Moderate Developmental Disabilities Research Articles

A large growing occipital meningocele with Dandy–Walker syndrome: A case report and review of the literature

Background: Dandy–Walker syndrome (DWS) is a well-known developmental anomaly. An occipital meningocele (OMC) is recognized as a malformation that is relatively often associated with DWS, but the association of DWS with OMC has been reported in approximately 40 cases. We present herein a rare clinical course of DWS with OMC, in which the sac was small at birth and became progressively larger. Case Description: A 5-day-old baby boy was referred to our hospital due to OMC. He was born at 33 gestational weeks due to premature rupture of the membranes. He was diagnosed as having DWS associated with OMC. The OMC was covered with skin and its maximum diameter at birth was 3 cm. Magnetic resonance imaging showed an occipital bone defect and continuity of the fourth ventricle, posterior fossa cyst, and OMC sac. The aqueduct was patent, and no hydrocephalus was found. The OMC sac increased progressively with moderate hydrocephalus and reached 7 cm at the age of 54 days when his weight was 2508 g. A cystoperitoneal shunt and repair were performed after sinus venography by contrast computed tomography (CT). At the age of 1 year and 8 months, he had moderate developmental disabilities. Conclusion: In most cases reported, the OMC was relatively small, and large and giant sizes were reported in only six cases. Almost all cases remained the same size as at birth and underwent surgical intervention as early as possible. It was possible to understand the relationship between the occipital bone defect and abnormal running of sinuses such as the superior sagittal sinus, torcular Herophili, and transverse sinus preoperatively from the CT venography (CTV) image. CTV may be an effective and important method for safely performing repair and shunt.

Long-Term Outcomes of 92 Cases of Fetal Hydrothorax Including Thoracoamniotic Shunting

Background: There have been no previous reports on the postnatal course, especially long-term outcomes, of fetal hydrothorax patients, including those treated with thoracoamniotic shunting (TAS) using a double-basket catheter.The outcomes of cases from a single center are reported. Methods: Cases of fetal hydrothorax managed at our center between 2005 and 2015 were enrolled retrospectively. TAS was performed if indicated. Long-term outcomes such as cerebral palsy, developmental disabilities, and others were analyzed. Results: Ninety-two cases of fetal hydrothorax were included. The causes were primary chylothorax, transient abnormal myelopoiesis, cardiac disease, pulmonary sequestration, mediastinal neoplasm, and infection. TAS was performed in 36 cases. Early neonatal death occurred in 19 cases. The 28-day survival rates for all cases and for TAS cases were 70% (48/69) and 72% (26/36), respectively. Of the cases that underwent TAS, one was treated with home oxygen therapy, one was diagnosed with cerebral palsy and severe intellectual disability, and five were diagnosed with mild or moderate developmental disabilities. Conclusions: The results showed that the survival rate and long-term outcomes of cases with hydrothorax have improved as TAS has become more prevalent. The reasons for these results need to be elucidated, and efforts are needed to further improve outcomes.

Making Sense of Self: An Autoethnographic Study of Identity Formation for Adolescents in Music Therapy

Abstract Told through my experiences of working with three teenagers who had mild and moderate developmental disabilities, this autoethnographic study considers identity formation as a core concept in music therapy clinical practice. In doing so, I explored theories of identity formation, including those described by Erikson (1950, Childhood and society, Norton), Marcia and colleagues (1993, Ego identity: A handbook for psychosocial research, Springer), and Crocetti, Rubini, and Meeus (2008, Journal of Adolescence, 31(2), 207–222), relating these concepts to each of the teenagers I worked with. This article, therefore, chronicles the ways in which my clinical thinking shifted from a skills-based approach to one that considers the client’s identity as a whole, leading to suggestions of ways to integrate identity formation theory into clinical practice.

