Most, but not all acute presentations are due to complications of sickle cell disease (SCD). Health care professionals who are unfamiliar with SCD sometimes fail to recognise a characteristic syndrome related to SCD and may attribute the symptoms and signs to a more familiar general medical diagnosis. This may result in incorrect management, with severe consequences. Conversely, in units where SCD is commonly seen, symptoms are assumed to be a ‘sickle crisis’ and insufficient consideration is given to alternative general medical conditions. This might happen in the situation of a young SCD child presenting with a febrile illness due to a viral infection, or an older patient with complications of diabetes or hypertension.

In this section we will describe the presentation, pathophysiology and management of complications of SCD. These are summarised in Table 4.1