This breaking apart of RBCs is called chronic hemolysis. RBCs generally live for about days. Sickle cells live for a maximum of 10 to 20 days. Hand-foot syndrome occurs when sickle-shaped RBCs block blood vessels in the hands or feet. This causes the hands and feet to swell. It can also cause leg ulcers. Swollen hands and feet are often the first sign of sickle cell anemia in babies. Splenic sequestration is a blockage of the splenic vessels by sickle cells.
It causes a sudden, painful enlargement of the spleen. The spleen may have to be removed due to complications of sickle cell disease in an operation known as a splenectomy. Some sickle cell patients will sustain enough damage to their spleen that it becomes shrunken and ceases to function at all. This is called autosplenectomy. Patients without a spleen are at higher risk for infections from bacteria such as Streptococcus , Haemophilus , and Salmonella species. Delayed growth often occurs in people with SCD.
Children are generally shorter but regain their height by adulthood. Sexual maturation may also be delayed. Seizures, strokes, or even coma can result from sickle cell disease. They are caused by brain blockages. Immediate treatment should be sought. Since SCD interferes with blood oxygen supply, it can also cause heart problems which can lead to heart attacks , heart failure , and abnormal heart rhythms. Damage to the lungs over time related to decreased blood flow can result in high blood pressure in the lungs pulmonary hypertension and scarring of the lungs pulmonary fibrosis.
These problems can occur sooner in patients who have sickle chest syndrome. Lung damage makes it more difficult for the lungs to transfer oxygen into the blood, which can result in more frequent sickle cell crises.
Priapism is a lingering, painful erection that can be seen in some men with sickle cell disease. This happens when the blood vessels in the penis are blocked. It can lead to impotence if left untreated. Gallstones are one complication not caused by a vessel blockage. Instead, they are caused by the breakdown of RBCs. A byproduct of this breakdown is bilirubin. Hemoglobin electrophoresis is a blood test that can determine if a person is a carrier of sickle cell, or has any of the diseases associated with the sickle cell gene.
Your doctor will consider your age, overall health and other factors when determining the best treatment for you. Early diagnosis and prevention of complications is critical in sickle cell disease treatment. Treatment aims to prevent organ damage including strokes, prevent infection, and treat symptoms. Treatment may include:. Any and all major organs are affected by sickle cell disease. The liver, heart, kidneys, gallbladder, eyes, bones, and joints can suffer damage from the abnormal function of the sickle cells and their inability to flow through the small blood vessels correctly.
Problems may include the following:. Sickle cell disease is a life-long condition. Although the complications of sickle cell disease may not be able to be prevented entirely, living a healthy life-style can reduce some of the complications. It is important to eat a healthy diet with lots of fruits, vegetables, whole grains, and protein, and drink lots of fluids. Do not take decongestants because they cause constriction of blood vessels and could trigger a crisis.
Other factors that may trigger a crisis include high altitudes, cold weather, swimming in cold water, and heavy physical labor. Avoid infections by getting an annual flu shot, washing your hands frequently, avoiding those who are sick, and getting regular dental exams. Health Home Conditions and Diseases. What causes sickle cell disease? Sickle cell is an inherited disease caused by a defect in a gene. A person will be born with sickle cell disease only if two genes are inherited—one from the mother and one from the father.
A person who inherits just one gene is healthy and said to be a "carrier" of the disease. A carrier has an increased chance of having a child with sickle cell disease if he or she has a child with another carrier. What are the risk factors for sickle cell disease? Having a family history of sickle cell disease increases your risk for the disease.
In the United States, it mainly affects African Americans. What are the symptoms of sickle cell disease? Symptoms and complications may include: Anemia.
Because sickled cells are short-lived or destroyed, there are less red blood cells available in the body. This results in anemia. Severe anemia can make you feel dizzy, short of breath, and tired. Pain crisis, or sickle crisis. People with sickle cell disease have it because they inherited two sickle cell genes , one from each parent. In some types of sickle cell disease, people can inherit a sickle cell gene from one parent and a different abnormal hemoglobin gene from the other parent.
A person who inherits the sickle cell gene from only one parent will not develop the disease, but will have something called sickle cell trait. People with sickle cell trait often don't have any signs of the disease, but they can pass the sickle cell gene to their children. Stem cell transplant also called bone marrow transplant is the only known cure for sickle cell disease. Transplants are complex and risky procedures, and for now are an option only for some patients.
Scientists are studying gene therapy as a treatment for sickle cell anemia. One day, it's hoped that doctors can stop the disease by changing or replacing the abnormal gene that causes it. But even without a cure, people with sickle cell disease can lead fairly normal lives if they follow their treatment plan.
Their plan might involve:. Reviewed by: Robin E. Miller, MD. Larger text size Large text size Regular text size. What Is Sickle Cell Disease? Episodes of pain. Periodic episodes of 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 your chest, abdomen and joints.
Pain can also occur in your bones. The pain varies in intensity and can last for a few hours to a few weeks. Some people have only a few pain crises a year. Others have a dozen or more pain crises a year. A severe pain crisis requires a hospital stay.
Some adolescents and adults with sickle cell anemia also have chronic pain, which can result from bone and joint damage, ulcers, and other causes. Sickle cell anemia is usually diagnosed in infancy through newborn screening programs. If you or your child develops any of the following problems, see your doctor right away or seek emergency medical care:.
Sickle cell anemia is caused by a mutation in the gene that tells your body to make the iron-rich compound that makes blood red and enables red blood cells to carry oxygen from your lungs throughout your body hemoglobin.
In sickle cell anemia, the abnormal hemoglobin causes red blood cells to become rigid, sticky and misshapen. If only one parent passes the sickle cell gene to the child, that child will have the sickle cell trait.
With one normal hemoglobin gene and one defective form of the gene, people with the sickle cell trait make both normal hemoglobin and sickle cell hemoglobin.
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