Congenital Disorders Flashcards
What is cystic fibrosis and what is its etiology?
An autosomal recessive disease that causes NaCl ion transport problems across cell membranes due to faulty CFTR protein. This makes for thick and viscous secretions
Where in the body would we find the impact of CF?
liver, pancreas, intestines, reproduction and respiratory systems
How does CF play a role in pulmonary dysfunction?
viscous mucous predisposes pt to chronic bacterial airway infections> obstructive lung disease>progressive loss of pulm fn
How does CF play a role in digestive dysfunction?
viscous mucous blocks ducts. Digestive enzymes can’t reach food in the small intestine and insulin/glucagon can’t get into the blood. > pancreatic dysfn. + malabsorption of vitamins + CF-related diabetes+ low body weight
CF can lead to exocrine pancreatic insufficiency which in turn leads to…
poor absorption, chronic constipation, distal intestinal obstruction syndrome
How does CF play a role in bone disease?
due to a decrease in insulin, there is a decrease in bone mineral density. So with CF, there are higher risks of osteopenia and osteoporosis.
How does CF play a role in reproductive dysfunction?
Female: delays puberty, egg fertilization is difficult
Male: bilat absence of vas deferens, obstructive azoospermia
How can we manage lung complications that accompany CF?
chest hygiene/postural drainage
drug: bronchodilators, mucolytics, decongestants, antibiotics
How can we manage digestive complications that accompany CF?
PT: chest hygiene
high fluid intake
Enzyme/Viatmin supplement
Diet! : as much as they like as long as it is rich in protein, calories, fat salt veggies and fruit
What are the effects of pulmonary edema on gas exchange?
IS fills with fluid>alveolar space fills>decreased surface available for gas exchange
what are the primary causes of pulmonary edema?
fluid overload
decreased serum albumin
lymphatic obstructions
increased pulmonary permeability (injury or immune response)
describe the progression of pulmonary edema.
- persistent cough w/ slight dyspnea, hypoxia, exercise intolerance
- more acute dyspnea, wheezing, hyperventilation
- productive and frothy bloody cough (medical emergency)
what is the treatment goal of pulmonary edema? Mortality rate?
40% , treatment is aimed @ improving oxygenation, maintaining MAP and reducing excess fluids
