Abstract
Abstract : Bardet-Biedl syndrome (BBS) is a rare, systemic, autosomal genetic disorder characterised by various physical and cognitive disabilities. Among the many characteristics is obesity, polydactyly, visual and auditory impairment. The vision of people diagnosed with BBS degenerates over time, resulting in complete blindness. This genetic disorder is more prevalent in boys than in girls. There is limited research on BBS because of its rarity and hardly any research that addresses the psycho-educational support needs for learners. A case of Gezani1, an adolescent, Tsonga boy diagnosed with BBS has been used to study the psycho-educational support needs for learners with this disorder. Semi-structured interviews were conducted with the principal, teacher, department of education district official and the geneticist. Medical reports from other health care professionals, such as an optometrist, dietician, and physiotherapist were also analysed. From a content data analysis, the following six themes emerged: cognitive support, visual support, emotional support, medical support, physical support, and speech support. Gezani needs an individualised support programme that will address all his psycho-educational support needs, and he needs ongoing remedial lessons to address academic delays. Gezani needs early training in both mobility and visual access, as his vision will degenerate with time. The training will enable him to cope when he loses his sight. Gezani and his family need emotional support due to the social isolation that they experience in their African community from disability stigmatisation. The family needs support and guidance as various family members also live with BBS. Gezani needs to go for regular medical check-ups related to the deteriorating BBS symptoms. The geneticist and dietician initiated a specific physical exercise and dietary programme to manage his obesity tendencies. Lastly, due to Gezani’s continued language difficulties, ongoing speech therapy is recommended. These themes affect Gezani’s learning, as teaching support needs are provided in all domains for him to learn at his optimal level. This study highlights the crucial role that educational psychologists play in diagnosing and supporting children with rare genetic disorders.
M.Ed. (Educational Psychology)