title: Impacts for Children Living with Genetic Muscle Disorders and their Parents - Findings from a Population-Based Study

authors: Jones, Kelly

subjects: Neuromuscular diseases

abstract: BACKGROUND: Genetic muscle disorders, including muscular dystrophies, congenital myopathies, and ion channel muscle diseases can be associated with significant disability. 
OBJECTIVE: This study aimed to explore child and parent perspectives of the impact of living with a genetic muscle disorder. 
METHODS: Eighty-three children (<16 years) with a clinical or molecular diagnosis were identified as part of a national prevalence study. Parents' experiences and needs were assessed using a study-specific questionnaire. Additional outcome measures included parent and child self-report versions of the Behavior Assessment System for Children and the Pediatric Quality of Life Inventory. Parents also completed the Hospital Anxiety and Depression Scale and Activlim.
RESULTS: Sixty-four percent of families had a combined annual household income below $60,000 NZD ($43,650 USD), being less than the national median income of $73,000 NZD ($53,112 USD). Parents reported needing more support than they were currently receiving (40%), particularly with household chores (23%) and transportation (17%). Few parents (13%) or children (4%) reported significant child behavioral difficulties. Risks of impaired quality of life were high (parent proxy 71%, child report 70%), and associated with co-morbid health conditions (p = 0.008), functional status (p = 0.001), wheelchair use (p = 0.001) and mechanical ventilation (p = 0.01). 
CONCLUSIONS: Findings are relevant to those involved in the care and support of children, and their families, who are impacted by genetic muscle disorders. Targeted guidelines are required to inform the provision of services, alongside promotion of existing community services to improve access to financial support, and assistance with day-to-day functioning. Future research should examine intervention and treatment options aimed at maximising affected children's quality of life.

text: Impacts for Children Living with Genetic Muscle Disorders and their Parents - Findings from a Population-Based Study BACKGROUND: Genetic muscle disorders, including muscular dystrophies, congenital myopathies, and ion channel muscle diseases can be associated with significant disability. 
OBJECTIVE: This study aimed to explore child and parent perspectives of the impact of living with a genetic muscle disorder. 
METHODS: Eighty-three children (<16 years) with a clinical or molecular diagnosis were identified as part of a national prevalence study. Parents' experiences and needs were assessed using a study-specific questionnaire. Additional outcome measures included parent and child self-report versions of the Behavior Assessment System for Children and the Pediatric Quality of Life Inventory. Parents also completed the Hospital Anxiety and Depression Scale and Activlim.
RESULTS: Sixty-four percent of families had a combined annual household income below $60,000 NZD ($43,650 USD), being less than the national median income of $73,000 NZD ($53,112 USD). Parents reported needing more support than they were currently receiving (40%), particularly with household chores (23%) and transportation (17%). Few parents (13%) or children (4%) reported significant child behavioral difficulties. Risks of impaired quality of life were high (parent proxy 71%, child report 70%), and associated with co-morbid health conditions (p = 0.008), functional status (p = 0.001), wheelchair use (p = 0.001) and mechanical ventilation (p = 0.01). 
CONCLUSIONS: Findings are relevant to those involved in the care and support of children, and their families, who are impacted by genetic muscle disorders. Targeted guidelines are required to inform the provision of services, alongside promotion of existing community services to improve access to financial support, and assistance with day-to-day functioning. Future research should examine intervention and treatment options aimed at maximising affected children's quality of life.

year: 2018

title: Complex uncertainty: Long-term risk management decision making in New Zealand local government

