Recommendations on the Training of Interveners for Students who are Deafblind

Preface The National Intervener Task Force, formed in January of 2002, is an informal network comprised of a variety of individuals representing state/multi-state deafblind projects, higher education institutions, agencies, and parents. The focus of the Task Force has been to develop a consistent understanding of issues and services related to the training and use of interveners in educational settings and early intervention settings. During the October 2002 Task Force meeting, participants requested that a small representative group be formed to summarize recommended intervener training practices and to review competencies recommended for intervener training. In response to this request, the SKI-HI Institute at Utah State University hosted a two-day meeting in February 2003 to review and recommend effective practices for the conceptualization and development of intervener training programs. Simultaneous to the Intervener Task Force activities, the SKI-HI Institute was also engaged in a variety of activities related to the identification of competencies for interveners. These activities included the development of multiple draft competency listings, field reviews, and revisions. In the fall of 2003, at the request of the SKI-HI Institute, staff with the National Technical Assistance Consortium for Children and Youth who are Deaf-Blind (NTAC) facilitated an external review of the proposed intervener competencies. The results of this review were used in the selection of the final intervener competencies. In the spring of 2004, the SKI-HI Institute and NTAC also facilitated the development of a national Community of Practice Focused on Interveners and Paraprofessionals Working with Children and Youth who are Deafblind. (Note: By definition, an intervener is a paraprofessional who has training and specialized skills in deafblindness.) Based on the needs and activities previously identified and initiated by the National Intervener Task Force and others, the identification of recommended practices related to the training and use of interveners and paraprofessionals was targeted as the initial focus of activities proposed to the Community. These include: • Refining and recommending competencies and training content for interveners and paraprofessionals working with students who are deafblind. • Recommending degrees of mastery, or levels of learning, for intervener training activities. • Developing a recommended crosswalk between the identified mastery levels and the identified training practices/pedagogy needed to reach the identified mastery levels. • Summarizing characteristics of existing training models. • Integrating training activities into larger state professional development infrastructures (i.e. No Child Left Behind (NCLB) and IDEA comprehensive systems of personnel development (CSPD). 2 • Providing examples of documenting training and acquisition of competencies. • Identifying recommended standards of practice related to the use of interveners and paraprofessionals in educational settings. This paper represents the authors' initial attempts at addressing some of the training issues and needs identified through the Intervener Task Force activities, and subsequently by the Community of Practice. It is not intended to be an exhaustive response, nor all-inclusive. It is intended to provide information and resources that can guide in the planning and development of intervener training programs. Other issues such as the identification of standards of practice related to the use of interveners and paraprofessionals will be addressed in subsequent documents. This paper provides: • A common understanding of the definition and role of an intervener • A comprehensive list of recommended intervener competencies and mastery levels necessary to become an intervener • A portfolio process for the documentation of intervener competencies • A discussion of recommended training practices • A checklist of considerations for developing an intervener training system It is hoped that this information will be useful and support the ongoing efforts related to the use of interveners and paraprofessionals with students and youth who are deafblind

Muscular dystrophy-dystroglycanopathy in a family of Labrador retrievers with a LARGE1 mutation

Alpha-dystroglycan (αDG) is a highly glycosylated cell surface protein with a significant role in cell-to-extracellular matrix interactions in muscle. αDG interaction with extracellular ligands relies on the activity of the LARGE1 glycosyltransferase that synthesizes and extends the heteropolysaccharide matriglycan. Abnormalities in αDG glycosylation and formation of matriglycan are the pathogenic mechanisms for the dystroglycanopathies, a group of congenital muscular dystrophies. Muscle biopsies were evaluated from related 6-week-old Labrador retriever puppies with poor suckling, small stature compared to normal litter mates, bow-legged stance and markedly elevated creatine kinase activities. A dystrophic phenotype with marked degeneration and regeneration, multifocal mononuclear cell infiltration and endomysial fibrosis was identified on muscle cryosections. Single nucleotide polymorphism (SNP) array genotyping data on the family members identified three regions of homozygosity in 4 cases relative to 8 controls. Analysis of whole genome sequence data from one of the cases identified a stop codon mutation in the LARGE1 gene that truncates 40% of the protein. Immunofluorescent staining and western blotting demonstrated the absence of matriglycan in skeletal muscle and heart from affected dogs. Compared to control, LARGE enzyme activity was not detected. This is the first report of a dystroglycanopathy in dogs