1. LAUREN BURNETT NUR3215 EBP CYSTIC FIBROSIS

2. OBJECTIVES By completion of this presentation, learners should be able to: Describe the effects Cystic Fibrosis has on the body Generate a nursing plan for patients with Cystic Fibrosis Differentiate between different manifestations of cystic fibrosis Compare and contrast between different treatment options for Cystic Fibrosis

3. WHAT IS IT? A genetic disorder of the exocrine glands that results from defective sodium and chloride transport Primarily affects respiratory system and GI tract

4. EPIDEMIOLOGY 30,000 people in the US 70,000 people worldwide 100 new cases a year Median age of diagnosis – 6 months Median age of survival – mid 30’s Primarily affects Caucasians

5. ETIOLOGY Mutation in the Cystic Fibrosis Transmembrane Conductive Regulator (CFTR) gene CFTR gene is responsible for production of a protein that regulates how salt and water are carried across membranes

6. PATHOPHYSIOLOGY Alteration in chloride, salt, and water transport Goblet cells increase in size and number Mucus becomes excessively thick and sticky Mucus clogs the airways (and other organ systems)

7. PATHOPHYSIOLOGY Bacteria grow inside mucus WBC responds to infection, further aggravating airway Frequent, persistent infections occur, damaging the lungs Respiratory failure and possible death

8. MANIFESTATIONS Respiratory manifestations are most common Persistent cough (maybe with purulent, green sputum) Multiple respiratory infections Possible wheezing Bronchiectasis Hemoptysis Tachypnea Shortness of breath Loss of appetite/weight loss

9. SYSTEMIC MANIFESTATIONS Gastrointestinal Cramps, pain, pancreatic abnormalities Endocrine Glucose intolerance, polydipsia, polyphasia, polyuria Reproductive Male sterility, pregnancy complications, delayed sexual development

10. DIAGNOSIS Newborn testing – heel stick Based on clinical symptoms Verified by diagnostic tests Sweat Chloride test CXR Pancreatic enzymes DNA analysis to detect CFTR gene mutation

11. HYPERINFLATION WITH BRONCHIECTASIS

12. MANAGEMENT Overall goal – Control/prevent infection Maintain clear airways Improve nutrition Psychosocial support Improve quality of life There is NO cure to treat all patients at this time Early detection and diagnosis is crucial to delay progression

13. NURSING MANAGEMENT Assessment – History, symptoms, activity, nutrition intake, medication use Diagnosis – Ineffective airway clearance Ineffective breathing pattern Risk for infection Imbalanced nutrition Goals of Care – Maintain adequate airway Reducing risk factors for infection Patient Education

14. MANAGEMENT Prophylaxis against infection Early antibiotic therapy when symptoms arise Chest physiotherapy Surgery Removal of nasal polyps Lung lobectomy Lung transplant Bronchodilators Tobramycin Azithromycin DNase

15. IS THERE A CURE? Ivacaftor (Kalydeco) A CFTR potentiator FDA approved for treatment of CF Only for 1 mutation type (there are over 1,500) Reduces viscosity of mucus Improves respiratory function and reduces risk of exacerbations Significant reduction in sweat chloride test 10 adverse effects on label

16. DRY POWDER TOBRAMYCIN Aminoglycoside antibiotic used for years as a nebulizer Very effective against Pseudomonas aeruginosa Now available as a dry powder to be inhaled (TIP) Before TIP (inhaled version), nebulizers were only option due to high dosage required Higher incidence of cough Faster administration time, more convenient and portable Greater patient satisfaction than nebulizer treatments

17. SO WHAT? Patient Education TB screening Immunizations Advocate for the patient

18. NCLEX QUESTION A patient comes into the ER complaining of a chronic cough. Upon assessment, the nurse learns this patient has a history of CF and is also experiencing shortness of breath. Which manifestation of CF should the nurse suspect? A.Respiratory infection B.Hemoptysis C.Bronchiectasis D.Emerging nasal polyps

19. NCLEX QUESTION A patient comes into the ER complaining of a chronic cough. Upon assessment, the nurse learns this patient has a history of CF and is also experiencing shortness of breath. Which manifestation of CF should the nurse suspect? A.Respiratory infection B.Hemoptysis C.Bronchiectasis D.Emerging nasal polyps

20. NCLEX QUESTION The nurse is teaching a mother of a child with CF about dietary improvements to make. Which type of diet would be an appropriate suggestion? A.High-calorie, High-protein diet B.Low-calorie, High-protein diet C.High-carb, High-fiber diet D.Low-protein, Low-fiber diet

21. NCLEX QUESTION The nurse is teaching a mother of a child with CF about dietary improvements to make. Which type of diet would be an appropriate suggestion? A.High-calorie, High-protein diet B.Low-calorie, High-protein diet C.High-carb, High-fiber diet D.Low-protein, Low-fiber diet

22. REFERENCES Brashers, V. L. (2012). Alterations in Pulmonary Function. In S. E. Huether, & K. L. McCance, Understanding Pathophysiology (pp. 685). St. Louis, Missouri: Elsevier. Brashers, V. L., & Gott, K. K. (2012). Alterations in Pulmonary Function in Children. In S. E. Huether, & K. L. McCance, Understanding Pathophysiology (pp. 718-720). St. Louis, Missouri: Elsevier. Cashion, A., Graff, C., Graves, R., & Wraa, C. (2014). Genetics. In R. Holleran, K. S. Osborn, A. B. Watson, & C. E. Wraa, Medical-Surgical Nursing: Preparation for Practice (pp. 142). Upper Saddle River, New Jersey: Pearson. Chiu, H., Song, j. C., & Yoon, J. (2012). Ivacaftor: A New Emerging Treatment Option in the Management of Cystic Fibrosis. Formulary, 47, 132-134, 139-140. Retrieved from http://eds.a.ebscohost.com/eds/pdfviewer/pdfviewer?sid=d06260d9-e989-4f9d-8070- ac489c1bfb9f%40sessionmgr4003&vid=1&hid=4203 http://eds.a.ebscohost.com/eds/pdfviewer/pdfviewer?sid=d06260d9-e989-4f9d-8070- ac489c1bfb9f%40sessionmgr4003&vid=1&hid=4203 McKeage, K. (2013). Tobramycin Inhalation Powder: A Review of Its Use in the Treatment of Chronic Pseudomonas aeruginosa Infection in Patients with Cystic Fibrosis. Drugs, 73, 1815-1827. doi: 10.1007/s40265-013-0141-0 Rhoades, C., & Wraa, C. (2014). Caring for the Patient with Lower Airway Disorders. In R. Holleran, K. S. Osborn, A. B. Watson, & C. E. Wraa, Medical-Surgical Nursing: Preparation for Practice (pp. 768-769). Upper Saddle River, New Jersey: Pearson.