Mouthpiece ventilation in Duchenne muscular dystrophy: a rescue strategy for noncompliant patients

Abstract

Objective:To evaluate mouthpiece ventilation (MPV) in patients with Duchenne muscular dystrophy (DMD) who are noncompliant with noninvasive ventilation (NIV). Methods:We evaluated four young patients with DMD who had previously refused to undergo NIV. Each patient was reassessed and encouraged to try MPV. Results:The four patients tolerated MPV well and were compliant with NIV at home. MPV proved to be preferable and more comfortable than NIV with any other type of interface. Two of the patients required overnight NIV and eventually agreed to use a nasal mask during the night. Conclusions:The advantages of MPV over other types of NIV include fewer speech problems, better appearance, and less impact on the patient, eliminating the risk of skin breakdown, gastric distension, conjunctivitis, and claustrophobia. The use of a mouthpiece interface should be always considered in patients with DMD who need to start NIV, in order to promote a positive approach and a rapid acceptance of NIV. Using MPV during the daytime makes patients feel safe and more likely to use NIV at night. In addition, MPV increases treatment compliance for those who refuse to use other types of interfaces.

Reliability and Validity of the Korean Version of the Duchenne Muscular Dystrophy Functional Ability Self-Assessment Tool

“…However, variability in ventilator use in DMD patients exists depending on the patient’s comfort and compliance with mechanical ventilation. In fact, some patients use only mouthpiece ventilation during the day, whereas others prefer nasal interfaces for ventilator support and alternate them throughout the day [ 25 ]. Additionally, rarely, some patients prefer ventilator support only when they are awake, so they don’t use the ventilator during the night [ 26 ].…”

Intermittent Abdominal Pressure Ventilation: An Alternative for Respiratory Support

“…Patients with the need for continuous noninvasive ventilation often present pressure ulcers from the mask, aerophagia, and intolerance to the mask due to interference with social life. These complications can lead to the failure of NIV [ 14 , 15 ]. IAPV maintains good ventilation and oxygenation and reduces complications related to positive pressure ventilation with a mask.…”

Continuous noninvasive ventilation for respiratory failure in patients with amyotrophic lateral sclerosis: current perspectives

“…The possibility to change the interfaces during the different daily activities represents an important improvement of QoL and adherence to treatment in patients who need continuous NIV. 39 The mouthpiece improves the social aspects of the patients, allowing them to speak and eat. Moreover, the patient can remove the mouthpiece to cough.…”

“…The mouthpiece could be considered during the NIV adaptation phase. 39 Bédard and McKim 41 recently studied the utilization of daytime mouthpiece ventilation in an ALS population using 24-hour NIV. They confirm the importance of preserved bulbar function and the ability to generate adequate peak cough flow for effective mouthpiece ventilation for survival.…”