Managing sickle cell disease
Advances are being made in the management of sickle cell disease for all age groups. Susan Claster and Elliott P Vichinsky discuss the progress in improving symptoms, problems unique to particular age groups, and the types of drugs and treatments currently under investigation
In 1973, the average life span of a patient with sickle cell disease was 14 years. Thirty years later, the development of comprehensive care models, day hospitals, and ongoing efforts in both basic sciences and clinical research has increased life expectancy to 50 years. Advances in the management of sickle cell disease have dramatically changed the outlook for patients (box 1).
Polymerisation of sickle haemoglobin is the catalyst in the development of vaso-occlusion. Additional factors are critical to the pathophysiology of sickling (the development of sickle cells in the blood), including abnormalities in coagulation, white cells, vascular endothelium, and damage to the membranes of red cells.1 The culmination of these factors results in red cells with reduced deformability that easily adhere to vascular endothelium, increasing the potential for decreased blood flow and vascular obstruction. As a result, patients develop both haemolytic anaemia and vasculopathy.2 Organ damage is also an ongoing