Examining the Function of Problem Behavior in Fragile X Syndrome: Preliminary Experimental Analysis

Abstract Fragile X syndrome is the most common inherited cause of intellectual and developmental disability. The influence of environmental variables on behaviors associated with the syndrome has received only scant attention. The current study explored the function served by problem behavior in fragile X syndrome by using experimental functional analysis methodology with 8 children with fragile X. No child met criteria for attention-maintained problem behavior, 5 children met criteria for escape-maintained problem behavior, and 4 children met criteria for tangible-maintained problem behavior. Results are discussed and compared with previous findings on the function of problem behavior in fragile X syndrome, and implications for intervention are discussed. It is noted that the external validity of these findings is limited by the small sample size.

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Behavioral Intervention for Problem Behavior in Children With Fragile X Syndrome

American Journal on Intellectual and Developmental Disabilities ◽

10.1352/1944-7558-116.6.457 ◽

2011 ◽

Vol 116 (6) ◽

pp. 457-478 ◽

Cited By ~ 18

Author(s):

Lauren J Moskowitz ◽

Edward G Carr ◽

V. Mark Durand

Keyword(s):

Quality Of Life ◽

Problem Behavior ◽

Fragile X Syndrome ◽

Behavioral Intervention ◽

Fragile X ◽

Family Quality Of Life ◽

Baseline Design ◽

Intervention Plan ◽

The Given

Abstract Parents and professionals typically report problem behavior as a significant concern for children with fragile X syndrome. In the present study, the authors explored whether behaviorally based interventions would result in a reduction in problem behavior and an improvement in quality of life for 3 children with fragile X syndrome and their families. A multiple baseline design was used to demonstrate intervention effects for specific high-priority contexts (i.e., bedtime, running errands, and toileting). A multicomponent intervention plan was developed to teach the parents and child to effectively cope with the particular context. After intervention, there were substantial improvements in problem behavior and family quality of life within the given contexts. Results of this study demonstrated the effectiveness of behavioral intervention for children with fragile X syndrome.

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Cooperative Parent-Mediated Therapy in Children with Fragile X Syndrome and Williams Beuren Syndrome: A Pilot RCT Study of a Transdiagnostic Intervention-Preliminary Data

Brain Sciences ◽

10.3390/brainsci12010008 ◽

2021 ◽

Vol 12 (1) ◽

pp. 8

Author(s):

Paolo Alfieri ◽

Francesco Scibelli ◽

Laura Casula ◽

Simone Piga ◽

Eleonora Napoli ◽

...

Keyword(s):

Fragile X Syndrome ◽

Preliminary Data ◽

Gold Standard ◽

Fragile X ◽

Autism Spectrum ◽

Small Sample ◽

Control Group ◽

Pilot Rct ◽

Experimental Group ◽

Communicative Skill

Children with fragile X syndrome and William Beuren syndrome share several socio-communicative deficits. In both populations, around 30/35% of individuals meets criteria for autism spectrum disorder on gold standard instruments. Notwithstanding, few studies have explored feasibility and validity of therapy for socio-communicative deficits in individuals with these genetic conditions. In this study, we present preliminary data on a pilot RCT aimed to verify the effectiveness of cooperative parent-mediated therapy for socio-communicative deficits in a transdiagnostic perspective in a small sample of 12 participants. Our preliminary data showed that the experimental group had significant improvement in one socio-communicative skill (responsivity) and in clinical global impression, while the control group in an adaptive measure of socialization and word production. Implications of these results are then discussed.

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Telehealth-enabled behavioral treatment for problem behaviors in boys with fragile X syndrome: a randomized controlled trial

Journal of Neurodevelopmental Disorders ◽

10.1186/s11689-020-09331-4 ◽

2020 ◽

Vol 12 (1) ◽

Author(s):

Scott S. Hall ◽

Katerina D. Monlux ◽

Arlette Bujanda Rodriguez ◽

Booil Jo ◽

Joy S. Pollard

Keyword(s):

