2.  SCA  Hemoglobin  How it is acquired  Symptoms  What happens in SCA  Treatment

3. Sickle-cell disease (SCD) is a hereditary or acquired blood disorder, characterized by an abnormality in the oxygen-carrying hemoglobin molecule in RBC.

4. Is the protein molecule in RBC that carries oxygen to the body's tissues and returns carbon dioxide from the tissues back to the lungs

5. Some drugs, toxins and conditions can induce anemia If a patient is hypoxic dehydrated acidotic their RBC can change into a sickle shape when the shape change it increase their vulnerability to destruction (hemolysis) types intravascular extravascular (eg in spleen during RBC filtration)

6. People who have SCD inherit two abnormal hemoglobin genes, one from each parent. In all forms of SCD, at least one of the two abnormal genes causes a person’s body to make hemoglobin S. When a person has two hemoglobin S genes, Hemoglobin SS, the disease is called sickle cell anemia. This is the most common and often most severe kind of SCD.

9.  Infections  Pain (can suddenly occur in the chest)  fatigue  Inflamed fingers and toes  Delayed puberty  Yellow skin (jaundice)  Gallstone  Shortness of breath  increase in immature RBC

11. Hemolysis and a decrease in the number of RBC and production of RBC occur. The amino acid sequence is altered; at the sixth position glutamic acid codon (GAG) is replaced by valine codon (GTG). This type of sickle cell anemia is called HgS

12.  Aplastic crisis is a temporary cessation of the RBC production the progenitor cells in the red bone marrow are infected by parvovirus B19. This shorts down the production of red blood cells.

13.  Occlusion blockage of a blood vessel by the dead red blood cell. Sickle-shaped cells are not flexible and can stick to vessel walls, causing a blockage that slows or stops the flow of blood causing hypoxia which leads to ischemia if this happens in bone its called dactylitis, in the lungs acute chest syndrome in the brain stoke

14. . Sequestration crisis is the enlargement of the spleen cause of trapping of red blood cells which leads to hemoglobin drop which leads to hypovolumetric shock

15. 20,000 to 200,000 cases per year in the US SCA can not be cured, but treatment may help.  blood transfusion is sometimes done.

16. Children born with SCA have to take 1 mg dose of folic acid daily for life. From birth to five years of age, they also have to take penicillin daily due to the immature immune system that makes them more prone to early childhood illnesses.

17. Bone marrow transplant has proven to be effective in children. Bone marrow transplants are the only known cure for SCA. However, bone marrow transplants are difficult to obtain because of the specific HLA (human leukocyte antigen) typing necessary. Ideally, a twin family member or close relative would donate the bone marrow necessary for transplantation.