WS – Assessing the Needs I

WS – Assessing the Needs I (05/2017 - 12/2017)

Challenges, Resources and Objectives in everyday life with Williams-Beuren Syndrome – Assessing the Needs for a tailored support

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Research projekt in collaboration with Bundesverband Williams-Beuren-Syndrom e.V.

 

Williams-Beuren-Syndrome: example of rare developmental impairments

Williams Beuren Syndrome (WS) is a rare, genetically determined developmental impairment usually goes hand in hand with an intellectual disability (review article: Pober, 2010). It is based on a random occurrence gene loss on chromosome 7 (Ewart et al., 1993). The prevalence of this rare disease is estimated to be 1: 7,500 (Stromme & Bjomstad, 2002).
Genetic, neurobiological and medical foundations of the WBS are now relatively well researched, but there is a lack of everyday educational insights that would be extraordinary helpful for people with WBS, their relatives and professional guidance (Prosetzky, 2014; Danielsmeier, 2014).

Initial Position: Transformation of the research field urgently required

Studies on the WS are almost exclusively based on a quantitative tradition of medical research (Prosetzky, 2014). As early as 2003, a transformation of the research field towards 1) greater pedagogical relevance 2) an expansion of the spectrum of methods and 3) an examination of complex relationships was required: "The Williams syndrome behavioral phenotype. The 'whole person' is missing" (Dykens, 2003, Title). Except for isolated studies (e.g. Fisher, 2014; Reis et al., 2016; Prosetzky, 2014; Danielsmeier, 2014), this transformation has so far failed to materialize.

Project Objectives

Provide impulses for support projects and stimulate discussion about the benefits of research

  • The first objective was to collect data from which impulses for the design of practical support projects for people with WBS and their families can be derived. Such projects could be: buddy or godfather projects, guidelines for relatives and educators, exchange forums, training for people with WBS, their relatives and / or professional companions - depending on what the respondents consider useful.

  • The second objective was to stimulate a specialist discussion about the practical usability of research on the WBS through the methodology and orientation of the own study.
    Ultimately, research paradigms would have to be discussed for integrated and holistic research, and professional and methodological gaps had to be overcome.

Project Results

(1) Comprehensive data set from 71 interviews provides the basis for educational guidelines

As part of a Cuncurrent Mixed Methods Design (Creswell, 2011), 71 interviews were carried out and transcribed in two partial studies on experiences and the need for support with relatives and professional companions. A large data set of qualitative data (1,800 pages of transcript) and associated quantitative data (N = 31) was collected. The interviews have so far been evaluated in five master theses and at the same time form the empirical basis for Vera Danielsmeier's dissertation, which started in 2017: "Challenges, opportunities and goals of people with WBS - An experience and needs analysis for tailored support offers" (cooperative doctorate with University of Bremen).

(2) Presentation of the work at the international WS conference

The study was presented and discussed as an ongoing project on November 10 and 11, 2017 at an international WBS conference (Research advances and integrative management "Building our future") at General Hospital La Paz, Madrid. The work was the only study presented there with a qualitative and open methodological focus. The approach met with great interest from the present researchers. There was a self-critical reflection of traditional research approaches, as well as networking with interested researchers.

(3) The research project as starting signal for a research group

At the Faculty of Social Sciences at the HSZG, the research group »WBS in Resonanz« was founded by Prof. Dr. Ingolf Prosetzky and Vera Danielsmeier in 2006. The data set collected will be further evaluated in the research group and the implementation of the support projects via the group will be guaranteed. The focus of his work is the evaluation of current international research publications, the development of own theoretical and empirical research contributions, international networking with other researchers and the implementation of regional theory-practice-practice projects. The long-term goal is to develop a pedagogical WBS competence and advice center in Görlitz.

