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SICKLE CELL ANEMIA
Definition
Sickle Cell Anemia or SCD (Sickle Cell Disease), is a group of inherited red blood cell disorders. If you have sickle cell anemia, there is a problem with your hemoglobin (MedlinePlus 2016). People who have SCD inherit two abnormal hemoglobin genes, one from each parent. In all forms of SCD, at least one of the two abnormal genes causes a person's body to make hemoglobin S. When a person has two hemoglobin S genes, Hemoglobin SS, the disease is called sickle cell anemia (National Heart, Lung, and Blood Institute 2017) Sickle cell anemia is inherited from your parents, it is a condition in which there are not enough healthy blood cells to carry adequate oxygen throughout your body (Mayo Clinic 2018). Red blood are usually shaped like round discs. Those that have sickle cell disease, their red blood cells are sickled shape, or crescent moons. This can cause painful episodes, serious infections, chronic anemia, and can damage organs in the body (Miller 2015). Each person that has sickle cell anemia, can have different complications. Some children remain relatively healthy, while others are frequently hospitalized. Due to recent advancements in early diagnosis and treatment, most children born with this disorder grow up to live relatively normal lives (Children's Cancer & Blood Foundation 2012).
Instructional Strategies
Useful Websites
Usually sickle cell anemia is diagnosed in infancy, if you or your child develops any of the following problems, see your doctor right away or seek emergency medical care:
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Unexplained episodes of severe pain, such as pain in the abdomen, chest, bones or joints.
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Swelling in the hands or feet.
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Abdominal swelling, especially if the area is tender to the touch.
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Fever. People with sickle cell anemia have an increased risk of infection, and fever can be the first sign of infection.
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Pale skin or nail beds.
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Yellow tint to the skin or whites of the eyes.
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Signs or symptoms of stroke. If you notice one-sided paralysis or weakness in the face, arms or legs; confusion; trouble walking or talking; sudden vision problems or unexplained numbness; or a headache, call 911 or call your local emergency number right away.
If sickle cell anemia is not treated or diagnosed right away, it can lead to complications:
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Stroke. A stroke can occur if sickle cells block blood flow to an area of your brain. signs of stroke include seizures, weakness or numbness of your arms and legs, sudden speech difficulties, and loss of consciousness. If your baby or child has any of these signs and symptoms, seek medical treatment immediately. A stroke can be fatal.
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Acute chest syndrome. This life-threatening complication causes chest pain, fever and difficulty breathing. Acute chest syndrome can be caused by a lung infection of by sickle cells blocking blood vessels in your lungs. It might require emergency medical treatment with antibiotics and other treatments.
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Pulmonary hypertension. People with sickle cell anemia can develop high blood pressure in their lungs (pulmonary hypertension). This complication usually affects adults rather than children. Shortness of breath and fatigue are common symptoms of this condition, which can be fatal.
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Organ damage. Sickle cells that block blood flow through blood vessels immediately deprive the affected organ of blood and oxygen. In sickle cell anemia, blood is also chronically low on oxygen. Chronic deprivation of oxygen-rich blood can damage nerves and organs in your body, including your kidneys, liver and spleen. Organ damage can be fatal.
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Blindness. Sickle cells can block tiny blood vessels that supply your eyes. Over time, this can damage the portion of the eye that processes visual images (retina) and lead to blindness.
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Leg ulcers. Sickle cell anemia can cause open sores, called ulcers, on your legs.
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Gallstones. The breakdown of red blood cells produces a substance called bilirubin. A high level of bilirubin in your body can lead to gallstones.
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Priapism. Men with sickle cell anemia can have painful, long-lasting erections, a condition called priapism. As occurs in other parts of the body, sickle cells can block the blood vessels in the penis. This can damage the penis and lead to impotence.
Research
This link will take you to the National Heart, Lung, and Blood Institute where you can find whatever you need to learn about sickle cell anemia. This site can tell you what exactly sickle cell anemia is, what causes it, risk factors, and how you can treat it.
https://www.nhlbi.nih.gov/health-topics/sickle-cell-disease
This link will take you to the Children's Cancer and Blood Foundation where you can learn about this disease, learn about the different forms, find out what causes sickle cell anemia, and so much more.
http://childrenscbf.org/content/sickle-cell-anemia-0#.Wru9atPwbxQ
This link will take you to the Mayo Clinic website where there is so much information about sickle cell anemia that it is a little overwhelming but the information is very helpful.
Sources
National Human Genome Research Institute. Last reviewed May 9, 2016. https://www.genome.gov/10001219/learning-about-sickle-cell-disease/
MedlinePlus Trusted Health Information for You. Last updated March 13, 2018. Topic last reviewed August 3, 2016. https://medlineplus.gov/sicklecelldisease.html
Robin E. Miller, MD. Kids Health from Nemours. Last reviewed July 2015. https://kidshealth.org/en/parents/sickle-cell-anemia.html
Children's Cancer & Blood Foundation. Last reviewed 2012. http://childrenscbf.org/content/sickle-cell-anemia-0#.Wru9atPwbxQ
National Heart, Lung, and Blood Institute. Last reviewed 2017. https://www.nhlbi.nih.gov/health-topics/sickle-cell-disease
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