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LEARNING OBJECTIVES

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LEARNING OBJECTIVES

Upon completion of the chapter, the reader will be able to:

  1. Explain the pathophysiology of cystic fibrosis (CF) and its multiorgan system involvement.

  2. Describe the common clinical presentation and diagnosis of CF.

  3. Consider long-term treatment goals with respect to clinical course and prognosis of CF.

  4. Identify nonpharmacologic therapies for CF management.

  5. Recommend appropriate pharmacologic therapies for chronic CF management.

  6. Design appropriate antibiotic regimens for acute pulmonary exacerbations of CF.

  7. Use pharmacokinetic principles when calculating drug doses in CF patients.

  8. Formulate monitoring plans for acute and chronic CF pharmacotherapy.

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KEY CONCEPTS

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  • Image not available. In cystic fibrosis (CF), the CF transmembrane regulator (CFTR) chloride channel is dysfunctional and usually results in decreased chloride secretion and increased sodium absorption, leading to altered viscosity of fluid excreted by the exocrine glands and mucosal obstruction.

  • Image not available. Pulmonary disease is characterized by thick mucus secretions, impaired mucus clearance, chronic airway infection and colonization, obstruction, and an exaggerated neutrophil-dominated inflammatory response.

  • Image not available. Maximizing nutritional status through pancreatic enzyme replacement and vitamin and nutritional supplements is necessary for normal growth and development and for maintaining long-term lung function.

  • Image not available. Airway clearance therapy is a necessary routine for all CF patients to clear secretions and control infection.

  • Image not available. Antibiotic therapy is indicated in three distinct situations over the course of CF: (a) early eradication and delay of colonization, (b) suppression of bacterial growth once colonization occurs, and (c) reduction of bacterial load in acute overgrowth.

  • Image not available. Antibiotic selection is based on periodic culture and sensitivity data, typically covering all organisms identified during the preceding year. If no culture data are available, empirical antibiotics should cover the most likely organisms for the patient's age group.

  • Image not available. Antibiotic regimens in severe CF exacerbations usually include an IV antipseudomonal β-lactam plus an aminoglycoside.

  • Image not available. CF patients have larger volumes of distribution for many antibiotics due to an increased ratio of lean body mass to total body mass and lower fat stores. CF patients also have an enhanced total body clearance, although the exact mechanism has not been determined.

  • Image not available. Titration of pancreatic enzyme doses is based on control of steatorrhea, stool output, and abdominal symptoms.

  • Image not available. Because CF-related diabetes results from insulin insufficiency, exogenous insulin replacement is usually required.

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INTRODUCTION

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Cystic fibrosis (CF) is an inherited multiorgan system disorder affecting children and, increasingly, adults. It is the most common life-shortening genetic disease among whites and the major cause of severe chronic lung disease and pancreatic insufficiency in children. Disease generally manifests as mucosal obstruction of exocrine glands caused by defective ion transport within epithelial cells. Due to the array of affected organ systems and complicated medical therapies, appropriate CF treatment necessitates multidisciplinary team collaboration.

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EPIDEMIOLOGY AND ETIOLOGY

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In the United States, CF most commonly occurs in whites, ranging from 1 in 1,900 to 3,700 individuals. CF is less common in Hispanics (1 in 9,000), African Americans (1 in 15,000), and ...

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