Having sickle cells is a condition that affects the blood, causing an abnormally low level of hemoglobin in a child’s blood. It also causes the spleen to become enlarged, causing pain and scarring. In addition, it is hazardous for children since it places them at risk of infection, a significant cause of death in children under five.
The protein Hemoglobin is responsible for carrying oxygen through the blood. This protein forms disc-shaped red blood cells in a healthy human body that move freely through blood vessels. But in sickle cell hemoglobin, this protein takes on a sickle-like shape. When it becomes sickled, the protein forms stiff rods within red blood cells, which attach to the blood vessel walls. This process slows blood flow, causing less oxygen to reach nearby tissues.
Symptoms of sickle cell disease vary between individuals. They can be mild or severe, depending on the type of sickle cell trait. In most cases, people with sickle cell trait only have one copy of the sickle gene. However, people with this trait have a 25% chance of developing hemoglobin SS sickle cell disease.
Children with hemoglobin SS sickle cell disease have been known to suffer from cognitive impairment. However, recent evidence indicates that cognitive impairment may also occur in children with sickle cell disease without an infarction. In addition, cognitive impairment has been associated with low hematocrit levels and abnormalities on MR imaging.
Hemoglobin SS sickle is caused by a mutation in the hemoglobin S gene. This mutation causes abnormal hemoglobin with two beta chains. The resulting sickle-shaped red blood cells obstruct the blood vessels and prevent blood from reaching body tissues. This condition also results in other complications, such as vision problems and delayed growth.
Mary’s infant daughter Mina has been diagnosed with sickle cell trait. Mary herself was not affected by sickle cell disease. Although there are no known cases in the family, other family members with hemoglobin SS may develop sickle cell disease later in life. For example, this can happen if they have malaria.
The symptoms of sickle cell disease include anemia and lethargy. The disease may also affect the brain, joints, and bones. A sickle cell disease can block the blood flow to the brain, resulting in severe damage. It is also associated with an increased risk of stroke.
Hemoglobin beta thalassemia
Sickle cell disease affects the production of red blood cells that carry oxygen. Usually, healthy red blood cells move through the body’s small blood vessels with minimal resistance. However, these red blood cells become abnormal and sticky with sickle cell disease, causing poor blood flow. As a result, patients with sickle cell disease may experience pain, fatigue, infection, and acute chest syndrome. In severe cases, it may lead to stroke.
Hemoglobin beta thalassemia is a sickle cell disease caused by abnormal beta globin. This form of the sickle disease is common in people with Mediterranean ancestry. Patients with the disease produce different levels of beta-globin, depending on the amount of beta gene in the patient’s blood. Those with no beta globin may experience similar symptoms to those with sickle cell disease.
Parents of children with sickle cell disease should be aware of early symptoms and follow the guidelines provided by their pediatrician and hematologist. They should learn to check the child’s temperature and to recognize any abnormality. They should also be taught to palpate the infant’s spleen to determine if the child has splenic enlargement. They should also understand the importance of prompt evaluation by a pediatric hematologist, particularly if the child has a fever greater than 101°F.
Fortunately, there is now a treatment for thalassemia. Stem cells can be used to repair damaged red blood cells. But it is not a standard procedure, and the risks are high. As with any other type of anemia, getting screened for thalassemia is essential. Early detection and treatment will reduce the risk of complications and the need for treatment.
Hemoglobin beta chain-related hemoglobinopathies are common and affect nearly one percent of the population. They are often found in people of Mediterranean, Middle Eastern, and Asian descent. However, sickle cell disease can be found in other countries as well.
Hemoglobin beta thalasis is caused by mutations in a person’s hemoglobin genes. These mutations decrease the production of alpha or beta globin in the blood. When this happens, the disease can be mild, moderate, or severe. In addition, it can be inherited, so it’s best to visit a doctor for a complete diagnosis.