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summer 2004 | early developments 1 Summer 2004 | Volume 8 #2 Fragile X Syndrome FPG Child Development Institute The University of North Carolina at Chapel Hill earlydevelopments2 early developments | summer 2004 news FPG project featured on CNN show ■ Young adults who received early educational intervention had signifi cantly higher mental test scores from toddlerhood through age 21 than did untreated controls. ■ Enhanced language skills in the children appears to have mediated the effects of early intervention on cognitive performance. ■ Reading achievement scores were consistently higher for individuals with early intervention. ■ Mathematics achievement showed a pattern similar to that for reading, with treated individuals earning higher scores. More information about the Abecedarian Study may be found at <www.fpg.unc.edu/~ABC/> The Abecedarian Study, a long- running FPG research project, was highlighted in a recent prime-time CNN show, “The Gap—50 Years After the Brown Decision.” Dr. Frances Campbell, who directs the study, was featured in the show. The Abecedarian Project is a carefully controlled study in which 57 infants from low-income families were randomly assigned to receive early intervention in a high quality child care setting and 54 were in a non- treated control group. The treated children received full-time educational intervention in a high quality child care setting from infancy through age 5. Investigators have now completed a young adult follow-up assessment of study participants. At age 21, cognitive functioning, academic skills, educational attainment, employment, parenthood, and social adjustment were measured. Major fi ndings of the young adult follow-up include these:summer 2004 | early developments 3 ISSN 1536-4739 Editors Pam Winton, Virginia Buysse Writer John Manuel Designer Gina Harrison Photographers Don Trull John Cotter Assistant Editor Satsuki Scoville www.fpg.unc.edu www.ncedl.org Early Developments is published three times a year by the FPG Child Development Institute at The University of North Carolina at Chapel Hill. To subscribe or to change your address contact Jay Hargrove CB #8185, UNC-CH Chapel Hill, NC 27599-8185 (919) 966-0888 email@example.com To order additional copies contact FPG Publications Offi ce (919) 966-4221 FPGPublications@unc.edu Periodicals postage paid at Chapel Hill, NC Total design, production, & printing costs of this issue of Early Developments were $8661. 12,000 copies of this document were printed at a cost of $6441, or 53¢ each. Photo Notes: Stock Photo page 14; Photos on pages 4, 9, 10, 16, courtesy of the Carolina Fragile X Project. Special thanks to the Hamilton family for their photos illustrating the article on page 9 and from which the cover illustration was derived. |ed| early developments Summer 2004 | Volume 8 #2 Director’s Notes 4 Going the Distance with Fragile X 6 All in the Family 9 Screening Newborns for FXS 11 The Behavioral Challenge 14 contents Speech, Language, and FXS 16 Finding Clues in Family Videos 18 Fragile X Info Center 19 Learn More About FXS at FPG 20 FPG Publications 22 4 early developments | summer 2004 IN 1993, some colleagues and I began a study of a little-known disorder—fragile X syndrome. The gene for fragile X had just recently been discovered, and there was lots of excitement, for many reasons. Although the disorder had been described for more than 20 years and it was almost certainly inherited, what went wrong and how it was inherited was not known. Offi cials at the NIH were excited because it was one of the fi rst examples of how the Human Genome Project, a massive effort to “map” the human genome, could lead to discoveries pinpointing the causes of hundreds, if not thousands of disorders. Genetic researchers were excited because this discovery helped them to understand an unusual inheritance pattern that could not previously be explained. Many parents were excited because they fi nally had a better understanding of the disorder affecting their child. And the fact that fragile X is a single gene disorder gave hope to many that with the advent of gene therapy, a cure might be possible. But of course, as with most discoveries, the answer to one question opened the door to many others. Knowing a gene fl aw or “mutation” doesn’t mean that you know everything about how it works. Genes, which are made up of DNA, provide instructions for making RNA, which then leads to proteins, which control all aspects of human biological function. Fragile X syndrome affects the production of a single protein (FMRP) known to be necessary for normal brain development. While much progress has been made in understanding the biology of fragile X syndrome, many mysteries remain unsolved, and a cure seems just as far away today as it did in the early 1990s. Of course, we don’t do basic biological research at FPG. But we do study children’s development and behavior over time, and we are very interested in both the biological factors and the environmental factors that affect behavior and development. When we started our research in 1993, no one had studied the earliest development of children with fragile X. So we began an early childhood study, starting with children in North Carolina, South Carolina, and Virginia under the age of six. Finding nearly a hundred families of young children with fragile X was quite a challenge. But with the help of a great research team and lots of people willing to help, we did it. We have been tracking these children ever since, and some of the oldest are now getting ready to enter high school. Along the way we have involved professionals from many different disciplines and have examined the many ways in which fragile X can affect both children and families. These studies are still under way, and we are now in the midst of planning a very large study to screen 1,000,000 newborns for fragile X. Director’s Notes by Don Baileysummer 2004 | early developments 5 This work has been immensely gratifying. Many different people at FPG and across the university are now actively involved in fragile X research. With a strong publication record and the most comprehensive longitudinal study of fragile X ever conducted, we are well positioned to continue this work into the future. However, what we have learned from all of this goes well beyond our publications and well beyond fragile X. X Biology is just as complicated as behavior. Compared with human development, which is enormously complicated and almost defies a “scientific” explanation, I thought biological function was relatively straightforward. In fact, genes regulate a complicated biological system with many different interactions and pathways that may never be fully understood. Although fragile X was thought to be a single gene disorder involving a single protein, in reality, it likely regulates and interacts with many other genes and many other proteins in a complex and changing pattern. X No one person or one discipline can fully understand any phenomenon. Over the years we have worked with psychologists, neurologists, psychiatrists, special educators, genetic counselors, anthropologists, speech and language pathologists, occupational therapists, and many others who have helped to provide insights into the multifaceted aspects of this disorder. X A focus on a single disorder still allows you to address “big” issues. For example, we now have the technology and knowledge to identify many disorders earlier than we currently are. Is it worth the investment needed to identify disorders earlier? Our work on newborn screening for fragile X will serve as a prototype for early detection of other disorders. X You don’t always know where your research will lead you. We started with a small study looking at some focused areas of development. One thing that has led to our success has been our willingness to look beyond the parameters