Status:
COMPLETED
Examining Cognitive Function and Brain Abnormalities in Adults With Sickle Cell Disease - Pilot Intervention Study
Lead Sponsor:
UCSF Benioff Children's Hospital Oakland
Collaborating Sponsors:
National Heart, Lung, and Blood Institute (NHLBI)
Conditions:
Anemia, Sickle Cell
Eligibility:
All Genders
21-55 years
Phase:
PHASE1
Brief 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 adult...
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 dise...
Eligibility Criteria
Inclusion
- 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
Exclusion
- 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.
- History of uncontrolled hypertension
- Any long-term disorder that may result in neurocognitive or brain dysfunction that is not secondary to SCD, including any of the following:
- Inflammatory arterial disorders (e.g., lupus, polyarteritis)
- History of cancer requiring chemotherapy and/or radiation
- Untreated hyperlipidemia
- Diabetes
- Ongoing active infection such as HIV, tuberculosis, or sarcoidosis
- History of long-term blood transfusion
- Long-term kidney failure/dialysis
- Long-term lung disease characterized by a need for oxygen
- Morbid obesity (i.e., weight greater than 115 kg)
- Heart disease, including a history of congestive heart failure, history of severe coronary artery disease characterized by angioplasty or surgery, or history of angina
- Active hepatitis or liver failure
- Acquired or congenital immune deficiency
- History of psychoses (e.g., delusions, hallucinations) and/or schizophrenia
- Neurodegenerative disorder
- Genetic disorder associated with neurocognitive dysfunction such as Down Syndrome
- Other long-term illness or disorder other than SCD that will adversely affect the person's performance in the study
Key Trial Info
Start Date :
December 1 2004
Trial Type :
INTERVENTIONAL
Allocation :
ESTIMATED
End Date :
December 1 2010
Estimated Enrollment :
36 Patients enrolled
Trial Details
Trial ID
NCT00850018
Start Date
December 1 2004
End Date
December 1 2010
Last Update
February 15 2017
Active Locations (14)
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1
Children's Hospital & Research Center at Oakland
Oakland, California, United States, 94609
2
Howard University
Washington D.C., District of Columbia, United States, 20001
3
Memorial Cancer Institute
Hollywood, Florida, United States, 33021
4
University of Miami Miller School of Medicine
Miami, Florida, United States, 33136