Kelly Sullivan

ADHD symptoms in children with FXS.

Parent‐ and teacher‐report of attention‐deficit/hyperactivity disorder (ADHD) symptoms were examined using problem behavior and DSM‐IV symptom inventory questionnaires for 63 children with full mutation fragile X syndrome (FXS) and 56 children without disabilities matched on mental age (MA). Prevalence rates of ADHD symptoms varied depending on type of measure (problem behavior or DSM‐IV criteria), subscale (ADHD‐inattentive or ADHD‐hyperactive), scoring method (continuous T‐scores or categorical scores based on DSM‐IV algorithm), and rater (parent or teacher). Overall, 54–59% of boys with FXS met diagnostic behavioral criteria for either ADHD‐inattentive type only, ADHD‐hyperactive type only, or ADHD‐combined type based on parent or teacher report. Boys with FXS were rated as having clinically high scores or met diagnostic criteria at higher rates than expected for the general population and had higher raw scores than their MA‐matched peers. Parent ratings of boys with FXS resulted in higher ADHD‐inattentive type and ADHD‐hyperactive type T‐scores than teachers. Boys who were rated as meeting DSM‐IV criteria were more likely to be taking psychotropic medication and to have younger mental ages. Parents were substantially more likely than teachers to rate boys as meeting DSM‐IV criteria for ADHD‐inattentive type, while teachers were only slightly more likely than parents to rate boys as meeting DSM‐IV criteria for ADHD‐hyperactive type. Teachers were more likely than parents to rate boys as meeting DSM‐IV criteria for ADHD when boys had lower levels of FMRP.

Executive functions in young males with fragile X syndrome in comparison to mental age-matched controls: baseline findings from a longitudinal study.

The performance of 54 boys with fragile X syndrome (FXS), ages 7 to 13 years, was compared to that of a group of typically developing boys who were matched on mental age (MA) and ethnicity across multiple measures of executive function (EF). Boys with FXS varied in their ability to complete EF measures, with only 25.9% being able to complete a set-shifting task and 94.4% being able to complete a memory for word span task. When compared to the control group, and controlling for MA and maternal education, boys with FXS showed significant deficits in inhibition, working memory, cognitive flexibility/set-shifting, and planning. No group differences were observed in processing speed. Mental age significantly impacted performance on working memory, set-shifting, planning, and processing speed tasks for both groups. In boys with FXS, MA significantly predicted performance on working memory and set-shifting tasks. Our findings suggest that deficits in EF in boys with FXS are not solely attributable to developmental delays but, rather, present as a true array of neurocognitive deficits.

Adaptive Behavior in Children With Fragile X Syndrome

Adaptive behavior over time in 70 children with fragile X syndrome, ages 1 to 12 years, was examined using the Vineland Adaptive Behavior Scales. With a mean of 4.4 assessments per child, adaptive behavior skills increased steadily and gradually over time. Children with less autistic behavior and higher percentages of FMPR expression showed better performance on all areas of adaptive behavior. Children without autistic behavior displayed higher scores and rates of growth on the Daily Living Skills domain, with the lowest scores in Socialization. Comparison to Brief IQs indicate that children with fragile X syndrome display nonverbal IQs superior to their adaptive behavior when they are below age 10 but that these skills seem to converge as they get older.