of what we were funded to do. When we find something interesting, we study it. X Good research takes a long time. Of course, we would all like immediate answers to questions. After more than 10 years, you would think we would have fragile X figured out. Although we know a lot more now than we did when we began, much remains to be learned. This is especially true when you do research that looks at how lives change over time. I cannot end this column without acknowledging several groups of people. First, are all of the individuals who have worked on this project throughout the years. I hesitate to mention names, because there are too many, but you know who you are. Suffice it to say that this has been a true team effort. Second, we appreciate the support of many different agencies and foundations. We have received funding from the Office of Special Education Programs, the National Institute for Child Health and Human Development, the FRAXA Foundation, the National Fragile X Foundation, and Ronald McDonald House Charities. Finally, throughout this project, we have had the privilege of getting to know a group of remarkable families. They have allowed us to study just about every aspect of their children’s lives and now their families’ lives as well. They have been amazingly tolerant and giving of their time and energy, hoping that this work will ultimately pay off. And they have inspired us to continue. We dedicate this work to them. |ed| 6 early developments | summer 2004 Going the Distance with Fragile X ELEVEN YEARS AGO, FPG Child Development Institute (FPG) launched a longitudinal study of a little known form of mental retardation known as fragile X syndrome (FXS). The Carolina Fragile X Project has since grown into a multidisciplinary team studying diverse aspects of the condition, ranging from early identification to school performance. The team’s research has led to wide understanding of what constitutes the condition, how it can be detected, strategies for intervention, and how to help families cope. FPG researchers have published thirty-two journal articles and chapters on FXS and many more are in the works. The team is working on seven different grants totaling more than $2.2 million a year. Next spring, FPG will publish a book on early intervention for young children with FXS. FPG Director Don Bailey has written the definition of fragile X for the online version of the World Book Encyclopedia. “You could say we have put fragile X on the map,” Bailey says. What is fragile X? Fragile X syndrome is the most common inherited form of mental retardation. First described as Martin-Bell syndrome in 1943, it became known as fragile X syndrome in 1969 when Herbert Lubs discovered an unusual constriction and occasional break at the end of the X chromosome. In 1991, scientists discovered that FXS results from a mutation known as a trinucleotide repeat expansion, in which a series of three nucleotides (CGG) in the DNA is expanded beyond its normal size. This genetic mutation disrupts A Fragile X Glossary autism: a complex developmental disability that typically appears during the first three years of life FMRP: protein necessary for brain development. Production is affected by fragile X syndrome; appears to be involved in synapse maturation and elimination fragile X syndrome: the most common inherited form of mental retardation. Effects can vary from subtle developmental delays to major impairments full mutation [of FXS]: is indicated by 200 or more repeats of the DNA sequence mutation; individuals will experience the impairments and delays associated with the syndrome genotype: the genetic makeup, as distinguished from the physical appearance, of an organism or a group of organisms hyperarousal: a tendency to become easily overstimulated and to overreact to changes in environment or routines hyposensitivity: less than the normal ability to respond to stimuli Individuals with Disabilities Education Act (IDEA): the law that guarantees all children with disabilities access to a free and appropriate public education, and provides services and resources to infants and toddlers with disabilities and their families phenotypic expression: the observable physical or biochemical characteristics of an organism, as determined by both genetic makeup and environmental influences. In reference to fragile X, refers to how the disorder appears in people’s behavior, development, and physical features premutation carrier [of FXS]: is indicated by 50–200 repeats of the DNA sequence mutation; individuals can transmit the disorder to their children, but may not be affected themselves vagal tone: an index of neural control of the heart summer 2004 | early developments 7 Research Focus the messages sent to make FMRP, a protein necessary for normal brain development. The normal range of CGG sequences is 5–50 repeats. An individual with 50–200 repeats is a premutation carrier of FXS, meaning they can transmit the disorder to their children, but may not be affected themselves. Individuals with 200 or more repeats have the full mutation of FXS and will experience the impairments and delays associated with the syndrome. For individuals with FXS, effects can vary from subtle developmental delays to major impairments. Males with the full mutation are more severely affected than females. Most males will have mild to severe mental retardation with cognitive and communication skills most likely to be affected. They may seem shy, anxious, and inattentive. Hyperarousal, a tendency to become easily overstimulated and to overreact to changes in environment or routines, is a common condition. Males have several distinguishing physical features, including large ears, loose joints and muscles, and an elongated face. Females generally have milder impairments. About one third will exhibit normal development, another third will exhibit learning disabilities, and about one third will have mental retardation. Females with FXS may also be shy and exhibit social problems. The early years The foundation of the Carolina Fragile X Project began with a series of longitudinal studies following the development of children with FXS beginning in preschool. Initially funded by the Office of Special Education Programs in the US Department of Education, these studies have been headed by Bailey with co-investigator Deborah Hatton. The first study, begun in 1993, focused on a group of 75 preschool boys. Successive grants in 1995 and 2001 have followed this same group of boys through elementary and middle school. A separate study on young girls was initiated in 1996. Collectively, those studies have yielded a wealth of information on FXS. They suggest that although parents usually notice problems much earlier, the typical child with FXS is not diagnosed as developmentally delayed until 21 months and not formally diagnosed with FXS until 32 months. They show considerable variability in early development, with some children progressing much faster than others. Boys have been found to have a higher activity level, to be slower to adapt to new situations, to be less persistent and more withdrawn than typical children. Roughly one quarter of boys with FXS are also classified as autistic, and experience more serious delays than children with autism or FXS alone. Researchers also have collected information about the early intervention or educational services that children with FXS receive, as well as parental perceptions of these services. This work led to a major “synthesis” conference in 2004 where experts discussed the features of an early intervention program most likely to be successful for children with FXS. Researchers are now planning a large study to determine the effectiveness of earlier intervention. School performance is a crucial part of the longitudinal studies. Analysis of elementary school experiences shows that by second grade, most boys are placed in self-contained classrooms that only serve children with disabilities and that by age 8, many children still experience challenges in performing basic functioning skills. Data on middle school performance are now being collected. 