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

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

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.
  2. 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). 
  3. 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 – 9/1/07

 

  1. PROGRESS TO DATE

Over the past year, we have recruited, inducted, and tested 18 individuals with cystinosis, 7 matched control subjects, and 1 cystic fibrosis subject.  Despite the efforts of our consultant Dr. Mark Pian, the pediatric pulmonologist at Children’s Hospital, we have been having difficulty recruiting and inducting cystic fibrosis patients due to their hectic schedules.  We are now planning to test CF children and adolescents when they are admitted into the hospital for medical care, as long as they are stable enough medically to complete the testing. We have also initiated plans to advertise for CF patients in Orange, Los Angeles, and Riverside counties, which should increase our numbers considerably.

  1. RESULTS

On all 26 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 just completing the first half of this research study, we have not yet analyzed any of the data.

 

 

 

 

 

 

 

 

 

Academic Functioning, page 2

 

  1. FUTURE PLANS

      In the next 6-month period, we plan to continue to recruit, induct, and test individuals with cystinosis, cystic fibrosis, and control participants, as we work toward the total projected participants.  We are arranging for cystinosis families to travel here to San Diego, as well as traveling to cystinosis families in various parts of the country.  Once final approval is granted by Children’s Hospital, we plan to increase our numbers of cystic fibrosis subjects by testing them while they are staying at Children’s Hospital in San Diego.  We also plan to travel to adjacent counties to test cystic fibrosis patients once they have been identified. Our efforts to recruit controls are also ongoing, and we have a plan in place to step-up the recruitment and induction of control participants.