Living with cystic fibrosis : a phenomenological study of children, adolescents, young adults and their parents
Creators: name: Jessup, MM
Publisher: name: University Of Tasmania
Year: 2023

description: In response to queries from nurses in an acute paediatric setting, this thesis investigates the experience of children and their parents who live with a chronic, lifethreatening and life-limiting disease - cystic fibrosis (CF). Affected families must follow a relentless regime of daily treatment, aware that acute, potentially fatal, exacerbations can occur at any time. What unique challenges and issues for care does this disease present, because of its converse chronicity yet impending life-threatening status? Anecdotally, nursing colleagues have conveyed their lack of insight into the concerns confronting these children and their parents, while being required to deliver sensitive and informed care. Because nurses generally encounter such families in an acute phase, they have expressed a need to know about their daily experience at home, before the exacerbation of the disease. Current literature features research with a medical focus, but a paucity of information for those seeking to understand the personal experience of living with CF. Cited studies tend to be situated in large, metropolitan centres, particularly in North America and Britain. That research does not consider an Australian perspective, nor the unique issues that result from isolation and rurality that may be encountered by those who reside in a small island setting. A phenomenological perspective has been used to frame the study. Data has been drawn from unstructured, conversational style interviews. It includes personal narratives, poetry and drawings that have been contributed by children, adolescents and young adults aged from two to twenty-one years old, plus their parents - eight families in all. Van Manen's (1990) four existentials are used to consider a lifeworld in which notions of time, body, space and relationship are indelibly altered. Analysis of the participants' contributions has realised eight distinct sub-themes that permeate their experience. From original fright, through ongoing dynamics of fear, fight, flight, form, familiarity and philosophy, they pursue a future that is both threatened and continually redefined. These sub-themes interplay in the paradox and contradiction of a life correlated with being \all at sea.\" Of particular magnitude is the parents' struggle in the search for new and accurate bearings - of information support and services. Their new reference point is the external reality of confronting life and death on a daily basis which although not always conscious is nonetheless implicit in the execution of each day's rigorous routine. The co-presence of these two dynamics situates those with CF in a life and death binary that is the essence of living that life. Children reveal a growing awareness of and adaptation to this life and death dynamic. This is a gradual process in which some participants are still engaged and about which they speak in comparison with school peers. The young adults have negotiated adolescence attended by extraordinary issues such as death of friends and lung transplantation. They talk freely about their plans for the future that on the one hand they once imagined they would never attain but on the other is still tentative. In light of the experience conveyed by participants implications for nursing education and practice are discussed. Potential areas for further research that have been generated by this study are then considered. New insight gained from this research project will enable a fresh consideration of those living with CF as the uncovered truths and impressions provide insight into a lifeworld that may not be as the enquiring nurses had imagined. As a result of enhanced understanding care can be delivered from an empathetic bearing towards those for whom it is not so much a bothersome routine but a life and death imperative."