1993 | Carolina Fragile X Project 1996 | Characteristics of Girls with FXS 1996 | LanguageDevelopment & Speech Characteristics in Boys with FXS 1996 | FMRP & Variability of FXS 1998 | Physiological/ Behaviorial Variables in Boys with FXS 1999 | EarlyIndicatorsof FXS8 early developments | summer 2004 Further refinement and recent studies Beginning in the mid-90s, FPG researchers began expanding the scope of their research, looking at particular aspects of FXS. n In a 1996 study funded by the National Institute on Disability and Rehabilitation Research, Joanne Roberts and Penny Mirrett examined the language development and speech characteristics of boys with FXS. n In another study, Bailey and co-investigators Hatton and Annette Taylor examined the relationship between levels of FMRP, the protein affected by FXS, and the variability in effects of FXS on individuals. n Bailey and Jane Roberts investigated the relationship between physiological and behavioral variables in boys with FXS. n In 1998, Jane Roberts received a grant from the FRAXA Foundation to examine classes of medication and their effects on the physiological and behavioral responses of boys with FXS. n The following year, Bailey, Hatton, Roberts, and Mirrett launched an investigation of the early development of infants, identified the earliest indicators of the syndrome, examined issues related to early screening of infants, and evaluated the usefulness of screening protocols used by pediatricians and other clinicians. This study has led to a planning grant from the NIH to develop a study for newborn screening for FXS. Since the year 2000, FPG researchers have focused much of their research on children’s development and the experience of families of children with FXS. n Joanne Roberts and co-investigator David Zajac of the School of Dentistry are continuing their research into speech and language with three studies funded by the National Institute of Child Health and Human Development and the March of Dimes. One is studying the language development of three groups of preschool and early elementary-aged boys—one group with FXS, another with Down syndrome, and one with typical development. The other two projects are examining the hearing, speech, and language development of young boys with FXS compared to young boys without the disorder. n Bailey and co-investigators Hatton, Stephen Hooper, Peter Ornstein, Jenni Schaaf, and Martie Skinner are midway through a project on Attention, Memory, and Executive Function in FXS. This project seeks to determine how children with FXS attend to important signals in their environment, remember key events and facts, and use this information to make decisions and solve problems. n Bailey and colleagues were awarded a major center grant from the National Institute for Child Health and Human Development. Entitled Family Adaptation to Fragile X Syndrome, this project seeks to describe and explain the variation in the extent to which parents of children with FXS experience a positive quality of life; construct environments that promote cognitive, language, academic, and adaptive development in their children; and promote social-emotional development and address challenging behaviors in their children with FXS. “The amazing thing about this research is the many directions it has led us over the years,” says Bailey. “We have learned so much from the children, from families, and from other researchers that has caused us to keep asking new questions and seeking new answers. Our ultimate goal is to do research that helps to shape future policy decisions about early identification and early intervention that provides appropriate support for both children and families.” |ed| Current Efforts 2000 | Family Adaptation to FXS 2001 |Language Development:Boys with & without FXS,Boys with Down Syndrome 2001 | AttentionMemory, & ExecutiveFunction:Children with & without FXS 2001 |Hearing: Boys with & without FXS 2003 | Speech:Boys with & without FXS,Boys with Down Syndrome 2003 | PlanningNewbornFXSScreeningsummer 2004 | early developments 9 All in the Family MUCH RESEARCH HAS BEEN CONDUCTED on the underlying biology of fragile X syndrome. Investigators are also well on their way to understanding and describing how FXS affects behavior and development. By comparison, little attention has been paid to the family consequences of fragile X. With the funding of FPG’s Fragile X Research Center, that is beginning to change. The Fragile X Research Center is a joint effort between The University of North Carolina at Chapel Hill and the University of Kansas. Its aim is to understand how families cope with FXS and what might be done to help support families. Eleven investigators from six disciplines—anthropology, developmental psychology, special education, speech and hearing sciences, quantitative psychology, and psychiatry—are collaborating in this effort.10 early developments | summer 2004 Why bother to study the family consequences of FXS? “In some respects, the challenges that families of children with FXS face are similar to those of children with other disabilities,” says Don Bailey, FPG director and head of the Fragile X Research Center. “But in many ways, the family dimensions of FXS are unique. This is particularly true in the areas of children’s learning, their behavior problems, and the inherited aspects of the disease.” Most children with FXS have signifi cant developmental delays that impact school performance and independent functioning. Families must constantly work to organize their home and fi nd specialized services to help their child learn language, cognitive, and academic skills. Children with FXS may also exhibit a wide range of behavioral and emotional problems, including social anxiety, self-injury, autistic behavior, and other challenging behaviors. Parents must deal with the stress and burden of regulating emotion and problem behavior to help their child adapt to the larger world. “We’ve found that although a child’s learning problems can be hard for families, it is the behavioral problems that are more likely related to serious family stress,” Bailey says. “Families of children with FXS may not be able to take their child out to a restaurant or the mall without that child acting out in some fashion. That can dramatically alter the family’s life.” Bailey says FXS is also unique for families in that it is inherited. Usually parents give birth without realizing they have the gene that carries the disease. Individuals who carry the gene must decide when and how to tell other family members and encourage them to get genetic testing. Women with the gene must consider that they risk having additional children with FXS. Mothers of children with FXS may feel guilty and suffer from depression. Mothers who have the full mutation themselves may face challenges in caring for their children. The Center is following three broad aims in its research. The fi rst is to describe and explain the extent to which parents of children with FXS experience a positive quality of life, are hopeful for the future, and are protected from adverse mental health outcomes (such as anxiety or depression). The second is to describe and explain the extent to which parents are able to provide environments that promote their children’s cognitive, language, academic, and adaptive development. The third aim is to describe and explain the extent to which parents are able to promote social-emotional development and deal with challenging behaviors. Center investigators are collaborating on three projects involving a total of one hundred families. Data are being collected using a range of methods, including surveys, semi-structured interviews, ethnographic interviews, and