Technological supports to promote choice opportunities by two children with fragile X syndrome and severe to profound developmental disabilities

Stasolla, Fabrizio; Damiani, Rita; Perilli, Viviana; Leone, Antonia Di; Albano, Vincenza; Stella, Anna; Damato, Concetta

doi:10.1016/j.ridd.2014.07.045

Cited by 38 publications

(15 citation statements)

References 35 publications

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“…Preliminary experience indicates that assistive technology (i.e. optic sensors such as photocells) generally may be of use to facilitate employment and opportunities of choice [17, 18]. …”

Section: Introductionmentioning

confidence: 99%

Characterization, treatment patterns, and patient-related outcomes of patients with Fragile X syndrome in Germany: final results of the observational EXPLAIN-FXS study

et al. 2016

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BackgroundAs data on the phenotype, characteristics and management of patients with Fragile X Syndrome (FXS) are limited, we aimed to collect such data in Germany in experienced centres involved in the treatment of such patients.MethodsEXPLAIN-FXS is a prospective observational (non-interventional) study (registry) performed between April 2013 and January 2016 at 18 sites in Germany. Requirements for patient participation included confirmed diagnosis of FXS by genetic testing (>200 CGG repeats) and written informed consent. Patients were followed for up to 2 years.ResultsSeventy-five patients (84.0 % males, mean age 16.7 ± 14.5 years, ranging from 2 - 82 years) were analysed. The mean 6-item score, determined according to Giangreco (J Pediatr 129:611-614, 1996), was 6.9 ± 2.5 points. At least one neurological finding each was noted in 53 patients (69.7 %). Specifically, ataxia was noted in 5 patients (6.6 %), lack of fine motor skills in 40 patients, (52.6 %), muscle tonus disorder in 4 patients (5.3 %), and other neurological disorders in 39 patients (51.3 %). Spasticity was not noted in any patient.Seizures were reported in 6 patients (8.1 %), anxiety disorders in 22 patients (30.1 %), depression in 7 patients (9.6 %), ADHD/ADD in 36 patients (49.3 %), impairment of social behavior in 39 patients (53.4 %), and other comorbidities in 23 patients (31.5 %). The mean Aberrant Behaviour Checklist Community Edition (ABC-C) score on behavioral symptoms, obtained in 71 patients at first documentation, was 48.4 ± 27.8 (median 45.0, range 5-115). The mean visual analogue scale (VAS) score, obtained in 59 patients at first documentation, was 84.9 ± 14.6 points (median 90; range 50 – 100).ConclusionsThis report describes the largest cohort of patients with FXS in Europe. The reported observations indicate a substantial burden of disease for patients and their caregivers. Based on these observations, an early expert psychiatric diagnosis is recommended for suspected FXS patients. Further recommendations include multimodal and multi-professional management that is tailored to the individual patient’s needs.Trial registrationThe ClinTrials.gov identifier is NCT01711606. Registered on 18 October 2012.

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“…Preliminary experience indicates that assistive technology (i.e. optic sensors such as photocells) generally may be of use to facilitate employment and opportunities of choice [17, 18]. …”

Section: Introductionmentioning

confidence: 99%

Characterization, treatment patterns, and patient-related outcomes of patients with Fragile X syndrome in Germany: final results of the observational EXPLAIN-FXS study

et al. 2016

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“…First, indices of happiness as a measure of quality of life have been investigated in previous studies targeting other behaviors (Stasolla, Damiani, Perilli, et al, ; Stasolla, De Pace et al, ; Stasolla, Perilli et al, ). The basic assumption is that through an MBP, an individual with multiple disabilities is provided with the ability to independently access preferred stimuli, and it is reasonable to assume that this would lead to increases in indices of happiness, but this remains unknown.…”

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confidence: 99%

A microswitch‐based program for promoting initial ambulation responses: An evaluation with two girls with multiple disabilities

Stasolla

Caffò

Perilli³

et al. 2017

J of App Behav Analysis

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We assessed the use of a microswitch-based program for promoting ambulation responses by two children with multiple disabilities. The goals of the study were to: (a) evaluate the importance of the contingency between the target behavior (forward step) and the programmed consequence (preferred stimuli), (b) measure effects of the intervention on indices of happiness, and (c) assess the social validation of the procedure using 20 physiotherapists as external raters. The intervention involved the automatic delivery of preferred stimuli contingent on forward steps. Results showed that both participants improved their performance (forward steps and indices of happiness) during contingent reinforcement phases compared to baseline and noncontingent reinforcement phases. Moreover, physiotherapists rated the intervention as socially valid.

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“…Most of the studies were published in the last decade ( n = 24); the remaining studies were published between 1997 and 2010. In 29 studies, a single case design was used (Dunphy & Hens, 2018 ; Gaskin et al, 2012 ; Hetzroni & Banin, 2017 ; Hronis et al, 2019 ; Kim et al, 2014 ; ; Lancioni, O’Reilly, et al, , 2006 , 2013 ; Lancioni, Singh, O’Reilly, Green, et al, 2014 ; Lancioni, Singh, et al, 2006 , 2013 ; Lancioni, Singh, O’Reilly, Sigafoos, et al, 2014 ; Lancioni, Smaldone, et al, 2006 ; Perilli et al, 2019 ; Simacek et al, 2017 ; Singh et al, 2017 ; Stasolla, Damiani, et al, 2014 ; Stasolla & O’Caffò, 2013 ; Stasolla et al, 2013 , 2015 , 2017a ; Stasolla, Perilli, Caffò, et al, 2017 ; Stasolla, Perilli, et al, 2014 ). In addition, two studies used a pre‐experimental design (Hoffman et al, 2017 ; Jonker et al, 2015 ) and one study used a randomized control trial (Cooney et al, 2017 ), while one study did not report about the study design (Monlux et al, 2019 ).…”

Section: Resultsmentioning

confidence: 99%

Psychological eHealth interventions for people with intellectual disabilities: A scoping review

Oudshoorn

Frielink

Nijs

et al. 2021

Research Intellect Disabil

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Technological supports to promote choice opportunities by two children with fragile X syndrome and severe to profound developmental disabilities

Cited by 38 publications

References 35 publications

Characterization, treatment patterns, and patient-related outcomes of patients with Fragile X syndrome in Germany: final results of the observational EXPLAIN-FXS study

Characterization, treatment patterns, and patient-related outcomes of patients with Fragile X syndrome in Germany: final results of the observational EXPLAIN-FXS study

A microswitch‐based program for promoting initial ambulation responses: An evaluation with two girls with multiple disabilities

Psychological eHealth interventions for people with intellectual disabilities: A scoping review

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