Razvijanje in spodbujanje pripovedovalne zmožnosti pri otroku s KBG sindromom

Lapanja, Mojca (avtor), Lipec Stopar, Mojca (mentor), Novšak Brce, Jerneja (komentor)

KBG sindrom je redka genska motnja, ki prizadene več telesnih organskih sistemov. Prvi ga je leta 1975 opisal J. Herrmann. Mutacija je na genu ANKRD11, vzorec dedovanja je avtosomno dominanten. Značilni simptomi pri posameznikih vključujejo neobičajne obrazne poteze in nepravilnosti skeleta. Zunanji znaki, ki so značilni za sindrom, so prisotni že ob rojstvu, vendar pa jih je mogoče spregledati, dokler se ne pojavi razvojni zaostanek, ki se opaža na vseh področjih razvoja. Največ oseb s KBG sindromom ima lažjo motnjo v duševnem razvoju. Prisotne so lahko tudi čustvene in socialne težave, motnje avtističnega spektra in motnja pozornosti z motnjo koncentracije in hiperaktivnostjo. Število oseb z diagnosticiranim KBG sindromom je v svetu izredno majhno, prav zato raziskav, ki bi zajemale izključno razvoj njihovega govorno-jezikovnega razvoja, ni zaslediti. Ker ima otrok s KBG sindromom, s katerim sem izvajala enoletno logopedsko obravnavo, pridruženo lažjo motnjo v duševnem razvoju, sem njegove dosežke s področja pripovedovanja primerjala z značilnostmi in dosežki otrok te skupine ter normami in smernicami mlajših in enako starih otrok značilnega razvoja. Usvajanje jezika pri otrocih z motnjo v duševnem razvoju poteka počasneje kot pri vrstnikih značilnega razvoja. Leksikalni razvoj jim ovirajo različni dejavniki – intelektualni primanjkljaji, ki vplivajo na sposobnost kategorizacije predmetov, generalizacijske zmožnosti in spomin, šibka pozornost, šibke spominske funkcije, težave s priklicem, manjše število strategij učenja besed in šibkejše besedišče. Manj zrele so tudi njihove komunikacijske zmožnosti. Primanjkljaji se pojavljajo na področjih produkcije, recepcije in ekspresije. Vse našteto ima vpliv tudi na zmožnost pripovedovanja. Pripovedovanje zgodbe daje uvid v jezikovni, spoznavni in socialni razvoj otroka. Pripovedovanje zahteva integracijo jezikovnih, spoznavnih in socialnih zmožnosti. Izsledki raziskav pri otrocih z motnjo v duševnem razvoju so pokazali, da je razvitost spretnosti pripovedovanja glede na dolžino povedi, morfološko in leksikalno raznolikost ter uporabo narativnih sredstev primerna njihovi mentalni starosti. Težave se kažejo pri slabši uporabi jezikovnih kohezivnih orodij, prepoznavanju čustvenih stanj nastopajočih junakov in referenčni komunikaciji ter ustreznosti. Prvi cilj najinih srečanj je bil dobiti uvid v zmožnost pripovedovanja zgodbe otroka s KBG sindromom. Na podlagi tega je bil oblikovan program, katerega cilj je bil razvijanje zmožnosti pripovedovanja. V začetku in ob koncu logopedske obravnave sem za oceno zmožnosti pripovedovanja zgodbe otroka s KBG sindromom uporabila Preizkus pripovedovanja zgodbe: Žabji kralj in Splošni govorni preizkus: Pisno sporočanje. Spremembe na ravni kohezivnosti in koherentnosti sem spremljala z analizo otrokovega pripovedovanja zgodbe po poslušanju predhodno prebrane mu zgodbe ob slikanici in samostojnem pripovedovanju zgodbe ob slikovnem gradivu ter s primerjavo pripovedovanega besedila (predstavitev sebe). Otrok s KBG sindromom in lažjo motnjo v duševnem razvoju je med izvajanjem individualne logopedske obravnave v razvoju svoje govorne kompetentnosti dosegel pomemben napredek. Otrok s KBG sindromom in lažjo motnjo v duševnem razvoju je bil ob začetku najinega dela star 9 let in 2 meseca, njegov razvoj govorno-jezikovne kompetentnosti pa je ustrezal 4–6 let starim otrokom značilnega razvoja. Ob koncu najinega dela je bil star 10 let in 2 meseca, njegovi dosežki v razvoju govorne kompetentnosti pa so bili normativni za skupino 8–9 let kronološko starih otrok značilnega razvoja. Velik napredek se je pokazal na področju koherentnosti, manjši pa na področju kohezivnosti.

