Anaesthesia for patients with sickle cell disease (and other haemoglobinopathies)

Sickle cell disease is a common inherited disorder of haemoglobin associated with significant morbidity and mortality. It is associated with the haemoglobin S variant of haemoglobin, which comprises abnormal beta globin chains that are the result of a mutation to chromosome 11. Under adverse conditions the beta globin chains undergo polymerization, leading to deformation of red cells and subsequent sickling crises. Individuals with sickle cell disease develop multisystem complications including anaemia, respiratory disease, cardiomegaly, cerebrovascular accidents, painful bone disease and renal impairment. Sickle crises may be precipitated by hypoxia, venous stasis, hypothermia, acidosis, and dehydration. The principle of perioperative care is prevention, though the active avoidance of these triggers. Supportive therapy and symptomatic relief may be required in the event of crisis.