authors: Robson, David

subjects: Local government

abstract: New Zealand’s natural hazardscape has long posed risks for government owned water infrastructure and its stakeholder communities. In the context of climate change, there are predictions that the frequency and intensity of natural hazard events are set to increase, but there are uncertainties about when such events might occur, and the severity and impact on water infrastructure. These threats are compounded by pressures such as population growth and constrained funding. There is a need, therefore, to consider the suitability of current governance arrangements and decision processes for the management of complex, long-term risk associated with state assets. This research, therefore, asks, are current local governance arrangements and associated decision-making processes for the management of water infrastructure appropriate for long-term risk management in an environment of uncertainty? The thesis addresses this question by critically evaluating the extent to which governance arrangements enable effective risk management decisions that protect government owned infrastructure from natural hazards. 
A novel conceptual framework which matches the type of problem within the scales of time and complexity is developed on the basis of a literature review on risk management, adaptability and resilience, and modes of governance across multiple levels. This framework is then used to inform an exploration of risk management decision processes. The examination of these processes involved a participatory design involving local and central government officials and associated risk management professionals. A mixed-methods approach was applied to explore experiences of risk management as it relates to water infrastructure within local government, within the context of wider relationships with central government. Following a pilot workshop with sector actors and risk professionals to establish the broad research focus, the research involved 36 semi-structured interviews and a further workshop with sector officials to discuss the findings and critique the resulting decision-making model.
The research found that current risk management decision making is characterised by, largely, a short-term horizon, this being connected with the tendency of elected representatives to operate within the three-year election cycle. The absence of a longer-term focus is explained in part by the autonomous environment within which councils operate, removed from central government oversight, and with little sharing of knowledge, expertise or costs of risk management. The key findings of the research indicate: a lack of consistent guidance and direction by central government to local government on adaptive risk management; a lack of coordination across the network of councils, specifically in terms of sharing knowledge, data and risk management activities; a lack of specificity in legislation regarding long term risk management which leaves risk decision making open to interpretation by councils; a culture of localised short term decision making in councils, with a low level of comprehension as to the management of long-term risks; short-term competing priorities for the financial resources required to manage risk; and a mistrust of internal guidance and advice due to the lack of in-house expertise, and a preference for external subject matter expertise. 
Overall, this research has identified that the complex nature of the environment of uncertainty posed by climate change has not been adequately acknowledged within local government and this is reflected in the prevailing risk management arrangements being largely reliant on annually renewed insurance arrangements based on in-house planning practices. The decision-making framework developed in this thesis acknowledges the nature of risk and the need for adaptive approaches within the constraints of time and complexity, and has potential to inform both governance arrangements and decision processes.

text: Complex uncertainty: Long-term risk management decision making in New Zealand local government New Zealand’s natural hazardscape has long posed risks for government owned water infrastructure and its stakeholder communities. In the context of climate change, there are predictions that the frequency and intensity of natural hazard events are set to increase, but there are uncertainties about when such events might occur, and the severity and impact on water infrastructure. These threats are compounded by pressures such as population growth and constrained funding. There is a need, therefore, to consider the suitability of current governance arrangements and decision processes for the management of complex, long-term risk associated with state assets. This research, therefore, asks, are current local governance arrangements and associated decision-making processes for the management of water infrastructure appropriate for long-term risk management in an environment of uncertainty? The thesis addresses this question by critically evaluating the extent to which governance arrangements enable effective risk management decisions that protect government owned infrastructure from natural hazards. 
A novel conceptual framework which matches the type of problem within the scales of time and complexity is developed on the basis of a literature review on risk management, adaptability and resilience, and modes of governance across multiple levels. This framework is then used to inform an exploration of risk management decision processes. The examination of these processes involved a participatory design involving local and central government officials and associated risk management professionals. A mixed-methods approach was applied to explore experiences of risk management as it relates to water infrastructure within local government, within the context of wider relationships with central government. Following a pilot workshop with sector actors and risk professionals to establish the broad research focus, the research involved 36 semi-structured interviews and a further workshop with sector officials to discuss the findings and critique the resulting decision-making model.
The research found that current risk management decision making is characterised by, largely, a short-term horizon, this being connected with the tendency of elected representatives to operate within the three-year election cycle. The absence of a longer-term focus is explained in part by the autonomous environment within which councils operate, removed from central government oversight, and with little sharing of knowledge, expertise or costs of risk management. The key findings of the research indicate: a lack of consistent guidance and direction by central government to local government on adaptive risk management; a lack of coordination across the network of councils, specifically in terms of sharing knowledge, data and risk management activities; a lack of specificity in legislation regarding long term risk management which leaves risk decision making open to interpretation by councils; a culture of localised short term decision making in councils, with a low level of comprehension as to the management of long-term risks; short-term competing priorities for the financial resources required to manage risk; and a mistrust of internal guidance and advice due to the lack of in-house expertise, and a preference for external subject matter expertise. 
Overall, this research has identified that the complex nature of the environment of uncertainty posed by climate change has not been adequately acknowledged within local government and this is reflected in the prevailing risk management arrangements being largely reliant on annually renewed insurance arrangements based on in-house planning practices. The decision-making framework developed in this thesis acknowledges the nature of risk and the need for adaptive approaches within the constraints of time and complexity, and has potential to inform both governance arrangements and decision processes.

year: 2021

title: Tribal guns, tribal gunners : A study of acculturation by Maori of European military technology during the New Zealand inter-tribal musket wars

authors: Bentley, Trevor William

abstract: The Musket Wars were New Zealand’s longest and most costly war. Some 20,000 Maori perished in the raids, battles and sieges that convulsed the tribal societies during the 1820’s and 1830’s. The rise of powerful musket armies set in train forty major hekes or tribal migrations that emptied vast reaches of country by 1840, paving the way for European settlement that proceeded largely unopposed until the 1860’s.

There is little known about the nature of Maori warfare in this period for the Musket Wars have yet to receive the same kind of intense study currently being given by New Zealand historians to the Northern and New Zealand Wars. While other nations have conducted exhaustive research into the military history of their indigenous people, the Musket Wars remain New Zealand’s least known war.

