Examining Cognitive Function and Brain Abnormalities in Adults With Sickle Cell Disease
Condition: Anemia, Sickle Cell
MeSH terms: Anemia, Sickle Cell
Study type: Observational

Summary: 
      Sickle cell disease (SCD), also known as sickle cell anemia, is an inherited blood disease
      that can cause intense pain episodes and may lead to organ failure. Preliminary studies have
      shown that adults with SCD may have brain abnormalities that contribute to problems with
      cognitive functioning, including attention and memory difficulties. This study will use brain
      magnetic resonance imaging (MRI) and neuropsychological testing to examine the differences in
      cognitive functioning in adults with SCD and adults without SCD.

      212 subjects participated in this cross-sectional study consisting of screening
      questionnaires, a neuropsychological testing battery, and MRI testing. Enrollment into this
      study ended in May 2008.
    

Detailed description: 
      SCD is an inherited blood disorder. Symptoms include anemia, infections, organ damage, and
      intense episodes of pain, which are called "sickle cell crises." In the past, SCD was
      considered a fatal disease, and many people with SCD died at a young age. Due to advances in
      medical care, people with SCD are now living longer lives; however, they often experience a
      deterioration in quality of life due to progressive organ failure. Past research has
      suggested that children with SCD commonly have frontal lobe dysfunction syndrome, which is a
      brain disorder that can affect cognitive functioning in areas such as attention,
      concentration, information processing, and decision making. Often times, however,
      neurocognitive and brain disorders are not diagnosed or treated in people with SCD. In
      preliminary brain imaging studies, at least half of adult participants with SCD had cognitive
      dysfunction that could be seen in images of the brain, while participants without SCD rarely
      had visible changes in the brain. Brain dysfunction may be one of the most important and
      least-studied problems affecting adults with SCD. The purpose of this study is to evaluate
      the extent of cognitive functioning problems in adults with SCD. The study will also
      determine if there is a connection between cognitive functioning problems and abnormalities
      seen on MRI brain images of adults with SCD.

      This study is an observational case/control study that will enroll adults with SCD and a
      control group of healthy adults who do not have SCD. At a study visit on Day 1, participants
      will undergo blood collection and will complete psychosocial questionnaires. Female
      participants will provide a urine sample for pregnancy testing. Study researchers will
      conduct a medical record review, a physical exam, and a neurological exam. They will also
      interview participants to collect medical history information. On Day 2, participants will
      undergo either a brain MRI or neuropsychological testing; on Day 3, the other procedure will
      be completed. On Day 4, study researchers will explain the study procedure results to
      participants. Participants will be asked if they are willing to take part in a second phase
      of the study in the future. Enrollment into this study ended in May 2008.

      A pilot interventional study follows this study, and is reported separately in
      ClinicalTrials.gov under NCT 00850018.
    

Intervention name(s): NP Battery; MRI
Intervention type(s): Behavioral; Procedure
Intervention description(s): Neuropsych Battery with 7 different tests that evaluate the patients neurological functioning.; The MRI is a standard procedure involving 30 minutes under the machine in order to obtain various images of the patients brain.
Other intervention name(s): The Weschler Adult Intelligence Scale - Third Edition; The Woodcock Johnson Revised - Test of Achievement; The Delis-Kaplan Function System; The Wisconsin Card Sorting test; The Test of Everday Attention; The California Verbal Learning Test; The Weschler Memory Scales - Third Edition
Observational model: Case-Control
Time perspective: Cross-Sectional
Sampling method: Non-Probability Sample
Study population: 
        212 participants, 160 will have sickle cell anemia, 52 will be matched controls based on
        gender, age, and education level
      
Keywords: Sickle Cell Disease; Sickle Cell Anemia; Hemoglobin SS; Hemoglobin SB0