Cystic Fibrosis-Nursing III/Exam 1

CF Causes…

thick, sticky mucus to build up in the lungs, which leads to life-threatening lung infections and ultimately respiratory failure

Describe the pathophysiology of cystic fibrosis

-It affects the cells that produce mucus, sweat, and digestive juices

-it causes the fluids to become thick and sticky with mucus

-mucus plugs up tubes, ducts, passageways of various organs in the body

The result of the CF mutation is…

decreased secretion of chloride and increased reabsorption of sodium into the cellular space

What organs are most commonly affected by CF?

Sinuses. Lungs, pancreas, biliary and hepatic systems, intestines, sweat glands

List the pulmonary clinical manifestations of CF

-wheezing

-hyperinflation of lung fields

-decreased pulmonary function test results

-airway bacterial colonization

-nasal polyps

List the non-pulmonary manifestations of CF

-GI problems (meconium plugs; black tarry stool)

-belly distended, won’t eat, puking green substance

-male and female infertility

-clubbing of the extremities

What are the commonn symptoms associated with CF?

wheezing and SOB

chronic cough

frequent lung infections

runny nose and congestion

salty skin

poor growth and weight gain

stomach pain and bloating

intestinal blockages

irregular/absent menstruation

frequent yeast infections

How do newborns present with CF/

20% are born with meconium ileus

-thick meconium plugs form due to the production of excessively thick stool

-present with abdominal distention, poor feeding, bilious emesis, and pain

When should CF be detected?

-delayed growth/FTT

-no bowel movements in first 24-48 hours of life

-repeated episodes of pneumonia when older

Trikafta

targets gene mutations

can cause liver failure

antibiotics

treat and prevent lung infections

anti-inflammatories

reduce airway swelling

hypertonic saline

mucus thinning drug

oral pancreatic enzyme

improves nutrient absorption in the digestive tract; must be taken every time you eat because natural enzyme is lacking

acid reducers

help pancreatic enzymes work better

What is the order of treatment when treating CF in a child?

Epinephrine, bronchodilators, hypertonic saline, suction, oxygen

Surgical treatments to treat cystic fibrosis

sinus surgery to remove polyps and treat recurrent sinusitis

-gastric tube to increase calories and nutrition

-intestinal surgery to relieve obstruction

-lung transplant: CF does not occur in new lungs

-liver transplant because of CF related cirrhosis