorate the clinical course of SCA in children and adults with three or more painful crises. Maximally tolerated doses of hydroxyurea may not be necessary to achieve a therapeutic effect. The long-term safety of hydroxyurea in patients with SCA is uncertain.