What is it? Serious disorder in which the body makes sickle-shaped red blood cells “Sickle-shaped” means that the red blood cells are shaped like a crescent Red blood cells contain an iron-rich protein called hemoglobin. This protein carries oxygen from the lungs to the rest of the body. Sickle cells contain abnormal hemoglobin called sickle hemoglobin or hemoglobin S. Sickle hemoglobin causes the cells to develop a sickle, or crescent, shape. Sickle cells are stiff and sticky. They tend to block blood flow in the blood vessels of the limbs and organs. Blocked blood flow can cause pain, serious infections, and organ damage.
Normal Blood Cells vs Sickle Blood Cells Normal Bone marrow is always making new red blood cells to replace old ones. Normal red blood cells live about 120 days in the bloodstream and then die. Sickle Blood Cells Sickle cells die after only about 10 to 20 days. Blood count is low. The bone marrow can't make new red blood cells fast enough to replace the dying ones.
Signs and Symptoms Usually show up in infants about 4 months old Anemia (shortage of red blood cells) Fatigue Episodes of Pain Result in blood cells block blood flow through tiny blood vessels to your chest, abdomen and joints. May last from a few hours to a couple of weeks. Hand Food Syndrome Swollen hands and feet may be the first signs of sickle cell anemia in babies. It is caused by the back flow of the red blood cells.
Signs and Symptoms Cont. Frequent Infections Sickle cell anemia can damage the spleen which help fight infections. Delayed Growth Sickle cells have difficulty providing oxygen and nutrients for someone to grow. A shortage of healthy red blood cells can slow growth in infants and children and delay puberty in teenagers. Vision Problems The blood vessels in your eyes can have back flow which can damage the retina.
Signs and Symptoms Cont. Pail skin or nail beds Yellow tint to the skin or whites of the eyes Sickle cell anemia may also cause strokes
Treatments and Cures Bone marrow transplant offers the only potential cure Finding a donor can be difficult and there are serious heath risks. Treatment for sickle cell anemia is usually aimed at avoiding crises, relieving symptoms and preventing complications.
Treatments and Cures Treatments may include: Antibiotics to prevent from infections from occurring Pain relieving medication to relieve pain episodes, may just be over the counter or prescription depending upon severity. Hydroxyurea reduces pain episodes and the need for blood transfusions. Also may include the production of fetal hemoglobin
Treatments and Cures Assessing a stroke risk will determine which children have a higher chance of a stroke and those that are have regular blood transfusions. Immunizations to prevent infection Blood transfusions to increase the number regular blood cells in your body. Supplemental Oxygen
The Cause For It All Sickle cell anemia is an autosomal recessive genetic disorder caused by a defect in the HBB gene. The presence of two defective genes (SS) is needed for sickle cell anemia. If each parent carries one sickle hemoglobin gene (S) and one normal gene (A), each child has a 25% chance of inheriting two defective genes and having sickle cell anemia; a 25% chance of inheriting two normal genes and not having the disease; and a 50% chance of being an unaffected carrier like the parents.
Random Facts In the United States, the disease most commonly affects African-Americans. About 1 out of every 500 African-American babies born in the United States has sickle cell anemia. Sickle cell disease is most common among people from Africa, India, the Caribbean, the Middle East, and the Mediterranean. The high prevalence of the defective gene in these regions may be due to the fact that carriers of a mutation in the beta-subunit of hemoglobin are more resistant to malaria.