Developing a screening instrument for underlying core domains of communicative competence in children with communication support needs.

A socio-neurocognitive approach to augmentative and alternative communication (AAC) shows several underlying domains of communicative competence: Attention, perception, cognition, memory, orientation, socio-emotional development, motor skills, and language. To determine developmental markers of these underlying core domains of communicative competence in children with communication support needs, we developed a new screening instrument. The present article consists of three consecutive studies. In study 1, we constructed the first version of the screening instrument based on a sample of both children without disabilities and children with Down syndrome. In study 2, we confirmed the reliability (i.e., internal consistency) of the screening instrument in a new group of young children with typical development and established concurrent validity with the Early Language Scale. In study 3, we established concurrent validity with the Communication Matrix in a clinical sample of children with communication support needs. The screening instrument can be used in clinical practice as part of AAC assessment to provide comprehensive insights into strengths and weaknesses in the underlying core domains of communicative competence of children with communication support needs.

A tutorial: self-created film as a semiotic resource in AAC.

Sharing personal stories with others is essential to human interaction and language development. To communicate, individuals use a variety of semiotic resources, including images, symbols, and written and spoken language. These modes are deployed in the co-construction of a daily face-to-face conversation. A self-created film can serve as a valuable resource to facilitate a deeper understanding of a personal experience, especially where spoken or written language may present a challenge, for example, for people who rely on augmentative and alternative communication (AAC). Although the AAC literature indicates that using videos delivers benefits for aided communicators, guidelines on how to self-create, use, and transcribe them are rare. The present paper, a tutorial, describes how people who use AAC can develop a personal-video-scene (PVS) via the Film as Observable Communication (FaOC) method to utilize self-created films in sharing their stories. The first part of this paper, the theoretical framework, describes theories, methods, and practices from the fields of AAC, social semiotics, and visual anthropology, on which the FaOC method is based. The second part provides a step-by-step tutorial delivering practical guidance on how to create, use, and transcribe the PVS as a resource in conversations.

Diagnosis and management in Pitt-Hopkins syndrome: First international consensus statement.

Pitt-Hopkins syndrome (PTHS) is a neurodevelopmental disorder characterized by intellectual disability, specific facial features, and marked autonomic nervous system dysfunction, especially with disturbances of regulating respiration and intestinal mobility. It is caused by variants in the transcription factor TCF4. Heterogeneity in the clinical and molecular diagnostic criteria and care practices has prompted a group of international experts to establish guidelines for diagnostics and care. For issues, for which there was limited information available in international literature, we collaborated with national support groups and the participants of a syndrome specific international conference to obtain further information. Here, we discuss the resultant consensus, including the clinical definition of PTHS and a molecular diagnostic pathway. Recommendations for managing particular health problems such as dysregulated respiration are provided. We emphasize the need for integration of care for physical and behavioral issues. The recommendations as presented here will need to be evaluated for improvements to allow for continued optimization of diagnostics and care.

Core vocabulary of young children with Down syndrome.

The aim of this study was to develop a core vocabulary list for young children with intellectual disabilities between 2 and 7 years of age because data from this population are lacking in core vocabulary literature. Children with Down syndrome are considered one of the most valid reference groups for researching developmental patterns in children with intellectual disabilities; therefore, spontaneous language samples of 30 Dutch children with Down syndrome were collected during three different activities with multiple communication partners (free play with parents, lunch- or snack-time at home or at school, and speech therapy sessions). Of these children, 19 used multimodal communication, primarily manual signs and speech. Functional word use in both modalities was transcribed. The 50 most frequently used core words accounted for 67.2% of total word use; 16 words comprised core vocabulary, based on commonality. These data are consistent with similar studies related to the core vocabularies of preschoolers and toddlers with typical development, although the number of nouns present on the core vocabulary list was higher for the children in the present study. This finding can be explained by manual sign use of the children with Down syndrome and is reflective of their expressive vocabulary ages.

Insights into Shared Decision-Making in Interprofessional Teams for a Boy with Down Syndrome with Communication and Language Issues: Simulation-Based Training for Medical and Allied Health Students.