direct observation. “We aren’t just looking at the negative consequences for families,” Bailey says. “Many families have rallied together, fi nding a common purpose in helping their child cope with this disease. In fact, we have met many remarkable families who inspire all of us with the amazing things they have done.” “Ultimately, we are trying to understand why some families do well and others are more challenged,” he adds. “Understanding this will help us provide the proper supports to families that need them. The bottom line is helping them achieve the quality of life for which they hope.” The Fragile X Research Center is funded by the National Institute of Child Health and Human Development. The Center was awarded $6 million for a fi ve-year period beginning in July of 2003. |ed| Acknowledgment: We are grateful to the Hamilton family for sharing photos from their family vacation album for this article. … although a child’s learning problems can be hard for families, it is the behavioral problems that are more likely related to serious family stress … . summer 2004 | early developments 11 KATHY MAY noticed there was a problem with her son, Sam, within weeks of his birth. He was not easy to console and always seemed to keep his hands clasped. At 6 weeks, she tried unsuccessfully to get Sam to look at her when she clapped her hands. At 15 months, Kathy’s doctor expressed concern that Sam’s language development was behind. She took Sam to an early intervention service provider who, suspecting he was autistic, recommended that he be tested at the local children’s hospital. At 20 months, the diagnosis was confirmed—Sam had fragile X syndrome. Compared to most families of children with FXS, the Mays were fortunate. Sam was diagnosed with FXS earlier than most children. According to a recent FPG study, the average boy with FXS is not diagnosed with FXS until nearly 3 years of age, and many are only identified much later. Had these children been identified earlier, they would have been immediately eligible for early intervention services under the Individuals with Disabilities Education Act. FPG’s study revealed another consequence of the late determination of FXS—more than half of the families surveyed had additional children without knowledge of the reproductive risk. Of the 191 children who were born to these families after the birth of their first child with FXS but before the FXS diagnosis, 109 (57%) also had FXS. Thus, a substantial proportion of the families ended up with two children with the disorder, imposing additional caregiving demands and stress. “Our study showed that parents perceive the discovery of FXS as a process that takes too long,” says Don Bailey, director of FPG. “One alternative is to establish a program of newborn screening.” Screening Newborns for Fragile X 112 early developments | summer 2004 With the discovery that FXS is a genetic disorder and the subsequent development of accurate DNA testing, it is now possible to genetically screen the population for FXS. Possible approaches include screening the following groups: women of childbearing age, pregnant women, newborns, and children at the first sign of developmental delay. FPG researchers have concluded that newborn screening provides distinct advantages over the other options. Whether that policy will become widespread remains to be seen. In the US, states determine whether to screen newborns for genetic disorders, which disorders to screen for, how to finance screening, and what follow-up mechanisms are in place to provide for treatment and support. Currently, all states mandate screening of newborns for phenylketonuria (PKU) and congenital hypothyroidism, and all states but one screen for galactosemia and sickle cell disease. Beyond that, there is wide variability among the states as to what they screen for. The question of which disorders to screen for remains the subject of considerable debate among policymakers, medical professionals, and the general public. Currently accepted guidelines rest on three fundamental criteria: 1) the disorder must be a significant public health problem that has major consequences for affected individuals, 2) an accurate, acceptable, and cost-effective procedure for screening the disorder must be available, and 3) a treatment must exist which, if provided early, can significantly alter the course of the disease or disorder. Bailey contends that FXS clearly meets the first of these criteria and could soon meet the second and third. “A recent review estimates that at least 1 in 4000 males are born with the full-mutation FXS, and 1 in 270 females may be a carrier. FXS exerts a clear and devastating effect on affected individuals, especially males, resulting in moderate to severe mental retardation, high levels of anxiety and arousal, and frequent instances of autism and self-injurious behavior. On that basis, I would contend that FXS presents a significant public health problem.” With respect to the second criterion, Bailey finds that while DNA testing for FXS is virtually 100% accurate, it is expensive, averaging between $200–$300 per test. However, new technologies may soon lower costs considerably. “It is quite likely that the cost of screening could be reduced to $10–$20 per child in the next 2–3 years, and other methods could be even cheaper,” he says. Currently, there are no medical treatments available for FXS, thus, this disorder does not appear to meet the third criterion (a treatment is available) using traditional standards. However, nonmedical treatment by early intervention programs may improve development among individuals with FXS and thus could be considered to alter the course of the disorder. “Recent research suggests that FMRP, the protein disrupted by FXS, appears to be involved in synapse maturation and elimination,” Bailey says. “If so, the most powerful interventions may be those that could be provided early in life during the period of rapid proliferation and pruning of neural connections. … at least 1 in 4000 males are born with the full-mutation FXS, and 1 in 270 females may be a carrier. 1early summer 2004 | early developments 13 “These facts and hypotheses, coupled with the existence of a nationwide system of services readily available for infants with disabilities, provide a strong logical basis in support of early intervention for children with FXS,” Bailey says. There are a number of negative consequences for delayed identifi cation of FXS. Parents experience frustration with professionals and doubts about themselves in their attempts to fi nd a professional who will acknowledge that their child’s development is atypical. Families, insurance companies, and the health care system face signifi cant fi nancial costs for the repeated visits that often happen before a diagnosis is made. Children and families miss out on the access to early intervention services provided by the Individuals with Disabilities Education Act (IDEA). Finally, families do not have access to information about carrier status of parents and thus may make future reproductive decisions without knowledge of the risk that a child will have FXS. Nonetheless, routine screening for FXS will not likely happen in the next 3–5 years since it does not fully meet the existing criteria for newborn screening, and also due to concerns about negative consequences for children (e.g., stigmatization), and controversies over issues related to reproductive decision-making and screening for carrier status. To help make decisions about the desirability of newborn screening, Bailey has embarked on an ambitious project. With planning grants from the National Institute of Health, the Maternal and Child Health Bureau, the Centers for Disease Control and Prevention, and the National Fragile X Foundation, he and his colleagues are developing plans for a massive research project. The goal is to conduct a study to determine the costs and benefi