Sickle Cell Disease

What comes to your mind when you hear “sickle cell disease”? The one thing that comes to my mind when I hear sickle cell disease is that it’s a type of disease where the cells in the human body are sick, but that’s sort of a way to put it. Sickle cell disease is a disease in which a group of disorders cause red blood cells to become misshaped and broken down which may cause pain.

According to the U.S. National Library of Medicine it states that sickle cell disease is a group of disorders that affects hemoglobin, the molecule in red blood cells that delivers oxygen to cells throughout the body. People with this disorder have atypical hemoglobin molecules called hemoglobin S, which can distort red blood cells into a sickle, or crescent, shape.

The rapid breakdown of red blood cells may also cause yellowing of the eyes and skin, which are signs of jaundice. Jaundice is also a disorder doctors pay close attention to after a baby is born. Then you have the painful episodes that can occur when sickled red blood cells, which are stiff and inflexible, get stuck in small blood vessels. These episodes deprive tissues and organs of oxygen-rich blood and can lead to organ damage, especially in the lungs, kidneys, spleen, and brain. A particularly serious complication of sickle cell disease is high blood pressure in the blood vessels that supply the lungs. Pulmonary hypertension occurs in about one-third of adults with sickle cell disease and can lead to heart failure. (Ashley-Koch A, Yang Q, Olney RS)

Sickle cell disease is most common in Africans and African-Americans. It is also found in other ethnic and racial groups, including people from South and Central America, the Caribbean, Mediterranean countries, and India

Patients with sickle cell disease may develop severe pain in the chest, back, arms, legs, and abdomen. Pain can occur anywhere in the body. Sickle red blood cells in the lungs can cause severe illness with chest pain, fever, and difficulty breathing. Sickle cell disease can also cause permanent damage to the brain, heart, kidneys, liver, spleen, and bones. The severity and symptoms vary greatly from person to person,

Even though it’s not easy having the disease, but those who suffer from it are some true warriors, because as myself I don’t think I could even bare the pain, and I wish those who have the disease could be cure, because nobody should suffer with the painful disease.

Hydroxyurea is the only FDA-approved medication that prevents painful episodes in sickle cell disease. Studies of patients with sickle cell disease show that the regular use of hydroxyurea decreases the frequency and severity of sickle cell crises and reduces the number of blood transfusions and hospitalizations.

Stem cell transplant is the only curative treatment for sickle cell disease. More than 200 patients with sickle cell disease have undergone stem cell transplants from a matched sibling donor. Stem cell transplant has a 5%-10% risk of death, but patients with successful transplants were completely cured of sickle cell disease, with no further episodes of pain.

Stem cell transplant is performed in young patients with severe sickle cell disease who have a matched sibling donor. Stem cell transplant using umbilical cord blood from a related donor has also been curative in a small number of