Higher oxygen saturation with hydroxyurea in paediatric sickle cell disease

Abstract

INTRODUCTION: Sickle cell disease (SCD) is one of the most common inherited diseases worldwide. It is associated with lifelong morbidity and reduced life expectancy. Hydroxyurea (HU) has been shown to reduce the frequency and severity of vaso-occlusive episodes in SCD. Hypoxaemia and intermittent nocturnal oxygen desaturations occur frequently in children with SCD and contribute to the associated morbidity, including risk of cerebrovascular disease. OBJECTIVE: To evaluate the effect of HU on oxygen saturation (SpO METHODS: A retrospective review of children with SCD and respiratory problems who attended two UK tertiary sickle respiratory clinics and were treated with HU. Longitudinal data were collected from 2 years prior and up to 3 years after the commencement of HU. RESULTS: Forty-three children, 23 males (53%) with a median age of 9 (range 1.8-18) years were included. In the 21 children who had comparable sleep studies before and after starting HU, mean SpO CONCLUSION: Children with SCD had higher oxygen saturation overnight and on daytime spot checks after starting HU. These data suggest HU may be helpful for treating persistent hypoxaemia in children with SCD pending more evidence from a randomised clinical trial.