ts of newborn screening for FXS. To do so will require screening 1,000,000 newborns 4 of all children born in the US in a given year) and conducting follow-up monitoring and intervention studies with those who have FXS. The Human Genome Project has led to a dramatic increase in the number of known genetic causes of developmental disability. With rapid changes in technology, soon it will be possible to screen for many of these conditions, perhaps hundreds of them, as cheaply and easily as one. But just because screen for them, does that mean we should? “This study is important,” says Bailey, “not only because of what FXS, but also about newborn screening for other disorders. We’re about to enter a new era of genetic information, and research is desperately needed so that when policy decisions are made, they can be informed by solid research.” |ed| ,” . (about 1/we can it can tell us about FXS 14 early developments | summer 2004 The Behavioral Challenge WHEN 3-YEAR-OLD JASON WAS PRESENTED WITH A NEW CHILD CARE PROVIDER, he began to bite the back of his own hand. He would not look at the woman, despite his mother’s attempts to introduce them. At one point, Jason swung at his mother, hitting her on the leg with his tiny fi st. “I don’t know what to do with him when he gets like this,” Jason’s mother said. “It’s making my life really diffi cult.” According to FPG research, behavior, rather than cognitive delays, may be the greatest concern of parents and teachers of children with fragile X syndrome. Not every child with FXS exhibits serious problems, but many do, and that has prompted the research community to examine how a child’s genetics, brain, behavior, and environment act as a result of this single gene disorder. Deborah Hatton, co-principal investigator of the Carolina Fragile X Project, has been conducting longitudinal studies of temperament and behavior of children with FXS since 1994. Temperament refl ects biologically-based individual differences, while behavior refl ects how a child acts out his or her feelings. “Temperament and behavior challenges of children with FXS range from shyness to aggression toward others or self, and are more common among boys with the condition than among girls,” Hatton says. Hatton’s early studies, based on parent questionnaires completed annually over a 5–year time period, revealed that 3- to 8-year-old boys with FXS are signifi cantly more active and less adaptable, approachable, persistent and intense than typically developing boys of the same age. “Parents noticed the same behaviors in their boys that we observed during our study: high activity, attention problems, and withdrawing behaviors,” Hatton says. “We also found considerable variability in the behavior of boys in the study.” More recently, Hatton’s research on boys between the ages of 4 and 12 showed that nearly half (49%) scored within the borderline or clinically signifi cant range on total behavior problems, while more than half (57%) scored in that range on the attention and thought problems. Boys with autistic behavior were more likely to have high scores on thought problems and social problems. Studies show that 25–47% of boys with FXS display autistic behavior. In her research, Hatton looked for a correlation between maternal education and reported problem behavior. Interestingly, mothers with higher education reported more problem behavior, and thought and attention problems, than did their less educated peers. Boys who were taking medication had more total problem behavior, more internalizing problems, and more social problems. Attention seemed to be an area of particular concern. summer 2004 | early developments 15 Other research found that 73% of boys with FXS met diagnosed criteria for attention defi cit hyperactivity disorder (ADHD) compared to 33% of their age and IQ matched peers. Boys with FXS are also likely to engage in self- injurious behavior at some point in their lives. Hatton’s colleague Frank Symons found that hand biting is a common form of self-injury in children with FXS, and most likely to be evidenced when the child’s preferred routines were disrupted, when requests were made of the child, or when the child was faced with a diffi cult task. FPG’s research suggests there is an underlying physiological basis for the emotional and self-regulation problems of children with FXS. FPG investigator Jane Roberts has examined the relationships between physiological arousal, as indexed by heart rate variability, and vagal tone (an index of neural control of the heart) in boys with FXS compared to a control group of typically developing boys. Results suggest that boys with FXS have higher heart rates, even when resting. Furthermore, they did not display expected patterns of heart activity in response to phases of increasing challenge. Other researchers have reported that children with FXS displayed elevated levels of electrodermal activity (perspiration measured on fi nger tips) during a variety of sensory tasks and that they may have higher levels of the stress hormone cortisol. All of this evidence suggests that there may be an underlying physiological basis for the behaviors observed in children with FXS. FPG’s recent work indicates that the temperament and behavior of children with FXS may change radically between infancy and 12 years of age. According to Hatton, infants appear to be under-responsive to sensory stimuli, while preschool aged children are overly responsive. “In our studies of preschool children, we were struck by their high activity, low attention and adaptability, impulsivity, hypersensitivity to sensory stimuli, and reluctance to approach new people or situations,” she says. “In contrast, our clinical impressions of infants with FXS at 9, 12, and 18 months suggests a general hyposensitivity (lack of response) in longitudinal observational protocols of temperament, development, and sensory function.” Families face particular parenting challenges in dealing with children with FXS. Research suggests that parental characteristics and child temperament interact to infl uence family adaptation and child outcome over time. Positive outcomes occur when families adapt levels of environmental demand to meet the temperamental characteristics of the child, a concept known as “goodness-of-fi t.” Unresponsive or poor parenting can exacerbate challenging behaviors and the temperamental characteristics of irritability, distress, and negativity. “A parent who is laid back and calm and who has little structure to daily routines may be well suited to a fussy and highly sensitive infant,” Hatton explains. “A parent who is rigid, highly structured, and anxious may fi nd such an infant more challenging.” Recently, FPG has been awarded a grant to study Family Adaptation to Temperament and Challenging Behavior in FXS. Hatton is principal investigator of this project and Steve Reznick is co-principal investigator. Jane Roberts is examining physiology in the children in the study to see how it relates to temperament and behavior. “The behavior of children with fragile X syndrome is intriguing— particularly the changes that we have observed clinically over time,” Hatton says. “The possibility of linking behavior to physiological characteristics, such as heart rate, and to genetic variables, is exciting. While learning more about the behavior of children with FXS, we will also be searching for strategies that families and teachers can use to promote positive behavior in these children. If behavioral problems are controlled, children will be more likely to experience success developmentally and academically, and parents and teachers will experience less stress. This research has the potential to improve the lives of children and families and the professionals who work with them.” |ed| Positive outcomes occur when families adapt levels of environmental demand to meet the temperamental characteristics of the child, a