Sickle Cell Disease Health Article

Psychosocial support

As in any lifelong, chronic disease, comprehensive care is important. Assistance with the emotional, social, family-planning, economic, vocational, and other consequences of sickle cell disease can enable affected individuals to better access and benefit from their medical care.

Prognosis

Sickle cell disease is characteristically variable between and within affected individuals. Predicting the course of the disorder based solely on genes is not possible. Several factors aside from genetic inheritance determine the prognosis for affected individuals, including the frequency, severity, and nature of specific complications in any given individual. The availability and access of comprehensive medical care also plays an important role in preventing and treating serious, acute complications, which cause the majority of sickle cell-related deaths. For those individuals who do not experience such acute events, life-expectancy is probably substantially greater than the average for all people with sickle cell disease. The impact of recent medical advances supports the hypothesis that current life-expectancies may be significantly greater than those estimated in the early 1990s. At that time, individuals with SS disease lived to the early-to mid-40s, and those with SC disease lived into the upper 50s on average. With early detection and comprehensive medical care, most people with sickle cell disease are in fairly good health most of the time. Most individuals can be expected to live well into adulthood, enjoying an improved quality of life including the ability to choose a variety of education, career, and family-planning options for themselves.

BOOKS

Beutler, Ernest. "The Sickle Cell Diseases and Related Disorders." In Williams Hematology, edited by Ernest Beutler, et al. 5th ed. New York: McGraw-Hill, 1995.

Bloom, Miriam. Understanding Sickle Cell Disease. Jackson, MS: University Press of Mississippi, 1995.

Embury, Stephen H., et al., eds. Sickle Cell Disease: Basic Principles and Clinical Practice. New York: Raven Press, 1994.

Reid, C.D., S. Charache, and B. Lubin, eds. Management and Therapy of Sickle Cell Disease. 3rd ed. National Institutes of Health Publication No. 96-2117, 1995.

PERIODICALS

Adams, R. J., et al. "Prevention of a First Stroke by Transfusions in Children with Sickle Cell Anemia and Abnormal Results on Transcranial Doppler Ultrasonography." New England Journal of Medicine, 339 (1998): 5-11.

Davies, Sally C. "Management of Patients with Sickle Cell Disease." British Medical Journal 315 (September 13,1997): 656.

Golden, C., L. Styles, and E. Vichinsky. "Acute Chest syndrome and Sickle Cell Disease." Current Opinion in Hematology 5 (1998): 89-92.

Hoppe, C., L. Styles, and E. Vichinsky. "The Natural History of Sickle Cell Disease." Current Opinion in Pediatrics 10(1998): 49-52.

Kinney, T.R., et al. "Safety of Hydroxyurea in Children with Sickle Cell Anemia: Results of the HUG-KIDS Study, A Phase I/II Trial." Blood 94 (1999): 1550-1554.

Platt, O., et al. "Mortality in Sickle Cell Disease: Life Expectancy and Risk Factors for Early Death." New England Journal of Medicine 330 (1994): 1639-1644.

Reed, W., and E.P. Vichinsky. "New Considerations in the Treatment of Sickle Cell Disease." Annual Review of Medicine 49 (1998): 461.

Schnog, J.B., et al. "New Concepts in Assessing Sickle Cell Disease Severity." American Journal of Hematology 58 (1998): 61-66.

Serjeant, Graham R. "Sickle-Cell Disease." The Lancet 350 (September 6, 1997): 725.

Singer, S.T., et al. "Erythrocytapheresis for Chronically Transfused Children with Sickle Cell Disease: An Effective Method for Maintaining a Low Hemoglobin S Level and Reducing Iron Overload." Journal of Clinical Apheresis 14 (1999): 122-125.

Xu, K., et. al. "First Unaffected Pregnancy Using Preimplantation Genetic Diagnosis for Sickle Cell Anemia." Journal of the American Medical Association 281 (1999): 1701-1706.

ORGANIZATIONS

Sickle Cell Disease Association of America, Inc. 200 Corporate Point Suite 495, Culver City, CA 90230-8727. (800) 421-8453. <Scdaa@sicklecelldisease.org>. <http://sicklecelldisease.org/>.

Jennifer Bojanowski