Sickle Cell Disease

Sickle cell disease (or sickle cell anemia) is an inherited blood disorder that affects red blood cells. Red blood cells contain an iron-rich protein called hemoglobin, which gives blood its red color and carries oxygen from the lungs to the rest of the body. With sickle cell disease, the red blood cells contain abnormal hemoglobin, making them stiff and sticky and likely to form clumps and get stuck in the blood vessels. The abnormal hemoglobin also causes red blood cells to be C-shaped (similar to a curved tool called a sickle), unlike normal red blood cells, which are disc-shaped and able to move easily through the blood vessels. These clumps of cells block the blood flow leading to the limbs and organs, causing pain, serious infections, or organ damage. Other symptoms include anemia (reduced number of red blood cells), a yellowing of the skin called jaundice, and the formation of gallstones, which are small stones that form in the gallbladder, a small organ that is part of the digestive system. Currently there is no cure for sickle cell disease, and it requires lifelong management. However, the symptoms and complications can be treated with antibiotics, intravenous fluids, blood transfusions, surgery, pain management, and counseling.

Established in 1995, UAB’s Adult Sickle Cell Clinic delivers the full spectrum of care for this chronic blood disease, helping improve the quality of life for patients by providing early intervention for preventable problems and reducing inpatient stays by promoting outpatient care. The clinic is held on Fridays from 8 am to 5 pm in the Hematology/Oncology Division of the Department of Medicine, located on the second floor of The Kirklin Clinic of UAB Hospital, though patients also are seen and evaluated throughout the week as needed. The clinic features 27 exam rooms, including one with a hospital bed, while the adjacent Infusion Therapy Unit provides 3 beds and 24 recliners for patients receiving blood transfusions, exchanges, and phlebotomies.The patients selected to transition from Children’s of Alabama to the UAB Adult Sickle Cell Clinic are those whose disease requires the continuing care of a hematologist. A transition team – including a physician and a social worker – meets regularly to develop plans and a timeline for patients who will be transitioned to the adult clinic. The UAB Medicine faculty members who work with our sickle cell programs routinely speak to various patient groups throughout Jefferson and surrounding counties, using these opportunities to educate patients on treatment options and ongoing research studies. In addition, UAB is actively involved in researching new therapies and potential cures for sickle cell disease.

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