• Jezik: Slovenski jezik
• Ključne besede: KBG sindrom
• Vrsta gradiva: Magistrsko delo/naloga
• Tipologija: 2.09 - Magistrsko delo
• Organizacija: PEF - Pedagoška fakulteta
• Leto izida: 2022
• PID: 20.500.12556/RUL-135689
• COBISS.SI-ID: 102227971
• Datum objave v RUL: 04.04.2022

Sekundarni jezik

• Jezik: Angleški jezik
• Naslov: Development and stimulation of child’s story-telling ability with KBG sindrom

Izvleček

KBG syndrome is a rare genetic disorder affecting several organ systems. It was first described by J. Herrmann in 1975. The mutation is located in the ANKRD11 gene and follows an autosomal dominant inheritance pattern. Typical symptoms include unusual facial features and skeletal abnormalities. These symptoms are present from birth, but may be overlooked until the onset of a general developmental delay. Most patients with KBG syndrome exhibit mild intellectual disability. Other common comorbidities include emotional and social difficulties, autism spectrum disorders, and attention deficit and hyperactivity disorder. The number of patients with a KBG syndrome diagnosis worldwide is extremely small, which is why there have been, to my knowledge, no studies dedicated exclusively to their speech and language development. The child with KBG syndrome who I provided with a year-long speech therapy treatment also had mild intellectual disability, which is why his storytelling scores were compared to those of children with the same intellectual disability, as well as to the expected scores for younger and same-age typical development groups. Language acquisition in children with intellectual disabilities is slower compared to typically developing children. Lexical development is impaired by a variety of factors – intellectual deficits affecting the ability to categorize objects and make generalizations, a short attention span, low memory functions and problems with recall, fewer strategies for word learning, and a less developed vocabulary. Their communicative skills are likewise less developed. The deficits manifest themselves in production, reception, and expression alike. Storytelling provides an insight into the linguistic, cognitive, and social development of a child. The activity requires an integration of all three abilities. Studies of children with intellectual disability have found that their storytelling ability levels in terms of sentence length, morphological and lexical variety, and the use of narrative devices is on a par with their mental age. However, they perform worse when it comes to the use of linguistic cohesion markers, recognizing the emotional state of characters, and appropriate use of reference. The initial goal of our sessions was to establish what the story-telling ability level of the child with KBG syndrome was. This served as the basis for a detailed work plan, with the aim of developing the storytelling ability further. At the beginning and the end of the treatment an assessment was made by means of two standardized tests – Preizkus pripovedovanja zgodbe: Žabji kralj1 and Splošni govorni preizkus: pisno sporočanje2. Improvements to the cohesion and coherence levels were tracked by means of an analysis of the child’s retelling of a story after listening to it being read from a picture book, his independent storytelling based on picture prompts, and his self-description. The child with KBG syndrome and mild intellectual disability achieved significant progress in language development during the course of the speech therapy sessions. At the start of the therapy the child was 9 years and 2 months old and his speech and language competence matched that of typically developing 4 to 6 year olds. At the end of the sessions the child was 10 years and 2 months and achieved the level of competence equivalent to that of typically developing 8 to 9 year olds. Major progress was observed in the area of coherence and minor progress in the area of cohesiveness.

• Ključne besede: KBG syndrome