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, which can cause pain, serious infections, and organ damage.

Other symptoms include anemia (a reduced number of red blood cells), a yellowing of the skin (jaundice), and the formation of gallstones, which are small stones that form in the small digestive organ called the gallbladder. 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, the UAB Adult Sickle Cell Clinic delivers complete care for this chronic blood disease, helping patients improve their quality of life by providing early treatment and outpatient care to help prevent hospital stays. The clinic is held on Fridays from 8 am to 5 pm on the first floor of the UAB Russell Clinic, though patients also are seen and evaluated throughout the week as needed. The patients selected to transition from Children’s of Alabama hospital to the UAB Adult Sickle Cell Clinic are those who require continuing care from a hematologist, a type of doctor who specializes in blood disorders. A team that includes a physician and a social worker meets regularly to develop plans and a timeline for patients who will be transitioned to the adult clinic.

UAB Medicine is actively involved in researching new therapies and potential cures for sickle cell disease. Also, the providers who work with our sickle cell programs often speak to various patient groups throughout Jefferson and surrounding counties, to help educate them on treatment options and ongoing research studies.

Care Providers

Dekozlymn Anderson, CRNP Emily Burke, NP Radhika Gangaraju, MD Benign Hematology, Blood Banking and Transfusion Medicine, Hematologic Coagulation Disorders Julie Kanter, MD Hematology, Pediatric Hematology, Sickle Cell Disorders Rachel Klein, MD Hematology Oncology, Internal Medicine, Pediatrics JaLesa Long, CRNP Hematology Oncology Calia Morais, PhD Hematology Oncology

Resources

• Comprehensive Sickle Cell Center

Related Specialties

• Alpha-1 Antitrypsin Deficiency