Cystic Fibrosis Flashcards
(Pathophysiology) Exocrine glands secrete excessive, thick,
tenacious mucus in lungs and pancreas
Sticky mucus ______ ducts
obstructs
In the lungs the air trapped resulting in
permanent damage
Provides and excellent medium for_____ growth
bacterial
Eventual respiratory failure or cor pulmonale
R CHF
Blocking of_______ duct results in
pancreas, lack of degestive enzymes and hterefore malabsorption of food and malnutrition
May also affect liver, _____ glands, ______ glands, small intestine, ________ system
salivary, sweat, reproductive
(ET) Genetic – on _____ chromosome, autosomal ______
7th, recessive
-May be diagnosed pre-natal
Clinical Findings
Meconium Salty Malabsorption Chronic Hypoxia Exercise intolerance Children not meet normal growth milestones
Meconium
ilium at birth
Salty
taste on skin
Malabsorption
stool changes (no weight gain) unable to absorb food
Chronic
cough/respiratory infections
Hypoxia
chest overinflated (air trapped)
Exercise
intolerance/fatigue
children not meet normal
growth milestones
Diagnostic tests
- Sweat test
- Stools
- X-rays
Medical treatment
- Team
- Enzyme replacement (pancrease)
- Well balanced diet, low fat
- Intense physical therapy including postural drainage, percussion and instruction in coughing
- Bronchi dilators/humidifiers/moderate exercise
- Hydrating fluids (decrease mucus thickness)
- O2
- Heart/lung transplants
