Cystic fibrosis Flashcards
what is the median survival for a cystic fibrosis(CF) patient
41 years
what organs are affected in CF
sweat glands, lungs, reproductive organs, pancreas/gall bladder/liver, intestines
what is the pneumonic to remeber the organs affected in CF
Some Lungs Retain Products = Infection
what is the best diagnostic indicator of CF
sweat test
what signs are seen on physical exam of CF patients
cyanosis, clubbing, low BMI, bilateral coarse crackles, nasal polyps
what respiratory clinical features are seen in CF
wet sounding cough, recurrent infection, nasal polyps, bronchiectasis
what reproductive sign is seen in CF
bilateral absence of the vas deferens
describe the new born screening for CF
dry heel prick test, not diagnostic, if +ve refer to CF centre to do sweat test and genetics
how are the frequent respiratory tract infections of CF managed
always 2 antibiotics(to reduce resistance),
for mild exacerbation = oral therapy 14 days
what kind of physiotherapy is used to help CF patients
autogenic draining, active cycle of breathing technique(ACBT)
what drugs other than antibiotics are used to manage CF
bronchodilators, mucolytics(Dnase), ivocaftor
what does the annual CXR CF patients receive check for
hyperinflation and bronchiectasis
