Sickle cell trait occurs when a person inherits a sickle cell gene from just one parent. It's not the same as sickle cell disease, in which a person inherits two sickle cell genes, one from each parent. People with sickle cell disease have just one kind of hemoglobin (hemoglobin S), which turns their normal, round red blood cells into abnormally curved (sickle) shapes. But people with sickle cell trait have both normal hemoglobin (hemoglobin A) and hemoglobin S, so they rarely have symptoms of sickle cell disease. But they are carriers and can pass the sickle cell gene to their children.
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People who inherit one sickle cell gene and one normal gene have sickle cell trait (SCT). 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.
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A blood transfusion is when a donor's blood is transferred to a patient. The blood is transferred into the patient's body through a vein. A blood transfusion can make up for the loss of blood.
The main reasons why a child may need a blood transfusion are:
• Loss of blood during surgery or from an injury or an illness.
• Too little blood is being made. Some illnesses and treatments can prevent the bone marrow from making blood (for example, chemotherapy decreases production of new blood cells).
• To prevent complications from a blood or bleeding disorder, such as sickle cell disease, thalassemia, or anemia caused by kidney disease, hemophilia, or von Willebrand disease.
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Sickle cell disease is a blood disorder that's inherited — meaning it's passed down from parents to their children. Babies are born with sickle cell disease when they inherit two abnormal genes (one from each parent). These genes cause the body's red blood cells to change shape.
Normal red blood cells are shaped like discs or donuts with the centers partly scooped out. They are soft and flexible so they can easily move through very small blood vessels and deliver oxygen throughout a person's body.
Sickle cells are stiffer. Instead of being disc shaped, they're curved like crescent moons, or an old farm tool known as a sickle. That's where the disease gets its name.
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Sickle cell disease (SCD) is a disease of hemoglobin, a molecule that carries oxygen within red blood cells. In SCD, defective hemoglobin causes the red blood cells to become stiff instead of flexible and form a sickle or a crescent.
SCD is an inherited disease, meaning that the disease is passed on from parents to children through genes. Genes are instructions that tell the body how to make all the different substances the body needs to work properly. Since SCD is inherited, the disease is present at birth.
In SCD, a child inherits two defective hemoglobin genes (called hemoglobin S), one from each parent. This causes the body to make faulty hemoglobin. If a child inherits just one defective hemoglobin S gene, and the other hemoglobin gene is normal (hemoglobin A), he or she will have sickle cell trait, and will not have any SCD symptoms.
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Sickle cell disease is a group of disorders that affects hemoglobin, the molecule in red blood cells that delivers oxygen to cells throughout the body. People with this disorder have atypical hemoglobin molecules called hemoglobin S, which can distort red blood cells into a sickle, or crescent, shape.
Signs and symptoms of sickle cell disease usually begin in early childhood. Characteristic features of this disorder include a low number of red blood cells (anemia), repeated infections, and periodic episodes of pain. The severity of symptoms varies from person to person. Some people have mild symptoms, while others are frequently hospitalized for more serious complications.
The signs and symptoms of sickle cell disease are caused by the sickling of red blood cells. When red blood cells sickle, they break down prematurely, which can lead to anemia. Anemia can cause shortness of breath, fatigue, and delayed growth and development in children. The rapid breakdown of red blood cells may also cause yellowing of the eyes and skin, which are signs of jaundice. Painful episodes can occur when sickled red blood cells, which are stiff and inflexible, get stuck in small blood vessels. These episodes deprive tissues and organs of oxygen-rich blood and can lead to organ damage, especially in the lungs, kidneys, spleen, and brain. A particularly serious complication of sickle cell disease is high blood pressure in the blood vessels that supply the lungs (pulmonary hypertension). Pulmonary hypertension occurs in about one-third of adults with sickle cell disease and can lead to heart failure.
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Sickle cell anemia is a blood disease that affects red blood cells. Normal red blood cells are round. In people with sickle cell anemia, hemoglobin – a substance in red blood cells – becomes defective and causes the red blood cells to change shape. The faulty hemoglobin is called hemoglobin S, (HgbS) and it replaces normal hemoglobin called: hemoglobin A (HgbA). Over time, the red blood cells become rigid and shaped like crescent moons or sickles.
The sickle-shaped red blood cells:
Clog blood vessels, causing episodes of pain and cutting off oxygen to tissues and organs.
Get trapped in the spleen (an organ that gets rid of old cells) where they are destroyed. The body cannot replace the lost cells fast enough. As a result, the body has too few red blood cells, a condition known as anemia.
Sickle cell anemia is a serious disease that can require frequent hospital stays. Children and young adults can die from the disease.
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Sickle cell disease is a genetic blood disorder. That means it’s inherited. It’s marked by abnormally shaped red blood cells that can ultimately lead to severe attacks of pain. Doctors call these attacks “crises.”
It’s passed down through families. If your parents have the sickle cell trait, you may get sick from the disease, or you may only “carry” the gene and never have symptoms. Learning how the trait is inherited can help you better understand what to expect.
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At some point in your life, you’ll accidentally drop something into the sink. If it’s small and of little value, like a marble, it’s not an issue. You let the water run and flush it through. But if your kid slips a big piece of Lego down the drain, you’re going to have some bigger problems when it gets stuck in your pipes.
That’s kind of like what happens during a sickle cell crisis. Red blood cells are usually round and have some give to them -- their shape lets them move easily throughout your body. But when you have sickle cell disease (SCD), some cells are curved -- like a sickle--and hard. They don’t flow as easily, and they can get stuck in the small blood vessels of your chest, belly, and joints. That’s when you have a sickle cell crisis.
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Sickle cell disease (SCD) is the most common inherited blood disorder. That means it’s passed down through families. You’re born with SCD. It is not something you catch or develop later in life.
The disease gets its name because when you have SCD, your red blood cells look like a sickle, which is a C-shaped farm tool.
Red blood cells contain a molecule called hemoglobin, which carries oxygen throughout the body. In a healthy person, hemoglobin is smooth, round, and flexible. That allows red blood cells to glide easily through your bloodstream. But if you have SCD, the hemoglobin’s shape is abnormal.
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