Adult Cystic Fibrosis Flashcards
What is the estimated life expectancy of a CF patient born in the 2000s?
> 40 years
What medical issues may CF patients face?
- 80% recurrent pulmonary infections and pancreatic insufficiency
- 15% recurrent pulmonary infections
- 5% gastrointestinal problems only
- (Diabetes, liver disease, osteoporosis, fertility issues)
Why do patients with CF experience persistent respiratory infections?
- CFTR abnormality
- Decreased mucociliary clearance
- Increased bacterial adherence
- Decreased endocytosis of bacteria
What does the effects of the CFTR abnormality lead to?
Bacterial colonisation which leads to:
- Inflammation
- Mucus plugging
- Airway ulceration
- Airway damage
- Bronchiectasis
What is responsible for chronic sputum production?
Aggressive progressive bronchiectasis
What respiratory problems do patients with CF face?
- Aggressive progressive bronchiectasis
- Recurrent lower respiratory tract infections
- Progressive obstructive airflow
- Respiratory failure
- Haemoptysis
- Pneumothorax
How is the progressive airflow obstruction managed?
Bronchodilators
How is respiratory failure managed?
- Oxygen, ambulatory oxygen
- Nocturnal NIV
- Symptomatic relief
- Bridge to transplantation
Describe haemoptysis in CF.
- Common, associated with infection
- If large may need embolization
- May be fatal
Describe pneumothorax in CF.
- Commoner in older males
- Associated with a poor prognosis
- Frequently requires surgical intervention
What pathogens are associated with CF?
- Pseudomonas aeruginosa
- Staphylococcus aureus
- Haemophilus influenza
- Burkholderia cepacia
- Stenotrophomonas maltophilia
What usually plays a role in endocytosis and destruction?
CFTR
What does colonisation increase with?
Age
Where is pseudomonas aeruginosa acquired from?
- Environment (particularly hospitals)
- Other CF patients
How does pseudomonas acquire multiple antibiotic resistance?
- Once colonised pseudomonas undergoes mucoid change, forms a biofilm, microcolonies in an alginate film
- Protected from host defences and able to rapidly acquire multiple antibiotic resistance
What is colonisation by pseudomonas associated with?
- Reduced life expectancy 28 vs 39 years
- Rapid decline in lung function
How is colonisation by pseudomonas managed?
- Attempt eradication with oral cirpofloxaicn and nebulised colomycin
- If fails, ceftazidime and nebulised colomycin
Where is burkholderia cepacia acquired from?
- Environment
- Other CF patients
What is colonisation by burkholderia associated with?
- Reduced life expectancy 16 vs 39 years
- Rapid decline in lung function
- Some patients with cepacia syndrome deteriorate very rapidly
How does burkolderia respond to antibiotics?
Has an innate resistance to most antibiotics
When does stenotrophomonas colonisation usually occur?
- Increasing frequent colonisation
- Usua;ly after pseudomonas but can occur as first Gram negative infection
How does stenotophomonas respond to antibiotics?
Multiple antibiotic resistance
What is mycobacterium abscessus resistant to?
All anti-tuberculous chemotherapy
What should be treated with oral antibiotics?
- Staph
- Haemophilus
- Pneumococcus
What should be treated with IV antibiotics?
- pseudomonas
- Stenotrophomonas
- Burholderia
How should 2 antibiotics be combined?
- B-lactam and aminoglycoside
- If multiple resistant then test for synergy between antibiotic combinations
- Large doses
- 2 week courses
What is ivacaftor?
First of a new class of drugs addressing the primary defect of CF
How does ivacaftor work?
- It is a CFTR potentiator
- Binds to CFTR which improves the transport of chloride ions
Who can be treated with ivacaftor?
-G551D patients only (Celtic mutation)
What does ivacaftor do?
- Improves lung function
- Weight gain
- Reduces sweat chloride
- Feel much better overall
What indications are there for double lung transplant?
- Rapidly deteriorating lung function
- FEV1< 30% predicted
- Life threatening exacerbations
- Estimated survival <2 years
