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- Dictionarysick·le cell a·ne·mi·a/ˌsikəl sel əˈnēmēə/
noun
- 1. a severe hereditary form of anemia in which a mutated form of hemoglobin distorts the red blood cells into a crescent shape at low oxygen levels. It is most common among those of African descent.
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Sickle cell disease is a group of inherited conditions that affect the red blood cells. The most serious type is sickle cell anaemia, which can cause painful episodes, infections, anaemia and organ damage.
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Sickle cell disease can cause a wide range of symptoms....
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Children and adults with sickle cell disease are supported...
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Sickle cell disease is caused by inheriting the sickle cell...
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Screening for sickle cell disease is offered to all pregnant...
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Testing for sickle cell carriers. A blood test can be done...
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A stem cell or bone marrow transplant replaces damaged blood...
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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 a genetic disorder that affects the shape and function of red blood cells. It can cause anemia, pain, infections, vision problems and organ damage. Learn more about the causes, risk factors, diagnosis and treatment of this condition.
Sep 30, 2024 · Sickle cell disease — also called sickle cell anemia — is a group of inherited disorders that affect hemoglobin , the major protein that carries oxygen in red blood cells. Normally, red blood cells are disc-shaped and flexible so they can move easily through the blood vessels.
Sickle cell anemia is a severe form of the inherited blood disorder, sickle cell disease. It’s caused by genetic changes (mutations) that lead to abnormal red blood cells and anemia. Symptoms are fatigue, infections and painful tissue damage.
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]
Sickle cell disease is caused by inheriting the sickle cell gene. It's not caused by anything the parents did before or during the pregnancy and you cannot catch it from someone who has it.
Sickle cell disease is a genetic disorder that causes abnormal red blood cells and chronic anemia. Learn about the symptoms, diagnosis, treatment, and complications of this inherited condition that affects people with African or Black American ancestry.
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