Assessing and managing people with sickle cell disease presenting with vaso-occlusive crisis in the emergency department.

Abstract

Sickle cell disease is a group of inherited blood disorders characterised by atypical haemoglobin molecule structures (haemoglobin S) which can manifest as painful vaso-occlusive crises, chronic anaemia and progressive organ damage. This article aims to raise greater awareness of sickle cell disease, its acute manifestations and the potential for rapid clinical deterioration that can occur in patients with this condition. The article outlines the main aspects of the assessment and management of patients presenting to the emergency department (ED) with vaso-occlusive crisis, describes the barriers to effective care that they commonly experience and explains how their care could be improved. The authors emphasise the importance of timely management of these patients by ED staff, including emergency nurses.