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Systems: Resp. WK > 29. Cystic Fibrosis: Adults > Flashcards

29. Cystic Fibrosis: Adults Flashcards

1
Q

What is cystic fibrosis?

A
  • one of the commonest autosomal recessive conditions affecting Caucasians
  • caused by mutations on chromosome 7 (on CF transmembrane conductance regulator;CFTR)
  • Defects in Cl channel; defects in Cl secretion and increased Na absorption across airway epithelium
  • induces changes in composition of airway surface liquid which predisposes lungs to chronic pulmonary infections and bronchiectasis
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2
Q

Which systems are affected by CF?

A

digestive and respiratory (digestive and lungs which become clogged up with thick mucus which can lead to organ damage and eventual failure)

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3
Q

What percentage of CF patients have recurrent pulmonary infections and pancreatic insufficiencies?

A

80%

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4
Q

What percentage of CF patients have recurrent pulmonary infections?

A

15%

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5
Q

What percentage of CF patients have gastrointestinal problems only?

A

5%

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6
Q

What are the most common health problems faced by CF patients? (7)

A
  1. (recurrent) pulmonary infections
  2. pancreatic insufficiencies
  3. gastrointestinal problems
  4. diabetes
  5. liver disease
  6. osteoporosis
  7. fertility issues
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7
Q

What is the main symptom of CF?

A

recurrent and persistent respiratory infections

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8
Q

What does CFTR stand for?

A

CF transmembrane conductance regulator (membrane proteins and chloride regulator)

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9
Q

What does the CFTR abnormality cause which leads to bacterial colonisation? (in terms of micromolecules) (3)

A
  1. decreased mucociliary clearance
  2. increased bacterial adherence
  3. decreased endocytosis of bacteria
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10
Q

What does bacterial colonisation lead to in a CFTR abnormality which results in bronchiectasis? (4)

A
  1. inflammation
  2. mucus plugging
  3. airway ulceration
  4. airway damage
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11
Q

What does CF eventually result in? (4)

A
  1. Agressive progressive and severe bronchiectasis
  2. recurrent LRT infections
  3. progressive airflow obstruction
  4. respiratory failure
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12
Q

What does aggressive progressive and severe bronchiectasis cause?

A

chronic sputum production (purulent; with pus)

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13
Q

What are features of progressive airflow obstruction?

A
  • increasing exertional dyspnoea

- survival related to FEV1

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14
Q

What is prescribed for progressive airflow obstruction?

A

bronchodilators (inhaled or nebulised)

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15
Q

What is type 1 respiratory failure?

A

Decrease in PaO2 (PaCO2 can be normal)

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16
Q

What is type 2 respiratory failure?

A

Decrease in PaO2 AND increase in PaCO2

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17
Q

What are treatment options in a respiratory failure patient? (4)

A
  • nocturnal NIV; non invasive ventilation
  • ambulatory oxygen (supplemental)
  • symptomatic relief
  • bridge for transplantation
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18
Q

Is haemoptysis common in CF? What can it be associated with?

A

Yes; associated with infection

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19
Q

What is required if large haemoptysis occurs in CF patient?

A

may need embolisation (blocking of abnormal blood vessels)

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20
Q

In what group of people is a pneumothorax more common?

A

commoner in older males (20%)

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21
Q

What is the general prognosis for a pneumothorax?

A

Generally poor and frequently requires surgical intervention

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22
Q

What is the main pathogen involved in CF?

A

Pseudomonas aerugirosa

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23
Q

What does colonisation of pseudomonas aerigirosa increase with?

