According to a study which was reported in the New England Journal of Medicine that children, who suffer from sickle cell anemia, reported 50% less incidence of silent strokes in the future, if they are regularly given blood transfusions.
Sickle cell anemia is a hereditary disorder in which the blood cells which are normally spherical in shape, attains sickle shape. It results in many health disorders, because of which the average life expectancy of a sickle cell patient is not more than 42 years.
The study points out to the need of regularly screening children suffering from sickle cell anemia for any evidence of silent stroke, something which is not done today.
Sickle cell anemia has no treatment and the argument was, why screen since there is no treatment. However, according to Dr. James Casella, vice chair of the clinical trial and director of pediatric hematology at Johns Hopkins Children’s Center in Baltimore, there seems to be something which can be done for sickle cell anemia children now. This is only a small step and there is more research required. One of the most important questions is –Do they have to have blood transfusion all their life?
Sickle cell anemia is an inherited disease and is mostly seen in people of South or Central American or Mediterranean and African descent. In America one in 500 black children are born with the condition, as per the data released by the U.S. National Institutes of Health. The RBC produced in sickle cell anemia is crescent shaped rather than disc shaped and the blood of such patients is sticky and tends to block the smooth flow of blood. One third of patients suffer from blood flow in the brain which can precipitate strokes and silent strokes. Silent strokes usually do not exhibit and symptoms and hence the name. However silent strokes leave behind damages to the brain tissues which can be dangerous in future.
The study involved the evaluation of Brain MRI scans of 1000 sickle cell patients in between the age of 5 and 15 years. 196 children with a previous incidence of stroke were arbitrarily assigned to one of two groups: one received monthly blood transfusions, and one stayed with usual care. In the next 3 years 6% of the transfusion group suffered from a fresh incidence of silent stroke while the same figure in the group which did not receive the blood transfusion was 14%.
The big question was to translate the findings of the study into clinical trials.
“The results of this trial are solid,” Steinberg said. “But it could be very difficult to do this in a community hospital setting, where the resources might not be there.”
Monthly transfusion of blood is not very easy and there is always a risk of iron overload. Another question is the duration of the blood transfusion to be given. In this study the effects of blood transfusion was studied for three years.
Steinberg agreed. “This is a difficult disease,” he said. “To make it better, the body has to make better red blood cells. Research is underway, and there are drugs under development. But they’re not here yet.”