Sickle Cell Disease: Programs and Treatments
Procedures performed or problems diagnosed and treated:
Consultative diagnostic and management services for infants, children and adolescents with the following conditions:
- Sickle cell anemia
- Hemoglobin sickle C disease
- Sickle beta thalassemia
- Other hemoglobinopathies
Description of services:
The Sickle Cell Disease Program at Children’s is one of the nation’s largest serving pediatric patients. It is a key component of the Southwestern Comprehensive Sickle Cell Center. It focuses on the prevention of disease complications and management using the newest treatment strategies, including hydroxyurea, chronic transfusions, stem cell (bone marrow) transplantation and state-of-the-art approaches to infection prevention, pain management and treatment of specific organ-related complications (chest syndrome, priapism, avascular necrosis of the femoral head, etc.).
- Children's is the principal clinical site for Southwestern Comprehensive Sickle Cell Center. The center is one of the first recipients of a multi-million dollar five-year grant from the National Institutes of Health to conduct cutting edge basic and clinical research to develop better treatments and a possible cure for sickle cell disease.
- Our widely acclaimed diagnostic and treatment program for children with sickle cell disease is one of the nation's largest, with more than 700 active patients.
- Children's offers the newest treatment modalities, such as chronic blood transfusion programs, hydroxyurea therapy and stem cell transplantation.
- Outreach clinics are conducted in Tyler and Paris, Texas, and account for approximately 15 percent of all outpatient sickle cell visits.
- Hematologists on the medical staff at Children's have spearheaded the development of a comprehensive set of diagnostic and management guidelines for children with sickle cell disease that have been endorsed by the Texas Department of Health.
- Sickle cell patient information, such as every hospitalization and major complication, has been collected over the past 20 years on a computerized database, which helps improve continuity of care and research.
Each year, our staff cares for more than 700 children with the various forms of sickle cell disease, often with the very treatments our researchers developed. These elements of our program stand out as exceptional:
Neurology: Neurological complications are common in sickle cell patients, particularly stroke and headache. Michael Dowling, M.D., Ph.D., M.S.C.S., is one of a handful of pediatric neurologists in the world dedicated to both the care and research of neurologic disease in sickle cell patients. Because of his collaboration, we can offer Transcranial Doppler (TCD) testing in our clinic as a part of our comprehensive visits.
Chronic transfusion: Proven to help prevent stroke in sickle cell patients, transfusions are a critical element of our overall program. Nurses and nurse practitioners are dedicated to this area, which manages transfusions for more than 80 children. Our transfusion program avoids Port-a-Cath placement in most patients. We are experienced with all available iron overload medication options and offer the erythrocytopheresis procedure, a specialized transfusion technique that prevents iron overload.
Transition: Alecia Nero, M.D., a hematologist in the Department of Medicine at UT Southwestern, manages a transition program that prepares our patients for the complex adult medical system. This unique resource allows teenage patients to develop a relationship with the same physician who will care for them after transition.
Bone marrow transplant: Bone marrow transplant is increasing as a treatment strategy for sickle cell patients in our program over the past few years. This specialized treatment is the only available cure for sickle cell disease.