Rationale: Noninvasive mechanical ventilation (NIV) is used for respiratory support in patients with Duchenne muscular dystrophy (DMD). Interfaces that cover the nose or the nose and mouth (oronasal interface) are the most common type of NIV, but they have several disadvantages. This case reports the success of the application of a mouthpiece ventilator for a patient who needs whole day ventilator care.
Patient concerns: A 25 year old male who was diagnosed with DMD in 2003 has exhibited respiratory symptoms such as dyspnea, failure of self-expectoration with fatigue, and headaches since 2019. The patient's initial blood gas analysis showed hypercapnic respiratory failure, and he initially began using a NIV with a face mask. After successful adaptation to the NIV, the face mask was changed to a nasaldeveloped progressive motor weakness with toe walking when he was 7-year old(2001). Weakness and gait diturbance was gradually aggravated through 2 years. And then he had had respiratory symptoms such as dyspnea, failure of self expectoration with fatigue, and headache since 2019.
Diagnosis: The patient’s initial arterial blood gas analysis showed hypercapnic respiratory failure, and he initially began using a NIV with a face mask. After successful adaptation to the NIV, the face mask was changed to a nasal mask with a chinstrap.
Interventions: The conventional NIV was very uncomfortable during the activities of daily life such as eating, talking, and drinking. Therefore, the patient was changed to mouthpiece ventilation (MPV) only during the day. After adaptation to MPV, the patient was able to successfully eat, talk, and drink with the necessary respiratory support.
Outcomes: Arterial blood gas sampling performed after the MPV application showed no significant difference between the nasal type NIV and MPV. The patient was satisfied with the MPV since he could talk, eat, and drink while being free from a device attached to his face, which made him uncomfortable and caused other problems such as skin injury and negative psychological consequences.
Conclusion: This case suggests that daytime mouthpiece ventilation (MPV) could successfully wash out CO2 for patients who need 24 hour ventilator care and improve their quality of life (QOL). Daytime MPV and nocturnal conventional NIV may be an ideal ventilation strategy for DMD patients to increase QOL even if they need all day ventilator care.
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