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The Scientific World Journal
Volume 2012, Article ID 949535, 55 pages
Review Article

Beyond the Definitions of the Phenotypic Complications of Sickle Cell Disease: An Update on Management

1Cardeza Foundation and Department of Medicine, Thomas Jefferson University, 1015 Walnut Street, Philadelphia, PA 19107, USA
2Wilmer Ophthalmological Institute, Johns Hopkins Hospital, 400 North Broadway, Baltimore, MD 21267, USA
3Department of Pediatrics, Aflac Cancer Center and Blood Disorders Service, Children’s Healthcare of Atlanta, Emory University School of Medicine, Atlanta, GA 30322, USA
4Department of Hematology, St. Jude Children’s Research Hospital, Memphis, TN 38105, USA
5Children’s Hospital & Research Center at Oakland, 744 52nd Street, Oakland, CA 94609, USA
6Division of Hematology, Children’s National Medical Center, 111 Michigan Avenue, N.W., Washington, DC 20010, USA
7Cincinnati Children’s Hospital Medical Center, Cincinnati, OH 45229, USA

Received 13 February 2012; Accepted 1 April 2012

Academic Editors: O. O. Akinyanju, M. F. Sonati, and F. Traina

Copyright © 2012 Samir K. Ballas et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


The sickle hemoglobin is an abnormal hemoglobin due to point mutation (GAG → GTG) in exon 1 of the β globin gene resulting in the substitution of glutamic acid by valine at position 6 of the β globin polypeptide chain. Although the molecular lesion is a single-point mutation, the sickle gene is pleiotropic in nature causing multiple phenotypic expressions that constitute the various complications of sickle cell disease in general and sickle cell anemia in particular. The disease itself is chronic in nature but many of its complications are acute such as the recurrent acute painful crises (its hallmark), acute chest syndrome, and priapism. These complications vary considerably among patients, in the same patient with time, among countries and with age and sex. To date, there is no well-established consensus among providers on the management of the complications of sickle cell disease due in part to lack of evidence and in part to differences in the experience of providers. It is the aim of this paper to review available current approaches to manage the major complications of sickle cell disease. We hope that this will establish another preliminary forum among providers that may eventually lead the way to better outcomes.