Oxygen is transported from our lungs to all parts of our body by haemoglobin, which is a major component of red blood cells. Sickle cell disease is an inherited disorder of haemoglobin. In people with sickle cell disease red blood cells become rigid once they have given up their oxygen and are often shaped like crescents. These rigid cells can block blood vessels, which causes problems throughout the body, including the lungs. The two most common chronic chest complications due to sickle cell disease are pulmonary hypertension and chronic sickle lung disease. Pulmonary hypertension is high blood pressure in the pulmonary artery that supplies blood to the lungs. High pressure in this artery is associated with an increased risk of death. Chronic sickle lung disease arises as a result of lung damage and loss of lung tissue.
Regular blood transfusions for people with sickle cell disease reduce the amount of the person's own sickled cells in their blood, replacing them with donated, non-sickled cells. Regular transfusions have already been shown to reduce the risk of strokes in people with sickle cell disease.The aim of this review was to find out if regular long-term blood transfusions in people with sickle cell disease lead to a reduction in new chronic chest complications or slow the progression of any chronic chest complications that have already developed. It also aimed to consider death rates due to chronic chest complications and any adverse effects of the transfusion programme. Only trials that compared the use of long-term blood transfusions to an alternative (or no) treatment were to be included in this review.
Unfortunately, no trials were found. There is a need for suitably designed trials to allow informed decisions about whether blood transfusions are effective and safe for the treatment of pulmonary hypertension and chronic sickle lung disease.