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 •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

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

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

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

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

Current Medications •Albuterol•DNase •Ibuprofen •Cephalexin •Pancrelipase•Multivitamin

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

Patient’s Outcome•Patient was strong enough for lung

transplant• Lung transplant was successful•Patient is currently enjoying their new

lungs

Nursing Diagnosis •Ineffective airway clearance related to

excess mucus

NCLEX 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 diet

D) high calorie diet

NCLEX QuestionsA 9-year-old child is admitted to the pediatric

unitfor treatment of cystic fibrosis. The nurse isassessing the child’s respiratory status. Which ofthe following findings is consistent with cysticfibrosis? A) Production of thick, sticky mucousB) Nonproductive, harsh coughC) StridorD) Unilateral decrease in breath sounds

A) Production of thick, sticky mucous

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