The paucity of information on inter-tribal; warfare in this period is sourced in the fact that native warfare was actively discouraged by the missionaries, judged barbaric by most early visitors and regarded as an inferior form of warfare by settlers and English military men. Later nineteenth century writers perpetuated a distorted view of the Musket Wars as chaotic inter-tribal conflicts in which Maori fought and destroyed Maori. This distorted view has its origins in the two widely divergent systems of values that operated in nineteenth century New Zealand and in the dominance of Victorian ideas about race and colonialism.

This thesis is revisionist only in its attempt to demolish the entrenched colonial view of Maori musket warfare as disordered and exclusively Maori. It endeavours to fill specific gaps that exist in the research on this period by examining the role and purpose of the European gun trading enterprise in New Zealand and the extent of European involvement in Maori warfare. The thesis explores the deployment and effectiveness of European guns during the three phases of Maori warfare that characterise the period; predatory raiding, the great rakau-musket battles and the musket to musket conflicts of the 1830’s. The thesis also develops the theme that while the Musket Wars were shaped by non-Maori influences they were distinctly Maori in style.
While the bulk of evidence is provided by contemporary European observers and nineteenth century historians, every attempt has been made to provide a more balanced picture of Maori warfare by reporting Maori experiences wherever possible. The thesis consequently draws on current Maori oral testimony, the evidence of contemporary Maori eyewitness and the substantial and well developed body of written tribal history currently available.

The Musket Wars represent a fascinating but largely forgotten military age in New Zealand where Maori armies of unprecedented size employed powerful armaments of flintlock guns and ships cannon on long distance campaigns against remote enemies. This period has provided a unique opportunity to research the ways an indigenous warrior society acculturated European firearms technology in established tribal rituals and in warfare, independent of European military influences and in a purely Maori context. Most importantly the Musket Wars have provided the opportunity to examine Maori tactical developments through the critical stage between classical Maori warfare and the clashes with British and Colonial forces during the Northern war of 1845 and New Zealand wars of the 1860’s.

text: Tribal guns, tribal gunners : A study of acculturation by Maori of European military technology during the New Zealand inter-tribal musket wars The Musket Wars were New Zealand’s longest and most costly war. Some 20,000 Maori perished in the raids, battles and sieges that convulsed the tribal societies during the 1820’s and 1830’s. The rise of powerful musket armies set in train forty major hekes or tribal migrations that emptied vast reaches of country by 1840, paving the way for European settlement that proceeded largely unopposed until the 1860’s.

There is little known about the nature of Maori warfare in this period for the Musket Wars have yet to receive the same kind of intense study currently being given by New Zealand historians to the Northern and New Zealand Wars. While other nations have conducted exhaustive research into the military history of their indigenous people, the Musket Wars remain New Zealand’s least known war.

The paucity of information on inter-tribal; warfare in this period is sourced in the fact that native warfare was actively discouraged by the missionaries, judged barbaric by most early visitors and regarded as an inferior form of warfare by settlers and English military men. Later nineteenth century writers perpetuated a distorted view of the Musket Wars as chaotic inter-tribal conflicts in which Maori fought and destroyed Maori. This distorted view has its origins in the two widely divergent systems of values that operated in nineteenth century New Zealand and in the dominance of Victorian ideas about race and colonialism.

This thesis is revisionist only in its attempt to demolish the entrenched colonial view of Maori musket warfare as disordered and exclusively Maori. It endeavours to fill specific gaps that exist in the research on this period by examining the role and purpose of the European gun trading enterprise in New Zealand and the extent of European involvement in Maori warfare. The thesis explores the deployment and effectiveness of European guns during the three phases of Maori warfare that characterise the period; predatory raiding, the great rakau-musket battles and the musket to musket conflicts of the 1830’s. The thesis also develops the theme that while the Musket Wars were shaped by non-Maori influences they were distinctly Maori in style.
While the bulk of evidence is provided by contemporary European observers and nineteenth century historians, every attempt has been made to provide a more balanced picture of Maori warfare by reporting Maori experiences wherever possible. The thesis consequently draws on current Maori oral testimony, the evidence of contemporary Maori eyewitness and the substantial and well developed body of written tribal history currently available.

The Musket Wars represent a fascinating but largely forgotten military age in New Zealand where Maori armies of unprecedented size employed powerful armaments of flintlock guns and ships cannon on long distance campaigns against remote enemies. This period has provided a unique opportunity to research the ways an indigenous warrior society acculturated European firearms technology in established tribal rituals and in warfare, independent of European military influences and in a purely Maori context. Most importantly the Musket Wars have provided the opportunity to examine Maori tactical developments through the critical stage between classical Maori warfare and the clashes with British and Colonial forces during the Northern war of 1845 and New Zealand wars of the 1860’s.

year: 1997