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Anne-Marie Martin explains how a person-centred intervention reduced the incidence of challenging behaviour exhibited by a person with intellectual disabilities
Abstract
This article presents a case study of how positive behaviour support improved the quality of life of a client, a young woman with a rare chromosomal disorder, in an Irish residential service for people with intellectual disability. The client's biological, psychological and social presentations are outlined, including one of her challenging behaviours, scatolia which appeared to be linked to each of these elements of her life. In addition, her behaviour support plan, based on a full assessment, is described.
Keywords
Goltz syndrome, positive behaviour support, scatolia
National and international policies and guidelines recommend prevention rather than management of challenging behaviours, and state that all support plans for clients who engage in such behaviours should focus on improvements to quality of life (Inclusion Ireland 2003, International Association for the Scientific Study of Intellectual Disabilities 2012). According to the Irish nurse regulator, An Bord Altranais (1997), nurses' primary objectives are maintaining the safety and wellbeing of, and preventing distress among, service users and others affected by challenging behaviours.
This article documents a comprehensive plan of care that was set out to support a client, a young woman with a rare chromosomal disorder called focal dermal hypoplasia, or Goltz syndrome. This is a rare, congenital, X-chromosome-dominant disorder, involving multiple systems of the body.
It is characterised by anomalies of the skin and other tissues, such as teeth and the lining of the mouth, anus, nostrils, hair and nails, that have ectoderms and mesoderms (Miranda et al 2005, Clements et al 2009, Tollefson and McEvoy 2009, Jain et al 2010). Originally known as atrophoderma linearis maculosa et papillomatosis congenitalis (Liebermann 1934), the condition was described as focal dermal hypoplasia by Goltz et al (1962). The specific gene associated with the condition was identified simultaneously by Wang et al (2007) and Grzeschik et al (2007).
Goltz (2010) states that only between 200 and 300 cases of focal dermal hypoplasia have been reported worldwide. About 90 per cent of affected individuals are female (Giam and Khoo 1998), with most affected males dying in the womb (Clements et al 2009). Presentation is variable, with mild to severe manifestations (Jain et...