People who are diagnosed with sickle cell disease should know what the result means and what symptoms to watch out for. This includes anemia, blood transfusions, and skin problems. However, it is important to note that a negative result does not necessarily mean the patient has Sickle Cell Disease. A person can also be sick with other conditions. This is where an Hb electophoresis test can help. While this is not a sure-fire way of diagnosing sickle cell disease, it can be a useful tool.
Blood transfusions
Patients with sickle cell disease may require blood transfusions. Transfusions replace sickle red blood cells with healthy blood. They involve the transfer of blood through the veins. These procedures can be done at a clinic, hospital, or even in the patient’s own home. Different types of blood can be used during transfusions, including whole blood, red blood cells, white-blood cells, platelets, and plasma. Partial exchange transfusions, in which a small amount of sickle blood is removed and replaced with healthy blood, may also be performed.
There are several risks with blood transfusions for sickle-cell-negative patients. In rare cases, the transfusion will lead to alloimmunization, which occurs when an individual’s immune system produces antibodies against donated blood. Approximately 30% of people with SCD who receive blood transfusions develop this type of reaction. The needle site may be sore or numb. Some people may even develop an infection, but this is rare. Another risk is delayed hemolytic transfusion reaction (DHTR), which occurs when a person develops antibodies to a donor’s blood. This can be life-threatening.
People with sickle cell disease should never receive blood transfusions containing RBCs containing Hgb S. A number of laboratories now test donor units for Hgb S before they are released. Donor units that are leukocyte reduced have the added benefit of decreasing the risks of cytomegalovirus transmission and febrile non-hemolytic transfusion reactions. Leukocyte reduced blood products can also reduce the risk of immune suppression, HLA alloimmunization, and RBC alloimmunization.
Before undergoing blood transfusion, the patient will be given a type and screen test to confirm their blood type. The doctor may also order a ferritin blood test to monitor the iron levels in the patient. The medical team will then access the patient’s blood vessels with an IV catheter. A large peripheral IV line may be sufficient for simple blood transfusions. Repeated or frequent transfusions may require a permanent access line. During the procedure, the doctor or nurse will review the patient’s medical history and any allergies.
Kidney problems
Whether you are sickle cell negative or positive for sickle cells is not always an easy choice. The fact of the matter is that kidney problems associated with SCD are quite common. The presence of this genetic abnormality is closely associated with higher rates of mortality and morbidity. This disease is a monogenic condition, affecting millions of people worldwide. This condition is particularly prevalent in malaria-endemic regions, where the sickle cells evolved as a protective mechanism.
If you have the trait, your doctor may recommend that you undergo a sickle cell test to see whether you are affected. This blood test can show you whether or not you have sickle cell disease, which is characterized by two sickle cell alleles and one normal beta allele. This condition has been associated with an increased incidence of COVID-19 mortality and kidney failure among adults with this blood disorder. If your family history is overwhelmingly African, you might have a higher risk of developing this condition than those without the trait.
In addition to kidney problems, the presence of sickle cells can cause a drop in blood oxygen levels in your body. This condition is often accompanied by seizures, numbness, loss of consciousness, and abdominal pain. Severe symptoms may also occur, and you should seek medical attention immediately. A large number of sickle cells can get trapped in your spleen and cause enlargement there. It’s best to regularly feel your child’s spleen to make sure it doesn’t enlarge.
Although the link between SCT and kidney problems is fairly well established, the pathophysiology of the condition is not fully understood. The common symptoms of SCT and kidney disease suggest that both disorders may result in injury to the renal medulla, which provides an optimum environment for sickling of RBCs. The ischaemic injury caused by the clinically silent episodes of sickling may also contribute to ongoing renal damage, such as progression to renal medullary carcinoma.
Skin problems
Sickle cells are short-lived, non-dividing blood cells that are abnormally large. These cells die faster than the body’s ability to filter them. This causes bilirubin to build up in the body, leading to jaundice. When sickled cells get stuck in a blood vessel, a pain crisis occurs. Pain is typically felt in the chest, although it can occur anywhere. Swelling of the fingers and hands is also common, especially in young children. In addition to the skin problems associated with sickle disease, the patient may have fever, shortness of breath, and pain in the fingers. In this case, the child should be treated as soon as possible in a hospital.
In addition to skin problems, patients with sickle cell disease may experience fever. While fever is not usually a symptom, it can be a warning sign of serious anaemia. The disease usually comes on gradually or suddenly, depending on the severity of the anaemia. In both cases, urgent treatment may be necessary. In addition, babies and small children may have pale skin. It is easiest to see pale skin on the eyelids, fingernails, and lips.
Affected individuals can also suffer from a stroke, which occurs when sickle cells block the blood flow to the brain. Symptoms of a stroke include seizures, numbness, and weakness, as well as loss of consciousness. A stroke is a dangerous condition that requires emergency medical attention. It can also lead to lung infections and require emergency treatment. It is also possible to develop skin problems associated with sickle cell disease, such as a rash or a bump.
Stroke
Many people with sickle cell disease also have a high risk for stroke. This is because the sickle-shaped cells can block the blood vessels that supply the brain. A person who has suffered a stroke may experience symptoms such as a severe headache, drooping facial muscles, speech difficulties, or even loss of consciousness. But not everyone with sickle cell disease will have obvious symptoms. Small strokes can occur, causing subtle brain injuries and making learning difficult.
When blood is transfused, a person with sickle cell disease is at a high risk for hemolytic reactions. Blood transfusions are often safer when the donor and recipient have the same disease. Transfusions of phenotypically matched blood prevent hemolytic reactions. The Cooperative Study of Sickle Cell Disease (CSSD) reported that an overall prevalence of alloimmunization was 18.6%. While these numbers may be slightly lower, the prevalence of SCD patient alloimmunization was significantly higher than in thalassemia. A person with SCD receives blood transfusions throughout their lives, and the donor pool is different from that of thalassemia.
SCD is most common in Africans and Hispanics from the Caribbean. People with the SCD gene are also found in the Middle East, South America, Mediterranean countries, and American Indians. Symptoms of SCD include anemia. The child may appear pale and tired. There may also be a family history of sickle cell disease. The doctor can order blood tests to determine whether the patient is a carrier.
Although the signs and symptoms of sickle cell disease are not immediately obvious, it is important to seek medical care as soon as possible. People with SCD are more likely to experience strokes. While strokes are a potentially serious issue, they can be deadly. And because the symptoms are not immediately noticeable, the person may not even be aware of them. So, the best way to protect against stroke is to take care of yourself and your family.
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