Table 3.

Practical recommendations for cough assistance in patients with LOPD. MAC, manually assisted coughing; I/E, insufflation/exsufflation; HFCWO, high frequency chest wall oscillation; NIV, non-invasive ventilation; TIV, tracheostomy invasive ventilation; PCF, peak cough flow; MEP, maximal expiratory pressure.

Symptoms	Mucus obstruction, recurrent desaturations, recurrent pulmonary infections
Testing	PCF, MEP
When to start	PCF < 270 L/min once during stable state independent of symptoms PCF < 160 L/min once during acute exacerbation MEP < 60 cm H2O with history of impaired airway clearance
Techniques	MAC	If patient is willing and able to co-operate Performed by respiratory therapists or trained caregivers Re-evaluate feasibility and effectiveness Switch to mechanical techniques if MAC is not feasible or proves ineffective
	Air stacking	Usually in combination with MAC Via bag valve mask in the non-invasive setting Via ventilator device (with NIV or TIV, respectively)
	I/E	If MAC/air stacking are not feasible or ineffective May be combined with MAC Individually titrate optimal pressure settings Feasible in both the NIV and TIV setting Re-evaluate using PCF as outcome measure Start early in case of pulmonary infection
	HFCWO	If MAC/air stacking are either not feasible or ineffective If I/E cannot be tolerated May be combined with MAC Individually titrate frequency and duration Feasible in both the NIV and TIV setting May be combined with suction Start early in case of pulmonary infection
Optional measures	Mucolysis	Hydration, mucolytics (with caution)
	Suction	If expectoration cannot be achieved by MAC, I/E, HFCWO alone