Cystic Fibrosis: Adults Flashcards
What percentage of patients have recurrent pulmonary infections and pancreatic insufficiency?
80%
Why is there persistent respiratory infections in CF?
Decrease in mucociliary clearance
Increase in bacterial adherence
Reduce in endocytosis of bacteria
What is the effect of CF on bronchiectasis?
Causes Aggressive progressive bronchiectasis: chronic sputum production (purulent
What is the result of decreased mucociliary clearance?
Increased bacterial colonisation
What is the result of increased bacterial colonisation?
Inflammation
Mucus plugging
Airway ulceration
Airway damage
What is the result of Inflammation
Airway plugging
Airway ulceration
Airway Damage?
Bronchiectasis?
What is the result of bronchiectasis?
More
Decreased mucociliary clearance
Increased bacterial adherence
Decreased endocytosis
Describe the onset of bronchiectasis in CF patients?
Aggressive and progressive - chronic sputum production
What respiratory problems besides bronchiectasis do CF patients face?
Recurrent lower respiratory tract infections
Progressive airflow obstruction (survival rate related to FEV1)
Increase in exertional dyspnoea
Haemoptysis (associated with infection - possible embolisation)
Pneumothorax- poor prognosis
Describe the onset of colonisation of pseudomonas aeruginosa
21% colonisation under 1 year
80% colonisation over 26 years
Where is pseudomonas aeruginosa acquired from?
Environment (particularly hospitals)
Other CF patients
How does pseudomonas aeruginosa protect itself from host defences?
Once colonised, it undergoes mucoid change. It forms a biofilm - rapidly acquires antibiotic resistance
What is colonisation of pseudomonas aeruginosa associated with?
Reduced life expectancy 28 vs 39 years
Rapid decline in lung function
How does the body respond to the presence of a biofilm?
Neutrophils and macrophages release proteinases and oxidants (free radicals) produce tissue damage
What is treatment for first isolation of pseudomonas Aeruginosa?
Attempt eradication with oral ciprofloxacin & nebulised colomycin 3/12
If fails, i.v. ceftazidime & nebulised colomycin
Where is Burkholderia cepacia acquired from?
Environment
Other CF patients
What is Colonisation of Burkholderia cepacia associated with?
Reduced life expectancy 16 vs 39 years
Rapid decline in lung function
Some patients ‘cepacia syndrome,’ very rapid deterioration
What is the resistance of burkholderia cepacia?
Innate resistance to most antibiotics
What does Genomovar 3 indicate (a group of Burkholderia cepacia-like organisms)?
Contraindication for transplantations
Which organism is usually secondary to pseudomonas?
Stentrophomonas maltophila
Which bacterium is resistant to all anti-tuberculosis chemotherapy and is a contraindication for transplantation.
Mycobacterium abscessus
How do you treat recurrent respiratory tract infections?
Early and aggressively with antibiotics
What bacteria would you treat with oral antibiotics?
Staph, Haemophilus, Pneumococcus
Which bacteria do you treat with IV antibiotics?
Pseudomonas, Stenotrophomonas, Burkholderia
What is the format of antibiotic administration?
Two antibiotics (b-lactam + aminoglycoside)
If multiply resistant, test for synergy between antibiotic combinations
Large doses (increased volume of distribution, increased clearance)
Two week courses
What is the function of the Ivacaftor?
Addresses primary defect of CTFR
CTFR potentiator, binds to CTFR and improves the transport of chloride ions
Improves lung function (10%)
Weight gain
Reduces sweat chloride
Feel much better
What are the indications for a double lung transplant?
Rapidly deteriorating lung function
FEV1 < 30% predicted
Life threatening exacerbations
Estimated survival <2 years
What is the likelihood of dying on the waiting list for a lung?
30-40% die on waiting list
What is survival rate for lung transplantation?
70-80% at 5 years
50% at 10 years
Gradual attrition due to bronchiolitis obliterans