concept known as “goodness-of-fi t.”16 early developments | summer 2004 Speech, Language, and FXS ASK FIVE-YEAR-OLD DAMON A QUESTION and his response is likely to be diffi cult to understand. When he meets new people, he becomes very quiet and avoids eye contact. Seven-year-old Tim answers questions in short sentences, but often is not on the topic being discussed. Tim and Damon’s speech characteristics are typical of boys with fragile X syndrome (FXS)—girls who carry this genetic mutation are considerably less affected. By identifying these characteristics early in childhood, researchers at FPG hope to aid in the assessment of children with FXS and prescribe intervention strategies that will improve the speech and language of these children as they mature into adults. Research into speech and language diffi culties among young boys with FXS began at FPG in 2001 under the direction of Joanne Roberts, FPG senior scientist and professor of pediatrics and speech and hearing sciences at the University of North Carolina at Chapel Hill. Roberts and co-investigators David Zajac and Jack Roush are assisted by two post-doctoral fellows, Elizabeth Hennon and Beth Barnes, project coordinator Kathleen Anderson, and research staff Anne Edwards, Cheryl Malkin, Julia Jurgens, Lauren Moskowitz, and Siara Cowan. Funded by the National Institute of Child Health and Human Development (NICHD) and the March of Dimes, the studies are examining the language of young boys with FXS in comparison to boys with Down syndrome and typically developing boys; and whether young boys with FXS have atypical hearing and auditory processing. The studies are also investigating the factors affecting poor speech intelligibility in conversational speech of young boys with FXS. summer 2004 | early developments 17 Roberts says most males with FXS will show moderate-to-severe delays in communication skills. “Phonological difficulties are common, including consonant substitutions and omissions, which is characteristic of developmentally younger children,” she says. “Conversational speech is often unintelligible, although single-word utterances often are understandable.” Other speech characteristics of some males with FXS include a rapid and fluctuating rate, and repetitive speech. Males may also have trouble repeating multisyllabic sequences and may demonstrate oral motor difficulties. With respect to language, boys with FXS often have delays in grammatical and vocabulary development. Some boys may have atypical pragmatic language, including frequent repetition of words, sentences, and topics, poor topic maintenance in conversation, difficulty answering direct questions, and gaze aversion. “The cause for these pragmatic impairments has often been attributed to hyperarousal, although word retrieval difficulties, syntactic difficulties, and executive function deficits also have been cited as possible causes,” Roberts says. Males with FXS often have prominent ears, although they do not appear to have hearing difficulties, Roberts says. FPG’s studies are among the first in the nation to examine the communication development of young children with FXS. Roberts hopes that her findings will aid both in the assessment of children with FXS and in developing appropriate intervention strategies. Because physical characteristics associated with FXS are not present in early childhood, language impairments may offer the first sign of the condition. Roberts hopes that her research will lead to increased awareness of these speech and language impairments and, thus, earlier detection. The cause of an individual’s speech and language difficulties and the particular domains affected will have important implications for intervention, although the specific contributing factors may be difficult to define. For example, if unintelligible speech is due to difficulties with sound patterns, then intervention should focus on particular sounds or suppression of specific phonological processes. However, if difficulties relate to such things as rate of speech, then slowing the rate of speech would be useful. For children whose communication difficulties are particularly evident in conversation, using scripts and routines in more structured environments and transitioning to more naturalistic environments with peers, teachers, and family members whenever possible would be more useful. “Our ultimate goal is to identify the types of intervention that are most appropriate for children with FXS,” Roberts says. “We want to know if there are specific strategies, different from those used with individuals with other forms of mental retardation, that we can recommend to help a child with FXS.” |ed| FPG’s studies are among the first in the nation to examine the communication development of young children with FXS. Photo: Don Trull, FPG Assessing speech and language skills involves pointing to pictures with verbal directions.18 early developments | summer 2004 Finding Clues in Family Videos Home videos are a great tool for preserving a family record, and especially for capturing those phases of early childhood that seem to pass so quickly. Now, FPG researchers are using retrospective analysis of home videos as a way of detecting previously unnoticed signs of fragile X syndrome among children aged 9 to 12 months. Signs of FXS are often very subtle among infants and toddlers and may not become obvious for months or even years. Even if parents suspect something may be wrong with their child, physicians may downplay their fears, believing that the child is at the late end of normal development, and defer developmental evaluations until later in the second year of life. The result is that FXS is typically not diagnosed (in children without a family history of developmental delays) until children reach two years of age. Valuable time is lost during which intervention strategies could have been used. Clearly, there is a need for better methods of early detection. But since children with FXS are rarely identified during the first year of life, studying their earliest development has been virtually impossible. Grace Baranek, FPG researcher and associate professor in UNC’s Department of Allied Health Sciences, has used an innovative strategy—retrospective video analysis—to address this issue. This approach involves collecting home videos of children later diagnosed with developmental problems and analyzing them to see if unusual behaviors or delays can be detected. Raters are trained to view the videos and code a variety of social and sensory-motor behaviors in the context of natural daily activities. Her first study using this technique showed that by 9 to 12 months of age, the behaviors of children later diagnosed with autism were markedly different from those of children with delayed or typical development. Baranek has just completed video analysis research on FXS conducted in a similar fashion to her autism research. She coded the same behavioral markers at 9 to 12 months, plus a few additional features such as repetitive movements that may reflect hyperarousal common to children with FXS. Findings so far confirm that even at this young age, children with FXS do exhibit markedly different behaviors than their normally developing peers, and can also be differentiated from children with autism. “Infants with FXS were showing significant delays in the way they played with toys,” Baranek says. “We also observed a high level of unusual postures, reflecting motor difficulties.” Baranek says her findings may not be practical yet. Although she has proved that early signs of developmental problems are evident during the first year of life, they may not be obvious to pediatricians or other professionals who don’t have the advantage of watching, rewatching, and coding videotapes. However, these findings provide important clues about how FXS is expressed in infancy and hopefully will stimulate other research on early