What interventions are available in secondary care to prevent chronic complications in people with sickle cell disease?
• The detailed management of sickle disease in secondary care is beyond the scope of this guidance. Children and adults will be followed up regularly in secondary care: • Parents or carers will receive comprehensive advice about caring for children with sickle cell disease. This should be reinforced in primary care if necessary. • Children will have regular psychological support. • Elective blood transfusion may be used in primary and secondary stroke prevention. • Hydroxycarbamide is used to prevent acute chest syndrome and acute painful crises. It has been found to improve survival. • Stem cell transplantation is the only cure, but this is usually done only in young people with very severe disease. • Surgery for sickle cell complications is used in the management of avascular necrosis. Splenectomy is recommended in people who have had two or more acute crises due to splenic sequestration.
