This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. The purpose of this research study is to determine the extent of memory and attention problems in adults with sickle cell disease. Adults who have not experienced a stroke or other brain damage will be asked to join the study. The study will also evaluate the link between these events or conditions and abnormal MRI. These findings will be compared to healthy people with similar backgrounds. For some participants, testing will show evidence of problems with memory and attention, potentially caused by the anemia that occurs among most patients with sickle cell disease. Correcting anemia has been linked in some non-sickle cell patients with better memory and attention. A second part of the study will involve treating anemia for six months in a subset of subjects who have completed the neuropsychological testing and MRI to assess the effect of trnasfusion on memory and attention. This second study phase phase is a randomized controlled clinical trial. Patients in the treatment arm of the study will receive six monthly transfusions as a potential treatment to improve memory. Patients in the non-intervention group will receive no transfusion therapy.
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