Cystic Fibrosis

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Cystic Fibrosis . Julianne Tamoney . Objectives. Brief discussion of pathophysiology Understand medical and nursing interventions Discussion of clinical relevance Understand interventions and care guidelines applied to specific patient . Pathophysiology. - PowerPoint PPT Presentation

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Cystic Fibrosis

Cystic Fibrosis Julianne Tamoney

Objectives Brief discussion of pathophysiologyUnderstand medical and nursing interventionsDiscussion of clinical relevance Understand interventions and care guidelines applied to specific patient 2Pathophysiology Multisystem genetic disease of the exocrine glandsLeads to an increase in thickness secretions that alter organ functionIn the lungs, thick secretions obstruct the bronchioles and predispose the lungs to chronic infections

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 354Intervention before lung transplant Patients with severe lung failure are put on a lung transplant waiting listECMO cannula is implanted through patients neck and sedation must be turned offPatient, still attached to the ECMO, walks around the hospital everyday while increasing their strengthWalking with ECMO 5OutcomesImproves strength and functional status Decrease length of stay in the hospitalLower hospital costs

Patient Scenario Female age 16Height is 55 weighing 93 lbBMI 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 downWheezing/ shortness of breath Current Medications AlbuterolDNase Ibuprofen Cephalexin PancrelipaseMultivitamin

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

Patients OutcomePatient was strong enough for lung transplant Lung transplant was successfulPatient is currently enjoying their new lungs

Nursing Diagnosis Ineffective airway clearance related to excess mucusNCLEX Questions When teaching the parents of an older infant withCF about the type of diet the child shouldconsume, which of the following would be mostappropriate?A) low protein dietB) high fat dietC) low carbohydrate dietD) high calorie dietD) high calorie diet

NCLEX QuestionsA 9-year-old child is admitted to the pediatric unitfor treatment of cystic fibrosis. The nurse isassessing the childs respiratory status. Which ofthe following findings is consistent with cysticfibrosis? A) Production of thick, sticky mucousB) Nonproductive, harsh coughC) StridorD) Unilateral decrease in breath soundsA) Production of thick, sticky mucous

Reference Albin, Amy. (July 2012). Lung transplant recipient defies all odds. defies-236093.aspxDukeMed. (Apr. 2012). ECMO -- Walking? (Dec 2011). Implementing early mobilization interventions in mechanically ventilated patients in the ICU., David A. (Dec 2011) Active rehabilitation and physical therapy during extracorporeal membrane oxygenation while awaiting lung transplantation: A practical approach. Medicine. (2000-2012). Nursing Central.