Table 4.

Management of acute respiratory failure (ARF) in patients with LOPD. ERT, enzyme replacement therapy; ICU, intensive care unit; RICU, respiratory intermediate care unit; NIV, non-invasive ventilation; TIV, tracheostomy invasive ventilation; I/E, insufflation/exsufflation; HFCWO, high frequency chest wall oscillation.

Admission to ICU or RICU [68] Broad spectrum antibiotics Avoid opiates and paralytics Always prefer NIV to TIV if possible If TIV is inevitable, aim for early closure of tracheostomy and re-start of NIV Aggressively treat airway secretions (I/E, HFCWO, bronchoscopy) Start respiratory rehabilitation as early as possible Evaluate patients without ventilatory support prior to ARF for NIV indication After rehabilitation, reinforce long-term prophylactic measures (e.g., cough assistance, immunizations) ERT not to be paused