Sickle cell disease (CSD) is an inherited blood disorder, characterized primarily by chronic anemia and periodic episodes of pain. The underlying problem involves hemoglobin, a component of the red cells in the blood. The hemoglobin molecules in each red blood cell carry oxygen from the lungs to the body organs and tissues and bring back carbon dioxide to the lungs.

By occluding blood vessels sickle cells cause vascular injury. This vaso-occlusion, which is responsible for most of the severe complications of sickle cell disease, can occur wherever blood flows. This process produces the periodic episodes of pain and ultimately can damage the tissues and vital organs and lead to other serious medical problems.

One of every 600 African Americans in the United States has sickle cell anemia. Fortunately, because of significant advances in treatment and preventive care, persons with sickle cell disease now survive into their fifth and sixth decades.

Health maintenance is extremely important in SCD and must include measures to prevent specific disease complications or at least to facilitate their diagnosis and minimize their impact.

Management of a crisis involves control of pain, maintenance of adequate hydration, and treatment of any underlying precipitating factors.

Get the Facts... With your MediFocus Guide

The Medifocus Guide on Sickle Cell Anemia provides answers to the following important questions and medical issues:

• What are the most common symptoms of sickle cell anemia?
  
• Are there any recognized risk factors for developing sickle cell anemia?
  
• What kinds of medical tests are used to establish the diagnosis of sickle cell anemia?
  
• What is the current standard of care for the treatment of sickle cell anemia?
  
• What treatment options are available for the management of sickle cell anemia?
  
• Are there any promising new developments or potential breakthroughs in treatment?
  
• Who are the most notable medical authorities who specialize in sickle cell anemia?
  
• Where are the leading hospitals and centers of research for sickle cell anemia?
  
• What are the most important questions to ask my doctor about sickle cell anemia?
  

What Your Doctor Reads:

This MediFocus Guide contains an extensive listing of citations and abstracts of recent journal articles that have been published about this condition in trustworthy medical journals. This is the same type of information that is available to physicians and other health care professionals. A partial selection of journal articles that are abstracted in this MediFocus Guide includes:

• Sickle cell disease in childhood: Part I. Laboratory diagnosis, pathophysiology and health maintenance.
  American Family Physician. 2000
• Reactive species in sickle cell disease.
  Annals of the New York Academy of Sciences. 2000
• Blockade of adhesion of sickle cells to endothelium by monoclonal antibodies.
  New England Journal of Medicine. 2000
• Screening for sickle cell disease and thalassaemia: a systematic review with supplementary research.
  Health Technology Assessment (South Hampton, NY). 2000
• The management of pain in sickle cell disease.
  Pediatric Clinics of North America. 2000
• Lessons from the Stroke Prevention Trial in Sickle Cell Anemia (STOP) study.
  Journal of Child Neurology. 2000
• Laparoscopic splenectomy for splenic sequestration crisis.
  AORN Journal. 2000
• Neonatal screening for sickle cell disease.
  Cochrane Database of Systematic Reviews [computer file]. 2000
• Prophylactic versus selective blood transfusion for sickle cell anaemia during pregnancy.
  Cochrane Database of Systematic Reviews [computer file]. 2000
• Sickle hemoglobin (HbS) allele and sickle cell disease: a HuGE review.
  American Journal of Epidemiology. 2000

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