Sickle Cell Disease AnaesthesiaUK

Description: Sickle cell disease is a haemoglobinopathy with autosomal recessive inheritance. Beta chain of HbA has valine substituted for glutamine at position 6 10% of black people have this condition in the UK In the homozygote, deoxygenated HbS becomes insoluble, leading to red cells becoming rigid and sickle shaped The onset of sickle cell disease is more likely in hypoxia, acidosis, low temperature or cellular dehydration Sickling is initially reversible but when potassium and water is lost, it becomes irreversible