development. Furthermore, “awareness of these early features of FXS may help a clinician to select evaluation methods and interventions that target the family’s specific needs,” Baranek says. “For example, activities designed to promote play skills, or environmental modifications that support engagement in daily activities would be recommended.” ■ 1early Photo: Don Trull, FPG Grace Baranek (upper left) examines home videos of infants at her laboratory with team members Fabian David and Cassandra Danko.summer 2004 | early developments 19 PARENTS AND PROFESSIONALS SEEKING MORE INFORMATION ON FRAGILE X will want to access FPG’s new website for the Fragile X Information Center (www.fpg.unc.edu/~fxic). The website provides a wealth of information, including the effects of FXS on young children, summaries of research findings, family experiences and intervention strategies, services available for children with disabilities, and how to advocate for those services. Lists of publications, personnel, and resources related to FXS are also included. Jane Roberts, manager of the information center, says FPG’s website is geared toward those who want detailed and objective information on FXS and related disabilities with references to specific studies. The website content is written by a team of authors and reviewed by a panel of parents and professionals. Parents who have previewed the site have given it positive reviews. “The fragile X website is an essential tool for educators and parents alike,” says Nichole Tooz, parent and educator. “Its friendly format provides relevant information that is not overwhelming or foreign, even to those new to fragile X. It addresses the most pertinent topics with a simple, yet thorough style. I personally have used information from this website in public presentations and with my son’s teachers and therapists. It has become an on-going resource, as the site is frequently updated with contributions from parents and professionals.” In the future, FPG plans to add content in the areas of temperament, academic achievement, educational transitions, educational services, toilet training, social skills, sibling issues, disability laws, alternative therapies, effects in permutation carriers, mental health issues, and communication skills. FPG’s Fragile X Information Center has been funded by a grant from the Ronald McDonald House Charities. Other funding sources include the Office of Special Education programs of the US Department of Education, the FRAXA Research Foundation, and the National Fragile X Foundation. Other websites available include those of the National Fragile X Foundation (www.fragilex.org) and the FRAXA Foundation (www.fraxa.org). |ed| FragileX Info Center 1Photo: John Cotter, FPG Jane Roberts, manager of the Fragile X Info Center, monitors the website.20 early developments | summer 2004 Newborn screening for fragile X syndrome. Bailey, D. B. (2004). Mental Retardation and Developmental Disabilities Research Reviews, 10, 3–10. Early development, temperament, and functional impairment in autism and fragile X syndrome. Bailey, D. B., Hatton, D. D., Mesibov, G., Ament, N., & Skinner, M. (2000). Journal of Autism and Developmental Disorders, 30, 49–59. Early developmental trajectories of males with fragile X syndrome. Bailey, D. B., Hatton, D. D., & Skinner, M. (1998). American Journal on Mental Retardation, 103, 29–39. Autistic behavior, FMR1 protein, and developmental trajectories in young males with fragile X syndrome. Bailey, D. B., Hatton, D. D., Skinner, M., & Mesibov, G. (2001). Journal of Autism and Developmental Disorders, 31, 165–174. Variability in FMRP expression and early development in males with fragile X syndrome. Bailey, D. B., Hatton, D. D., Tassone, F., Skinner, M., & Taylor, A. K. (2001). American Journal on Mental Retardation, 106, 16-27. Autistic behavior in young boys with fragile X syndrome. Bailey, D. B., Mesibov, G., Hatton, D. D., Clark, R. D., Roberts, Jane E., & Mayhew, L. (1998). Journal of Autism and Developmental Disorders, 28, 499-508. The nature and consequences of fragile X syndrome. Bailey, D. B., & Nelson, D. (1995). Mental Retardation and Developmental Disabilities Research Reviews, 1, 238-244. Identifying infants and toddlers with fragile X syndrome: Issues and recommendations. Bailey, D. B., Roberts, Jane E., Mirrett, P., & Hatton, D. D. (2001). Infants and Young Children, 14(1), 24-33. Family experiences and factors associated with diagnosis of fragile X syndrome. Bailey, D. B., Skinner, D., Hatton, D., & Roberts, Jane E. (2000). Journal of Developmental and Behavioral Pediatrics, 21, 315-321. Discovering fragile X syndrome: Family experiences and perceptions. Bailey, D. B., Skinner, D., & Sparkman, K. (2003). Pediatrics, 111, 407–416. Delayed diagnosis of fragile X syndrome—United States, 1990–1999. Bailey, D. B., Skinner, D., Sparkman, K., Moore, C. A., Olney, R. S., & Crawford, D. C. (2002). Morbidity and Mortality Weekly, 51, 740–742. Sensory processing correlates of occupational performance in children with fragile X syndrome: Preliminary findings. Baranek, G. T., Chin, Y. H., Hess, L. M. G., Yankee, J. G., Hatton, D. D., & Hooper, S. R. (2002). American Journal of Occupational Therapy, 56, 538-546. Behavior and autonomic nervous system function assessed via heart period measures: The case of hyperarousal in boys with fragile X syndrome. Boccia, M. L., & Roberts, Jane E. (2000). Behavior Research Methods, Instruments, and Computers, 32, 5-10. Fragile X syndrome and autism. Hatton, D. D., & Bailey, D. B. (2001). In E. Schopler, L. Marcus, C. Shulman, & N. Yirmiya (Eds.), The research basis of autism intervention outcome (pp. 75–89). New York: Kluwer Academic/Plenum Publishers. Behavioral style of young boys with fragile X syndrome. Hatton, D. D., Bailey, D. B., Hargett-Beck, M. Q., Skinner, M., & Clark R. D. (1999). Developmental Medicine and Child Neurology, 41, 625-632. Early intervention services for young boys with fragile X syndrome. Hatton, D. D., Bailey, D. B., Roberts, J. P., Skinner, M., Mayhew, L., Clark, R. D., Waring, E., & Roberts, Jane E. (2000). Journal of Early Intervention, 23, 235-251. Ocular status of boys with fragile X syndrome: A prospective study. Hatton, D. D., Buckley, E., Lachiewicz, A., & Roberts, Jane E. (1998). Journal of the American Association for Pediatric Ophthalmology and Strabismus, 2, 298-302. Problem behavior in boys with fragile X syndrome. Hatton, D. D., Hooper, S. R., Bailey, D. B., Skinner, M., Sullivan, K., & Wheeler, A. (2002). American Journal of Medical Genetics, 108, 105–116. Adaptive behavior in children with fragile X syndrome. Hatton, D. D., Wheeler, A. C., Skinner, M. L., Bailey, D. B., Sullivan, K.M., Roberts, J. E., Mirrett, P., & Clark, R.D. (2003). American Journal on Mental Retardation, 108, 373-390. Nonverbal assessment of cognitive abilities in children with fragile X syndrome: The utility of the Leiter International Performance Scale-Revised. Hooper, S. R., Hatton, D. D., Baranek, G. T., Roberts, J. P., & Bailey, D. B. (2000). Journal of Psychoeducational Assessment, 18, 255-267. Family and professional congruence in communication assessments of preschool boys with fragile X syndrome. Jackson, S. C., & Roberts, Joanne E. (1999). Journal of Early Intervention, 22, 137-151. Developmental screening and detection of developmental delays in infants and toddlers with fragile X syndrome. Mirrett, P., Bailey, D. B., Roberts, Jane E., & Hatton, D. D. (2004). Developmental and Behavioral Pediatrics, 25, 21–27. Early intervention practices and communication intervention strategies for young males with fragile X syndrome. Mirrett, P., Roberts, Joanne E., & Price, J. (2003). Language, Speech and Hearing Services in Schools, 34, 331. Cardiovascular indices of physiological arousal in boys with fragile X syndrome. Roberts, Jane E., Boccia, M. L., Bailey, D. B., Hatton, D. D., & Skinner, M. (2001). Developmental