Paediatrics (RCH) - Theses

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    Clowns in the midst: understanding clown doctors at The Royal Children's Hospital Melbourne
    Brockenshire, Naomi Anne ( 2018)
    Clown doctors are a feature in paediatric hospitals, visiting children and families, providing a welcome escape from the reality of hospitalisation. Though the use of humour to improve health and wellbeing has been widely researched, limited exploration of the clown doctors has occurred. This study aims to elucidate the work of clown doctors within a major paediatric hospital. This was an ethnographic study. Ethnography is an innovative approach to paediatric research, giving an intricate view that is otherwise difficult to attain. Participants for this study included the clown doctors employed at The Royal Children’s Hospital, and every person they had a meaningful encounter with during the course of their work, including patients, families, clinical and non-clinical staff. Data was collected via participant observation, with approximately 1,500 hours of ‘clown ward rounds’ documented over one year. Furthermore, 25 hour-long semi-structured interviews were conducted with a range of key informants. A constructivist framework was used to analyse emergent concepts. Constructivism explores how relationships and interactions create the individual’s understanding of the world. Furthermore, how different understanding, or meaning, can be derived from interactions based on individual context, background, culture and personal history. When asking people about the clown doctors, most ascribed a function, such as: distraction, anxiety reduction and procedural assistance; entertainment and making people laugh; emotional support and providing comfort; and communication, including translating clinical information to families. These functional elements of the clown doctors are the result of a more complex, intimate human connection that develops due to the nature of clown doctors being low-status, open, vulnerable and, in particular, existing as outsiders to the medical establishment. Clown doctors use humour to break down the emotional barriers created by illness, which they achieve through being person-centric and offsetting medically driven interactions the hospital often demands. They empower patients, returning a sense of control that is generally absent for hospitalised children. While almost universally acknowledged as a positive addition to the hospital, most people who encounter the clown doctors have little conception about the scope of their work. Although clown doctors are often described in concrete clinical terms, their real power lies in their ability to connect with people, and the psychosocial advantages that connection provides. The results of this descriptive study deliver valuable insight and a comprehensive understanding of clown doctors and the complexity of human relationships within a major paediatric hospital. Through this research we can identify what the clown doctors bring to the hospital environment, how paediatric staff can employ their unique skills more effectively, and finally give long-overdue credence to the notion that laughter, mirth, creativity and child-like wonder has as much place in a hospital as medicine.
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    Proximal femoral osteotomy in children and adolescents with cerebral palsy
    Zhou, Leena ( 2018)
    Background Cerebral Palsy (CP) is the most common cause of physical disability affecting children in developed countries. Approximately one third of children with CP may develop hip displacement. Non-ambulant children at Gross Motor Function Classification System (GMFCS) levels IV and V are at highest risk. Without early detection through surveillance programs, hip displacement can progress to hip dislocation, which is frequently painful and negatively impacts health-related quality of life (HRQoL). Injections of Botulinum Neurotoxin A (BoNT-A) have no role, and soft tissue surgery has a limited role in preventing hip displacement in non-ambulant children with CP. Bony hip reconstruction surgery such as a proximal femoral osteotomy (PFO) is effective in stabilising the hip and HRQoL. PFOs include Femoral Derotation Osteotomies (FDO) which aim to improve the gait of an ambulant child (GMFCS I-III), and Varus Derotation Osteotomies (VDRO) which aim to contain the hips in non-ambulant children (GMFCS IV-V). However, PFOs can carry high risks, especially in children with medical co-morbidities such as respiratory disease, nutritional deficiencies, hypertonia and osteopenia. Aim This thesis involved a series of three studies, which aimed to expand our knowledge of the trainee learning curve, outcomes and adverse events relating to PFO in children and adolescents with CP. Method and Results A new implant combining locking and cannulated technology (Locking Cannulated Blade Plate, LCBP) was recently developed for use in PFO. A pilot study was performed on the first 25 patients who had surgery with the LCBP, at the Royal Children’s Hospital (RCH), Melbourne. This study established safety for use in children as young as three, with weights as low as eleven kilograms. A further prospective, parallel cohort study of 90 consecutive children with CP was conducted to compare the LCBP against with existing non-cannulated, non-locking implant (Angled Blade Plate, ABP). Technical and radiological outcomes of surgery were similar between implants. However, the surgical technique was reported by trainees to be easier when using the LCBP, with less technical errors. Approximately 60 percent of the children experienced minor adverse events including: constipation, inadequate pain control, and respiratory compromise. However, a CP specific tool was not available to classify the severity of events. Study three was performed to clarify the Modified Clavien-Dindo (MCD) system for lower limb surgery in children with CP and test its’ reliability for classifying adverse events. Very good reliability was demonstrated amongst members within a multidisciplinary team. Conclusion Novel findings from these studies may help improve the safety and efficacy of the management of hip displacement in children with CP. Further research should address the long-term outcomes of PFO in children with CP, evaluate the validity of the MCD for children in CP and determine if the MCD can be embedded in the electronic medical records (EMR) as a routine tool for audit and clinical research.
