Fragile X Syndrome and School Behavior

Abstracted presentation at Texas Association of School Nurses Annual Conference (Corpus Christi, November ’97) — (Marcia Braden, PhD)

Fragile X syndrome may be underlying behavioral concerns in the classroom for undiagnosed students, especially in early childhood. Behaviors reflect obsessive-compulsive tendencies, hyperactivity, attention deficit, and autism.

Fragile X syndrome is the second most common chromosomal cause of mental retardation. The severity of cognitive, language and behavioral outcomes may be related to the actual number of CCG amino acid repeats at the affected site on the lower end of the X chromosome.

Sensory integration problems are chiefly responsible for "acting-out" behaviors. Behaviors such as crawling under a table or tantrums are efforts to escape the sensory overload they perceive. These children can be hypersensitive to lighting, noise, food and clothing textures, odors, touch, and sudden changes in routine. Careful observation and documentation about what preceded "acting out" can assist the teacher and occupational therapist to plan intervention strategies. For example, "white noise" audiotapes may mask distracting sounds, and fluorescent lights should be replaced by natural recessed lighting.

Eye contact is more aversive to a child with fragile X syndrome than to autistic students. Forced eye contact can trigger arousal and extreme discomfort which is expressed in hand flapping, perseverative speech, hand rubbing (sweaty palms), and aggressive behavior. Interventions include not forcing eye contact, allowing student to wear sunglasses, or asking educational diagnostician to wear sunglasses when administering appraisal tests. Students with fragile X syndrome already tend to do poorly on IQ tests because it is a change in routine, but they perform better on achievement tests which are related to classroom routines.

COMMENT: Dr. Braden’s book, Fragile X: Handle with Care (1997), is an excellent resource for school staff and families to understand the school-age child and adult with fragile X syndrome and contains specific guidelines to address the diverse social, language, behavioral and physical issues. It is available through Avanta Publishing at 800/434-0322 or www.fragilex.com. As a result of the session and the book, I plan to review the assessment information on some of my Special Education students whose teachers have been concerned about "acting out" behaviors. -J.O.