The efficacy of hydroxyurea in decreasing transfusion requirements and hospital admission in children with sickle cell disease

Abstract

The burden of sickle cell disease lies in Africa where resources are limited . Hydroxyurea may be an affordable treatmentoption for these patients. Purpose To asses whether hydroxyurea has any effect in reducing vaso-occlusive crises, hospitalization and transfusion requirements in children with homozygous sickle cell a n a e m i a . To e v a l u a t e t h e t o x i c i t y o f h y d r o x y u r e a . M e t h o d s T h i s i s a r e t r o s p e c t i v e , d e s c r i p t i v e s t u d y o f c l i n i c a l and h a e m a t o l o g i c a l o u t c o m e s i n c h i l d r e n w i t h s i c k l e c e l l a n a e m i a t r e a t e d w i t h h y d r o x y u r e a . R e s u 1t s Ten p a t i e n t s w e r e e v a l u a t e d . H y d r o x y u r e a d e c r e a s e d t h e r a t e o f v a s o - o c c 1 u s i o n and d e c r e a s e d t h e t r a n s f u s i o n r e q u i r e m e n t s . The c l i n i c a l and h a e m a t o l o g i c a l b e n e f i t s w e r e g r e a t e s t when f o e t a l h a e m o b l o b i n was m a x i m a l . T h e r e was no s h o r t - t e r m t o x i c i t y . C o n c l u s i o n H y d r o x y u r e a a m e l i o r a t e s symptoms in sickle cell disease. On going studies are n e e d e d t o a s s e s s l o n g - t e r m e f f e c t s .