Timing of endoscopic third ventriculostomy in pediatric patients with congenital obstructive hydrocephalus: assessment of neurodevelopmental outcome and short-term operative success rate

The purpose of this study was to determine the impact of early (⩽6months old), midterm (6–12months old) and late (>12months old) endoscopic third ventriculostomy (ETV) on the operative success rate and postoperative neurodevelopmental outcome of children with congenital obstructive hydrocephalus. We divided 63 children into three groups according to whether they underwent early, midterm or late ETV. Their preoperative developmental quotient (DQ) was assessed using the Gesell developmental diagnosis schedule (GDDS). Three and 6months after the initial procedure, GDDS was used to obtain postoperative DQ from two assessments (blinded and non-blinded). Meanwhile, two observers studied the operative success rate of initial ETV. There were no substantial differences between blinded and non-blinded assessments. The success rate of early ETV was only 20.8%. By contrast, this rate was 55% and 73.7% for midterm and late ETV, respectively. Before operation, we observed severe developmental abnormalities in all children (DQ score<40). However, children in midterm and late ETV groups achieved improvement after the operation, which was particularly remarkable in late ETV group. Six months after the first surgery, 16 (84.2%) children in the late ETV group, nine (45%) in the midterm ETV group and four (16.7%) in the early ETV group had moderate developmental disability. Nevertheless, overall prognosis for the three groups was not optimistic. There were no children with mild neurodevelopmental disability or normal function. Our data confirmed that age is a determinant for ETV effectiveness and overall prognosis.

Teaching Students With Developmental Disabilities Daily Living Skills Using Point-of-View Modeling Plus Video Prompting With Error Correction

A primary goal of instruction for students with developmental disabilities is to enhance their future quality of life by promoting skill acquisition, which will enable them to live, function, and participate in the community. One instructional method that can help students with developmental disabilities improve independence in performing daily living skills is video prompting (VP). This study investigated the effectiveness of a VP plus error correction procedure on skill acquisition when teaching daily living skills to four adolescents with mild and moderate developmental disabilities. A multiple baseline across participants design demonstrated that all four participants rapidly acquired dish washing skills upon introduction of the intervention. Future research should further explore the efficacy of error correction procedures used with VP and the impact that these procedures have on student learning and skill retention.

Nutrition and Food Skills Education: For Adults with Developmental Disabilities

Individuals with developmental disabilities have poor eating habits and are at increased risk for cardiovascular disease, obesity, and osteoporosis. Needs and preferences for a nutrition education and foods skills program were explored in adults with developmental disabilities, agency managers, and support workers. Twenty-eight adults with mild to moderate developmental disabilities participated in individual interviews; seven managers and 21 support workers took part in three focus group discussions. Concurrent data collection and analysis, data saturation, and a constant comparative method guided the research. All participants indicated a need for nutrition education and cooking programs for this population. Seven major themes emerged: poor eating habits, safety concerns, low transferable skills, social relationships, staff training needs, resource needs, and limited funding. Individuals with developmental disabilities also expressed feelings of self-efficacy in learning to cook healthy food. There is a strong interest in and need for nutrition education and food skills programs for adults with developmental disabilities. The collaboration of multiple community partners in program implementation and delivery is essential. At the policy level, the needs of individuals with aging caregivers must be addressed and access to registered dietitians must be improved for this population.

Development and initial validation of the Environmental Restriction Questionnaire (ERQ)

The purpose of this manuscript was to develop and test the psychometric properties of the Environmental Restriction Questionnaire (ERQ) a parent-reported questionnaire for measuring perceived environmental restrictions for young children participation. Reliability and homogeneity were tested by Cronbach's alpha and inter-item correlations. Construct validity was computed by factor analysis and known group differences analysis. Convergent and divergent validities were calculated by correlation with the Children Participation Questionnaire (CPQ). Participants were 290 children and their parent. Seventy-five children who were referred to occupational therapy evaluation as consequence of moderate developmental disabilities and 215 children without any disability (mean age±standard deviation for total sample, 5 y, 3 mo±.65 y; range, 3 y, 11 mo to 6 y, 10 mo). The ERQ has good internal reliability. Cronbach's alpha for the ERQ measures ranged between .75 and .91, indicating adequate homogeneity. Factor analysis yielded three factors that explained almost 48% of the total variance. Significant differences were found between known groups. Convergent and divergent validity were supported by various correlations with the Children Participation Questionnaire (CPQ). The ERQ has demonstrated good psychometric properties and can be used as a reliable and valid measure to assess perceived environmental restriction at the age of 4–6 y.

