What to Know About Sickle Cell Disease

Sickle cell disease affects millions of people all over the world. This inherited blood disorder is characterized by flawed hemoglobin—the protein in red blood cells that carries oxygen to the tissues of the body. While normal red blood cells are smooth, disk-shaped and move easily through the blood vessels, cells with sickle cell hemoglobin are stiff, sticky and have the tendency to clump together. The resulting blockages of small blood vessels and the flow of healthy oxygen-rich blood can cause pain and other complications. 

Here are four things you may not know about sickle cell disease. 
 

1. It’s a disease inherited from your parents.
   Sickle cell disease is caused by a defect in a gene—a person must inherit two of these genes, one from their father and one from their mother, in order to have sickle cell disease.
    
   “A person who inherits just one gene is considered healthy, but is a carrier of the disease and more likely to pass it on if they have a child with someone who has the disease or is also a carrier,” said Erev Tubb, MD, FACP, medical oncologist, hematologist and medical director of Inspira’s Leading-Edge Cancer Center.. For parents who are both carriers of the sickle cell gene, there is a one in four chance that their child will have sickle cell disease. 
    
2. It occurs more often in people from countries where malaria is or was common. 
   This disease is particularly common among those whose ancestors are from sub-Saharan Africa, South American, the Caribbean, Central America, Saudi Arabia, India, Turkey, Greece and Italy. “Because of the occurrence in countries where malaria is or was common, it’s believed that people who carry the sickle cell gene are less likely to have severe forms of malaria,” said Dr. Tubb. 
   
   In the United States, sickle cell disease mainly affects African Americans. According to the Centers for Disease Control and Prevention (CDC), it's estimated that one in 13 Black or African American babies is born with the sickle cell trait. 
    
3. The symptoms may mimic other blood disorders. 
   The most common complications for sickle cell disease include pain episodes, anemia, stroke and increased infections. “Because it is a blood disorder, sickle cell disease can affect all major organs,” said Dr. Tubb. “The liver, heart, gallbladder, eyes, kidneys, bones and joints are all at risk of being damaged by the abnormal function of sickle cells.” 
   
   Sickle cell disease is most often found during routine newborn screening tests, but it can be diagnosed at any time through a blood test, so it’s important to visit your doctor if you have any concerning symptoms that could be related to a blood disorder. 
    
4. It’s a lifelong condition. 
   Currently, the only cure for sickle cell disease is a bone marrow or stem cell transplant—risky procedures that have serious side effects. However, mild symptoms can be managed with pain medications, proper hydration, vaccinations and frequent visits to their health care provider. 

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