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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. High levels of bilirubin can lead to gallstones.
These are also called pigment stones. Sickle chest syndrome is a severe type of sickle cell crisis. It causes severe chest pain and is associated with symptoms such as cough, fever, sputum production, shortness of breath, and low blood oxygen levels. Abnormalities observed on chest X-rays can represent either pneumonia or death of lung tissue pulmonary infarction. The long-term prognosis for patients who have had sickle chest syndrome is worse than for those who have not had it.
All newborns in the United States are screened for sickle cell disease. Prebirth testing looks for the sickle cell gene in your amniotic fluid. In children and adults, one or more of the following procedures may also be used to diagnose sickle cell disease. Your doctor may want to test you for sickle cell anemia if you have any of the symptoms mentioned above.
Hb electrophoresis is always needed to confirm the diagnosis of sickle cell disease. It measures the different types of hemoglobin in the blood. Bone marrow transplant has been used to treat sickle cell anemia. Children younger than 16 years of age who have severe complications and have a matching donor are the best candidates.
The prognosis of the disease varies. Some patients have frequent and painful sickle cell crises. Others only rarely have attacks. Sickle cell anemia is an inherited disease. This can help you understand possible treatments, preventive measures, and reproductive options. A hemoglobin electrophoresis test is a blood test your doctor may ask you to take to screen for blood disorders. It can tell if your child is a carrier of sickle cell. It can also tell if your child has any of the diseases linked to the sickle cell gene.
Other blood tests may also be done. It will also depend on how severe the condition is. Early diagnosis and preventing further problems is important in treating this disease. Your child's healthcare provider will refer you to a hematologist. This is an expert in blood disorders. Other specialists may also be involved in your child's care.
Drinking plenty of water daily 8 to 10 glasses. This helps prevent and treat pain crises. In some cases, IV intravenous fluids may be needed. Blood transfusions. These are used to treat anemia, chronic pain, acute chest syndrome, and splenic sequestration, and to prevent stroke. Regular eye exams. These are done to screen for an eye condition called retinopathy. Stem cell transplant.
Transplants can cure some children with SCD. Studies of this treatment are ongoing. Transplants are only done at certain medical centers. This is a medicine that can reduce the number of sickle cells in the blood. It reduces complications, painful episodes crises , and hospital stays. Long-term anemia. This may lead to delayed healing and delayed growth and development. In severe cases, your child may need treatment in a hospital.
Over time, many episodes of acute chest syndrome can cause lasting permanent lung damage. When red blood cells build up in the spleen, it becomes enlarged and painful. The spleen can be damaged and scarred after many episodes of splenic sequestration. By age 8, many children with SCD have had their spleen removed. Or they may have lasting damage from repeated splenic sequestration.
The risk for infection is a major concern for children without a working spleen. Infection is the major cause of death in children younger than age 5. If the blood vessels to the brain are blocked, a stroke can occur. Serious long-term problems may result. A child who has had a stroke is more likely to have another one.
The sickle cells block the blood vessels in the penis, causing great pain. If not treated right away, this can lead to the inability to have an erection impotence.
Advances in preventive care and new medicines have reduced the life-threatening problems of sickle cell. But it is still a severe, chronic, and sometimes fatal disease.
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