Deadly Lung Disorder in Sickle Cell Disease (cont.)

"This is similar to what occurred in the mid-nineties. We saw that use of a transcranial Doppler exam changed the screening and management of pediatric patients with sickle cell disease in that it defined important clinical decision points that successfully led to preventing - or at least improving - the odds of reducing strokes in children and adolescents," added Dr. Rodgers.

Of the 195 individuals evaluated in the current study, 20 percent with pulmonary hypertension died and all but two of the patients without the condition survived. Even patients with mild arterial pressure in the lungs had a high rate of fatality.

Sickle cell treatment continues to improve and people with the disease are living longer. "This," said co-investigator Dr. Oswaldo Castro, Acting Director, Howard University Center for Sickle Cell Disease, "may be why we are seeing such a high prevalence of pulmonary hypertension in sickle cell patients."

Since the study identified those sickle cell patients at the highest risk of death certain therapies targeting pulmonary hypertension could improve survival rates. "Several types of interventional therapies are now available but so far have been tried in only a few sickle cell patients," said Dr. Castro. He referenced inhalants such as oxygen and nitric oxide; blood exchanges through transfusions; and use of vaso-dilator drugs such as Viagra that open the blood vessels or arteries.

Detection of high blood pressure in the lungs may also account for the unexplained or mistakenly explained sudden deaths in adult patients with sickle cell disease. "We see a high frequency of sudden death in those with sickle cell and this study helps to clarify what is causing those fatalities; particularly in the absence of coronary artery disease (hardening of the arteries) as the cause," explained lead investigator Dr. Gladwin.

The co-authors agree that this study's findings represent an opportunity to address a major cause of disability and death in the adult sickle cell disease population - pulmonary hypertension. They believe the research data supports universal screening of individuals with sickle cell disease for this condition and clearly shows the immediate need for clinical trials to further investigate therapies that may combat a serious complication of sickle cell disease that is associated with an ominous outcome.

The research team included collaborators from the Critical Care Medicine Department, Warren Grant Magnuson Clinical Center, NIH; the Cardiovascular Branch, National Heart, Lung, and Blood Institute, NIH; the Hematology Branch, National Institute of Diabetes and Digestive and Kidney Diseases, NIH; and the Center for Sickle Cell Disease, Howard University College of Medicine.

Source: National Institutes of Health Press Release, February 25, 2004 (

Last Editorial Review: 12/28/2004