Parenting Stress ◽

Problem Behavior ◽

Fragile X Syndrome ◽

Problem Behaviors ◽

Fragile X ◽

Treatment Acceptability ◽

Stress Index ◽

Treatment As Usual ◽

Increased Risk ◽

Behavioral Treatments

Abstract Background Children with fragile X syndrome (FXS) are at increased risk for exhibiting problem behaviors such as aggression and self-injury. However, many children with FXS have limited access to behavioral treatments that have known efficacy due to the low availability of treatment providers and the wide geographical dispersion of families with FXS across the country. Telehealth may offer a cost-effective and practical solution to overcome these significant barriers. We examined the effect of administering an established behavior analytic intervention called functional communication training (FCT) via telehealth on levels of problem behaviors exhibited by boys with FXS. We also examined treatment acceptability, as well as the effect of the treatment on levels of parenting stress. Methods Boys with FXS, aged 3 to 10 years, who displayed problem behaviors daily, were randomized to receive FCT via telehealth (n = 30) or treatment as usual (n = 27) over 12 weeks. Outcome measures included in-session observations of problem behavior, the Aberrant Behavior Checklist—Community (ABC-C), the Treatment Acceptability Rating Form—Revised (TARF-R), and the Parenting Stress Index, 4th edition (PSI-4). Results Intention-to-treat analyses indicated that scores on the irritability subscale of the ABC-C, our primary outcome measure, decreased significantly for boys who received FCT via telehealth compared to boys who received treatment as usual (p < .001, Cohen’s d = 0.65). In-session observations conducted for those who received treatment showed that levels of problem behavior decreased by 91% from baseline. Levels of parenting stress related to child behavioral problems were also lower following FCT treatment, and caregivers reported that the intervention was acceptable. Conclusions These findings support telehealth-enabled FCT as a framework for expanding access to behavioral treatments for problem behaviors in children with FXS. Expanded delivery of behavior analytic treatment via telehealth also has the potential to lower healthcare costs, improve child and family quality of life, and lead to advances in the treatment of problem behavior in the broader population of individuals with neurodevelopmental disorders. Trial registration ClinicalTrials.gov, NCT03510156. Registered 27 April 2018

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Alterations to Functional Analysis Methodology to Clarify the Functions of Low Rate, High Intensity Problem Behavior

Behavior Analysis in Practice ◽

10.1007/bf03391815 ◽

2012 ◽

Vol 5 (1) ◽

pp. 27-39 ◽

Cited By ~ 3

Author(s):

Barbara J. Davis ◽

SungWoo Kahng ◽

Jonathan Schmidt ◽

Lynn G. Bowman ◽

Eric W. Boelter

Keyword(s):

Functional Analysis ◽

Problem Behavior ◽

High Intensity ◽

Analysis Methodology ◽

Functional Analysis Methodology ◽

Low Rate

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Examining the Specificity of Forms and Functions of Aggressive Behavior in Boys With Fragile X Syndrome

American Journal on Intellectual and Developmental Disabilities ◽

10.1352/1944-7558-125.4.247 ◽

2020 ◽

Vol 125 (4) ◽

pp. 247-259

Author(s):

Tobias C. Britton ◽

Ellen H. Wilkinson ◽

Scott S. Hall

Keyword(s):

Aggressive Behavior ◽

Fragile X Syndrome ◽

Developmental Disability ◽

Fragile X ◽

Psychotropic Medications ◽

Developmental Period ◽

Limited Information ◽

Intellectual And Developmental Disability ◽

Functional Assessments ◽

Critical Developmental Period

Abstract Limited information is available concerning the specificity of the forms and functions of aggressive behavior exhibited by boys with fragile X syndrome (FXS). To investigate these relationships, we conducted indirect functional assessments of aggressive behavior exhibited by 41 adolescent boys with FXS and 59 age and symptom-matched controls with intellectual and developmental disability (IDD) and compared the data between groups. Results showed that boys with FXS were more likely to exhibit specific forms of aggressive behavior (i.e., scratching others and biting others) compared to controls, but the sources of reinforcement identified for aggression were similar across groups. Boys with FXS who were prescribed psychotropic medications were more likely to be older and to exhibit more forms of aggression. The implications for the treatment of aggressive behavior during this critical developmental period in FXS are discussed.