BACKGROUND: Shared decision-making is one key element of interprofessional collaboration. Communication is often considered to be the main reason for inefficient or ineffective collaboration. Little is known about group dynamics in the process of shared decision-making in a team with professionals, including the patient or their parent. This study aimed to evaluate just that. METHODS: Simulation-based training was provided for groups of medical and allied health profession students from universities across the globe. In an overt ethnographic research design, passive observations were made to ensure careful observations and accurate reporting. The training offered the context to directly experience the behaviors and interactions of a group of people. RESULTS: Overall, 39 different goals were defined in different orders of prioritizing and with different time frames or intervention ideas. Shared decision-making was lacking, and groups chose to convince the parents when a conflict arose. Group dynamics made parents verbally agree with professionals, although their non-verbal communication was not in congruence with that. CONCLUSIONS: The outcome and goalsetting of an interprofessional meeting are highly influenced by group dynamics. The vision, structure, process, and results of the meeting are affected by multiple inter- or intrapersonal factors.

Emotion dysregulation as cross-disorder trait in child psychiatry predicting quality of life and required treatment duration.

Background: Emotion dysregulation (ED) is increasingly under investigation as a cross-disorder trait, and is by some considered as the core feature in mental disorders. The aims of this study were to scrutinize the overlapping and distinct characteristics of ED for internalizing, externalizing and neurodevelopmental disorders and to identify the most pertinent ED characteristics to guide clinicians in treatment choice. Methods: Information on clinical diagnosis (Attention Deficit/Hyperactivity Disorder ADHD, Autism Spectrum Disorder, Oppositional Defiant Disorder/Conduct Disorder, Anxiety and Mood Disorders), ED (measured by the CBCL-Emotion Dysregulation Index), Quality of Life (Qol, measured by the Kidscreen-27), and treatment duration (measured by Electronic Health Records) was retrieved from two large samples of toddlers (1.5-5 year old; N = 1,544) and school aged children (6-18 year old; N = 7,259). Frequency scores and logistic regression were used to study symptom profiles of ED, as measured with CBCL-EDI, across all disorders. Linear regression was used to determine the predictive value of ED (CBCL-EDI total score) regarding QoL and treatment duration in addition to-and in interaction with-clinical diagnosis. Results: Across disorders, equal levels of total ED were found, which predicted lower QoL and a longer treatment duration in addition to clinical diagnosis. The majority of items (11/15 and 16/18) were of equal relevance to the disorders; items that were not, largely reflected disorder specific DSM definitions (i.e., externalizing symptoms in ODD/CD and internalizing symptoms in Anxiety and Mood disorders). Conclusion: ED is a clinically useful cross-disorder trait to predict severity of impairment as well as required treatment duration. In addition, ED is largely composed of shared features across disorders, with certain disorder specific colored elements.

Predictors of receptive and expressive vocabulary development in children with Down syndrome.

PURPOSE: There is a lack of longitudinal data on predictors of vocabulary development in children with Down syndrome (DS). In typically developing children, many internal and external predictors of vocabulary development have been determined before. The purpose of the present study was to investigate the role of these variables in the receptive and expressive vocabulary development of children with DS. METHOD: The present study used a longitudinal design in young children with DS to study the vocabulary development over a period of 1.6 years and investigated the possible predictive role of child-related and environmental variables. RESULT: Receptive vocabulary development was best predicted by the adaptive level of functioning and early receptive vocabulary skills. Expressive vocabulary development was best predicted by the adaptive level of functioning, receptive vocabulary, maternal educational level, level of communicative intent of the child, attention skills and phonological/phonemic awareness. CONCLUSION: A wide range of internal and external predictors for vocabulary development of children with DS was found. Predictors resemble those predicting vocabulary development in peers with typical development between 1 and 6 years of age, as identified in other studies.

Food preferences in young Dutch children and recommendations for feeding intervention in developmental disabilities.

Total and chronic food refusal (i.e., the refusal of all types of food during a prolonged period) in young children with developmental disabilities can be treated effectively using a combination of environmental interventions. However, no guidelines for the selection of food items to offer the child in these interventions are available. The aim of the present study was to assess the preferences for specific food items of young Dutch nondisabled children (N=254) in order to enable trainers to select food items that maximize success of feeding interventions. Results indicate that 54 out of 107 food items were found to be preferred. The mean appreciation scores of boys and girls did not differ significantly for these preferred food items, except for raisins and brown bread. Also, there were no differences between the distinguished age-groups, except for peach. Recommendations for the selection of food items within feeding intervention for total and chronic food refusal in young children with developmental disabilities are given.