Examining Cognitive Function and Brain Abnormalities in Adults With Sickle Cell Disease - Pilot Intervention Study

Completed · Phase 1

Conditions studied: Anemia, Sickle Cell

In brief

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 who are treated for anemia with monthly blood transfusions for 6 months versus adults with SCD who receive usual care for 6 months.

Key facts

Study ID

NCT00850018

Run by

UCSF Benioff Children's Hospital Oakland

People needed

36

Starts

2004-12-01

Expected to finish

2010-12-01

Last updated by the study team

2017-02-15

Who can join

Age: 21 and older, up to 55. Sex: any. Healthy volunteers: accepted.

You may qualify if…

• Completion of all components of the Phase 1 study (NCT00528801)
• Wechsler Adult Intelligence Scale (WAIS) III-Performance IQ (PIQ) score less than or equal to 90
• Hemoglobin less than or equal to 9.0 g/dL
• People who did not complete Phase I of the study are eligible for enrollment in this study if they meet all of the following criteria:
• Capable of giving informed consent for the study
• Willing to undergo transfusion therapy for 6 months
• African descent
• Proficient/fluent in English
• Hemoglobin electrophoresis confirming hemoglobin SS or SB0 (less than or equal to 15%)
• WAIS III-PIQ score less than or equal to 90
• Hemoglobin less than or equal to 9.0 g/dL
• Mini-Mental Status Examination (MMSE) score of greater than or equal to 20
• Profile of Mood States (POMS) score on the Depression-Dejection Subscale less than or equal to 40

You may not qualify if…

• People who meet any of the following criteria are disqualified from enrollment in this study:
• History of life threatening or serious transfusion complications
• Lack of venous access
• Current enrollment in the Arginine study (NCT00513617)
• Pregnant
• Refusal of transfusion
• History of unexplained severe hemolytic transfusion reaction
• History of serious allergic, pulmonary transfusion reaction requiring hospitalization
• Positive auto-immune hemolytic anemia (direct Coombs test with IgG and complement)
• Multiple (three or more) clinically significant allo-antibodies, due to common antigens (e.g., EC, Kel)
• Uncommon, clinically significant antibody that results in difficulty in finding matched units (e.g., anti-JKB)
• Currently taking Hydroxyurea and not on a stable dose in the 6 months before study entry
• Creatinine level greater than 1.7 mg/dL
• Ferritin level greater than 1,500 ng/mL or quantitative liver iron level greater than 7 mg/g dry weight and not currently on iron chelation therapy. (This is a pilot transfusion in which only 6 months of transfusion will be utilized. The likelihood of iron overload induced toxicity from the transfusions over the 6 months is very small. Furthermore, ferritin is disproportionately elevated in SCD and overestimates the iron burden. Therefore, a quantitative liver iron and/or ferritin level has been included as criteria for exclusion.)
• Major infarct identified on Phase I MRI
• Currently on Procrit or related drug that stimulates red blood cell production
• In addition to the exclusion criteria listed above, people who did not complete Phase I (or who completed Phase I more than 1 year prior to enrollment into this study) are disqualified for enrollment in this study if they meet any of the following criteria:
• Overt stroke
• Previous evidence of an abnormal MRI or computed axial tomography (CT) scan other than small periventricular or watershed lesions
• History of head injury that resulted in neurological symptoms or medical visit
• Abnormal neurological exam with focal findings
• Alcohol consumption exceeding 14 drinks/week if female or 21 drinks/week if male
• Drug abuse, as defined as using non-prescribed medication
• History of claustrophobia and/or presence of metallic implants such as pacemakers, surgical aneurysm clips, or known metal fragments embedded in the body
• Baseline blood pressure greater than 140/90 mm Hg on two repeated measurements. A second measurement is needed only if the first is greater than 140/90 mm Hg.

Where it is running

• Children's Hospital & Research Center at Oakland — Oakland, California, United States
• Howard University — Washington D.C., District of Columbia, United States
• Memorial Cancer Institute — Hollywood, Florida, United States
• University of Miami Miller School of Medicine — Miami, Florida, United States
• Medical College of Georgia — Augusta, Georgia, United States
• Johns Hopkins — Baltimore, Maryland, United States
• Boston Medical Center — Boston, Massachusetts, United States
• Karmanos Cancer Institute at Wayne State University — Detroit, Michigan, United States
• Washington University — St Louis, Missouri, United States
• University of North Carolina at Chapel Hill — Chapel Hill, North Carolina, United States
• Duke University Medical Center — Durham, North Carolina, United States
• Cincinnati Children's Hospital — Cincinnati, Ohio, United States
• University of Cincinnati Medical Center — Cincinnati, Ohio, United States
• University of Texas Medical Branch — Galveston, Texas, United States
• Children's Hospital & Research Center at Oakland — Oakland, California, United States
• Howard University — Washington D.C., District of Columbia, United States
• Memorial Cancer Institute — Hollywood, Florida, United States
• University of Miami Miller School of Medicine — Miami, Florida, United States
• Medical College of Georgia — Augusta, Georgia, United States
• Johns Hopkins — Baltimore, Maryland, United States
• Boston Medical Center — Boston, Massachusetts, United States
• Karmanos Cancer Institute at Wayne State University — Detroit, Michigan, United States
• Washington University — St Louis, Missouri, United States
• University of North Carolina at Chapel Hill — Chapel Hill, North Carolina, United States
• Duke University Medical Center — Durham, North Carolina, United States

Full record on ClinicalTrials.gov

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