Sickle Cell

Sickle cell disease is an inherited blood disorder that affects the ability of red blood cells, or hemoglobin, to carry oxygen to the cells throughout the body.

The disease mostly affects African-Americans, Hispanics, and people of Caribbean descent, though it has been found in individuals of Middle Eastern, Indian, Latin American, Native American, and Mediterranean heritage. Each year, an estimated 1,000 children are born with sickle cell disease in the United States. One in 12 African-Americans has the sickle cell trait.

Symptoms and Crises

All major organs can be affected by sickle cell disease. The spleen, in particular, suffers damage from the sickle cells blocking healthy oxygen-carrying cells. Without a normal functioning spleen, individuals with sickle cell are more at risk for infections. Infants and young children are at risk for life-threatening infections.

Other risk factors include:

• Acute Chest Syndrome: Also called sickling in the chest, can be a life-threatening complication of sickle cell disease; often occurs suddenly, when the body is under stress from infection, fever, or dehydration, causing the sickle cells to stick together and block the flow of oxygen in the tiny vessels of the lungs; resembles pneumonia and can include fever, pain, and a violent cough
  
• Anemia: The most common symptom of all the sickle cell diseases; can make a person pale and tired when severe, due to reduced oxygenation of tissues; can delay normal growth and development
  
• Jaundice: Yellowing of the skin, eyes, and mouth, due to an excess of bilirubin building up in the liver as the fast-dying sickle cells are processed
  
• Pain Crisis or Sickle Crisis: Also called “vaso-occlusive crises,” occurring when blood flow is blocked by sickled cells stuck in the blood vessel; can occur anywhere, but most often occurs in the chest, arms, and legs; in infants and children under age 3, painful swelling of the fingers and toes
  
• Splenic Sequestration (pooling): A sudden drop in hemoglobin that can be life-threatening if not treated promptly and can cause the spleen to become enlarged and painful from the increase in blood volume; permanent spleen damage after repeated episodes
  
• Stroke: A sudden and severe complication caused by misshapen cells blocking the major blood vessels that supply the brain with oxygen; can result in devastating neurological impairment
  

More About Sickle Cells

Normal hemoglobin cells are smooth, round, and flexible, like the letter “O.” They move through the vessels in our bodies easily. Sickle cell hemoglobin cells are stiff and sticky and form into the shape of a sickle, or the letter “C,” when they lose their oxygen.

These sickle cells tend to cluster together, and can’t move easily through the blood vessels. The cluster causes a blockage and stops the movement of healthy, normal oxygen-carrying blood. This blockage causes the painful and damaging complications of sickle cell disease.

Sickle cells only live for about 15 days, while normal hemoglobin can live up to 120 days. Sickle cells also risk being destroyed by the spleen because of their shape and stiffness. The spleen helps filter infections from the blood, and sickle cells get stuck in this filter and die. Because of the decreased number of hemoglobin cells circulating in the body, a person with sickle cell disease is chronically anemic.