1. Cystic Fibrosis Julianne Tamoney

2. Objectives Brief discussion of pathophysiology Understand medical and nursing interventions Discussion of clinical relevance Understand interventions and care guidelines applied to specific patient

3. Pathophysiology Multisystem genetic disease of the exocrine glands Leads to an increase in thickness secretions that alter organ function In the lungs, thick secretions obstruct the bronchioles and predispose the lungs to chronic infections

4. Symptoms Skin has a characteristic taste of salt Experiences repeated upper respiratory infections Does not gain weight Clubbing of the nails Barrel-chested May not achieve developmental milestones Number one genetic killer of children and young adults in the United States CF patients only live to be about 35

5. Intervention before lung transplant Patient’s with severe lung failure are put on a lung transplant waiting list ECMO cannula is implanted through patient’s neck and sedation must be turned off Patient, still attached to the ECMO, walks around the hospital everyday while increasing their strength Walking with ECMO

6. Outcomes Improves strength and functional status Decrease length of stay in the hospital Lower hospital costs

7. Patient Scenario Female age 16 Height is 5’5 weighing 93 lb BMI is 16 Sweat test showed increased chloride in sweat Diagnosed with end stage cystic fibrosis Patient complains of cold that is worsening Developed influenza causing the clogged lungs to shut down Wheezing/ shortness of breath

8. Current Medications Albuterol DNase Ibuprofen Cephalexin Pancrelipase Multivitamin

9. Interventions Patient is placed on ECMO due to severe lung failure Patient walks around the hospital three to four hours a day with the help of nurses, physical therapists, and respiratory therapists

10. Patient’s Outcome Patient was strong enough for lung transplant Lung transplant was successful Patient is currently enjoying their new lungs

11. Nursing Diagnosis Ineffective airway clearance related to excess mucus

12. NCLEX Questions When teaching the parents of an older infant with CF about the type of diet the child should consume, which of the following would be most appropriate? A) low protein diet B) high fat diet C) low carbohydrate diet D) high calorie diet

14. NCLEX Questions A 9-year-old child is admitted to the pediatric unit for treatment of cystic fibrosis. The nurse is assessing the child’s respiratory status. Which of the following findings is consistent with cystic fibrosis? A) Production of thick, sticky mucous B) Nonproductive, harsh cough C) Stridor D) Unilateral decrease in breath sounds

15. A) Production of thick, sticky mucous

16. Reference Albin, Amy. (July 2012). Lung transplant recipient defies all odds. http://newsroom.ucla.edu/portal/ucla/lung-transplant- recipient defies-236093.aspx DukeMed. (Apr. 2012). ECMO -- Walking? http:// www.dukehealth.org/health_library/health_articles/ecmo- walking PubMed. (Dec 2011). Implementing early mobilization interventions in mechanically ventilated patients in the ICU. http:// www.ncbi.nlm.nih.gov/pubmed/22147819 Turner, David A. (Dec 2011) Active rehabilitation and physical therapy during extracorporeal membrane oxygenation while awaiting lung transplantation: A practical approach. http:// journals.lww.com/ccmjournal/Abstract/2011/12000/ Active_rehabilitation_and_physical_therapy_during.1.aspx Unbound Medicine. (2000-2012). Nursing Central. http:// nursing.unboundmedicine.com/nursingcentral/ub