Resp Care
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Hospitalization can occur at any age for patients with cystic fibrosis (CF). The leading cause for admission is an acute worsening of signs and symptoms that can be called a pulmonary exacerbation. The reasons for admission are usually the need for intravenous antibiotics and aggressive airway clearance with good nutritional support. ⋯ An important aspect of the hospitalization is the interaction between the RT and the patient. To give good therapy is to be a great coach. From infection control to following proper nebulizer protocol, to consistency with airway clearance, to education, the CF RT is there for the life of the patient.
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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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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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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.