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Sickle cell disease (SCD), also simply called sickle cell, is a group of hemoglobin-related blood disorders typically inherited. [2] The most common type is known as sickle cell anemia. [2] It results in an abnormality in the oxygen-carrying protein haemoglobin found in red blood cells. [2]
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•Complications
Symptoms of sickle cell anemia usually appear around 6 months of age. They vary from person to person and may change over time. Symptoms can include:
•Anemia. Sickle cells break apart easily and die. Typical red blood cells usually live for about 120 days before they need to be replaced. But sickle cells usually die in 10 to 20 days, leaving a shortage of red blood cells. This is known as anemia. Without enough red blood cells, the body can't get enough oxygen. This causes fatigue.
•Episodes of pain. Periodic episodes of extreme pain, called pain crises, are a major symptom of sickle cell anemia. Pain develops when sickle-shaped red blood cells block blood flow through tiny blood vessels to the chest, abdomen and joints.
The pain varies in intensity and can last for a few hours to a few days. Some people have only a few pain crises a year. Others have a dozen or more a year. A severe pain crisis requires a hospital stay.
Some people with sickle cell anemia also have chronic pain from bone and joint damage, ulcers, and other causes.
•Swelling of hands and feet. Sickle-shaped red blood cells block blood circulation in the hands and feet, which can cause them to swell.
See your healthcare professional right away if you or your child has symptoms of sickle cell anemia, including fever or stroke.
Infections often start with a fever and can be life-threatening. Because children with sickle cell anemia are prone to infections, seek prompt medical attention for a fever greater than 101.5 degrees Fahrenheit (38.5 degrees Celsius).
Seek emergency care for symptoms of stroke, which include:
•One-sided paralysis or weakness in the face, arms or legs.
•Confusion.
•Difficulty walking or talking.
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Sickle cell anemia is caused by a change in the gene that tells the body to make hemoglobin. Hemoglobin is the iron-rich compound in red blood cells that allows these cells to carry oxygen from the lungs to the rest of the body. The hemoglobin associated with sickle cell anemia causes red blood cells to become rigid, sticky and misshapen.
For a child to have sickle cell anemia, both parents must carry one copy of the sickle cell gene and pass both copies to the child.
If only one parent passes the sickle cell gene to the child, that child will have the sickle cell trait. With one typical hemoglobin gene and one sickle cell gene, people with the sickle cell trait make both typical hemoglobin and sickle cell hemoglobin.
Their blood might contain some sickle cells, but they generally don't have symptoms. They're carriers of the disease. That means they can pass the gene to their children.
For a baby to have sickle cell anemia, both parents must carry a sickle cell gene. In the United States, sickle cell anemia most commonly affects people of African, Mediterranean and Middle Eastern descent.
Sickle cell anemia can lead to a host of complications, including:
•Stroke. Sickle cells can block blood flow to the brain. Signs of stroke include seizures, weakness or numbness of the arms and legs, sudden speech difficulties, and loss of consciousness. If your child has any of these signs or symptoms, seek medical treatment right away. A stroke can be fatal.
•Acute chest syndrome. A lung infection or sickle cells blocking blood vessels in the lungs can cause this life-threatening complication. Symptoms include chest pain, fever and difficulty breathing. Acute chest syndrome might need emergency medical treatment.
•Avascular necrosis. Sickle cells can block the blood vessels that supply blood to the bones. When the bones don't get enough blood, joints may narrow and bones can die. This can happen anywhere but most often happens in the hip.
•Pulmonary hypertension. People with sickle cell anemia can develop high blood pressure in their lungs. This complication usually affects adults. Shortness of breath and fatigue are common symptoms of this condition, which can be fatal.
•Organ damage. Sickle cells that block blood flow to organs deprive the affected organs of blood and oxygen. In sickle cell anemia, blood also is low in oxygen. This lack of oxygen-rich blood can damage nerves and organs, including the kidneys, liver and spleen, and can be fatal.
If you carry the sickle cell trait, it can help to see a genetic counselor before you get pregnant. A counselor can help you understand your risk of having a child with sickle cell anemia. You also can learn about possible treatments, preventive measures and reproductive options.
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By Mayo Clinic Staff
Dec 22, 2023
1.Sickle cell disease. National Heart, Lung, and Blood Institute. https://www.nhlbi.nih.gov/health-topics/sickle-cell-disease. Accessed Aug. 4, 2023.
2.Field JJ, et al. Overview of the management and prognosis of sickle cell disease. https://www.uptodate.com/contents/search. Accessed Aug. 4, 2023.
•Ask the Mayo Mom: Managing sickle cell disease in children and teens
•Mayo Clinic Minute: Sickle cell disease explained
Sickle cell anemia is an inherited disorder that affects the shape and function of red blood cells. Learn about the signs, causes, risk factors, and possible treatments of this condition that can cause pain, infection, organ damage and stroke.
Apr 22, 2024 · Sickle cell disease is a genetic disorder that affects hemoglobin and causes red blood cells to become sickle-shaped and block blood flow. Learn about the symptoms, complications, and new therapies for this lifelong illness from the NHLBI.
3 days ago · Learn about sickle cell disease (SCD), a group of inherited blood disorders that affect red blood cells and cause pain and other serious problems. Find out the causes, diagnosis, prevention, treatment, and resources for SCD from the Centers for Disease Control and Prevention (CDC).
Aug 3, 2023 · Learn about sickle cell disease, a genetic disorder that affects red blood cells and causes pain, anemia and organ damage. Find out the different types, how they are inherited, and how to manage and prevent complications.
Sickle cell disease is an inherited blood disorder that affects the oxygen-carrying protein in red blood cells. It can cause pain, anemia, infections, stroke, and other complications. Learn about the diagnosis, prevention, and treatment options from Johns Hopkins Medicine.
Learn about sickle cell disease, a group of inherited red blood cell disorders that cause hard and sticky cells. Find data, statistics, communication resources, and steps to better health from CDC.