Mapping nonverbal IQ in young boys with fragile X syndrome

This study examines the developmental changes in nonverbal intellectual functioning evident in males with fragile X syndrome (FXS) measured by the Leiter International Performance Scales‐Revised (Leiter‐R). The Leiter‐R provides both IQ scores and associated growth scores which permit the examination of both age‐based IQ scores and overall intellectual growth. Participants were 45 males with full mutation FXS and ranged in age from 4.0 to 13.8 years. Each child was assessed annually using the Leiter‐R as part of a larger longitudinal battery for an average of 3.5 assessments per child and a range of 2–6 assessments, representing a total of 156 assessment occasions. Longitudinal analyzes of Leiter scores consisted primarily of hierarchical linear modeling, with the impact of chronological age, maternal education, fragile X mental retardation 1 protein (FMRP), autistic behaviors also being assessed. Findings revealed a significant linear decline in nonverbal IQ scores, with no effects of maternal education, autistic behaviors, or FMRP on mean level or rate of change in IQ scores over time. The decline slowed significantly around 8 years of age, but scores continued to decline into the 12th year of age. In contrast, a significant linear increase was observed in Leiter‐R growth scores, which was negatively influenced by autistic behaviors. The rate of increase did not change over time, and neither mean level nor rate of increase was influenced by maternal education or FMRP levels. These findings suggest that declines in IQ are the result of steady, but suboptimal intellectual growth, rather than a true deterioration in overall intellectual functioning.

Sustained attention and response inhibition in boys with fragile X syndrome : Measures of continuous performance

Sustained attention and response inhibition were examined in boys with full mutation fragile X syndrome (FXS) using adapted visual and auditory continuous performance tests (CPTs). Only 61% of 56 boys with visual CPT data and 54% of 52 boys with auditory data were able to demonstrate sufficient understanding to complete the visual and auditory CPTs, respectively. Mental age (MA) predicted whether boys with FXS were able to demonstrate understanding of the CPTs. The performance of boys with FXS who were able to complete the CPTs was compared to a sample of boys without disabilities matched on MA. Boys with FXS demonstrated similar or smaller declines in sustained attention over task time than their MA‐matched peers on the visual and auditory CPTs, respectively, but consistently demonstrated greater declines in response inhibition over task time than their MA‐matched peers. There were no differences between groups for response time of hits. Higher MAs consistently predicted better sustained attention and response inhibition over task time on the visual and auditory CPTs. Furthermore, boys taking psychotropic medication performed better at the beginning of most tasks, although their performance deteriorated at a faster rate over time, and boys rated as meeting diagnostic criteria for ADHD‐hyperactive type had more difficulty over task time with response inhibition on the auditory CPT. For both boys with FXS and their MA matches, performance was better on the visual CPT than on the auditory CPT though this effect may be attributable to a number of factors other than the modality.

Developmental Trajectories of Young Girls with Fragile X Syndrome.

To describe the early phenotype of girls with full mutation fragile X, we used 54 observations of 15 girls between the ages of 6 months and 9 years to examine developmental trajectories as measured by the Battelle Development Inventory. In this sample, autistic behavior was associated with poorer developmental outcomes, primarily due to interactions of age with autistic behavior, even though autistic behavior, measured continuously, was relatively mild. Although this small sample, ascertained primarily through male relatives with fragile X syndrome, limits generalizability, considerable variability in developmental outcome in young girls was documented. In addition, findings support previous research suggesting that even mild autistic behaviors in girls can be associated with developmental outcomes.

Effectiveness of a Statewide Abusive Head Trauma Prevention Program in North Carolina

