A Rehabilitation Program in Children With Sickle Cell Disease and Cognitive Deficits: a Pilot Study

Not Applicable

Completed

Conditions: Sickle Cell Disease

Interventions: Behavioral: General Tutoring
Behavioral: Memory Training

Registration Number: NCT00895154

Lead Sponsor: Washington University School of Medicine

Brief Summary: The overall goal of this project is to determine the feasibility of conducting a cognitive intervention for children with sickle cell disease.

Detailed Description: The primary aims of this clinical trial are:

1. Determine the effectiveness of targeted learning and memory strategy remediation in students with sickle cell disease and cognitive deficits.

2. Determine if there is improvement in academic achievement after targeted memory strategy remediation.

Recruitment & Eligibility

Status: COMPLETED

Sex: All

Target Recruitment: 21

Inclusion Criteria

Sickle cell disease that is confirmed by hemoglobin analysis
Has a brain magnetic resonance imaging (MRI) document reading by a neuroradiologist
Cognitive deficits, which are defined by one standard deviation lower than the normal ranges of an age-based standard score in cognitive tests that are evaluated by a psychologist
Must be a student who will remain in school for at least 2 years
Must provide informed consent with assent in accordance with the institutional policies (institutional review board approval)

Exclusion Criteria

Known developmental delays (these participants are ineligible because their learning potential may be biased from a prior insult)
Intelligent quotient is less than 50 (may have impaired learning potential)
Aphasic (i.e., unable to produce intelligible speech)

Study & Design

Study Type: INTERVENTIONAL

Study Design: PARALLEL

Arm && Interventions

Group	Intervention	Description
General Tutoring Group	General Tutoring	Participants will receive general tutoring in the subject of his/her choice.
Tutoring + Memory Training Group	Memory Training	Participants will receive tutoring and memory training.
Tutoring + Memory Training Group	General Tutoring	Participants will receive tutoring and memory training.

