Respiratory physician Footnote 1

Respiratory physicians are responsible for monitoring lung health, as this is expected to decline alongside muscle strength

Respiratory complications include respiratory muscle fatigue, mucus plugging, atelectasis, pneumonia and respiratory failure

Ambulatory phase
Monitor:
  • FVC annually
  • Sleep apnoea or sleep-disordered breathing
Administer:
  • Immunisation with pneumococcal vaccines and inactivated influenza vaccine
Early non-ambulatory phase
Monitor:
  • FVC, MIP/MEP, PCF, SpO2 and petCO2/pctCO2 twice-yearly
  • Sleep apnoea or sleep-disordered breathing
Administer:
  • Immunisation as before
  • Lung volume recruitment when FVC ≤60% of predicted
Late non-ambulatory phase
Monitor:
  • FVC, MIP/MEP, PCF, SpO2 and petCO2/pctCO2 twice-yearly
  • Sleep apnoea or sleep-disordered breathing
Administer:
  • Immunisation as before
  • Lung volume recruitment when FVC ≤60% of predicted
  • Assisted coughing when FVC <50% predicted; PCF 270 L/min or MEP <60 cm H2O
  • Nocturnal assisted ventilation with backup rate of breathing when there are signs or symptoms of sleep hypoventilation or other sleep-disordered breathing (FVC <50% predicted, MIP <60 cm H2O)
  • Addition of assisted daytime ventilation when daytime SpO2 <95% pCO2 >45 mmHg, or symptoms of waking dyspnoea present