Resp Care
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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.
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Chronic airways infection and inflammation is the greatest source of morbidity and mortality in cystic fibrosis (CF) patients. Many organisms can be found in the lower respiratory tract of CF patients, but infection with mucoid Pseudomonas aeruginosa is common, is associated with poorer outcomes, and is the main target for antimicrobial strategies in CF. Aerosol antibiotics achieve high local concentrations in the airways, reduce systemic toxicity, and have been used successfully for chronic suppressive treatment for established P. aeruginosa infections. ⋯ Liposome formulations are being studied as a way to penetrate mucoid biofilms and prolong the residence time of the antibiotic in the lungs. Light, porous, dry-powder formulations are also in clinical trials to reduce delivery time. These new formulations and delivery systems promise to expand our armamentarium against microbes while reducing the time burden for patients.
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Review
Infection control in cystic fibrosis: cohorting, cross-contamination, and the respiratory therapist.
Cystic fibrosis (CF) is a complex genetic disease characterized by lung infections that lead to early morbidity and death. Pathogens that commonly infect the lungs of patients with CF include Staphylococcus aureus, Haemophilus influenzae, Pseudomonas aeruginosa, and Burkholderia cepacia. Aggressively treating pulmonary infection with antibiotics has contributed to improved survival in patients with CF but has also promoted multiple-drug-resistant bacteria. ⋯ Increasing evidence of patient-to-patient transmission of CF pathogens led the Cystic Fibrosis Foundation to produce evidence-based infection-control recommendations, which stress 4 principles: standard precautions, transmission-based precautions, hand hygiene, and care of respiratory equipment. Respiratory therapists need to know and follow these infection-control recommendations. Cohorting patients infected with B. cepacia complex is one of several interventions successful at keeping the spread of this pathogen low, but cohorting patients who are infected/colonized with other microbes is controversial, the main argument of which is not being certain of a patient's present respiratory culture status at any given patient visit.
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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.