Current opinion in pulmonary medicine
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This review will examine what is now known about palliative and end-of-life care for cystic fibrosis (CF) patients, including the changing demographics and context of such care, and then outline a brief research agenda to guide further work in this area. ⋯ The time has come for national, multicenter studies of palliative and end-of-life care practices in CF, followed by systematic evaluation of the efficacy of existing interventions. Studies of the symptoms and treatment burdens in CF should continue with an emphasis on interventions to improve health-related quality of life throughout the lifespan of children and adults with CF.
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Understanding the molecular and cellular processes responsible for the development of lung disease in cystic fibrosis (CF) has led to evaluation of a broad range of new therapies within multiple therapeutic classes. For these reasons, clinical research in CF is accelerating, as new agents progress through the early stages of drug development, move into clinical trials and are offered to study patients. This review focuses on the most notable clinical trials of pulmonary therapies reported in the last year. ⋯ The pace of drug development in CF will require an expanding pool of patients willing to participate in clinical research to test new agents. Although these potential therapies will likely improve quality of life for people with CF and contribute to improved survival, it will be important to avoid adding excessively to the already high burden of treatment. If the demands on patients' time continue to grow, a decrease in adherence to effective therapies may paradoxically lead to worse outcomes and negate the benefits new treatments bring.
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This review will focus on recent research documenting baseline adherence to infection control recommendations and barriers to their implementation as experienced by multidisciplinary cystic fibrosis (CF) care providers. In addition, controversies regarding optimal infection control will be discussed. Finally, suggestions to improve infection control in CF will be proposed. ⋯ These data suggest that access to national infection control guidelines and written local policies are critically important to improving infection control for CF.
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Oral appliances are an alternative to continuous positive airway pressure (CPAP) for the treatment of obstructive sleep apnea (OSA). Although CPAP is a highly efficacious treatment, there is a need for other treatment options because the clinical effectiveness of CPAP is often limited by poor patient acceptance and tolerance, and suboptimal compliance. ⋯ There is robust evidence of the efficacy of oral appliances for improving polysomnographic indices and modifying the health risk associated with OSA. The current evidence suggests a similar impact on health outcomes as CPAP. However, further research is required to address a number of unresolved issues, including the influence of device design, titration procedures, prediction of treatment outcome and the clinical effectiveness of oral appliances for modifying the adverse health consequences of OSA.
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This review aims to highlight and place in context recent advances in and insights into the natural history, diagnosis, and management of idiopathic pulmonary fibrosis (IPF). ⋯ Although IPF remains a diagnostic and therapeutic challenge to even the most experienced of clinicians, our knowledge of the natural history of the disease, diagnostic accuracy, and therapeutic approach continue to advance.