Development and initial validation of the Children Participation Questionnaire (CPQ)

Purpose. To develop and test the psychometric properties of a parent-reported questionnaire measuring participation of preschool children (Children Participation Questionnaire; CPQ) aged 4–6 years in their everyday activities.Methods. Reliability was tested by Cronbach's α and by test–retest. Construct validity was computed by known group differences analysis. Convergent and divergent validities were calculated by correlation with the Vineland Adaptive Behaviour Scale (VABS). Two hundred thirty-one children with mild to moderate developmental disabilities (mean age 5.16 ± 0.66 years old) were compared to 249 children without disability (mean age 5.13 ± 0.72 years old).Results. The CPQ has good internal reliability. Cronbach's α for the participation measures ranged between 0.79 and 0.90, indicating good homogeneity. The temporal stability of the CPQ was supported with intra-class correlations ranging from 0.71 to 1.00. Significant differences were found between children with and without disabilities in all the CPQ measures. The CPQ could also differentiate between age groups and groups of varying socio-economic status. Convergent and divergent validity were supported.Conclusions. The CPQ has demonstrated good psychometric properties and can be used as a reliable and valid measure to assess children's participation at the age of 4–6 years.

Development and initial validation of the Performance Skills Questionnaire (PSQ)

The objectives of this study was to develop and test the psychometric properties of the Performance Skills Questionnaire (PSQ), addressed to measure performance skills of preschoolers, as reported by their parents. Participants included 231 children ranging in age from 4 to 6 years old, with mild to moderate developmental disabilities and 240 children without disabilities at same age range. Internal consistency, test–retest, construct validity, and divergent and convergent validity were assessed. The PSQ has shown good internal reliability, and temporal stability. Construct validity was supported by factor analysis which yielded 3 factors that explained almost 52% of the total variance. Significant differences were found between known groups. Convergent and divergent validity were supported by significant correlations with Visual-Motor Integration (VMI) test, and the Children Participation Questionnaire (CPQ). The PSQ is a unique tool that measures performance skills based on preschool children's everyday function. Results provide evidence in support of the PSQ as a reliable and psychometrically sound instrument.

Barriers to Supported Employment for Persons With Developmental Disabilities

Nurses have long played a role in caring for persons with developmental disabilities (DD) and therefore require an understanding of the issues of quality of life for this population. Meaningful employment is important to life quality and a challenge for persons with DD. This retrospective study examined supported employment barriers for 112 adults with mild to moderate DD. Inattention, interpersonal, and behavior problems were frequent barriers (37.5%) to maintaining employment. Poor attendance, inadequate work quality, or interpersonal problems were responsible for 20.8% of involuntary employment terminations. An understanding of work-related issues for people with DDs may assist nurses to better address the psychosocial needs of this population.

Influenza Vaccination in a “Special Needs” Extended Care Facility

ISSUE: The Conway Human Development Center (CHDC) is a 515 bed extended care residential facility in Conway, Arkansas. Our clients, who range in ages from 10 to 70 years, have profound (82%) to moderate developmental disabilities. Influenza vaccines are indicated for residents and healthcare workers at long-term care facilities. Many residents in our facility are immunosuppressed or suffer from poor respiratory status due to physical deformity, cerebral palsy or chronic illness. With the threat of pneumonia always present in this population, we are dedicated to preventing anything that is known to increase the risk for pneumonia or severe respiratory disease in our residents – including influenza.

