Resp Care
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Since the detection of the underlying gene defect, our knowledge of how the genetic mutations in cystic fibrosis cause lung disease has increased substantially, but we still lack a complete understanding of some of the pieces in the puzzle. Nevertheless, the information gained has led to new therapeutic approaches that address key factors of cystic fibrosis pathophysiology. ⋯ The efficacy of these treatments still needs to be established, but early studies look promising for several compounds. This review summarizes our current understanding of the pathophysiology and treatment of cystic fibrosis lung disease.
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Bacteria infect the respiratory tract early in the course of cystic fibrosis disease, often fail to be eradicated, and together with an aggressive host inflammatory response, are thought to be key players in the irreversible airway damage from which most patients ultimately die. Although incompletely understood, certain aspects of the cystic fibrosis airway itself appear to favor the development of chronic modes of survival, in particular biofilm formation; this and the development of antibiotic resistance following exposure to multiple antibiotic courses lead to chronic, persistent infection. ⋯ Future work should aim to develop clinically applicable methods to identify these and to determine which have the potential to impact pulmonary health. We outline the basic tenets of infection control and treatment.
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The role of the respiratory therapist in the care of patients with cystic fibrosis has expanded throughout the years. As key members of the multidisciplinary team, respiratory therapists actively participate in the medical management of patients with cystic fibrosis along the continuum of care, from acute in-patient stays to the out-patient clinic and/or home setting. Through their involvement in diagnostic testing, administering therapy, or direct bedside care, patient and caregiver education, and disease management, respiratory therapists strive to preserve lung function, maintain overall health, and improve the patient's quality of life.
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Measurements of disease severity provide a guide for the physician to tailor therapies, for the patient and family to gauge progress, and are required for clinical trials. For many respiratory diseases, including cystic fibrosis, sensitive, noninvasive measurements are few, and some of those that are available are applicable only to certain subgroups of patients or lack sufficient sensitivity. We discuss currently available measurements in 4 groups: physiology, infection, inflammation, and radiology. For each group we highlight strengths and weaknesses, ask how we could improve upon these, and provide details of alternative methods.
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Earlier diagnosis, treatment of exacerbations, and the use of long-term therapies have all improved the lifespan of patients with cystic fibrosis (CF). However, the natural history of CF airways disease remains one of worsening bronchiectasis and obstructive airways impairment. The progression of airways disease leads to eventual respiratory failure, but some will suffer other acute respiratory complications that require intervention, including pneumothorax, massive hemoptysis, and respiratory failure. ⋯ The respiratory therapist also plays a key role in the care of the patient with respiratory failure. Here is also discussed the role of ventilatory support and airway-clearance therapies in the patient with advanced stage disease. Now, more than ever, the patient needs caregivers with the knowledge and sensitivity to provide appropriate palliative care.