Publications

  • Danielsmeier, V. (2017): Understanding Auditory Aversions in Williams Syndrome. A Longitudinal Mixed Methods Approach. Forschungsposter, präsentiert auf dem International Meeting on Williams-Beuren Syndrome "Building our Future". Madrid: Genral Hospital La Paz.
  • Danielsmeier, V. (2018): „Ich sehe was, das Du nicht siehst“. Welche Rolle spielen Emotionen bei der Fähigkeit die Perspektive Anderer einzunehmen? In: Wir üBer unS/Umschau. Zeitschrift des Bundesverbandes WBS. Heft 46, S. 71-78.
  • Köster, T. P. (2018): Herausforderungen und Chancen pädagogischer Arbeit mit Menschen, die mit dem WBS leben - erhoben anhand von Interviews mit Bezugspersonen aus dem fachlich/professionellen Umfeld der Betroffenen. Unveröff. Master-Thesis.
  • Prosetzky, I. (2018): Ausgewählte Impulse zur konkreten pädagogischen Unterstützung von Personen mit WBS. Bericht vom International Meeting on Williams-Beuren Syndrome "Building our Future" in Madrid/Spanien. In: Wir üBer unS/Umschau. Zeitschrift des Bundesverbandes WBS. Heft 46, S. 60-65.
  • Danielsmeier, V.; Groba, L.; Prosetzky, I. (2017): Neue Forschungsgruppe der Hochschule Zittau/Görlitz startet Forschungsreihe auf dem Bundesverbandstreffen 2017/. In: Wir üBer unS/Umschau. Zeitschrift des Bundesverbandes WBS. Heft 45, S. 72-74
  • Pestrup, M. (2017): Welche Copingstrategien nutzen Personen, die unter den Bedingungen des WBS leben? Eine Explorationsstudie aus Sicht von professionell Begleitenden. Unveröff. Master-Thesis.
  • Prosetzky, I. (2017): Aktuelle pädagogische und alltagsrelevante Erkenntnisse zum WBS. In: Wir üBer unS/Umschau. Zeitschrift des Bundesverbandes WBS. Heft 45, S.70-71
  • Wrieden, A. (2017): Zum Verständnis von Belastungen im Rahmen der Adoleszenz von Personen, die unter den Bedingungen des WBS leben - Hinweise aus der Sicht von professionell Begleitenden. Unveröff. Master-Thesis.

Bibliography

  • Creswell, J. W., & Plano Clark, V. L. (2011). Designing and conducting mixed methods research (2. Aufl.). Los Angeles: SAGE Publications.
  • Danielsmeier, V. (2014). Das Musik- und Geräuscherleben von Menschen mit dem Williams-Beuren-Syndrom im Kontext psychischer und sozialer Bedingungsfaktoren. Eine ressourcenorientierte Studie. Unveröff. Diplomarbeit im Fach Psychologie an der Universität Bremen.
  • Dykens, E. M. (2003). The Williams syndrome behavioral phenotype. The ‘whole person’ is missing. Current Opinion in Psychiatry, 16 (5), 523–528. doi.org/10.1097/00001504-200309000-00006 
  • Ewart, A. K., Morris, C. A., Atkinson, D., Jin, W., Sternes, K., Spallone, P., Keating, M. T. (1993). Hemizygosity at the elastin locus in a developmental disorder, Williams syndrome. Nature Genetics, 5(1), 11–16. doi:10.1038/ng0993-11
  • Fisher, M. H. (2014). Evaluation of a stranger safety training programme for adults with Williams syndrome. Journal of intellectual disability research : JIDR, 58 (10), 903–914. doi.org/10.1111/jir.12108
  • Prosetzky, I. (2014). Mehr als die Summe seiner Symptome: Zur kulturhistorischen Neuropsychologie und Pädagogik des Williams-Beuren-Syndroms (neue Ausg). Schriftenreihe International Cultural-historical Human Sciences: Vol. 48. Berlin: Lehmanns.
  • Reis, S. M., Schader, R., Milne, H. & Stephens, R. (2016). Music & Minds. Using a Talent Development Approach for Young Adults with Williams Syndrome. Exceptional Children, 69 (3), 293–313. doi.org/10.1177/001440290306900303
  • Schreier, M. (2013). Qualitative content analysis in practice (reprinted.). Los Angeles: Sage.
  • Stromme, P., Bjomstad, P. G., & Ramstad, K. (2002). Prevalence Estimation of Williams Syndrome. Journal of Child Neurology, 17(4), 269–271. doi:10.1177/088307380201700406
  • Pober, B. R. (2010). Williams–Beuren Syndrome. New England Journal of Medicine, 362(3), 239–252. doi:10.1056/NEJMra0903074

Project Funding

The research projekt is co-financed by tax funds on the basis of the budget decided by the members of the Saxon State Parliament.

Contact

Project Lead

Foto: Prof. Dr. phil. Ingolf Prosetzky
Prof. Dr. phil.
Ingolf Prosetzky
Fakultät Sozialwissenschaften
Campus 02826 Görlitz
Furtstraße 2
Building I, Room 2.07
+49 3581 374-4287
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