Academic Functioning in Cystinosis: A Comprehensive Study of the Process of Achievement

Academic Functioning in Cystinosis: 
A Comprehensive Study of the Process of Achievement

Angela O. Ballantyne, Ph.D.  Principal Investigator
Amy M. Spilkin, Ph.D., Co-Principal Investigator
Doris A. Trauner, M.D. Co-Investigator

Six-Month Progress Report - Cystinosis Research Foundation
9/1/06 – 3/1/07

 

A.  SPECIFIC AIMS
      The specific aims for this project are:

  1. To be the first study to comprehensively examine academic functioning in children with cystinosis, compared to children with another chronic illness (cystic fibrosis) and typically developing controls, using a comprehensive academic achievement battery.  Our goal is to delineate the academic profile of school-age children with cystinosis to provide a more thorough understanding for parents and school personnel.  Moreover, these results may elucidate a potential non-verbal learning disability profile in children with cystinosis.
  1. To use academic testing scores within a context as opposed to viewing the scores in isolation to allow for a more complete picture of the academic functional level of the child.  This will be done by assessing academic competence through questionnaires from the perspective of the child’s teacher, as well as the child, in multiple domains (e.g., academic skills, interpersonal skills, academic motivation, study skills, classroom engagement). 
  1. To examine processes underlying potential areas of deficit, by pinpointing where breakdowns in academic competence occur.  The assessment measures we are proposing are designed not only to assess the amount of academic knowledge a child has, but also how the information is utilized (i.e., the process).  Understanding the process will allow for the future application of precise intervention strategies that pinpoint the specific areas of difficulty for children with cystinosis.  

PROGRESS TO DATE 9/1/06 – 3/1/07

  1. PROGRESS TO DATE

Over the first 6 months of the study we obtained IRB approval for the study, ordered testing supplies, and trained a research assistant on the measures to be administered.  In addition, we have successfully recruited, inducted, and tested 12 individuals with cystinosis and 5 control participants.  We have also made contact with Dr. Mark Pian, the pediatric pulmonologist at Children’s Hospital, and to date he has referred 1 cystic fibrosis patient to us who we are currently attempting to schedule for testing (the patient is currently in the hospital).  Hence, we have made substantial progress toward the goals of this study.

  1. RESULTS

On all 17 participants tested to date, we have collected WJ-IIIs, Wechsler IQs, and the age-appropriate academic functioning questionnaires (ACES and/or SMALSI).  We are also in the process of scoring, reliability checking, and data entering all of the test and questionnaire data collected so far.  Since we are in the first quarter of this research study, we have not yet analyzed any of the data.

  1. FUTURE PLANS

In the next 6-month period, we plan to continue to recruit, induct, and test individuals with cystinosis, cystic fibrosis, and matched control participants, as we work toward the total projected participants (i.e., 30 with cystinosis, 30 with cystic fibrosis, and 30 controls).