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

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

Study Overview

• Status: Completed
• Conditions: Sickle Cell Disease
• Intervention / Treatment: Behavioral: General Tutoring; Behavioral: Memory Training

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.

• Study Type: Interventional
• Enrollment (Actual): 21
• Phase: Not Applicable

Contacts and Locations

Study Locations

• United States
  • Missouri
    • Saint Louis, Missouri, United States, 63110
      • Washington University School of Medicine/St. Louis Children's Hospital

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 8 years to 18 years (Child, Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

Description

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 Plan

How is the study designed?

Design Details

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

Number of Arms

2

Arms and Interventions

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

What is the study measuring?

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

Collaborators and Investigators

• Sponsor: Washington University School of Medicine
• Collaborators: National Heart, Lung, and Blood Institute (NHLBI); Medical College of Wisconsin; American Society of Hematology
• Investigators: Principal Investigator: Allison King, MD, MPH, Washington University School of Medicine

Publications and helpful links

General Publications

• Yerys BE, White DA, Salorio CF, McKinstry R, Moinuddin A, DeBaun M. Memory strategy training in children with cerebral infarcts related to sickle cell disease. J Pediatr Hematol Oncol. 2003 Jun;25(6):495-8. doi: 10.1097/00043426-200306000-00014.
• King AA, White DA, McKinstry RC, Noetzel M, Debaun MR. A pilot randomized education rehabilitation trial is feasible in sickle cell and strokes. Neurology. 2007 Jun 5;68(23):2008-11. doi: 10.1212/01.wnl.0000264421.24415.16.

Study record dates

Study Major Dates

• Study Start: September 1, 2003
• Primary Completion (Actual): July 1, 2014
• Study Completion (Actual): December 1, 2014

Study Registration Dates

• First Submitted: May 6, 2009
• First Submitted That Met QC Criteria: May 6, 2009
• First Posted (Estimate): May 8, 2009

Study Record Updates

• Last Update Posted (Actual): May 15, 2019
• Last Update Submitted That Met QC Criteria: April 23, 2019
• Last Verified: April 1, 2019

More Information

Terms related to this study

• Keywords: Memory; Sickle Cell Disease; Learning; Sickle Cell; Achievement
• Additional Relevant MeSH Terms: Mental Disorders; Neurocognitive Disorders; Hematologic Diseases; Genetic Diseases, Inborn; Anemia; Anemia, Hemolytic, Congenital; Anemia, Hemolytic; Hemoglobinopathies; Cognitive Dysfunction; Anemia, Sickle Cell; Cognition Disorders
• Other Study ID Numbers: 653; K23HL079073 (U.S. NIH Grant/Contract)