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Neuropsychological Dysfunction and Neuroimaging Abnormalities in Neurologically Intact Adults With Sickle Cell Disease


N/A
21 Years
55 Years
Not Enrolling
Both
Anemia, Sickle Cell

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Trial Information

Neuropsychological Dysfunction and Neuroimaging Abnormalities in Neurologically Intact Adults With Sickle Cell Disease


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.


Inclusion Criteria:



Individuals who meet all of the following criteria are eligible for enrollment as cases
into the study:

1. Adult between the ages of 21 and 55

2. African descent

3. Proficient/fluent in English

4. Hemoglobin electrophoresis confirming hemoglobin SS or SB0 (%A <= 15)

5. Hemoglobin <= 10 g/dL

6. Capable of giving informed consent for the protocol

Individuals who meet all of the following criteria are eligible for enrollment as
community controls into the study:

1. Adult between the ages of 21 and 55

2. African descent

3. Proficient/fluent in English

4. Capable of giving informed consent for the protocol

Exclusion Criteria:

Individuals who meet any of the following criteria are disqualified from enrollment in the
case group of the study:

1. Overt stroke

2. Previous evidence of an abnormal MRI or CT other than small peri-ventricular or
watershed lesions

3. History of head injury that resulted in neurological symptoms or medical visit

4. Abnormal neurologic exam with focal findings

5. Mini-Mental Status Examination (MMSE) score of < 20

6. Profile of Mood States (POMS) score on the Depression-Dejection Subscale suggestive
of a clinical depression (score > 40)

7. Alcohol consumption exceeding 14 drinks/week if female, 21 drinks/week if male

8. Drug abuse, defined as using non-prescribed medication

9. History of claustrophobia and/or presence of metallic implants such as pacemakers,
surgical aneurysm clips, or known metal fragments embedded in the body

10. Pregnancy

11. Baseline blood pressure > 140/90 on two repeated measurements. A second measurement
is needed only if the first is > 140/90

12. History of uncontrolled hypertension

13. Any chronic disorder that may result in neurocognitive or brain dysfunction that is
not secondary to SCD including:

1. Inflammatory arterial disorders (lupus, polyarteritis)

2. History of cancer requiring chemotherapy and/or radiation

3. Untreated hyperlipidemia

4. Diabetes

5. Ongoing active infection such as HIV, tuberculosis, sarcoidosis

6. History of chronic transfusion

7. Chronic renal failure/Dialysis

8. Chronic lung disease characterized by need for oxygen

9. Morbid obesity (weight >115 kg)

10. Heart disease: history of congestive heart failure, history of severe coronary
artery disease characterized by angioplasty or surgery, or history of angina

11. Active hepatitis or liver failure

12. Acquired or congenital immune deficiency

13. History of psychoses (delusions, hallucinations) and/or schizophrenia

14. Neurodegenerative disorders

15. Genetic disorder associated with neurocognitive dysfunction such as Down
Syndrome

16. Other chronic illness or disorder other than SCD that will adversely affect the
subject's performance in the study

14. Currently on Procrit or related drug that stimulates red blood cell production

Individuals who meet any of the following criteria are disqualified from enrollment as
community controls in to the study:

1. Hb electrophoresis other than AA

2. Abnormal Hb (females: < 12 g/dL; males: < 13.5 g/dL)

3. Overt stroke

4. Previous abnormal MRI or CT

5. History of head injury that resulted in neurological symptoms or medical visit

6. Abnormal neurologic exam with focal findings

7. Mini-Mental Status Examination (MMSE) score of < 20

8. Profile of Mood States (POMS) score on the Depression-Dejection Subscale suggestive
of a clinical depression (score > 40)

9. Alcohol consumption exceeding 14 drinks/week if female, 21 drinks/week if male

10. Drug abuse, defined as using non-prescribed medication

11. History of claustrophobia and/or presence of metallic implants such as pacemakers,
surgical aneurysm clips, or known metal fragments embedded in the body

12. Pregnancy

13. Baseline blood pressure > 140/90 on two repeated measurements. A second measurement
is needed only if the first is > 140/90

14. History of uncontrolled hypertension

15. Any chronic disorder that may result in neurocognitive or brain dysfunction
including:

1. Inflammatory arterial disorders (lupus, polyarteritis)

2. History of cancer requiring chemotherapy and/or radiation

3. Untreated hyperlipidemia

4. Diabetes

5. Ongoing active infection such as HIV, tuberculosis, sarcoidosis

6. History of chronic transfusion

7. Chronic renal failure/Dialysis

8. Chronic lung disease characterized by need for oxygen

9. Morbid obesity (weight > 115 kg)

10. Heart disease: history of congestive heart failure, history of severe coronary
artery disease characterized by angioplasty or surgery, or history of angina

11. Active hepatitis or liver failure

12. Acquired or congenital immune deficiency

13. History of psychoses (delusions, hallucinations) and/or schizophrenia

14. Neurodegenerative disorders

15. Genetic disorder associated with neurocognitive dysfunction such as Down
Syndrome

16. Other chronic illness or disorder that will adversely affect the subject's
performance in the study

16. Currently on Procrit or related drug that stimulates red blood cell production

Type of Study:

Observational

Study Design:

Observational Model: Case Control, Time Perspective: Cross-Sectional

Outcome Measure:

Wechsler Adult Intelligence Scale (WAIS)-III Performance IQ

Outcome Description:

Extent of neurocognitive dysfunction in neurologically asymptomatic adult patients with sickle cell disease as measured by WAIS-III performance IQ. This quotient is based on an average of 100, with a standard deviation of 15. The Wechsler intelligence scales are not considered adequate measures of extremely high and low intelligence (IQ scores above 160 and below 40, respectively). The performance IQ is derived from scores on seven subtests: picture completion, picture arrangement, block design, object assembly, digit symbol, matrix reasoning, and symbol search.

Outcome Time Frame:

Within 2 months of signing informed consent.

Safety Issue:

No

Principal Investigator

Elliott Vichinsky, MD

Investigator Role:

Principal Investigator

Investigator Affiliation:

Northern California CSCC (Children's Hospital Oakland)

Authority:

United States: Federal Government

Study ID:

480

NCT ID:

NCT00528801

Start Date:

December 2004

Completion Date:

May 2008

Related Keywords:

  • Anemia, Sickle Cell
  • Sickle Cell Disease
  • Sickle Cell Anemia
  • Hemoglobin SS
  • Hemoglobin SB0
  • Congenital Abnormalities
  • Anemia
  • Anemia, Sickle Cell

Name

Location

University of Texas Medical BranchGalveston, Texas  77555-1329
Boston Medical CenterBoston, Massachusetts  02118
Medical College of GeorgiaAugusta, Georgia  30912
Duke University Medical CenterDurham, North Carolina  27710
University Of Cincinnati Medical CenterCincinnati,, Ohio  45267-0589
University of Southern CaliforniaLos Angeles, California  90033
University of North Carolina at Chapel HillChapel Hill, North Carolina  27599
Cincinnati Children's HospitalCincinnati, Ohio  45229
University Of Miami Miller School Of MedicineMiami, Florida  33010
Memorial Cancer InstitutePembroke Pines, Florida  33028
Children's Hospital & Research Center at OaklandOakland, California  94609
Children's Medical Center at DallasDallas, Texas  75390