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PHTY3020 - WK 9 CF & paed resp and physio management

Terms in this set (90)

what is cystic fibrosis? (pathophysiology)
CF is an autosomal recessive disorder that mainly affects the lungs but also the pancreas, liver, kidney and intestines. It is caused by the presence of mutations in both copies of the gene for the protein CFTR. This results in exocrine gland dysfunction, with chloride unable to move from the cell and therefore sodium water remains in the cell. This results in thick dehydrated secretions which contributes to a number of abnormalities.
what does CFTR stand for?
cystic fibrosis transmembrane conductance regulator
What chromosome in CF is affected?
long arm of chromosome 7 which codes for epithelial protein (CFTR)
what are some common screening techniques for CF?
guthre test - heel prick
sweat tests - Cl >60Meq/L = gold standard
genetic testing
faecal fat
what systems does CF affect?
resp
GIT
liver
endocrine
reproductive
what are signs/symptons/ indications of infection in CF patients?
increase cough
moist cough
more productive
yellow-green sputum
SOB
wheezing
lethargy
loss of apetite
decrease ex tolerance
what are signs/symptons/ indications of disease progression in CF patients?
respiratory distress
clubbing
FRC increases
chest deformity
reduction of function
respiratory failure
briefly describe the epidemiology of CF?
northern european descent
1:2500 birthsq
1:25 carry CF gene
36 infants year in NSW
how is Staphylococcus Aureus spread? how many children and adults with CF have experienced it??
skin to skin, touching infected surfaces or droplet infection
50% children > 6 years
35% in adults
how is Haemophilus Influenzae spread?how many children and adults with CF have experienced it??
person to person in air or contact with similar surfaces
25% 2-5 years
5% adults
what percentage of adults and children have experienced Pseudomonas Aeruginosa? which version is more resistant to Abs and chronic?
under 5 years 25%
adults 80%
mucoid version
what percentage of adults and children have experienced Burkholderia Cepacia? how is it spread and where is it found?
2% children % adults
soil, plants, water. person to person
what are some infection control strategies for CF patients?
segregation
isolation
universal precuations e.g. hand washing
cleaning of respiratory equipment
what is Ivacaftor (kalydeco)? what are its effects?
it is a CFTR potentiator
improves FEV1, weight gain, sweat chloride decreases towards normal range
what are some examples of inhalation therapy for CF? name5
bronchodilators (e.g. salbutamol)
preventors e.g. seretide, flixotide
hypertonic saline
bronchitol (mannitol - similar to HS)
pulmozyme (improve PFT's)
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