• Describe the early development of boys with fragile X syndrome
  
• Describe stability and change in the behavioral and temperament characteristics of boys with fragile X syndrome
  
• Describe the strengths of boys with fragile X syndrome
  
• Understand family challenges associated with fragile X syndrome
  
• Identify factors associated with variability in development
  
• Determine the perceptions of parents, teachers and allied health professionals regarding the unique learning features associated with fragile X syndrome and the strategies they have adopted to address these needs.
  

Elementary School Boys Study

• Describe the developmental, functional and school achievement patterns of boys with fragile X syndrome;
  
• Identify factors that influence their development and achievement;
  
• Describe the development of social competence and peer relations;
  
• Identify the factors that influence social competence and peer relations;
  
• Describe the nature and quality of school experiences provided during the elementary years;
  
• Survey the needs and concerns of teachers working with boys with fragile X syndrome;
  
• Identify effective strategies for enhancing achievement and social competence; and
  
• Describe patterns of family coping and adaptation during the elementary years.
  

Methods

Participants

• 75 boys with fragile X syndrome and their families living in the southeastern United States were recruited to participate.
  
• Boys birth to six years of age were eligible for enrollment. Most of the boys joined the study between the ages of 30 and 54 months, although some were as young as 9 months old.
  
• Varying economic, racial, and ethnic backgrounds are represented within the group.
  

Staff


• A multi-disciplinary staff of professionals with experience serving young children with special needs was recruited to conduct the research assessments.
  

Data Collection

• The research design was intentionally "family-centered" in order to make participation as stress-free as possible for children and families.
  
• Project staff travels to the home community of each participant for semi-annual (and later annual) assessments.
  
• Developmental testing with children takes place in a familiar environment, their home, school, or child care center. Assistance is sought from parents, teachers, and therapists to assure the childís comfort.
  
• Interviews with parents coincide with the child assessments and surveys and questionnaires are collected during visits.
  
• A summary report from each assessment and a small participation stipend are provided for parents.
  
• Classroom observations are made to document educational practices, engagement in learning activities, and instructional modifications.
  
• An evaluation of an instructional strategy in classrooms is being conducted with the collaboration of several teachers using a sub-set of study participants.
  

Measures

• Development, Achievement, and Cognitive Skills
  
  • Battelle Developmental Inventory (BDI) for boys eight and younger
    
  • Woodcock-Johnson Tests of Achievement, Revised
    
  • Beery-Buktenica Developmental Test of Visual-Motor Integration (VMI)
    
  • Leiter International Performance Scale-Revised
    
  • Mullen Scales of Early Learning
    
  • Vineland Adaptive Behavior Scales
    
  • ABILITIES Index
    
• Temperament and Behavior
  
  • Carolina Record of Individual Behavior (CRIB)
    
  • Childhood Autism Rating Scale (CARS)
    
  • Child Behavior Checklist, Teacher and Parent Versions
    
  • Carey Temperament Scales
    
  • Rothbart Temperament Scales
    
  • Self-Injurious Behavior Questionnaire, Teacher and Parent Versions
    
  • Social Skills Rating System, Parent and Teacher Versions
    
  • Friendship Survey
    
  • Sensory Profile
    
  • School Function Assessment
    
  • Eye Contact and Stereotypic Behavior Rating
    
  • Physical Features Checklist
    
• Family
  
  • FAMILIES Index
    
  • Family Support Scale
    
  • Family Needs Survey
    
  • Transition and Service Satisfaction Interview
    
• Education
  
  • Teacher and Therapist Surveys and Interviews
    
  • Practices in Early Elementary Classrooms
    

Results

1. Developmental and educational assessments. Data collection is ongoing; however preliminary findings suggest that there is great variability in the development of young boys with fragile X syndrome. As a group, development appears to proceed at about half the anticipated rate of typical children. Children who also meet the diagnostic criteria for autism using the CARS show significantly greater delays and slower developmental progress throughout the preschool and early elementary years.
   
2. Temperament. So far, data have been analyzed for 45 boys, ages 47 through 88 months. Results indicated considerable variability in temperament profiles for these boys. A characteristic temperament profile was not identified for fragile X syndrome; however, boys with fragile X differed significantly from the reference sample of typical children on five of the nine temperament dimensions. They were more active and less intense, approachable, adaptable, and persistent. No significant differences were found in distractibility, rhythmicity, mood or sensory threshold. Only 36% of the boys were classified as "easy", "difficult" or "slow-to-warm-up". The rest fell in intermediate ranges. Severity of disability and temperament ratings were not related, and scores were stable over time. Our results suggest that many of the behaviors observed in boys with fragile X syndrome may actually be related to temperament.
   

References

• Bailey DB, Hatton DD, Skinner M (1998). Early developmental trajectories of males with fragile X syndrome. American Journal of Mental Retardation, 103, 29-39.
  
• Hatton, D. D., Bailey, D. B., Jr., Hargett, M. Q., Skinner, M., & Clark, R. D. (1999). Behavioral Style of Young Boys with Fragile X Syndrome. Developmental Medicine and Child Neurology, 41, 1-8.
• Bailey, D. B., Hatton, D. D., Mesibov, G., Ament, N., & Skinner, M. (2000). Early development, temperament, and functional impairment in autism and fragile X syndrome. Journal of Autism and Developmental Disorders, 30(1), 49-59.
• Bailey, D. B., Mesibov, G., Hatton, D. D., Clark, R. D., Roberts, J. E., & Mayhew, L. (1998). Autistic behavior in young boys with fragile X syndrome. Journal of Autism and Developmental Disorders, 28, 499-508.
• Bailey, D. B., & Nelson, D. (1995). The nature and consequences of fragile X syndrome. Mental Retardation and Developmental Disabilities Research Reviews, 1, 238-244.
• Hatton, D. D., Bailey, D. B., Roberts, J. P., Skinner, M., Mayhew, L., Clark, R. D., Waring, E., & Roberts, J. E. (2000). Early intervention services for young boys with fragile X syndrome. Journal of Early Intervention, 23, 23-37.
• Hatton, D. D., Buckley, E. G., Lachiewicz, A. M., & Roberts, J. E. (1998). Ocular status of boys with fragile X syndrome: A prospective study. Journal of the American Association for Pediatric Ophthalmology and Strabismus, 2, 298-302.
  
• Hatton, D. D., Hooper, S. R., Bailey, D. B., Skinner, M. L., Sullivan, K. M., & Wheeler, A. (2002). Problem behavior in boys with fragile X syndrome. American Journal of Medical Genetics, 108, 105-116.
• Roberts, J. E., Hatton, D. D., & Bailey, D. B. (2001). Development and behavior of male toddlers with fragile X syndrome. Journal of Early Intervention, 24(3), 207-223.
• Symons, F. J., Clark, R. D., Roberts, J. P., & Bailey, D. B. (2001). Classroom behavior and academic engagement of elementary school-aged boys with fragile X syndrome. Journal of Special Education, 34(4), 194-202.
• Symons, F. J., Clark, R. D., Hatton, D. D., Skinner, M., & Bailey, D.B. (2003). Self-injurious behavior in young boys with fragile X syndrome. American Journal of Medical Genetics, 118A(2), 115-121.