A Preschooler
with Fragile X Syndrome

Jamie is an animated four year-old boy who has fragile X syndrome. In spite of being in perpetual motion, he is an astute observer who doesn’t miss much. Jamie is curious and interested in people, toys, and activities and can be engaging and fun to be around. However, his personality is a study in extremes-- his likes are almost obsessions, and his dislikes can produce catastrophic responses. For example, going into the auditorium at his school makes him cling to his teacher, cry, stiffen, and howl in fearful protest. These unpredictable and extreme responses have posed significant challenges to his parents, making major re-organization of family life necessary. His parents, Beth and Michael, when describing his impact said, “For the first three years of his life, we just stayed home. It was too stressful to go anywhere. He still doesn’t sleep well, and sometimes we are just worn out.”

When he was three and a half, Jamie was diagnosed with fragile X syndrome. Learning about FXS gave his parents a better understanding of the link between Jamie’s behavior and his sensory processing difficulties. Beth and Michael realized that many of Jamie’s symptoms, such as avoiding groups, resisting touch, and mouthing objects, were common in children with fragile X. His puzzling behaviors began to make sense. They now understand that he mouths objects, smells them and overstuffs his mouth with food as a means of seeking out sensory input. His tantrums often happen when he becomes over-stimulated by his surroundings. Things that do not bother most people are annoying to Jamie because he processes tactile information differently. For instance, he always wants to wipe his hands as soon as they get messy, whereas most children his age don't mind the mess. Beth and Michael found several articles that contained suggestions on calming activities for children with sensory problems. Many of the ideas were simple to implement, such as giving Jamie pretzels or sugarless gum to replace his habit of chewing on his shirt collar.

Jamie communicates with two- and three-word phrases but often has difficulty comprehending what others say to him. He is difficult to understand due to the hoarse quality of his voice and rapid rate of speech. His parents describe him as an affectionate, loving child who enjoys interacting with other people, but only with one or two people at a time. He becomes overwhelmed when too many people are present. They also say that Jamie thrives on routine and structure. When faced with unexpected schedule changes or interruptions, he becomes non-compliant. For instance, Beth recently had to take her daughter to the doctor unexpectedly. When she asked Jamie to get ready to go with them, he defiantly shook his head, crossed his arms and said, “Uh-uh.” By the time she got him in the car, he was having a full-fledged tantrum, which included pulling off his socks and shoes. Although he is usually able to calm himself after some regrouping time, he does tend to “shut down” if too many things upset him. When he is in such a state, he becomes quiet, sometimes closes his eyes and is unresponsive when others try to talk to him.

His mother, Beth, recalls that his first experience in the regular childcare center was “ a nightmare.” Jamie started attending the center two days per week and gradually increased until he was there five days per week from 8:30 a.m. - 12:30 p.m. The other 12 children in his class were typically developing 2 to 3 year-olds. At first, Jamie had a difficult time adjusting to the new setting. He threw tantrums each time Beth dropped him off, and his teachers reported that it took a long time for him to calm down afterwards. After the first two weeks, though, the tantrums became less intense and Jamie only cried for about 5 minutes each time.

Jamie often laughed and smiled when he interacted with his teachers one-on-one, but his behavior became a problem during group activities. He resisted his teachers' attempts to coax him into sitting with the other children during circle time, so his teachers had to gently bounce him on their laps in order to get him to stay. Despite their good intentions, circle time was just too overwhelming for Jamie, and he could only tolerate it for about 2-3 minutes before he began to whine and tantrum. Beth finally had to ask the teachers to let Jamie stay in the play area during circle time in order to avoid these tantrums.

Over the next few months, Beth struggled to help Jamie do well at the center because she felt strongly that he needed the experience of being around typically developing children. He used many words but was not yet putting two words together. She wanted him to have appropriate role models so that his language and social skills could develop further. The stress of trouble-shooting Jamie’s problems at day care, however, began to take its toll.

After four months at the center, Beth began to seriously consider other placement options for Jamie. The staff loved Jamie and wanted to work with him, but his behavior problems indicated that he was unhappy and stressed by the childcare environment. Each day when Beth picked him up, Jamie began to strip off his socks, shoes and sometimes even his shirt on the way home. She also noticed that he was scratching the skin on his arms to the point where it was red and inflamed. She and her husband had never seen these behaviors before and knew they had to do something different for Jamie.

Beth and Michael began to search for other options. They discussed the issue with Jamie's early intervention coordinator, Gina, who agreed that he seemed to do best with one-on-one attention in a more subdued environment. She recommended a special education preschool class, since the classroom was highly structured and offered low child-to-adult ratios. Both parents were initially opposed to this placement. A visit to the classroom, however, changed their point of view. They thought the structure in the class would be good for Jamie, and they were hopeful that it would reduce the frequency of his tantrums. In addition, they liked the emphasis on developing the language and social skills of the children.

When Jamie was almost three, he began attending the special education preschool class, which he currently attends. His class has six other children, one teacher and two assistants. Jamie’s first week at school was very rough, but, once his teacher established his routine, he settled in well. He now loves going to school, and his parents are happy with the progress he is making there. Many of his troubling behaviors -- such as stripping off clothing have ceased, and his tantrums are much less frequent. He continues to receive OT and speech services. His occupational therapist uses sensory integration techniques to help him calm down and reorganize throughout the day.

Recently Jamie has been talking more, and his parents have decided to encourage his language development by bringing him two afternoons per week to a friend’s home day care. They chose to place Jamie in this setting because it is smaller and less noisy than a regular childcare center. The home childcare provider cares for three typically developing children, ranging from ages 2 to 5. When Jamie is there he uses language more frequently than at his preschool, and his parents are thrilled with this progress.

Jamie’s parents work daily toward their goal of making Jamie as independent as possible. His current challenge is becoming toilet trained. Although he often urinates in the toilet when taken to the bathroom, he does not initiate going on his own. His bowel movements have always been loose, and he has had no success controlling them. He still wears pull-ups due to his frequent accidents, and he is on an hourly bathroom schedule at school.

Jamie's parents are considering medication to address his hyperactivity. Finding the right preschool setting helped improve his behavior significantly, but they are thinking ahead to kindergarten and wondering how increasing demands will affect him. They continue to struggle with his poor sleep habits and hope he will soon begin sleeping through the night. Naturally, they wonder about his future and what he will be like when he is older. “But right now,” sighs Beth, “we're just taking one day at a time.”