Sickle cell disease is a genetic disease that occurs predominantly in people of African descent. Patients suffer from severe attacks of pain from blood vessels being blocked by red blood cells that become rigid and form a sickle shape when de-oxygenated. High blood pressure in the lungs is known as pulmonary hypertension. Specialists say it has nothing to do with blood pressure measured by a cuff on your arm. Instead, pulmonary hypertension occurs when the blood vessels that supply the lungs narrow and their walls thicken, so they can't carry as much blood. The pressure builds up and the heart has to work harder trying to force the blood through. Eventually the heart can't keep up and patients become tired, dizzy and short of breath.
A new study pinpoints a major complication in adults with sickle cell disease that can often lead to death. The research shows nearly one-third of adults with sickle cell disease develop high blood pressure in their lungs and that the condition increases their risk of death. For the study researchers followed 195 sickle cell disease patients for two years. Each patient underwent a Doppler echocardiography, which is a test that uses waves to see the heart. Researchers say 32 of the patients suffered from pulmonary hypertension.During the study period, researchers say 20 percent of the patients with pulmonary hypertension died and all but two of the patients without the condition survived. Researchers say even patients with mild arterial pressure in the lungs had a high rate of fatality.
Thus researchers say the Doppler echocardiography is a reasonably priced, non-invasive test that should be offered to adults with sickle cell disease. They say once the patients are identified as having pulmonary hypertension, there are several interventional therapies that could help. They also point out that there is a high rate of sudden death in sickle cell patients and that may be explained by pulmonary hypertension. Researchers say this study gives doctors an opportunity to address a major cause of disability and death in the adult sickle cell disease population and move forward with clinical trials to investigate therapies.