Sickle Cell Anemia

Sickle cell anemia is a hereditary condition characterized by destruction of red blood cells in the body. It is noticed that sickle cell anemia affects people of African ancestry and those of Mediterranean and Middle Eastern descent. The soft round red blood cells develop an abnormal shape that resembles a sickle or crescent moon. Their shape stops them from flowing easily and they get clogged up inside smaller blood vessels. This stops the blood from flowing properly. Important organs like the brain, heart, or kidneys need constant blood flow to stay healthy. Although our body attacks and destroys these sickle cells, it can't make new blood cells fast enough to replace the older ones. This results in the decrease in number of red blood cells leading to anemia. Sickle cell anemia is generally caused by genetic defects or disease. A child with sickle cell anemia has inherited a defective hemoglobin gene.

Sickle shaped blood cells get stuck within the small blood vessels and obstruct blood flow to some parts of the body, thereby leading to pain. Symptoms of sickle cell anemia include fatigue, breathlessness, rapid heart rate, delayed growth and puberty, susceptibility to infections, excessive penis pain, chest pain and decreased fertility. Since the spleen is damaged by the sickle cells, the resistance to infections is reduced considerably. Hands and feet tend to swell (Hand-foot syndrome) and the body faces stunted growth. Retinal damage can occur due to the obstruction of blood flow to the eyes. In about 10% of the cases, a stroke can occur if the sickle cells block blood vessels in the brain. Acute chest syndrome is another complication of this form of anemia. In this condition, the patient suffers symptoms similar to pneumonia and will need to be treated in a hospital.

There is no cure for sickle cell anemia but treatment can be followed to prevent complications and improve the quality of life of the patient. The patient will need to be under the guidance and care of a hematologist or genetic counselor. Blood test for hemoglobin 5 gives an indication as to the presence of this disease. A microscopic analysis of a blood sample will exhibit the distinctly shaped sickle cells. Bone marrow transplant can offer potential cure for this form of anemia, provided the right donor is found. Red blood cells from a donor can be given intravenously to a patient. But it carries inherent risks. Penicillin is given to keep young affected kids from life-threatening infections. Painkillers like acetaminophen and ibuprofen can relieve the pain associated with sickle cell anemia. Children diagnosed with sickle cell anemia must be given regular childhood vaccinations as well as Hib (Hemophilus Influenzae B) vaccine and the pneumococcal vaccine.