SICKLE CELL ASSOCIATION OF TEXAS MARC THOMAS FOUNDATION

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What is Sickle Cell Disease?


Sickle cell disease is an inherited life-threatening blood disorder that affects the red blood cells. These sickled cells carry less oxygen to parts of the body and when these misshaped cells are clumped together causes pain crisis. It affects African  Americans, Hispanics, Greeks, Italians, East Indians, Saudi Arabians, Asians, Syrians, Turks, Cypriots, Sicilians, Caucasians, and others. Sickle cell disease is quietly devastating. Its symptoms can occur in any part of the body. Persons with the disease extremely vulnerable to infections. They may also suffer from jaundice deterioration of joints, kidney infections, recurrent severe pain episodes, strokes, blindness and a shortened life expectancy. Sickle cell disease can lead to other ailments. These include strokes, kidney and liver problems. There is no cure.

‚ÄčSpecifically, Sickle cell disease is characterized by defective hemoglobin (a protein in red blood cells that carries oxygen to the tissues of the body).

Sickle cell disease inhibits the ability of hemogloblin in red blood cells to carry oxygen. Cells containing normal hemoglobin are smooth, disk-shaped, and flexible, like doughnuts without holes, so they can move through the vessels in our bodies easily. Cells containing sickle cell hemoglobin are stiff and sticky and form into the shape of a sickle or crescent, like the letter C, when they lose their oxygen. These sickle cells tend to cluster together, and cannot easily move through the blood vessels. The cluster causes a blockage in small arteries or capillaries and stops the movement of healthy, normal oxygen-carrying blood. This blockage is what causes the painful and damaging complications of sickle cell disease.

Sickle cells only live for about 10 to 20 days, while normal red blood cells can live up to 120 days. Also, sickle cells risk being destroyed by the spleen because of their shape and stiffness. The spleen is an organ that helps filter the blood of infections. Sickled cells get stuck in this filter and die. Due to the decreased number of healthy red blood cells circulating in the body, a person with sickle cell disease is chronically anemic. The spleen also suffers damage from the sickled cells blocking healthy oxygen carrying cells and typically infarcts in the first few years of life. Without a normal functioning spleen, these individuals are more at risk for infections. Infants and young children are at risk for life-threatening infections.


What is Sickle Cell Trait?


  • 1 in 12 African Americans carry the "silent" Sickle Cell Trait
  • 1 in 36 Hispanics carry the "silent" Sickle Cell Trait


Sickle Cell Trait is not Sickle Cell Disease. Sickle Cell Trait (SCT) occurs when one person inherits one sickle cell gene and one normal gene. People with SCT usually do not have any of the symptoms of sickle cell disease (SCD), but they can pass the trait on to their children, that is why education is vital. Knowing if you have SCT can be the difference of giving birth to a child with the disease or not. By knowing your status you can break the sickle cycle. To get tested contact us at 1-844-994-HOPE.