A
  • age (80% colonised over 26 years and 21% colonised <1 year)
  • possibly role of repeated antibiotics
24
Q

How is pseudomonas aerigirosa acquired? (3)

A
  1. environment (particularly hospitals)
  2. other CF patients (epidermic strains, antibiotic resistant, virulent)
  3. segregation and disinfection policies
25
What does CFTR membrane protein play a role in?
- endocytosis (and destruction) | - plays a role in conducting chloride and thiocyanate ions
26
What occurs to pseudomonas once it's colonised? (4)
1. pseudomonas undergo mucoid change 2. pseudomonas forms a biofilm, microcolonies in an alginate film 3. they are protected from host defences 4. rapidly acquires multiple antibiotic resistance
27
What is pseudomonas aeruginosa colonisation associated with? (2)
- reduced life expectancy (28 vs 39 years) | - rapid decline in lung function
28
What should be done as soon as patient is identified with CF?
first isolation
29
What should be done on first isolation of a CF patient? (what 2 medications should be used)
- attempt eradication with oral ciprofloxacin and nebulised colomycin 3/12
30
What 2 medications should be administered if oral ciprofloxacin and nebulised colomycin don't work?
- IV ceftazidime | - nebulised colomycin
31
Where is Burkholderia cepacia acquired from? (3)
1. environment | 2. other CF patients (epidermic strains are virulent) 3. segregation policies
32
What is colonisation of Burkholderia cepacia associated with? (3)
- reduced life expectancy 16 vs 39 years - rapid decline in lung function - some patients have "cepacia syndrome" with very rapid deterioration
33
What is the main disadvantage of Burkholderia cepacia which makes it more difficult to treat?
its innate resistance to most antibiotics
34
What is a contraindication for treating Burkholderia cepacia (Genomovar III)? i.e. it would do patient more harm
transplantation
35
What are the 4 most common pathogens that CF patients are at risk of?
1. pseudomonas aeruginosa 2. burkholderia cepacia 3. stenetrophomonas maltiphilia 4. mycobacterium abscessus
36
After which group of pathogens does stenotrophomonas maltiphilia usually occur in?
after pseudomonas (but can also occur as a first gram negative infection)
37
What is the main disadvantage of stenotrophomonas maltiphilia?
Multiple antibiotic resistance
38
What is mycobacterium abscessus resistant to specifically?
all anti-tuberculous chemotherapy
39
What is the contraindication for treating stenotrophomonas maltiphilia i.e. it would do patient more harm?
transplantation (infection on the increase in isolated patients)
40
What is the rule for treating respirator infections in CF patients?
Treat early and very aggressively with antibiotics
41
What 3 pathogen groups are oral antibiotics used to treat?
1. staph 2. haemophilus 3. pneumococcus
42
What 3 pathogen groups are IV antibiotics used to treat?
1. pseudomonas 2. strenotrophomonas 3. burkholderia
43
What 2 antibiotic families are used to treat TB?
1. Beta lactams | 2. aminoglycoside
44
What do you do if pathogens multiply and become resistant to the 2 classes of antibiotics?
- test for synergy between antibiotic combination
45
What are large doses of the two types of antibiotics linked with? (2)
- increased volume of distribution | - increased clearance
46
What is the duration of antibiotic courses for treating respiratory infections in CF patients?
2 weeks
47
What is special about Ivacaftor, the new CF drug?
first of new class of drug addressing the primary defect in CF
48
Describe the action of Ivacaftor on CFTR and its effect.
- CFTR potentiator and binds to CFTR | - improves transport of Cl ions
49
What percentage of patients can be considered for Ivacaftor therapy?
5-10% patients ( only those with Celtic mutations; G551D mutation)
50
What are the main advantages of Ivacaftor? (4)
1. improves lung function 10% predicted 2. weight gain 3. reduced sweat chloride 4. patient feels much better (normal)
51
What is the main disadvantage to Ivacaftor?
VERY expensive; £180, 000 per year
52
What are 4 indications for a double lung transplant in CF patients?
1. rapidly deteriorating lung function 2. FEV1< 30% predicted 3. life threatening exacerbations 4. estimated survival< 2 years
53
What percentage of CF patients die on the waiting list for a double lung transplant?
30-40%
54
What is interesting about survival rates following a double lung transplant in CF patients?
- Gradual attrition/ reduction in strength and effectiveness - 70-80% of survival rate at 5 years post transplantation - 50% survival rate at 10 years post transplantation
55
Why is there a decline in survival rate for double lung transplantation in CF patients?
due to bronchiolitis obliterans developing
56
What is the main aim of lung transplantation in CF patients?
- measure to prolong survival | - measure to improve quality of life

Systems: Resp. WK (40 decks)

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