IMPORTANCE Abusive head trauma (AHT) is a serious condition, with an incidence of approximately 30 cases per 100,000 person-years in the first year of life. OBJECTIVE To assess the effectiveness of a statewide universal AHT prevention program. DESIGN, SETTING, AND PARTICIPANTS In total, 88.29% of parents of newborns (n = 405 060) in North Carolina received the intervention (June 1, 2009, to September 30, 2012). A comparison of preintervention and postintervention was performed using nurse advice line telephone calls regarding infant crying (January 1, 2005, to December 31, 2010). A difference-in-difference analysis compared AHT rates in the prevention program state with those of other states before and after the implementation of the program (January 1, 2000, to December 31, 2011). INTERVENTION The Period of PURPLE Crying intervention, developed by the National Center on Shaken Baby Syndrome, was delivered by nurse-provided education, a DVD, and a booklet, with reinforcement by primary care practices and a media campaign. MAIN OUTCOMES AND MEASURES Changes in proportions of telephone calls for crying concerns to a nurse advice line and in AHT rates per 100,000 infants after the intervention (June 1, 2009, to September 30, 2011) in the first year of life using hospital discharge data for January 1, 2000, to December 31, 2011. RESULTS In the 2 years after implementation of the intervention, parental telephone calls to the nurse advice line for crying declined by 20% for children younger than 3 months (rate ratio, 0.80; 95% CI, 0.73-0.87; P < .001) and by 12% for children 3 to 12 months old (rate ratio, 0.88; 95% CI, 0.78-0.99; P = .03). No reduction in state-level AHT rates was observed, with mean rates of 34.01 person-years before the intervention and 36.04 person-years after the intervention. A difference-in-difference analysis from January 1, 2000, to December 31, 2011, controlling for economic indicators, indicated that the intervention did not have a statistically significant effect on AHT rates (β coefficient, -1.42; 95% CI, -13.31 to 10.45). CONCLUSIONS AND RELEVANCE The Period of PURPLE Crying intervention was associated with a reduction in telephone calls to a nurse advice line. The study found no reduction in AHT rates over time in North Carolina relative to other states. Consequently, while this observational study was feasible and supported the program effectiveness in part, further programmatic efforts and evaluation are needed to demonstrate an effect on AHT rates.

Designing and Testing a Shaken Baby Syndrome Prevention Program—The Period of PURPLE Crying: Keeping Babies Safe in North Carolina

Each year infants die or are permanently disabled at the hands of their caregivers by abusive head trauma, more commonly known as Shaken Baby Syndrome (SBS). Current medical research confirms the central role of infant crying in triggering shaking by caregivers; anecdotally, inconsolable crying is the most common explanation given by abusers. Although SBS prevention efforts have been initiated, most remain unevaluated. This article provides an overview of a 5-year, evidence-based SBS-prevention program called The Period of PURPLE Crying: Keeping Babies Safe in North Carolina from a social marketing perspective. The project includes three components: (1) in-hospital education for parents of every newborn at all 86 hospitals/birthing centers in North Carolina; (2) reinforcement in community settings such as prenatal visits to health departments or well-child care visits to primary care providers; and (3) a media campaign.

Trauma-Informed Care for Children in the Child Welfare System: An Initial Evaluation of a Trauma-Informed Parenting Workshop

An essential but often overlooked component to promoting trauma-informed care within the child welfare system is educating and empowering foster, adoptive, and kinship caregivers (resource parents) with a trauma-informed perspective to use in their parenting as well as when advocating for services for their child. In this first evaluation of the National Child Traumatic Stress Network’s trauma-informed parenting workshop (Caring for Children who Have Experienced Trauma, also known as the Resource Parent Curriculum), participant acceptance and satisfaction and changes in caregiver knowledge and beliefs related to trauma-informed parenting were examined. Data from 159 ethnically diverse resource parents were collected before and after they participated in the workshop. Results demonstrate that kinship and nonkinship caregivers showed significant increases in their knowledge of trauma-informed parenting and their perceived self-efficacy parenting a child who experienced trauma. Nonkinship caregivers increased on their willingness to tolerate difficult child behaviors, whereas kinship caregivers did not show a significant change. Participants also demonstrated high levels of satisfaction with the workshop. Although these preliminary results are important as the first empirical study supporting the workshop’s effectiveness, the limitations of this study and the directions for future research are discussed.

Process evaluation of a statewide abusive head trauma prevention program

The current study used four dimensions of the RE-AIM framework (Reach, Adoption, Implementation, and Maintenance) to evaluate the implementation of a statewide abusive head trauma prevention program. Numerous methods, including telephone surveys, paper and pencil questionnaires, site visits, and program administrative data were used to conduct the process evaluation. Results indicate that the intervention was successfully implemented in all birthing hospitals (n=86) across the state with a high degree of fidelity. Furthermore, the majority of the hospitals reported incorporating the program into unit procedures and employee training. More than three-fourths indicated that they plan to continue the program after the study ends. The RE-AIM framework was applied and served as a useful guide for the process evaluation of a multifaceted, multi-system, universal public health intervention.