Hydroxyurea for Sickle Cell Disease | oneSCDvoice
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reference materials

Hydroxyurea for Sickle Cell Disease

key information

source: Cochrane Library

year: 2001

authors: Ashley P Jones, Sally C Davies, Adebayo Olujohungbe


Sickle cell disease is one of the most common inherited diseases worldwide. It is associated with lifelong morbidity and a reduced life expectancy. Hydroxyurea, an oral chemotherapeutic drug, is expected to ameliorate some of the clinical problems of sickle cell disease, in particular that of pain, by raising fetal haemoglobin.

To assess the effects of hydroxyurea therapy in people with sickle cell disease (all types), of any age, regardless of setting.

Search methods:
We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Register, comprising of references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.

Selection criteria:
All randomised or quasi-randomised controlled trials comparing the use of hydroxyurea for one month or longer with placebo, standard therapy or other interventions for the treatment of people with sickle cell disease.

Data collection and analysis:
Three authors independently assessed study quality and extracted data from the two included studies.

Main results:
Two studies found by the searches, which reported results from a total of 324 adults and children, were suitable for inclusion in the review. From the data provided in the published reports, only one study (the MSH study to the United States of America) could be analysed. This study showed marked differences in favour of hydroxyurea treatment as compared with placebo in terms of annual crisis rate, use of transfusions, and life-threatening complications (in particular, acute sickle chest syndrome). Both studies documented the expected rise in fetal haemoglobin. No serious adverse effects were reported from either study.

Authors’ conclusions:
While hydroxyurea appears both effective and safe in severely affected SS adults over a two-year period; further studies are required to elucidate its role in other patient groups and for other conditions.

DOI: 10.1002/14651858.CD002202

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