Standards for the management of sickle cell disease in children

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Sickle cell disease is now the commonest genetic condition in England with a birth prevalence of 1 in 2000. It causes significant morbidity and mortality particularly in the early years unless prophylactic measures have been put in place. By the end of 2006 newborn screening had been introduced all over England, its prime aim being to minimise mortality from pneumococcal sepsis in the first few years of life. Plans are currently underway to roll out programmes in Scotland and Wales. Standards and guidelines for the care of children with sickle cell disease were written to accompany the newborn screening programme and to offer a management plan for those working in areas of low prevalence where resources and expertise were possibly less well developed. This article describes the existing evidence base for treatment and the current consensus of good practice. It is acknowledged that more work needs to be carried out to develop guidelines further.

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