Hydroxyurea is not a cure for sickle cell disease so at present should be offered only to patients whose clinical condition is severe and who have been fully informed about this and other treatment options. The tendency to form sickle cells depends on the concentration of haemoglobin S within the red cell relative to that of other haemoglobins present. Loss of water from the cells results in an increase in the concentration of haemoglobin S and an increased risk of sickling. Their use has not been conclusively proven and their effect at best seems modest which limits their clinical usefullness and uptake. There is compelling evidence that increased adherence by aggregates of sickled red cells to blood vessel wall lining occurs which results in tissue damage and blockage accompanied by pain.
Blood transfusion remains a cornerstone in the management of patients with sickle cell disease.
Both studies demonstrate the use of novel techniques for identifying individuals at risk of this devastating complications. The dilemma will be how to effectively produce matched blood in the quantities required for treatment while reducing the incidence of transfusion associated complications like iron overload, red cell antibody formation with consequent transfusion reactions, blood clots in implanted venous devices and blood borne infections. Attempts at curing sickle cell disease. When employed, it should be undertaken early in the course of the disease before the onset of organ damage which reduces the chances of a success. Our experience of carrying out transplantation is improving such that with time we may be able to push back the age limit which is currently 16 years.