Blood transfusions for treating acute chest syndrome in people with sickle cell disease

Sickle cell disease is an inherited blood condition affecting over 250 million people worldwide and is particularly common in Sub-Saharan Africa, South and Central America, Saudi Arabia, India and a number of Mediterranean countries. It is characterised by the presence of sickle-shaped red blood cells which are capable of blocking the blood vessels causing pain and severe damage to several organs of the body. People with sickle cell disease may have the acute onset of chest problems which may include fever, this is called acute chest syndrome. It may have several different causes which include infection and blockage of blood vessels. Fever, coughing, shortness of breath which are accompanied with chest pain are the most common symptoms. Treatment will depend on the individuals' clinical condition and the severity of the symptoms. Standard treatment consists of supportive care, antibiotics, intravenous fluids and blood transfusion, either simple or exchange, may also be indicated. Simple transfusions, which consist of packed red cells administered intravenously, are useful if there is moderate to severe illness. In severe or rapidly progressive illness exchange transfusion is used to remove the sickled cells and replace them with normal haemoglobin and thereby reducing the blood viscosity. The authors of this review did not find any trials showing how effective blood transfusions might be for treating acute chest syndrome in people with sickle cell disease and concluded that future research should provide evidence for people to make informed decisions about whether blood transfusions are effective.