Sickle Cell Disease

Contact Help Group: Sickle Cell Society (based in London)
Tel: 0044 020 8961 7795
E: mail: info@sicklecellsociety.org

Sickle cell disease is an inherited disease of red blood cells. It is characterized by pain episodes, anemia (shortage of red blood cells), serious infections and damage to vital organs. The symptoms of sickle cell disease are caused by abnormal hemoglobin. Hemoglobin, the main protein inside red blood cells, carries oxygen from the lungs and takes it to every part of the body. Normally, red blood cells are round and flexible and flow easily through blood vessels. But in sickle cell disease, the abnormal hemoglobin causes red blood cells to become stiff and, under the microscope, may look like a C-shaped farm tool called a sickle. These stiffer red blood cells can get stuck in tiny blood vessels, cutting off the blood supply to nearby tissues. This is what causes pain (called a sickle cell pain episode or crisis) and sometimes organ damage in sickle cell disease. Sickle-shaped red blood cells also die and break down more quickly than normal red blood cells, resulting in anemia.

Medical problems: that can occur for sufferers of sickle cell disease are: infections, pain episodes, hand-foot syndrome, stroke, acute chest syndrome, vision problems, slow growth.

Is there a cure for sickle cell disease: A small number of children with severe sickle cell disease have been cured through a blood stem cell transplant. The stem cells (immature cells that develop into blood cells) come from bone marrow, or less frequently from umbilical cord blood, usually donated by siblings who are a good genetic match. Most children with sickle cell disease, however, do not have siblings who are good genetic matches. For this reason, researchers have recently begun performing stem cell transplants using umbilical cord blood from unrelated donors with apparent success.