People with sickle cell disease have abnormal hemoglobin, called sickle hemoglobin or hemoglobin S. If your child has sickle cell disease, her red blood cells do not last as long because the sickle hemoglobin damages them. This means your child has fewer red blood cells than normal, a condition called anemia. The exact level may be different depending on the type of sickle cell disease and the person.
Blood tests done over a period of time will tell the doctor what is normal for your child. A reticulocyte is a young red blood cell that is forming in the bone marrow. Normally, reticulocytes stay in the bone marrow until they develop into red blood cells and enter the blood.
The reticulocyte count is a test that measures the number of reticulocytes in the blood. For most people, the number is very low because most reticulocytes stay in the bone marrow. If your child has sickle cell disease, she may have a higher reticulocyte count. A normal amount of reticulocytes in the blood is between 0. If your child has sickle cell disease, she may have a reticulocyte count of 2—3 percent or more.
The number of reticulocytes is different for each person with sickle cell disease. This could be dangerous.
The St. White blood cells help the body fight infection. When the white blood cell count is low, it is easier to get an infection and harder to get over it. If your child has sickle cell disease, her white blood cell count will usually be normal or higher than normal. However, illness and some medicines can make the white blood cell count go up or down for a short time. The normal hemoglobin types include hemoglobin A, hemoglobin A2, and hemoglobin F in adults while the type of sickle cell hemoglobin is hemoglobin S.
Normal hemoglobin contains glutamate at position 6 on the surface of the beta chain while in sickle cell hemoglobin, valine replaces glutamate in the same position.
Glutamate is strongly hydrophilic in normal hemoglobin while valine makes the sickle cell hemoglobin more hydrophobic. Under low oxygen concentrations, normal hemoglobin is less likely to polymerize while sickle cell hemoglobin tends to polymerize into crystals, distorting red blood cells into a sickle shape.
Normal hemoglobin makes red blood cells become biconcave in shape while sickle cell hemoglobin makes red blood cells to become crescent in shape. Normal hemoglobin is more soluble under low oxygen concentrations, while sickle cell hemoglobin is less soluble. Normal hemoglobin allows red blood cells to freely flow through the veins while sickle cell hemoglobin causes sickle red blood cells to become sticky at the branching points of the veins. Mutations of normal hemoglobin cause hemoglobinopathies and thalassemia while sickle cell hemoglobin causes sickle cell disease and sickle cell anemia.
Normal hemoglobin is the type of hemoglobin present in regular blood cells. Generally, hemoglobin A is the most common form of normal hemoglobin in adults. Besides, normal hemoglobin does not undergo polymerization and it allows normal red blood cells to maintain their biconcave structure. Therefore, under low oxygen concentrations, normal red blood cells freely float through the veins.
In contrast, sickle cell hemoglobin is a mutation of normal hemoglobin with valine replacing the glutamate at position 6 on the surface of the beta chain. Consequently, this makes sickle cell hemoglobin to more hydrophobic, polymerizing the hemoglobin tetramers and distorting the normal biconcave shape into a sickle shape. Additionally, under low oxygen concentrations, this type of hemoglobin to become sticky at the branching points of the veins. Ultimately, sickle cell hemoglobin causes sickle cell anemia.
It is not a deadly disease but rather a manageable one. However, early diagnosis is better to prevent further complications. In SCD, or sickle cell disease, the hemoglobin in the cells can be either normal or may cause the cell to become sickle-shaped which can be called sickle cell hemoglobin. Let us try to find out the differences between normal hemoglobin and sickle cell hemoglobin.
Normal RBCs are circular in shape or disc-shaped while sickle cells are shaped like a half moon or crescent shape. When these cells move and flow into the bloodstream, normal red blood cells which contain normal hemoglobin move in a very smooth way. The sickle cell hemoglobin usually form strands that cause the sickling shape of the RBCs or red blood cells. When this happens, sickle cells will cause sickle cell anemia.
As we all know, in anemia, there is a reduced number of RBCs. This causes anemia since there is little normal hemoglobin that carries the oxygen from the lungs to the rest of the body. So symptoms such as fatigue and drowsiness may occur in the child. RBCs are produced in the bone marrow and are replaced in the body every three months.
This is in the case of normal RBCs. However, in SCD, it is replaced rapidly at about days only. The bone marrow has great difficulty producing red blood cells.
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