Sickle Cell Information

Sickle Cell Disease - Sickle cell disease is an inherited blood disorder that affects red blood cells. People with sickle cell disease have red blood cells that contain mostly hemoglobin* S, an abnormal type of hemoglobin. Sometimes these red blood cells become sickle-shaped (crescent shaped) and have difficulty passing through small blood vessels. When sickle-shaped cells block small blood vessels, less blood can reach that part of the body. Tissue that does not receive a normal blood flow eventually becomes damaged. This is what causes the complications of sickle cell disease. There is currently no universal cure for sickle cell disease.

There are several types of sickle cell disease. The most common are: Sickle Cell Anemia (SS), Sickle-Hemoglobin C Disease (SC), Sickle Beta-Plus Thalassemia and Sickle Beta-Zero Thalassemia.

Sickle Cell Trait (AS) - An inherited condition in which both hemoglobin A and S are produced in the red blood cells, always more A than S. Sickle cell trait is not a type of sickle cell disease. People with sickle cell trait are generally healthy.

Sickle cell conditions are inherited from parents in much the same way as blood type, hair color and texture, eye color and other physical traits. The types of hemoglobin a person makes in the red blood cells depend upon what hemoglobin genes the person inherits from his or her parents. Like most genes, hemoglobin genes are inherited in two sets...one from each parent.

Source: www.sicklecelldisease.org  

Diagnosis & Complications - Sickle cell trait is diagnosed with a simple blood test. People at risk of having sickle cell trait can talk to a doctor or health clinic about getting this test. It is possible for a person with sickle cell trait to experience complications of sickle cell disease, such as splenic sequestration, pain crisis, and, rarely, sudden death.

This can happen under extreme conditions of:
  • High altitude (flying, mountain climbing, or cities with a high altitude)
  • Increased pressure (scuba diving)
  • Low oxygen (mountain climbing or exercising extremely hard, such as in military boot camp or when training for an athletic competition)
  • Dehydration (too little water in the body)
Source: www.cdc.gov  

Statistics - Sickle cell disease affects millions of people throughout the world and is particularly common among those whose ancestors come from sub-Saharan Africa, Spanish-speaking regions in the Western Hemisphere (South America, the Caribbean, and Central America), Saudi Arabia, India, and Mediterranean countries such as Turkey, Greece, and Italy.

In the United States, more than 70,000 people have sickle cell disease. Sickle cell disease occurs in 1 in every 500 African American births. 2 million people have sickle cell trait. 1 in 12 African Americans have sickle cell trait.

Data Source - National Heart, Lung and Blood Institute, National Institute of Health. Sickle cell anemia: Who is at risk? Available at: www.nhlbi.nih.gov/health/dci/Diseases/Sca/SCA_WhoIsAtRisk.html

Signs & Symptoms - Guidelines for Early Detection - A SIMPLE PAINLESS BLOOD TEST followed by a laboratory technique called Hemoglobin Electrophoresis will determine the type of hemoglobin you have. When you pass an electric charge through a solution of hemoglobin, distinct hemoglobins move different distances, depending on their composition. This technique differentiates between normal hemoglobin (A), Sickle hemoglobin (S), and other different kinds of hemoglobin (such as C, D, E, etc.).

Source: www.cdc.gov   

New Treatment - Research on bone marrow transplants, gene therapy, and new medicines for sickle cell anemia is ongoing. The hope is that these studies will provide better treatments for sickle cell anemia. Researchers also are looking for a way to predict the severity of the disease. Researchers are studying several new medicines for sickle cell anemia. These include:
  • Butyric acid. This is a food additive that may increase normal hemoglobin in the blood.
  • Nitric oxide. This medicine may make sickle cells less sticky and keep blood vessels open. People who have sickle cell anemia have low levels of nitric oxide in their blood.
  • Decitadine. This medicine increases hemoglobin F levels (this type of hemoglobin carries more oxygen). It may be a good choice instead of hydroxyurea.
Source: www.nhlbi.nih.gov

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