Children’s Healthcare of Atlanta announced the launch of a unique brain mapping study of sickle cell disease. This study consists of two parts: (1) a magnetic resonance imaging (MRI) study to obtain detail pictures of the brain-also called brain morphometrics and (2) written paper and pencil tests-also called neuropsychological tests. These tools are new or improved and are designed to study the relationships between the brain and behavior. The purpose of this study is to better examine how sickle cell disease affects the brain, even when no serious problems, such as strokes, have occurred.
“Through this study, we are able to examine the brain’s cortical thickness and how sickle cell disease effects the development of the brain, which will ultimately lead to a better understanding of the specific areas that are particularly vulnerable to the effects of the disease, said Richard Jones, Ph.D., MRI Physicist, Children’s Healthcare of Atlanta. “Our goal is to provide the basis for an improved understanding of how to better treat pediatric sickle cell patients.”
Children’s researchers will study the brains of children ages 12 to 21 years who (1) have sickle cell disease, but no known neurological problems and who (2) do not have sickle cell disease or a known neurological problem, but do have a sibling who has sickle cell disease. All children with sickle cell disease participating in the study are current/former patients at the Aflac Cancer Center and Blood Disorders Service of Children’s Healthcare of Atlanta. “Children’s researchers and physicians hope to prove the predictability of strokes in children with sickle cell disease through future studies like the Brain Mapping Study”
Sickle cell disease (SCD) is considered one of the most prevalent diseases in the United States, afflicting roughly 1 in 400 African American children. Acute pain episodes and chronic pain are considered the most debilitating symptoms of SCA. However, as the disease progresses, various parts of the body can be compromised such as the brain. Neurologic complications secondary to cerebral infarction are an common source of disability for children with sickle cell disease (SCD). The chance of having a first overt cerebrovascular accident (CVA) by 20 years of age is estimated at 11% for patients with homozygous SS disease (Hb SS). Previous clinical studies also show that a still larger number of children with Hb SS have cerebral infarctions that are not associated with obvious symptoms but are documented by magnetic resonance imaging (MRI). The prevalence of these “silent infarctions” is approximately 20%.
Brain morphometry uses detail radiology pictures of the brain collected by MRI to model the size and structure of specific areas of the brain. Currently, brain morphometry is being used in research settings to help describe the growth and function of the brain in adults and children. Neuropsychology is the study of behavior and learning. Neuropsychologists use standardized tests to measure learning skill and cognitive performance. A select set of these tests have been grouped together by Children’s neurophsycologists for this study. This group of tests should help researchers identify neurological, behavioral and psychological factors that could affect learning in children with sickle cell disease.
The results of the MRI will be collected and grouped by the study subject’s sickle cell disease status. Researchers, who are unaware of clinical problems of the study participants, will identify and compare differences between the sickle cell and normal control groups. These results will then be compared with the results of the neuropsychological tests to observe ways in which the findings compare.
The study aims to identify regions of abnormal brain development in sickle cell children who would be diagnosed as normal using conventional MRI techniques. By using a group analysis to compare the results of the brain morphometry, diffusion and MRI, the study will determine if subjects are experiencing similar patterns of abnormal development. If so, researchers will then analyze the individual data to see if these techniques will allow an accurate assessment of the brain of individual patients. The results from the cerebral blood flow and susceptibility weighted imaging studies performed with MRI will be evaluated on a qualitative basis by one or more radiologists. The end-point for the study will occur when the indicated number of study and control subjects has been recruited.
This study is intended to learn how sickle cell disease affects the brain’s growth and development through basic interdisciplinary research.
Principal Investigator: Richard Jones, Ph.D.
Co-Investigators: Clark Brown M.D., Greg Kirk M.Sc., Susan Palasis, M.D. and Thomas Burns, Psy.D.