Cystic Fibrosis - Adults Flashcards
What are some of the conditions associated with CF?
Recurrent pulmonary infections Pancreas insufficiency GI problems Diabetes Liver disease Osteoporosis Fertility issues
Why do those with CF get recurrent resp infections?
CFTR abnormality:
- Decreased mucociliary clearance
- Increased bacterial adherence
- Decreased endocytosis of bacteria
What does the bacterial colonisation caused by CF cause?
Inflammation
Mucus plugging
Airway ulceration
Airway damage
What can the effects of bacterial colonisation lead to?
Bronchiectasis
What sort of sputum is produced with bronchiectasis?
Purulent
What type of respiratory failure will a sufferer of CF go into first?
Type 1, then Type 2
What respiratory symptoms are experienced in CF?
Purulent sputum Reccurent LRTI Dyspnoea - progressive airflow obstruction Respiratory failure Haemoptysis Pneumothorax
What are some of the bacteria that commonly infect CF patients?
Pseudomonas aeruginosa Staph aureus H influenza Burkholeria cepacia Stenotrophomonas maltophilia
How does pseudomonas aeruginosa protect itself from host environment?
Forms a biofilm (microcolonies in an alginate film):
Protection from host defences
Acquires multiple antibiotic resistance
What do you treat the first isolation of pseudomonas aeruginosa with?
Oral ciprofloxacin and nebuliser colomycin
What is the second treatment for pseudomonas aeruginosa if first treatment fails?
IV ceftazidime and nebulised colomycin
What is the effect of pseudomonas aeruginosa on the prognosis of a CF patient?
Reduces life expectancy (28 vs 39yrs)
Rapid decline in lung function
What is the effect of Burkholderia Cepacia on the prognosis of a CF patient?
Reduces life expectancy (16 vs 39yrs)
Rapid decline in lung function
Maybe ‘cepacia syndrome’ - very rapid deterioration
Does mycobacterium abscessus affect the ability of the patient to have a lung transplant?
Yes, it is a contraindication
Which bacteria types are given oral antibiotics?
Staph
Haemophilus
Pneumococcus
