Management of sickle cell disease: out-patient and community aspects

Sickle Cell Disease (SCD) is the commonest inherited disorder in England, affecting 1 in 2000 births, the majority born in London and of Black African family origins. Newborn bloodspot screening for SCD has now been implemented across the whole of England and this review considers the neonatal screening pathway and aspects of out-patient and community care which should be delivered within the comprehensive care networks. Morbidity and mortality during childhood has declined due to implementation of effective care programmes which include infection and stroke prophylaxis. Children still suffer from complications of SCD affecting long-term health, quality of life, self esteem, school performance, and attainment. Treatment options for long-term control of the disease include hydroxycarbamide, regular transfusion and sibling allogeneic bone marrow transplantation. The indications, evidence of efficacy, and adverse effects of these treatments are discussed.