Social Inclusion—The Next Step: User-Friendly Strategies to Promote Social Interaction and Peer Acceptance of Children with Disabilities

Research tells us that children with developmental disabilities and delays have reduced social interaction and reduced social acceptance by other children in comparison with their same-aged peers within early childhood settings. The consequence of this is that these children have limited access to the possible developmental opportunities within the peer culture, leading to long-term social maladjustment. This can be counteracted by the implementation of carefully planned social interventions; however, the strategies informed by current research are often unable to be implemented because of the lack of user-friendliness for staff. This paper is a quantitative and qualitative case study of a four-year-old child with a moderate developmental disability attending a community kindergarten. The authors investigated the effect of strategies planned in collaboration with kindergarten staff to increase social interaction and acceptance for the focal child within the existing peer culture.

The effects of low-dose fentanyl on emergence agitation and quality of life in patients with moderate developmental disabilities

Study Objective To analyze whether emergence agitation could be reduced by using a low dosage of fentanyl without causing an increase in postoperative adverse effects and/or affecting the patient's quality of life after discharge. Design Randomized, controlled crossover trial. Setting University medical center. Patients One hundred ten ASA physical status I and II ambulatory patients with moderate developmental disabilities who received anesthesia for dental care. Interventions During their first visit, patients were randomly treated either with a placebo or a 1- to 1.5- μg/kg dose of fentanyl before inserting a reinforced laryngeal mask airway and treated with the remaining method during their second visit 6 to 12 months later. Measurements Measured variables included anesthetic and emergence period, the occurrence of emergence agitation, and postanesthetic adverse effects such as nausea and vomiting. After discharge, the patient's quality of life was evaluated based on disturbances in daily activity, eating habit, and sleeping behavior. Results were analyzed by Student t test and McNemar test ( P < 0.05). Main Results Between the two treatments, there was a significant difference in the frequency of emergence agitation ( P = 0.04) but no postoperative adverse effects such as postoperative nausea and vomiting. We also found no significant disturbances in the patient's quality of life according to daily activity, eating habits, or sleeping behavior. Conclusion Emergence agitation may be reduced by using 1 to 1.5 μg/kg of fentanyl during induction while maintaining anesthesia by sevoflurane in patients with moderate developmental disabilities undergoing dental care, without causing an increase in postoperative adverse effects and affecting the patient's life quality after discharge.

ACCeL system – A new way of controlling computers using pen and paper: Some user experiences

This paper is based on the experiences from a project, the purpose of which was to support people with mild to moderate developmental disabilities in using the Internet. During the first year of the project, we studied how a web site should be designed so that people in the target group could understand and make use of it. The goal was to develop guidelines that cognitively supported the user [2]. Since each user is unique, it is also desirable to let him or her control the design to as great an extent as possible. In another study within the framework of the project, we examined how people with mild to moderate developmental disabilities managed to navigate the Internet. Seven test subjects were asked to move between different web sites using Microsoft Internet Explorer’s tools. Observations were made of how they managed to move forward, back, up, down and to open or close a web site. Their ability to carry out a search or type in a URL address was also observed. The results from this study demonstrated that the test persons managed to navigate without much difficulty [3]. Problems arose, however, when navigation required the subjects to process text. The study indicated that there were two cog

How people with developmental disabilities navigate the Internet

We live at a time when the Internet is used increasingly for communication, for information, and for the exchange of goods and services. Questions arise about how people with learning disabilities make use of this new technology. In this article, Bjorn Harrysson, with two of his colleagues, A. Svensk and G. I. Johansson, from the Department of Design Sciences at the Lund Institute of Technology in Sweden, explores the opportunities and difficulties experienced by members of this group when navigating the Internet.Harrysson, Svensk and Johansson observed seven people, aged between 15 and 44 and with mild to moderate developmental disabilities, as they navigated between different web pages using the general tools of Microsoft Internet Explorer Web Browser. The authors describe some of the strategies that were used for moving within and between web pages and for opening web pages, carrying out searches and finding preferred web sites.The results of the study are partly optimistic. The people involved made good use of many of the features of the general software. They experienced greater difficulties when it became necessary to use text to navigate and explore the huge potential of the Internet. Harrysson, Svensk and Johansson close their article by making a series of recommendations for developments that would facilitate ease of access and independence in the use of the Internet for people with developmental disabilities.