Primary Outcome Measures

Name	Time	Method
Performance IQ (PIQ) (WASI)	After >/= 50 hours of tutoring	The PIQ score is comprised from the Matrix Reasoning and Block Design subtests of the WASI-4. The raw scores from these subtests are converted to T scores using score charts in the manual. The T scores are summed together for a Performance Sum of T Scores. This score is converted to a Composite PIQ score using a score chart in the manual. Higher scores = better Performance IQ. Range = 45-160. Average = 100; standard deviation +/- 15.
Full Scale IQ (FSIQ) (WASI)	After >/= 50 hours of tutoring	The WASI is a brief, reliable measure of intelligence. Participants engage in 4 subtests: Block Design, Vocabulary, Similarities, and Matrix Reasoning. Participants receive a raw score on each of these 4 subtests. This raw score is converted to a T score using a score chart in the WASI manual. The T scores from all 4 subtests are summed together to obtain a Full Scale Sum of T Scores. This Full Scale Sum of T Scores is converted to a Full Scale IQ-4 Composite Score using a score chart published in the WASI manual. Higher scores indicate better intelligence. FSIQ-4 Range: 40-160. Average FSIQ-4 =100, standard deviation is +/- 15.
Verbal IQ (PIQ) (WASI)	After >/= 50 hours of tutoring	The VIQ score is comprised from the Vocabulary and Similarities subtests of the WASI-4. The raw scores from these subtests are converted to T scores using score charts in the manual. The T scores are summed together for a Verbal Sum of T Scores. This score is converted to a Composite VIQ score using a score chart in the manual. Higher scores = better Verbal IQ. Range = 45-160. Average = 100; standard deviation +/- 15.
Letter Word Identification T Score (WJ-III)	After >/= 50 hours of tutoring	This sub-test measures a student's word identification skills. A participant's raw score (# of items answered correctly) is converted to a T score using a program (Compuscore for the WJ III, Version 2.1). Higher T scores = better performance. Range: 0-100. Average T score = 50; standard deviation =/- 10.
Reading Fluency T Score (WJ-III)	After >/= 50 hours of tutoring	This sub-test measures a student's ability to read simple sentences quickly. A participant's raw score (# of items answered correctly) is converted to a T score using a program (Compuscore for the WJ III, Version 2.1). Higher T scores = better performance. Range: 0-100. Average T score = 50; standard deviation =/- 10.
Calculations T Score (WJ-III)	After >/= 50 hours of tutoring	This sub-test measures a student's ability to perform paper and pencil math computations.A participant's raw score (# of items answered correctly) is converted to a T score using a program (Compuscore for the WJ III, Version 2.1). Higher T scores = better performance. Range: 0-100. Average T score = 50; standard deviation =/- 10.
Math Fluency T Score (WJ-III)	After >/= 50 hours of tutoring	This sub-test measures a student's ability to solve simple addition, subtraction and multiplication facts quickly. A participant's raw score (# of items answered correctly) is converted to a T score using a program (Compuscore for the WJ III, Version 2.1). Higher T scores = better performance. Range: 0-100. Average T score = 50; standard deviation =/- 10.
CVLT-C Trials 1-5 Long Delay Recall T Score	T scores were obtained at baseline and again at follow-up (after partcipant completed 50 hours of tutoring)	The CVLT®-C measures multi-trial learning and long-term recall abilities for verbal information. The Raw scores from all trials (1-5) are summed to calculate a Total Raw Score for Trials 1-5. This Raw score is converted to a T score using the CVLT-II Comprehensive Scoring System (computer system). A higher T score indicates better performance. Range = 0-100. Average =50 with a standard deviation of =/- 10 points.
Spelling T Score (WJ-III)	After >/= 50 hours of tutoring	This sub-test measures a student's ability to write orally presented words correctly. A participant's raw score (# of items answered correctly) is converted to a T score using a program (Compuscore for the WJ III, Version 2.1). Higher T scores = better performance. Range: 0-100. Average T score = 50; standard deviation =/- 10.
California Verbal Learning Test-Children's Version (CVLT-C) List A Trials 1-5 Immediate Recall T Score	After >/= 50 hours of tutoring	The CVLT®-C measures multi-trial learning and long-term recall abilities for verbal information. The Raw scores from all trials (1-5) are summed to calculate a Total Raw Score for Trials 1-5. This Raw score is converted to a T score using the CVLT-II Comprehensive Scoring System (computer system). A higher T score indicates better performance. Range = 0-100. Average =50 with a standard deviation of =/- 10 points.
CVLT-C Trials 1-5 Short Delay Recall T Score	After >/= 50 hours of tutoring	The CVLT®-C measures multi-trial learning and long-term recall abilities for verbal information. The Raw scores from all trials (1-5) are summed to calculate a Total Raw Score for Trials 1-5. This Raw score is converted to a T score using the CVLT-II Comprehensive Scoring System (computer system). A higher T score indicates better performance. Range = 0-100. Average =50 with a standard deviation of =/- 10 points.
CVLT-C Trials 1-5 Semantic Clustering T Score	After >/= 50 hours of tutoring	The CVLT®-C measures multi-trial learning and long-term recall abilities for verbal information. The CVLT-C consists of five learning trials of 15 words which can be organized into three semantic categories. The Raw scores from all trials (1-5) are summed to calculate a Total Raw Score for Trials 1-5. This Raw score is converted to a T score using the CVLT-II Comprehensive Scoring System (computer system). A higher T score indicates better performance. Range = 0-100. Average =50 with a standard deviation of =/- 10 points.
CVLT-C Trials 1-5 Recognition Discriminability T Score	After >/= 50 hours of tutoring	TThe CVLT®-C measures multi-trial learning and long-term recall abilities for verbal information. The Raw scores from all trials (1-5) are summed to calculate a Total Raw Score for Trials 1-5. This Raw score is converted to a T score using the CVLT-II Comprehensive Scoring System (computer system). A higher T score indicates better performance. Range = 0-100. Average =50 with a standard deviation of =/- 10 points.
Delis Kaplan Executive Function System (D-KEFS) Number/Letter Switching T Score	After >/= 50 hours of tutoring	The D-KEFS allows for the assessment of executive functions. There are 9 stand alone tests that can be individually or group administered. The Number-Letter Switching condition of the Trails Making sub-test specifically assesses flexibility of thinking on a visual-motor sequencing task. A participant's raw score (# of items answered correctly) is converted to a T score using tables provided in the manual. Higher T scores = better performance. Range: 0-100. Average T score = 50; standard deviation =/- 10.