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    Improving oxygen therapy for children and newborns in Nigerian hospitals
    Graham, Hamish Robert ( 2018)
    Oxygen is a long-established medical therapy that can be life-saving for severely ill children admitted to hospital. Effective provision of oxygen to patients requires a multifaceted system that involves technical, clinical, supply chain, financing, and other managerial and policy elements. However, this system is currently difficult to achieve in many low-resource settings resulting in poor access to, and use of, oxygen and subsequent excess mortality. Previous work has shown that improved hospital oxygen systems can improve patient access to oxygen therapy and reduce inpatient case fatality rates from childhood pneumonia. However, studies have shown variable impact in different settings due to contextual factors that are not well understood. In addition, the burden of hypoxaemia and impact of oxygen on conditions other than pneumonia is unclear – particularly in the large neonatal population. This study aimed to understand how to improve oxygen systems in particular contexts, and generate evidence to support the scale-up of effective oxygen systems in Nigeria and globally. My realist review of past oxygen projects developed a theoretical framework describing how improved oxygen systems could improve clinical outcomes in particular contexts – highlighting the interaction between efforts to improve oxygen access and the clinical use of oxygen. My oxygen needs assessment in 12 secondary-level Nigerian hospitals provided detailed data on existing oxygen systems – highlighting the impact of poor power supplies, weak maintenance systems, and lack of pulse oximetry. My prospective cohort study provided new data on the epidemiology of hypoxaemia in the Nigerian context. This study showed high prevalence of hypoxaemia among admitted children and neonates with a range of conditions, highlighted the strong association between hypoxaemia and death, and demonstrated poor accuracy of clinical signs to predict hypoxaemia (particularly for children with non-respiratory conditions). My mixed-methods realist evaluation identified how pulse oximetry could be most effectively adopted into routine paediatric and neonatal care – highlighting the role of key influencers to model behaviour, practical training and ongoing encouragement, personal experience of benefit, and the reasons why nurses valued pulse oximetry. My stepped wedge trial evaluated the effect of our intervention (improved oxygen system) on clinical outcomes and care practices. This study demonstrated mortality benefit for children admitted with pneumonia, and suggested that the introduction of pulse oximetry generated most of this benefit by stimulating better use of existing oxygen supplies. We found no mortality benefit for children with other conditions or neonates, and detected an unexpected trend towards higher mortality in the “full oxygen system” period compared to the “pulse oximetry period” for neonates. Post-hoc analysis tested some potential explanatory theories for these findings – highlighting the effects of pre-existing oxygen access and external factors. In conclusion, improving oxygen systems is complex. The studies contained in this thesis have helped fill evidence gaps that are hindering oxygen policy and planning decision-making in Nigeria and globally. They have directly informed national and global policies and program planning.