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Developmental Trajectories of Young Girls With Fragile X Syndrome

American Journal on Intellectual and Developmental Disabilities ◽

10.1352/1944-7558-114.3.161 ◽

2009 ◽

Vol 114 (3) ◽

pp. 161-171 ◽

Cited By ~ 21

Author(s):

Deborah D. Hatton ◽

Anne Wheeler ◽

John Sideris ◽

Kelly Sullivan ◽

Alison Reichardt ◽

...

Keyword(s):

Fragile X Syndrome ◽

Fragile X ◽

Developmental Trajectories ◽

Developmental Outcomes ◽

Small Sample ◽

Considerable Variability ◽

Full Mutation ◽

Autistic Behavior ◽

Young Girls ◽

Autistic Behaviors

Abstract 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.

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An Indirect Examination of the Function of Problem Behavior Associated with Fragile X Syndrome and Smith-Magenis Syndrome

Journal of Autism and Developmental Disorders ◽

10.1007/s10803-011-1229-6 ◽

2011 ◽

Vol 42 (2) ◽

pp. 201-209 ◽

Cited By ~ 24

Author(s):

Paul Langthorne ◽

Peter McGill

Keyword(s):

Problem Behavior ◽

Fragile X Syndrome ◽

Fragile X

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Revisiting vitamin D status and supplementation for inpatients with intellectual and developmental disability in the north of England, UK

BJPsych Open ◽

10.1192/bjo.2021.95 ◽

2021 ◽

Vol 7 (S1) ◽

pp. S14-S14

Author(s):

Vlad Ciausu ◽

Marcin Ostrowski ◽

Bethany Dudley ◽

Iain McKinnon ◽

Chris Ince

Keyword(s):

Vitamin D ◽

Physical Health ◽

Developmental Disability ◽

Small Sample Size ◽

Vitamin D Supplementation ◽

Small Sample ◽

Inpatient Service ◽

Intellectual And Developmental Disability ◽

Vitamin D Levels ◽

Secure Unit

AimsVitamin D deficiency is common among people with Intellectual and Developmental Disability (IDD) and is linked to worse health outcomes.Our aims were to re-evaluate vitamin D testing and supplementation among inpatients with IDD, examine any correlates with physical health conditions including COVID-19 and make recommendations for the current regime of supplementation and testing within inpatient IDD services.MethodThe study population comprised inpatients who were in any of the Northgate Hospital IDD inpatient services in Northumberland, UK. The wards sampled were the Medium Secure Unit, Low Secure Unit, Hospital Based Rehabilitation Wards and Specialist Autism Inpatient Service. Records of all inpatients between January 2019 and July 2020 were examined for 25-hydroxyvitamin D [25(OH)D] level, ward area, supplementation status, test seasonality, medication, and health status.We performed a correlation to see whether there was an association between vitamin D level and length of time on treatment. In addition, comparison of the replete and inadequate group for age, ethnicity, seasonality, ward location and psychotropic medication was undertaken.Data on physical health risk factors, obesity and COVID-19 infection were also collected. The physical comorbidities were described in order to evaluate whether any emerging patterns relating to COVID-19 infection were emerging.ResultThere were 67 inpatients in Northgate IDD services on 1 January 2019, with 11 further patients admitted up to the end of the sampling period on 31 July 2020. Nineteen patients were discharged during that period, so the sample comprised 78 patients.Ages were comparable across three of the ward areas, except for an older group of patients in the hospital-based rehabilitation setting. Mean 25(OH)D level for supplemented (800IU/day) patients was 75nmol/l (SD 20) compared to 40nmol/l (SD 19) in the non-supplemented group (p < 0.001).Thirty-eight percent of those who were inpatients during the first wave of the COVID-19 pandemic developed symptoms, but the small sample size could not establish vitamin D levels as a predictor of outcome.ConclusionOur findings show that clinicians continue to offer vitamin D supplementation for inpatients, at a dose of 800IU (20μg) per day.The mean vitamin D levels we observed were higher for those on supplements compared to our 2013 baseline data, whereas patients not on supplementation now had levels akin to those found previously. Vitamin D (800IU/day) supplementation is effective but adequacy of the nationally recommended dose of 400IU/day is unclear. Links to COVID-19 merit further research.

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