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01. Respiratory System > Adult Cystic Fibrosis > Flashcards

Adult Cystic Fibrosis Flashcards

1
Q

What is the estimated life expectancy of a CF patient born in the 2000s?

A

> 40 years

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2
Q

What medical issues may CF patients face?

A
  • 80% recurrent pulmonary infections and pancreatic insufficiency
  • 15% recurrent pulmonary infections
  • 5% gastrointestinal problems only
  • (Diabetes, liver disease, osteoporosis, fertility issues)
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3
Q

Why do patients with CF experience persistent respiratory infections?

A
  • CFTR abnormality
  • Decreased mucociliary clearance
  • Increased bacterial adherence
  • Decreased endocytosis of bacteria
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4
Q

What does the effects of the CFTR abnormality lead to?

A

Bacterial colonisation which leads to:

  • Inflammation
  • Mucus plugging
  • Airway ulceration
  • Airway damage
  • Bronchiectasis
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5
Q

What is responsible for chronic sputum production?

A

Aggressive progressive bronchiectasis

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6
Q

What respiratory problems do patients with CF face?

A
  • Aggressive progressive bronchiectasis
  • Recurrent lower respiratory tract infections
  • Progressive obstructive airflow
  • Respiratory failure
  • Haemoptysis
  • Pneumothorax
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7
Q

How is the progressive airflow obstruction managed?

A

Bronchodilators

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8
Q

How is respiratory failure managed?

A
  • Oxygen, ambulatory oxygen
  • Nocturnal NIV
  • Symptomatic relief
  • Bridge to transplantation
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9
Q

Describe haemoptysis in CF.

A
  • Common, associated with infection
  • If large may need embolization
  • May be fatal
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10
Q

Describe pneumothorax in CF.

A
  • Commoner in older males
  • Associated with a poor prognosis
  • Frequently requires surgical intervention
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11
Q

What pathogens are associated with CF?

A
  • Pseudomonas aeruginosa
  • Staphylococcus aureus
  • Haemophilus influenza
  • Burkholderia cepacia
  • Stenotrophomonas maltophilia
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12
Q

What usually plays a role in endocytosis and destruction?

A

CFTR

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13
Q

What does colonisation increase with?

A

Age

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14
Q

Where is pseudomonas aeruginosa acquired from?

A
  • Environment (particularly hospitals)

- Other CF patients

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15
Q

How does pseudomonas acquire multiple antibiotic resistance?

A
  • 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
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16
Q

What is colonisation by pseudomonas associated with?

A
  • Reduced life expectancy 28 vs 39 years

- Rapid decline in lung function

17
Q

How is colonisation by pseudomonas managed?

A
  • Attempt eradication with oral cirpofloxaicn and nebulised colomycin
  • If fails, ceftazidime and nebulised colomycin
18
Q

Where is burkholderia cepacia acquired from?

A
  • Environment

- Other CF patients

19
Q

What is colonisation by burkholderia associated with?

A
  • Reduced life expectancy 16 vs 39 years
  • Rapid decline in lung function
  • Some patients with cepacia syndrome deteriorate very rapidly
20
Q

How does burkolderia respond to antibiotics?

A

Has an innate resistance to most antibiotics

21
Q

When does stenotrophomonas colonisation usually occur?

A
  • Increasing frequent colonisation

- Usua;ly after pseudomonas but can occur as first Gram negative infection

22
Q

How does stenotophomonas respond to antibiotics?

A

Multiple antibiotic resistance

23
Q

What is mycobacterium abscessus resistant to?

A

All anti-tuberculous chemotherapy

24
Q

What should be treated with oral antibiotics?

A
  • Staph
  • Haemophilus
  • Pneumococcus
25
Q

What should be treated with IV antibiotics?

A
  • pseudomonas
  • Stenotrophomonas
  • Burholderia
26
Q

How should 2 antibiotics be combined?

A
  • B-lactam and aminoglycoside
  • If multiple resistant then test for synergy between antibiotic combinations
  • Large doses
  • 2 week courses
27
Q

What is ivacaftor?

A

First of a new class of drugs addressing the primary defect of CF

28
Q

How does ivacaftor work?

A
  • It is a CFTR potentiator

- Binds to CFTR which improves the transport of chloride ions

29
Q

Who can be treated with ivacaftor?

A

-G551D patients only (Celtic mutation)

30
Q

What does ivacaftor do?

A
  • Improves lung function
  • Weight gain
  • Reduces sweat chloride
  • Feel much better overall
31
Q

What indications are there for double lung transplant?

A
  • Rapidly deteriorating lung function
  • FEV1< 30% predicted
  • Life threatening exacerbations
  • Estimated survival <2 years

01. Respiratory System (82 decks)

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