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

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

Detailed Description
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The primary aims of this clinical trial are: Determine the effectiveness of targeted learning and memory strategy remediation in students with sickle cell disease and cognitive deficits. Determine if there is improvement in academic achievement after targeted memory strategy remediation.

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

Conditions
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Sickle Cell Disease

Intervention / Treatment
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* Behavioral: General Tutoring
* Behavioral: Memory Training


Participation Criteria
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Eligibility Criteria
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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)

Ages Eligible for Study
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Minimum Age: 8 Years
Maximum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Treatment
Allocation: Randomized
Intervention Model: Parallel Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Active Comparator: General Tutoring Group<br>Participants will receive general tutoring in the subject of his/her choice. | Behavioral: General Tutoring<br>* Participants will be tutored for approximately 8-10 hours per month during the school year.<br>|
| Experimental: Tutoring + Memory Training Group<br>Participants will receive tutoring and memory training. | Behavioral: General Tutoring<br>* Participants will be tutored for approximately 8-10 hours per month during the school year.<br>Behavioral: Memory Training<br>* Participants will receive memory training for approximately 8-10 hours per month during the school year.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Full Scale IQ (FSIQ) (WASI) | 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. | After >/= 50 hours of tutoring | 
| Verbal IQ (PIQ) (WASI) | 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. | After >/= 50 hours of tutoring | 
| Performance IQ (PIQ) (WASI) | 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. | After >/= 50 hours of tutoring | 
| Letter Word Identification T Score (WJ-III) | 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. | After >/= 50 hours of tutoring | 
| Reading Fluency T Score (WJ-III) | 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. | After >/= 50 hours of tutoring | 
| Calculations T Score (WJ-III) | 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. | After >/= 50 hours of tutoring | 
| Math Fluency T Score (WJ-III) | 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. | After >/= 50 hours of tutoring | 
| Spelling T Score (WJ-III) | 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. | After >/= 50 hours of tutoring | 
| California Verbal Learning Test-Children's Version (CVLT-C) List A Trials 1-5 Immediate Recall T Score | 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. | After >/= 50 hours of tutoring | 
| CVLT-C Trials 1-5 Semantic Clustering T Score | 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. | After >/= 50 hours of tutoring | 
| CVLT-C Trials 1-5 Short Delay Recall T Score | 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. | After >/= 50 hours of tutoring | 
| CVLT-C Trials 1-5 Long Delay Recall T Score | 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. | T scores were obtained at baseline and again at follow-up (after partcipant completed 50 hours of tutoring) | 
| CVLT-C Trials 1-5 Recognition Discriminability T Score | 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. | After >/= 50 hours of tutoring | 
| Delis Kaplan Executive Function System (D-KEFS) Number/Letter Switching T Score | 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. | After >/= 50 hours of tutoring | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Sickle Cell Disease, Sickle Cell, Memory, Learning, Achievement