Testing newborn babies for sickle cell diseases

Sickle cell diseases are inherited and affect mainly people of African origin. The red blood cells are abnormally (sickle) shaped, which can lead to life-threatening complications. They are most likely to be fatal in the first few years of life since affected children are at higher risk of serious infections. Regular antibiotics and immunisations reduce the risk of infections, and if sickle status is known, can be started early. Screening babies allows early diagnosis and therefore early treatment. Screening may also have disadvantages. This review aims to assess whether screening compared to diagnosis from symptoms leads to less morbidity and mortality. The authors were not able to find trials that assessed the benefits and harms of screening. There is evidence that starting treatment early is of benefit. Early treatment is made possible by screening in the neonatal period. There are some reports in non-trial literature which suggest that newborn screening is appropriate based on currently available evidence. Healthcare providers must assess whether these reports are relevant to their practice and situation when deciding whether to screen for SCD in the neonatal period. Practice recommendations could be made from the results of a prospective randomised controlled trial. Such a trial may be thought to be unethical given the proven benefit of early preventative treatment of children with penicillin. There are no trials included in the review and we have not identified any relevant trials up to July 2008. We therefore do not plan to update this review until new trials are published.