Curing Sickle Cell

November 2, 2009 8:12:12 AM PST
Sickle cell disease is an inherited disorder affecting red blood cells. Red blood cells are typically round. But they turn into crescent shapes in many sickle cell patients. These crescent shaped cells have a hard time traveling through small blood vessels, and sometime even block the vessel. Blocked blood vessels result in less blood reaching that part of the body, according to the Sickle Cell Disease Association of America. When tissue does not receive the right amount of blood, it becomes damaged, leading to the main complications of sickle cell anemia. Red blood cells carry the oxygen from the air in our lungs to the rest of our body through the main substance of the cell, hemoglobin. Typical red blood cells contain hemoglobin A, and live for about 120 days until a new cell replaces them. Sickle cell patients have red blood cells with hemoglobin S, a form of hemoglobin A, and only live about 16 days. The type of hemoglobin a person has is inherited from both of their parents, much like hair and eye color. A simple blood test can tell a person which type of hemoglobin is in their red blood cells.

SYMPTOMS: Sickle cell symptoms can vary from mild to severe. Because it is an inherited disease, infants are born with the disease, although it can not be detected in the first four months of life. The most common symptom is fatigue, or feeling week and tired, according to the National Heart Lung and Blood Institute. Additional symptoms include shortness of breath, dizziness, headache, coldness in the hands and feet, pale skin and chest pain. "Sickle cell crisis" refers to a symptom of the disease causing pain, affecting bones, lungs, joints, and the abdomen. This pain and organ damage is caused by the clumping of cells in a vessel, blocking blood from reaching its destination limbs and organs. Pain level varies and can last days, weeks or months. Patients may have less than one crisis a year, or experience fifteen or more crises.

TREATMENT OPTIONS: There is no universal cure for sickle cell disease, according to the Sickle Cell Disease Association of America, but there are treatments to control complications, relieve associated pain, and prevent infection, eye damage and strokes. Antibiotics aid in prevention of infection in children from age two to five. Over the counter pain relievers may help alleviate pain during crises. Hydroxyurea is prescribed to patients to reduce the frequency of crises and reduces the need for blood transfusions. It works by stimulating the production of fetal hemoglobin, the kind found in infants to prevent the formation of sickle cells, according to the Mayo Clinic. More comprehensive reatment options include bone marrow transplantation and gene therapy. In bone marrow transplants, finding a donor is often difficult. In addition, there are serious risks and complications to a transplant, including death, according to the Mayo Clinic.

Lakshmanan Krishnamurti, M.D., Director of the Comprehensive Sickle Cell Program at the Children's Hospital of Pittsburgh of UPMC, is pioneering a new form of bone marrow transplant that reduces the risk of the life-threatening complications of a traditional transplant. The key is administering immunosuppressive drugs in combination with chemotherapy rather than heavy doses of chemotherapy to prepare the body for new bone marrow. "The important thing about the reduced intensity transplant is that it could be a treatment option for patients with advanced sickle cell disease, for whom conventional transplants were not an option," Dr. Krishnamurti told Ivanhoe.

FOR MORE INFORMATION, PLEASE CONTACT:

Marc Lukasiak
Public Relations
Children's Hospital of Pittsburgh of UPMC
(412) 692-7919


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