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The Effect of Sickle Cell Disease on the Lung

Graham, LeRoy M. Jr. MD, FCCP

doi: 10.1097/01.cpm.0000140181.02195.b7
Colleagues in Respiratory Medicine

Sickle cell disease is the most common inherited disease in African Americans, affecting at least 50,000 individuals. At present, there is no cure. Despite improvement in survival, median life expectancy remains 42 years for males and 48 years for females. There is an increasing appreciation of both the acute and chronic pulmonary manifestations of sickle cell disease and their role in the morbidity and mortality of this disease. Acute manifestations include the acute chest syndrome, pulmonary thromboembolism, and episodic respiratory symptoms that suggest an asthma-phenotype. Chronic manifestations include abnormal pulmonary function, hypoxemia, evolving pulmonary hypertension, and exercise intolerance. Sickle chronic lung disease is a clinical continuum characterized by dyspnea, exercise intolerance, pleuritic chest pain, and progressive deterioration in pulmonary function. Timely cardiopulmonary assessment in both the acute and chronic setting provides ample opportunity for effective intervention. Recent insights into the pulmonary pathophysiology of sickle cell disease suggest ongoing lung injury may be related to ischemia/reperfusion, pulmonary vasculopathy, and alterations in nitric oxide metabolism. These insights offer the potential for novel therapies and management strategies that may alter the natural history of sickle cell disease.

Acute and chronic pulmonary manifestations of sickle cell disease account for much of the morbidity and mortality experienced by the nearly 50,000 Americans affected. This paper reviews these manifestations and emphasizes the importance of timely cardiopulmonary assessment as a basis for effective intervention. Recent insights into pulmonary pathophysiology are discussed as a basis for novel therapies and management strategies that might alter the natural history of this debilitating and as-yet incurable disease.

From the Department of Pediatrics, Morehouse School of Medicine, Georgia Pediatric Pulmonology Associates, PC Children's Healthcare of Atlanta, Atlanta, GA.

Address correspondence to: Dr. LeRoy M. Graham MD, FCCP, Georgia Pediatric Pulmonology Associates, Suite 450, 1100 Lake Hearn Drive, Atlanta, GA 30342. E-mail: LMG254@aol.com.

© 2004 Lippincott Williams & Wilkins, Inc.