cystic fibrosis- adults Flashcards
what are some of the problems associated with CF?
recurrent pulmonary infections pancreas insufficiency gastrointestinal problems diabetes liver disease osteoporosis fertility issues
why do sufferers of CF suffer from persistent respiratory infections?
decreased mucocilliary clearance
increased bacterial adherence
decreased endocytosis of bacteria
what does the bacterial infection caused by CF cause?
inflammation
mucus plugging
airway ulceration
airway damage
what can CF ultimately lead to with recurrent respiratory infections?
bronchiectasis
what sort of sputum is produced with bronchiectasis?
purulent
what sort of respiratory failure will a sufferer of CF go into first?
type 1 (then into type 2)
why may haemoptosis be caused by CF?
it is associated with infection and bronchiactisis
what are some respiratory complications of CF?
bronchiectasis recurrent LRTIs progressive airflow obstruction respiratory failure haemoptysis pneumothorax
what are some of the bacteria that commonly infect CF patients?
pseudomonas aeruginosa staphylococcus aureus haemophilus influenzae stenotrophomonas maltophilia burkholderia cepacia mycobacterium abscessus
what does pseudomonas aeruginosa form on infection?
a biofilm
what is the purpose of a biofilm?
protects the bacteria from host defences and antibiotics
why is pseudomonas aeruginosa difficult to treat?
it produces a biofilm and rapidly acquires multiple antibiotic resistance
what do you treat the first isolation of pseudomonas aeruginosa with?
oral ciprofloxacin and nebulised colomycin
what is the second treatment for pseudomonas aeruginosa if first treatment fails?
ceftazidime and nebulised colomycin
what does a colonisation of pseudomonas aeruginosa do to the prognosis for a CF patient?
reduced life expectancy
rapid decline in lung function
what does a colonisation of burkholdena cepacia do to the prognosis of a CF patient?
reduces life expectancy
rapid decline in lung function
what is cepacia syndrome?
patient has a very large immune response to burkholdena cepacia infection causes very rapid deterioration
does mycobacterium abscessus affect the ability of the patient to have a lung transplant?
yes it is a complete contraindication
what are the two antibiotics given to CF patients for recurrent respiratory tract infections?
B-lactam and aminoglycoside (together)
what is the name of the new drug with the possibility to cure CF in some patients?
ivacaftor
what are the indications for lung transplantation in CF patients?
- Rapidly deteriorating lung function
- FEV1 < 30% predicted
- Life threatening exacerbations
- Estimated survival <2 years
