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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
Q

What does CFTR membrane protein play a role in?

A
  • endocytosis (and destruction)

- plays a role in conducting chloride and thiocyanate ions

26
Q

What occurs to pseudomonas once it’s colonised? (4)

A
  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
Q

What is pseudomonas aeruginosa colonisation associated with? (2)

A
  • reduced life expectancy (28 vs 39 years)

- rapid decline in lung function

28
Q

What should be done as soon as patient is identified with CF?

A

first isolation

29
Q

What should be done on first isolation of a CF patient? (what 2 medications should be used)

A
  • attempt eradication with oral ciprofloxacin and nebulised colomycin 3/12
30
Q

What 2 medications should be administered if oral ciprofloxacin and nebulised colomycin don’t work?

A
  • IV ceftazidime

- nebulised colomycin

31
Q

Where is Burkholderia cepacia acquired from? (3)

A
  1. environment

2. other CF patients (epidermic strains are virulent) 3. segregation policies

32
Q

What is colonisation of Burkholderia cepacia associated with? (3)

A
  • reduced life expectancy 16 vs 39 years
  • rapid decline in lung function
  • some patients have “cepacia syndrome” with very rapid deterioration
33
Q

What is the main disadvantage of Burkholderia cepacia which makes it more difficult to treat?

A

its innate resistance to most antibiotics

34
Q

What is a contraindication for treating Burkholderia cepacia (Genomovar III)? i.e. it would do patient more harm

A

transplantation

35
Q

What are the 4 most common pathogens that CF patients are at risk of?

A
  1. pseudomonas aeruginosa
  2. burkholderia cepacia
  3. stenetrophomonas maltiphilia
  4. mycobacterium abscessus
36
Q

After which group of pathogens does stenotrophomonas maltiphilia usually occur in?

A

after pseudomonas (but can also occur as a first gram negative infection)

37
Q

What is the main disadvantage of stenotrophomonas maltiphilia?

A

Multiple antibiotic resistance

38
Q

What is mycobacterium abscessus resistant to specifically?

A

all anti-tuberculous chemotherapy

39
Q

What is the contraindication for treating stenotrophomonas maltiphilia i.e. it would do patient more harm?

A

transplantation (infection on the increase in isolated patients)

40
Q

What is the rule for treating respirator infections in CF patients?

A

Treat early and very aggressively with antibiotics

41
Q

What 3 pathogen groups are oral antibiotics used to treat?

A
  1. staph
  2. haemophilus
  3. pneumococcus
42
Q

What 3 pathogen groups are IV antibiotics used to treat?

A
  1. pseudomonas
  2. strenotrophomonas
  3. burkholderia
43
Q

What 2 antibiotic families are used to treat TB?

A
  1. Beta lactams

2. aminoglycoside

44
Q

What do you do if pathogens multiply and become resistant to the 2 classes of antibiotics?

A
  • test for synergy between antibiotic combination
45
Q

What are large doses of the two types of antibiotics linked with? (2)

A
  • increased volume of distribution

- increased clearance

46
Q

What is the duration of antibiotic courses for treating respiratory infections in CF patients?

A

2 weeks

47
Q

What is special about Ivacaftor, the new CF drug?

A

first of new class of drug addressing the primary defect in CF

48
Q

Describe the action of Ivacaftor on CFTR and its effect.

A
  • CFTR potentiator and binds to CFTR

- improves transport of Cl ions

49
Q

What percentage of patients can be considered for Ivacaftor therapy?

A

5-10% patients ( only those with Celtic mutations; G551D mutation)

50
Q

What are the main advantages of Ivacaftor? (4)

A
  1. improves lung function 10% predicted
  2. weight gain
  3. reduced sweat chloride
  4. patient feels much better (normal)
51
Q

What is the main disadvantage to Ivacaftor?

A

VERY expensive; £180, 000 per year

52
Q

What are 4 indications for a double lung transplant in CF patients?

A
  1. rapidly deteriorating lung function
  2. FEV1< 30% predicted
  3. life threatening exacerbations
  4. estimated survival< 2 years
53
Q

What percentage of CF patients die on the waiting list for a double lung transplant?

A

30-40%

54
Q

What is interesting about survival rates following a double lung transplant in CF patients?

A
  • 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
Q

Why is there a decline in survival rate for double lung transplantation in CF patients?

A

due to bronchiolitis obliterans developing

56
Q

What is the main aim of lung transplantation in CF patients?

A
  • measure to prolong survival

- measure to improve quality of life

Systems: Resp. WK (40 decks)

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