Developmental progress of children with congenital heart defects requiring open heart surgery

Recent advances for infants requiring early open heart surgery have resulted in a dramatic decline in mortality and severe morbidity. The developmental progress of these new survivors is currently being defined. Causes contributing to brain injury are multifactorial, and may involve preoperative, intraoperative, and postoperative events. Before surgery, these children often exhibit hypotonia, poor state regulation, microcephaly, and developmental delays. These findings are particularly prevalent in newborns. In the acute postoperative period, neurodevelopmental deficits continue to manifest clinically. Long-term follow-up studies indicate that subtle neurological deficits and global developmental lags are characteristic of this population. Overall, severe neurological sequelae are uncommon; however, mild to moderate developmental disabilities are prevalent. Functional limitations, academic achievements, and health-related quality of life are areas that deserve further attention.

Outpatient Assessment of Aberrant Behaviour for a Child with Developmental Disability and Attention Deficit/Hyperactivity Disorder

We describe a multidisciplinary outpatient assessment service for children with developmental disability who exhibit aberrant behaviour such as aggression and self-injury. The assessment and treatment planning process of this service is described with a 7-yr-old boy who was diagnosed with moderate developmental disabilities and attention deficit/hyperactivity disorder. He had recently been expelled from special school due to aberrant behaviour. Referring professionals wished to identify methods to control his behaviour with a view to returning him to school. Results of the assessment indicated that aberrant behaviour was maintained primarily by escape and avoidance of academic tasks. Aberrant behaviour also seemed to be exacerbated by overactivity and poor attention during academic tasks. Recommendations based on the assessment findings included redesigning the classroom curriculum and a trial of stimulant medication.

User Interface Design Guidelines for Computer Accessibility by Mentally Retarded Adults

An exploratory three-phase study examined the ability of adults diagnosed as moderately developmentally disabled to successfully use a personal computer, input devices preferred, and user interface design factors to be considered when designing or selecting applications for this population. Phase I observed the reaction of the participants, none of whom had ever used a computer, to a graphical user interface. In Phase II usability tests compared the mouse, the trackball, and the touchscreen to gather heuristic data on input device preference and develop user interface design guidelines for applications for the target population. Phase III tested the guidelines by developing two prototype games: “Shopping,” designed to teach money-handling skills, and “Getting Dressed,” to teach a basic life skill. Phase I showed that participants liked and understood the graphical user interface. All could use the touchscreen, and most could use the mouse. Phase II usability testing found that the mouse was preferred over the touchscreen and the trackball, although its drag-drop times were longer. Reasons given were less fatigue and greater control. Phase III found participants preferred screens that allowed them to control the action, that quick or unexpected screen responses were upsetting, and that strong, realistic visual feedback was important. The study is seen as a first step in developing guidelines to make the computer accessible to those with moderate developmental disability.

Using Self-Scheduling to Promote Self-Directed Leisure Activity in Home and Community Settings

The effects of self-scheduling plus training to implement weekly schedules on the frequency, diversity, and novelty of self-directed leisure activities by three adults with moderate developmental disabilities living in the community were examined in this study. Self-scheduling consisted of the adults choosing desired leisure/recreational activities via picture cards, and then placing the cards in a sequenced activity book for each day of the week. Training for schedule implementation consisted of a daily prompt to look at the picture schedule plus a nightly review of engaged activities. All participants learned to independently self-schedule their leisure activity as a result of training and maintained their performance on probes conducted between 8 weeks and 6 months after training. Self-scheduling and training schedule implementation resulted in substantial increases in the weekly frequency and diversity of self-directed leisure activity, as well as increases in the cumulative novelty of activity. These results were also maintained long term. This study emphasizes the importance of incorporating choice and self-management in leisure education for persons with severe and moderate intellectual disabilities.