Common and Severe Complications of Sickle Cell Disease

A doctor talking to a male patient while holding a prescription bottleA team of scientists with the National Heart, Lung, and Blood Institute (NHLBI) of the National Institutes of Health has found that a hormone detected in a simple blood test can identify patients with sickle cell disease who have developed a life-threatening complication called pulmonary hypertension. The team has also found that the same hormone is a clear predictor of death in adult sickle cell patients.

The hormone, called brain natriuretic peptide or BNP, is released
by the heart ventricles and helps predict death in heart failure patients. The
new study is published in the July 19 issue of the Journal of the American
Medical Association.

“This is an important leap forward in research on sickle cell
disease,” said NHLBI Director Elizabeth G. Nabel, M.D. “Having a marker in the blood that will not only help identify sickle cell patients with this deadly complication but also predict those at the highest risk — will aid in the care and treatment of these patients.”

Sickle cell anemia is one of the most common genetic blood disorders in the United States. About 30 percent of sickle cell patients have pulmonary hypertension. In this condition, there is constant high blood pressure in the pulmonary arteries that supply the lungs. This pressure leads to narrowed arteries, causing the heart to work harder to pump blood.

Pulmonary hypertension often leads to heart failure and it is a major risk factor for death in adults with sickle cell disease. Currently, echocardiograms and other heart tests are used to diagnose pulmonary hypertension, but there has not been a blood test to help detect the condition.