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    Hypoplastic left heart syndrome: development of a new animal model for hypothesis testing
    Hattam, Andrew Theodore ( 2018)
    INTRODUCTION: Hypoplastic left heart syndrome (HLHS) is a congenital heart defect that involves severe underdevelopment of the left-sided heart structures. Despite the severity of the cardiac deformity, HLHS is compatible with fetal life. In-utero survival of HLHS is facilitated by compensatory blood flow patterns facilitated by the presence of fetal shunts and the parallel arrangement of the fetal circulation. As the left ventricle (LV) is unable to perfuse the systemic circulation in HLHS, the fetal right ventricle (RV) is able to perfuse territories normally supplied by the LV during development via flow across the ductus arteriosus (DA) and aortic isthmus. However, after birth the DA closes and the RV is no longer able to compensate for the inadequate LV. As a result, newborns with HLHS develop circulatory collapse and die. At present, without intervention HLHS is universally fatal, with palliative surgery being the treatment option of choice offered by most institutions worldwide. Fundamental to the future study of HLHS and progress toward curative therapy is the creation of a clinically relevant mammalian animal model that facilitates hypothesis testing. Thus, the overall aim(s) of this thesis were to utilize an established fetal lamb model of the fetal cardiovascular system to develop a new stable, chronic, and clinically relevant fetal lamb model of congenital aortic stenosis (AoSt) (a condition known to evolve into HLHS in-utero); and quantify the growth-related changes in haemodynamics and cardiac morphology associated with the in-utero progression of the model using serial fetal echocardiography (Study 1). Additional studies using invasive cardiovascular instrumentation aimed to characterise the cardiovascular physiology of the fetal lamb model of congenital AoSt during fetal life (Study 2), the transition from fetus to newborn (Study 3), and closure of the DA (Study 4). METHODOLOGY: Surgery was performed in n=19 anaesthetised time-dated twin-pregnancy ewes (mean 91±3 days; term=147 days), with one fetus undergoing non-constrictive ascending aortic banding, and the second serving as the un-operated control. Serial fetal echocardiography studies were performed under maternal sedation at fortnightly intervals until near-term (140±1 days) in order to measure structural and functional changes in cardiac growth (Study 1). At near-term, sets of twin fetuses (n=12) were anaesthetised and instrumented with aortic (Ao), pulmonary arterial (PA) and left atrial (LA) fluid-filled pressure (P) catheters, and in a subgroup of fetuses (control; n=9, banded; n=4), an LV micromanometer catheter was placed. Haemodynamic and arterial blood gas measurements were made during fetal life (Study 2), as well as during the transition from fetus to newborn (Study 2), and after closure of the DA (Study 4). RESULTS: Echocardiography (Study 1) performed after non-occlusive banding of the ascending aorta of mid-gestation fetal lambs increased the peak ascending aortic (trans-stenosis) pressure gradient from 8±4mmHg at mid-gestation to 26±13 mmHg at near-term (p<0.001). Versus control animals, this pressure gradient was associated with increases in LV posterior wall (15%; p<0.05), and septal (14%; p<0.05), thickness, as well as a reduction in LV chamber short axis dimension during both systole (26%; p<0.001) and diastole (22%; p<0.001). These changes were also coupled with near-term increases in RVO (28%; p<0.005), HR (20%; p<0.001) and CVO (21%; p<0.001) compared to control animals. Invasive instrumentation (Study 2) demonstrated AoP, PAP and HR were similar between control and banded fetuses. However, LAP was paradoxically lower in banded fetuses versus controls (6.2±1.9 vs. 4.3±1.7 mmHg; p<0.05), while LV systolic pressure (56±9 vs. 75±7 mmHg; p<0.05) and LV dP/dtmax (1415±285 vs. 1930±504 mmHg; p<0.05) were higher in banded fetuses. Birth (Study 3) in banded fetuses was related to attenuation of the normal birth-related drop in PAP, as well as absence of the normal birth-related rise in AoP. Higher fetal LV systolic pressures were maintained after birth in banded animals, whereas LV dP/dtmax was reduced (p<0.05). Closure of the DA (Study 4) in banded animals was not associated with the normal rise in AoP seen in control animals. LAP decreased in banded animals after DA closure (32%; p<0.05) but remained unchanged in controls. Further, banded animals sustained hypoxic changes, manifest via reduced arterial blood pO2 (74.1±15.2 vs. 61.7±12.2 mmHg; p<0.05) after DA closure. CONCLUSION: Non-occlusive ascending aortic banding in mid-gestation fetal sheep is associated with a significant chronic rise in LV afterload, as well as substantial changes in LV growth patterns; manifest not only via alterations in LV wall thickness and cavity dimensions, but also in LV chamber geometry. Moreover, these changes are associated with increased right-heart blood flow and CVO, allowing fetuses to remain compatible with life in-utero. In addition, arterial blood pressures were maintained and the blood gas status largely unimpaired in fetal sheep after mid-gestation aortic banding. Although not associated with dramatic changes in haemodynamics and arterial blood gas status during fetal life, birth was associated with findings suggestive of changes in systemic, pulmonary and intra-cardiac blood flow patterns, as well as cardiac function; all of which have potential relevance to the clinical arena and early management of the newborn with HLHS. Finally, closure of the DA was associated with reduced systemic perfusion pressures, and hypoxia, findings akin to those seen in circulatory collapse of infants with HLHS. Therefore, given its favourable characteristics, this model is suitable for future hypothesis testing in the pathogenesis and pathophysiology of AoSt and HLHS.