Titlebook: Cystic Fibrosis in Primary Care; An Essential Guide t Douglas Lewis, MD, FAAFP Book 2020 Springer Nature Switzerland AG 2020 cystic fibrosi

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Exercise for CF,ugh many studies. Not everyone uses the term exercise. Physical activity has been used in multiple studies as a synonymous term for exercise. For our chapter, we will be using the terms exercise and physical activity interchangeably. Exercise has been demonstrated to improve quality of life, disease

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Inflammation in Cystic Fibrosis,sregulation of inflammatory mediators. The inflammatory component of cystic fibrosis begins early in life and is sustained and intense. This inflammation leads to airway damage in the form of bronchiectasis which is the main cause of morbidity and mortality for the cystic fibrosis patient. There are

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CF Airway Clearance,lized recombinant human DNase (RhDNase, dornase alfa) is the most widely used mucolytic agent. It decreases sputum viscosity and should be considered in all CF patients ages 6 and older to help reduce pulmonary exacerbations (PEs) and improve lung function. Hypertonic saline, which serves to promote

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Acute and Chronic Infection Management in CF,asis. All of these manifestations of cystic fibrosis contribute to a rich milieu for lower respiratory pathogens in patients affected by the disease. CF patients are, therefore, highly susceptible to chronic colonization with many pathogens such as . and .. They are also uniquely prone to acute infe

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Sexual and Reproductive Health and Counseling,n that patients desire to learn about the physiologic, genetic, and psychosocial aspects of SRH [1]. This chapter addresses pubertal development, family planning, and other SRH concerns, along with infertility, genetic screening, and pregnancy management.

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Gastrointestinal Disease and Nutrition in Cystic Fibrosis,f chloride. The mutation in this gene creates tenacious secretions in the lung, pancreas, intestinal tract, and hepatobiliary systems. The focus in this chapter will be pancreatic insufficiency, intestinal diseases, hepatobiliary disease, and nutritional factors (Sabharwal S, Schwarzenberg SJ. Cysti

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Electrolyte Abnormalities in CF,ride. Appropriately evaluating patients is imperative to treatment and prevention of long-term sequelae. Once hyponatremia is diagnosed, volume status is assessed to determine etiology. Patients with acute hyponatremia and severe neurologic findings are admitted to the hospital and have rapid sodium