cycstic fibrosis Flashcards
what is the prevalence of cystic fibrosis
1 : 2,500
how does CF effect patients fertility
men - 95% infertile
females are fertile - though poor nutritional sate may hinder that
what are the options for the infertile male CF patients
intra-cytoplasmic sperm injection (30% success rate)
adoption
sperm donor
what are the contraindications for CF lung transplant
other organ failure
malignancy within 5 years
significant peripheral vascular disease
addiction
what is the problem with lung transplant
there is a goldilocks zone
too good
or
too bad
when are CF patients bad enough
FEV1 <30% predicted
sever weight loss
hypoxia
how has CF median survival got better
CF centres
multi-disciplinary teams
physio
nutrition/ enzymes
antibiotics
what is the main cause of death in CF patients
respiratory failure
what is the pathology of CF
a mutation with the CFTR gene (cystic fibrosis transmembrane conductance regulator)
what does a mutation with the CFTR
problems with cilia
causing decreased mucociliary clearance and endocytosis of bacteria
increase of bacterial adherence
what are the presenting features of CF for neonatal
screening
what are the presenting features of CF in infants and young people
Recurrent chest infection
failure to thrive
what are the presenting features of CF in older children and adults
recurrent chest infections
male infertility
bronchiectasis
hyperinflation
clubbing
what is the neonatal screening for CF
new-born bloodspot day 5
how do you make a CF diagnosis
clinical assessment
sweat test
repeat CF mutation analysis
what is CF in relation to the rest of the body
it is a multi system disease
what is one system that is often damaged by CF
pancreatic insufficiency
85 - 90%
abnormal stools
how is pancreatic insufficiency from CF treated
enteric coated enzyme pellets
high energy diet
fat soluble vitamin and mineral supplements
what are the cardinal features of CF
recurrent bronchopulmonary infections
bronchiectasis
fibrosis
scarring
what is the vicious cycle of CF
mucus obstruction
leads to chronic infection
leading to increased inflammation
leading to fibrosis, scarring, bronchiectasis
what treatments of mucus obstruction
airway clearance physiotherapy, hypertonic saline
what is used to treat the chronic infections
antibiotics
what is used to treat the increase inflammation
azithromycin
ibuprofen
what are the common infective organism in CF
staph aureus
H. influenze
what are the cornerstones for treatments
proactive treatment
good nutrition
active lifestyle
where is the potential for a cure
CFTR modulator treatment
gene therapy/ editing
what do images of CT show with patients that have CF
tramlines
signet rings (circles, with calcifications)
consolidation
how does type two diabetes tie in with CF
poor lung function
leading to inactive
leading to High BMI
what is the treatment of pulmonary infections in CF
treat early and aggressively
what other treatments for generally quality of life in CF patients
bronchodilators
airway clearance
mucolytics
what are some other disease that CF patients often get
type 2 diabetes
osteoporosis
pneumothorax
haemostasis
what is one of the most common if not the most common infection in CF patients
pseudomonas aeruginosa
what is the pseudomonas aeruginosa - how is it treated
acquired form environment or CF patients
reduced life expectance
rapid lung decline
attempt eradication
