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ItemEffect of Reduced Agalsidase Beta Dosage in Fabry Patients: The Australian Experience(SPRINGER-VERLAG BERLIN, 2012)BACKGROUND: In Australia, enzyme replacement therapy (ERT) for Fabry Disease (FD), both Agalsidase alfa (Replagal, Shire HGT) and beta (Fabrazyme, Genzyme), is funded and monitored through a specific government program. Agalsidase beta supply has been rationed by Genzyme since 2009 due to manufacturing issues. Consequently, the Australian Fabry Disease Advisory Committee has treated patients on Agalsidase beta at 50% of their usual dose from mid-2009, with a further reduction to 30% for some patients from late 2009. AIM: To determine the clinical effect of Agalsidase beta dose reduction in the Australian FD patient cohort. METHODS: A questionnaire assessing FD symptoms was administered to 40 patients on long-term ERT. Clinical data from The Fabry Registry for patients receiving Agalsidase alfa or beta, for at least 2 years prior to the time of enforced Agalsidase beta dose reduction, were reviewed. Disease burden and quality of life (QOL) were graded using the Disease Severity Scoring System, Mainz Severity Score Index, Brief Pain Inventory and Short Form 36 Health Survey at 2 years before dose reduction, at the time of dose reduction and at the most recent clinical review following dose reduction. RESULTS: Disease severity and QOL scores did not change between the ERT groups. Males on Agalsidase beta reported lower energy levels after dose reduction, while no change was reported by females on either product or by males on a stable dose of Agalsidase alfa. CONCLUSION: This study suggests that energy levels in male patients worsen after dose reduction of Agalsidase beta.
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ItemSevere Infusion Reactions to Fabry Enzyme Replacement Therapy: Rechallenge After Tracheostomy(SPRINGER-VERLAG BERLIN, 2012)A 34-year-old male patient with Fabry disease (OMIM 301500) commenced enzyme replacement therapy (ERT) with Agalsidase alfa, with positive clinical response. Infusion reactions, initially mild and easily managed, commenced during his 13th infusion, and continued over the next 3 years. Severity of reactions subsequently increased despite very slow infusion, extended prophylactic medication and attempted desensitisation, requiring regular intensive care unit (ICU) admissions. Facial oedema and flushing, throat tightness, headache and joint pain typically occurred 4-36 h after completion of most infusions, responding rapidly to subcutaneous adrenaline. Low titre specific IgG seroconversion was noted at 12 months, with subsequent reversion to negative after 5 years, despite persistence of infusion reactions. Specific IgE and skin testing was negative. Trial of ERT product switch to Agalsidase-beta resulted in no improvement in reactions. At 5 years, ERT was ceased in the face of recurrent ICU readmissions. In the face of progressive clinical deterioration, he underwent tracheostomy to allow recommencement of ERT. Two years later, he has clinically improved on regular attenuated dose Agalsidase-beta, administered by slow infusion in a local hospital setting.
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ItemCarpal Tunnel Syndrome in Fabry Disease(SPRINGER-VERLAG BERLIN, 2012)Carpal tunnel syndrome (CTS) is a common peripheral mononeuropathy affecting up to 4% of the general population, typically women in late middle age. The incidence in patients with Fabry disease (FD) is unclear, but may affect 25% of patients with this X-linked lysosomal storage disease. We report three cases of CTS in young Caucasian male patients with classical FD, who developed CTS symptoms with supportive nerve conduction study (NCS) findings. Two patients had bilateral CTS and two had evidence of concurrent ulnar nerve neuropathy on NCS, suggesting a systemic process contributed to nerve compression. All were receiving enzyme replacement therapy (ERT) and had a moderate burden of FD complications. It is possible that an increase in connective tissue in the intracarpal canal in FD patients may be incited by injury to fibroblasts, via either accumulation of globotriaosylceramide (GL3) or local ischaemia through endothelial injury. The former hypothesis may be a more plausible explanation for the development of CTS, as histology of the flexor retinaculae from our patients has demonstrated fibroblasts with characteristic vacuolation and excessive myxomatous stroma, despite endothelial clearance of GL3 in these patients receiving ERT. CTS should not be overlooked in FD patients and young patients presenting with CTS should be evaluated for an underlying systemic or genetic disorder. Surgical carpal tunnel decompression was effective in our patients, already troubled by long-standing acroparesthesia, in providing sustained relief of symptoms.
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ItemA festival in disarray: the 2002 Adelaide festival: A debacle or another model of arts organization and leadership?(HELDREF PUBLICATIONS, 2004-01-01)
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ItemPrivilege or problem: The distinct role of government in arts development in South Australia(ROUTLEDGE JOURNALS, TAYLOR & FRANCIS LTD, 2005-01-01)
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ItemDoes it matter who is in charge? The influence of the business paradigm on arts leadership and management.(International Graduate School of Management, University of South Australia, 2005)
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ItemDoes the art end when the management begins? The challenges of making ‘art’ for both artists and arts managers(Arts & Cultural Management Program, University of South Australia, School of Management, 2010)
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ItemArts and business: The impact of business models on the activities of major performing arts organisations in Australia(University of Queensland, School of Languages and Comparative Cultural Studies, 2010)Managerial business models were first introduced to Australian subsidised performing arts organisations by the then Howard Coalition government in 2000. Until the early 1990s, Australian arts organisations were contextualised as 'not for profit' entities, with an overall objective of producing good art. Over the past decade, however, major Australian performing arts organisations have been viewed more frequently as part of an 'industry' and, within this industry construct, framed as 'business entities', with a need to prove positive financial outcomes as a first priority. This article explores what is meant by business models in the context of Australian major performing arts organisations and looks at the impact of this approach.
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ItemFestivals, artists and entrepreneurialism: the role of the Adelaide Fringe Festival(Griffith University, Australia, 2011)This paper addresses the role of the Adelaide Fringe Festival in facilitating entrepreneurialism amongst participating artists. Tracing the discursive development of the notion of the entrepreneur, the paper identifies how entrepreneurialism has been taken up by the discourses of the creative industries. While we note that entrepreneurialism is a key strategy within the creative industries framework, it would appear for artists the concept does not necessarily connote the achievement of commercial outcomes. The paper argues that these cultural entrepreneurs are defined by self-reliance, the focus on the development of their craft, and the cultural value of their work.
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ItemNo Preview AvailablePerceptions of sex-role stereotypes, self-concept, and nursing role ideal in Chinese nursing students(BLACKWELL PUBLISHING LTD, 2002-02)AIM: This study examined the relationship between sex-role stereotypes, self-concept and the requisite personality characteristics of an ideal nurse in a cohort of Hong Kong nursing students. METHODS: To rate these concepts a measure of eight comprehensive dimensions of personality perception was administered to 177 nursing students, studying on preregistration and postregistration programs at a Hong Kong tertiary institution. Both male and female nursing students perceived an ideal nurse to possess a profile of traits including being high on the dimensions of emotional stability, application, intellect, helpfulness and restraint. RESULTS: No significant difference between the self-ratings of the male and female students was found, indicating that male students had undergone a highly self-selective process when choosing nursing education under the influence of Chinese cultural stereotypical attitudes towards nursing. A typical Chinese nurse was rated as similar to the typical female in Chinese society by both male and female nursing students. A typical Chinese nurse was rated relatively low on the masculine dimensions of openness, extroversion and assertiveness. The self-ratings of male nursing students more closely approximated the ideal nurse than did the self-ratings of female nursing students. CONCLUSION: The conclusions highlight implications for the recruitment and education of both male and female nursing students in Hong Kong society.