Psychobiology, 39, 107–123. Development and behavior of male toddlers with fragile X syndrome. Roberts, Jane E., Hatton, D. D., & Bailey, D.B. (2001). Journal of Early Intervention, 24, 207–223. Learn More About fxs@fpgsummer 2004 | early developments 21 FPG Fragile X Websites Carolina Fragile X Project www.fpg.unc.edu/~fx/ Communication of Young Males with Fragile X Syndrome www.fpg.unc.edu/~carolinacommunicationproject/ Fragile X Information Center www.fpg.unc.edu/~fxic/ Early communication, symbolic behavior, and social profi les of young males with fragile X syndrome. Roberts, Joanne E., Mirrett, P., Anderson, K., Burchinal, M., & Neebe, E. (2002). American Journal of Speech-Language Pathology, 11, 295–304. Receptive and expressive communication development of young males with fragile X syndrome. Roberts, Joanne E., Mirrett, P., & Burchinal, M. (2001). American Journal on Mental Retardation, 106, 216-230. Screening for fragile X syndrome: Parent attitudes and perspectives. Skinner, D., Sparkman, K., & Bailey, D. B. (2003). Genetics in Medicine, 5, 378-384. Classroom behavior and academic engagement of elementary school-aged boys with fragile X syndrome. Symons, F. J., Clark, R. D., Roberts, J. P., & Bailey, D. B. (2001). Journal of Special Education, 34, 194–202. Self-injurious behavior in young boys with fragile X syndrome. Symons, F. J., Clark, R. D., Hatton, D. D., Skinner, M., & Bailey, D. B. (2003). American Journal of Medical Genetics, 118A, 115–121. National Fragile X Awareness Day US Senator John Edwards (NC-Dem) joined researchers at FPG to declare July 21, 2000, as National Fragile X Awareness Day. The goal of the declaration was to increase public awareness of the need for research, early diagnosis, and treatment of FXS, a disease that Edwards said “remains unknown to the vast majority of the public and even to many physicians.” Referring to the announcement at FPG, Edwards said, “I am proud of the efforts that the Carolina Fragile X Project has undertaken. It is fi tting that our commemoration is being held at one of the leading facilities in the nation for the treatment of those who inherited the syndrome.” In 1999, Edwards and Sen. Chuck Hagel of Nebraska introduced a bill that would dedicate funds to establish research centers on FXS. The bill was incorporated and passed into law as part of the Children’s Health Act. The National Institutes of Health subsequently held a competition and funded three centers, including the one at FPG. Don Bailey (left) accepts the declaration from Senator John Edwards. Photo: Don Trull, FPG22 early developments | summer 2004 fpg for more information about FPG publications, see the FPG website at www.fpg.unc.edu or contact the FPG Publications Office at 919•966•4221 or FPGpublications@unc.edu Early Intervention Services Assessment Scale (EISAS)—Conceptualization and development of a program quality self-assessment instrument. Aytch, L., Castro, D., & Selz-Campbell, L. (2004). Infants and Young Children, 17(3), 236–246. Newborn screening for fragile X syndrome. Bailey, D. B. (2004). Mental Retardation and Developmental Disabilities Research Reviews, 10, 3–10. First experiences with early intervention: A national perspective. Bailey, D., Hebbeler, K., Scarborough, A., Spiker, D., & Mallik, S. (2004). Pediatrics, 113, 887–896. School readiness for ALL children: Ensuring that children with disabilities or other special needs are included in California’s school readiness efforts. Brault, L. M., Knapp, P., & Winton, P. J. (2004). Los Angeles: UCLA Center for Healthier Children, Families and Communities. Available at http://www.healthychild.ucla.edu/First5CAReadiness/ChildrenDisabilitiesOtherNeeds.asp Home visiting and family literacy programs. Bryant, D., & Wasik, B. H. (2004). In B. H. Wasik (Ed.), Handbook of family literacy (pp. 329–346). Mahwah, NJ: Lawrence Erlbaum Associates, Inc. 2early developments | summer 2004 A framework for understanding the consultation process: Stage-by-stage. Buysse, V., & Wesley, P. W. (2004). Young Exceptional Children, 7(2), 2–9. Resources for supporting young children with challenging behaviors and their families. Catlett, C., Winton, P., & Hemmeter, M. L. (2004, Winter). Young Exceptional Children, 7(2), 30. Integrating services for family literacy. Harbin, G. L., Herrmann, S., Wasik, B. H., Dobbins, D., & Lam, W. K. (2004). In B. H. Wasik (Ed.), Handbook of family literacy (pp. 373–398). Mahwah, NJ: Lawrence Erlbaum Associates, Inc. Developmental screening and detection of developmental delays in infants and toddlers with fragile X syndrome. Mirrett, P., Bailey, D. B., Roberts, Jane E., & Hatton, D. D. (2004). Developmental and Behavioral Pediatrics, 25, 21–27.summer 2004 | early developments 23 recent publications Selected New FPG Products Many products developed at FPG are available for free online. You may also purchase products from the FPG Publications Office, phone 919.966.4221 or email FPGpublications@unc.edu. The FPG online catalog currently includes information on more than 300 publications. Visit the FPG website at www.fpg.unc.edu to learn more. 2003 North Carolina needs and resources assessment. Bryant, D., Maxwell, K., & Taylor, K. (2003). www.fpg.unc.edu/~ncnr_assessment/ Child and program characteristics of the North Carolina More at Four pre-kindergarten program: Year 1 (January–June 2002). Peisner-Feinberg, E. S. (2003) www.fpg.unc.edu/~mafeval/pages/publications.cfm Prekindergarten policy framework. National Prekindergarten Center. (2004). Chapel Hill: The University of North Carolina at Chapel Hill, FPG Child Development Institute.www.fpg.unc.edu/~npc/framework/index.cfm Child care and its impact on young children (2–5). Peisner-Feinberg, E. S. (2004). In R. E. Tremblay, R. G. Barrs, & R. DeV. Peters (Eds.), Encyclopedia on Early Childhood Development [online]. Montreal, Quebec, Canada: Centre of Excellence for Early Childhood Development. Available at http://www.excellence-jeunesenfants.ca/documents/Peisner-FeinbergANGxp.pdf Effects of age and developmental status of partners on play of preschoolers with disabilities. Skinner, M. L., Buysse, V., & Bailey, D. B. (2004). Journal of Early Intervention, 26(3), 34–43. Earliest literacy: From birth to age 3. Sparling, J. (2004). In B. H. Wasik (Ed.), Handbook of family literacy (pp. 45–56). Mahwah, NJ: Lawrence Erlbaum Associates, Inc. An eco-cultural perspective on early literacy: Avoiding the perils of school for non-mainstream children. Vernon-Feagans, L., Head-Reeves, D., & Kainz, K. (2004). In B. H. Wasik (Ed.), Handbook of family literacy (pp. 427–448). Mahwah, NJ: Lawrence Erlbaum Associates, Inc. Handbook of family literacy. Wasik, B. H. (Ed.). (2004). Mahwah, NJ: Lawrence Erlbaum Associates, Inc. 224 early developments | summer 2004 ed early developments FPG Child Development Institute CB 8185, UNC-CH Chapel Hill, NC 27599-8185 Address Service Requested Non-Profi t Org US Postage Paid Permit 177 Chapel Hill, NC ISSN 1536-4739 Visit us online www.fpg.unc.edu www.ncedl.org
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|Description||Vol. 8, no. 2 (summer 2004)|
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summer 2004 | early developments 1
Summer 2004 | Volume 8 #2 Fragile X Syndrome
The University of North Carolina
at Chapel Hill earlydevelopments2 early developments | summer 2004 news
FPG project featured on CNN
show ■ Young adults who received early
educational intervention had
signifi cantly higher mental test
scores from toddlerhood through
age 21 than did untreated controls.
■ Enhanced language skills in the
children appears to have mediated
the effects of early intervention
on cognitive performance.
■ Reading achievement scores were
consistently higher for individuals
with early intervention.
■ Mathematics achievement showed
a pattern similar to that for reading,
with treated individuals earning
More information about the
Abecedarian Study may be found at