Flashcards in Cystic Fibrosis - Unit 2 Deck (62)
Cystic Fibrosis - defective gene? If so, where is it?
Inherited defective gene, located on chromosome 7.
Cystic fibrosis - leads out to defective chloride ion transport. T/F?
Both parents cannot be carriers. T/F?
FALSE - they ARE.
If both parents are carriers, there is a ____ % chance they will have an affected child, ___ % chance the child will be a carrier, and a ___% chance the child will not be a carrier or have the disease.
Is there universal screening for cystic fibrosis?
No - just DNA analysis.
Cystic fibrosis - dysfunction of the exocrine glands that are the ___ producing glands.
CF - more prevalent in blacks. T/F?
FALSE - usually whites. 95% are white!
__ in ___ caucasians are symptom free carriers.
1 in 29
CF mostly affects epithelia cells in the airway and pancreas. T/F?
CF - no abnormal transport of sodium and chloride. T/F?
What's the life expectancy? How many are 18 and older?
1966 - 7.5 years.
1996 - 31 years.
40% are 18 years and older
How has life expectancy increased?
improved therapies, CPT, gene therapy, aerosolized antibiotics, improved nutrition.
Increased viscosity of mucus secretions - - what does that do?
Obstructs small passageways of bronchioles and pancreas.
Increases sodium and chloride in saliva and sweat - T/F?
Sodium and chloride in sweat is 2-5 times greater than children without CF. T/F?
Kids with CF can taste salty when kissed. T/F?
Do CF kids have autonomic nervous system abnormalities?
What is the sweat chloride test?
Pilocarpine iontophoresis - 2 separate samples. Sweat production is stimulated with electric current and then that sweat is collected on filter paper.
Sweat test - easy on newborns. T/F?
FALSE - they don't really sweat!
What's the normal sweat chloride test?
No correlation between severity of disease and amount of electrolytes found in sweat. T/F?
Cilia in the lung have difficulty moving the thick mucus - T/F?
What happens then?
True - so they do not effectively cough and remove secretions.
Since they can't cough well and remove secretions, what happens? *hint, think of leaving shit in there....*
Infection - mucus serves as a medium for bacterial growth!
In addition to hypoxia, hypercarbia, and acidosis, what else might occur in the child with severe lung involvement secondary to CF?
Pulmonary Hypertension and Cor Pulmonale
What is pulmonary hypertension?
Increased pressure in pulmonary vessels
Cor Pulmonale - what is it?
Right ventricular enlargement and failure due to lung disease.
What are common organisms found in children with CF?
P. aeruginosa, Burkholderia cepacia, S. aureus, H. influenza, E. coli, K. pneumoniae
Which organisms cause the most challenges for children with CF?
Burkholderia cepacia (can rapidly lead to sepsis and rapid pulmonary function deterioration) and P. aeruginosa (pseudomonas, difficult for the macrophages to destroy and develops resistance to antibiotics)
What are some respiratory manifestations of CF?
Wheezing, coughing (chronic), a cough that can begin as dry and non productive, respiratory infections, increased dyspnea, hyperinflation and areas of atelectasis, barrel shaped chest, cyanosis, finger and toe clubbing