Sickle cell disease is a multisystem disease characterised by chronic haemolytic anaemia, painful vasoocclusive
crises and acute and chronic end-organ damage. It is one of the most common serious inherited
single gene conditions worldwide and has a major impact on the health of affected individuals. Peri-operative
complications are higher in patients with sickle cell disease compared with the general population and may
be sickle or non-sickle-related. Complications may be reduced by meticulous peri-operative care and
transfusion, but unnecessary transfusion should be avoided, particularly to reduce the risk of alloimmunisation.
Planned surgery and anaesthesia for patients with sickle cell disease should ideally be
undertaken in centres with experience in caring for these patients. In an emergency, advice should be sought
from specialists with experience in sickle cell disease through the haemoglobinopathy network
arrangements. Emerging data suggest that patients with sickle cell disease are at increased risk of COVID-19
infection but may have a relatively mild clinical course. Outcomes are determined by pre-existing
comorbidities, as for the general population.