CF & non-CF bronchiectasis

Question 1

Describe briefly the pathology of CF bronchiectasis.

Answer 1

Inherited mutation to CFTR gene.

Progressive, genetic disease that involves the destruction of airway cells that widen and scar the airway.

Question 2

Briefly describe the pathophysiology of CF bronchiectasis.

Answer 2

1. The CTFR dysfunction causes loss of inhibition sodium channels –> excessive sodium and water reabsorption (dehydration).
2. Loss of chloride removal prevents correction of low surface water volume -> reduction of lubricating layer between mucus and epithelium.
3. Cilia is compressed and less effective, impairing MCC and cough effectiveness.
4. Decreased MCC allows for bacterial colonisation of airways creating an inflammatory response –> airway destruction.

Question 3

What symptoms might the patient describe if they had CF bronchiectasis? And why?

Answer 3

Fatigue and exercise intolerance - SOB

Persistent cough that produces thick mucus - impaired MCC and reduced cough effectiveness

Chest pain - secondary symptom from persistent cough

Question 4

What signs might the patient with CF bronchiectasis have? Why?

Answer 4

• abnormal POB
• mucus plugging and thickening of bronchial walls on CXR
• auscultation: reduced breath sounds, wheeze, coarse crackles

Question 5

What might you hear if you auscultated a patient with CF bronchiectasis? Why?

Answer 5

course crackles:
- air moving past excessive secretion

wheeze:
- narrowed airways - increased airway resistance

reduced breath sounds:
- reduced airflow/blockage of airway

Question 6

What can you observe and palpate?

Answer 6

Observation:

• wheezing and reduced breath sounds
• SOB - air can’t flow through airways
• hyperventilating

Palpation:
- upper chest breathing - using accessory muscles to assist with breathing

Question 7

What features might you see on a CXR for a patient with CF bronchiectasis? Why?

Answer 7

Thickening of bronchial walls + irregularly shaped bronchioles

• inflammation preventing normal mucus drainage
• mucus build up means bacteria is trapped causing more infections –> scarring and thickening of walls

Widespread “patchy” opacities + mucus plugging -
- large patches of mucus secretion built up in the airways

Question 8

What features might you see on PFT? What do they mean?

Answer 8

Normal/slightly reduced FVC
- may be reduced because of remodelling of the lungs due to scarring but otherwise normal.

Decreased FEV1
- increased airway resistance due to build up of thick mucus -> airway obstruction

Decreased FEV1/FVC because FEV1 is reduced

Reduced DLCO
- bronchial obstruction decreases area of diffusion

Question 9

What impairments would someone with CF bronchiectasis have? What are the signs/symptoms to support this?

Answer 9

Gas movement impairment:
- reduced oxygen movement and CO2 movement due to obstructive airways (mucus)

(SOB, reduced breathing pattern, wheezing)

Secretion movement impairment:

• defective CFTR gene causes secretions to become thick and sticky.
• mucus plugging occurs in the airways.
• dehydration of airways + reduced cilia effectiveness - secretion movement impairment

(excessive sputum production, ineffective cough)

Question 10

What is the pathology of non-CF bronchiectasis?

Answer 10

• Progressive, irreversible condition that causes the bronchial airways to dilate.
• Dilation occurs when supporting structures are weakened by chronic inflammatory changes associated with secondary infections.
• Loss of cilia –> build up of mucus and bacteria.

Question 11

Briefly describe the pathophysiology of non-CF bronchiectasis.

Answer 11

• usually idiopathic but can also be caused by other issues e.g. COPD, asthma and recurrent pulmonary infections.
• MCC impairment due cilia damaged by inflammatory processes.
• Remodelling of airways: bronchial walls dilate and become floppy –> mucus production.
• Dilated and floppy airways –> ineffective cough and airway clearance.
• Permanent scarring and damage to lung parenchyma (alveoli)

Question 12

What are the impairments for non-CF bronchiectasis?

Answer 12

Gas movement impairment:

• walls of airway are thickened and damaged (gas movement impairment)
• overtime, there is scarring of the lung parenchyma so alveoli is damaged –> oxygen exchange impairment

Secretion movement impairment:

• damage to cilia means impairment of MCC and mucus builds up in the airways and bacteria colonises in the airways
• inflammation from bacteria –> airways dilate and are floppy (ineffective cough) –> excess mucus production