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Diurnal ventilation via mouthpiece: survival in end-stage Duchenne patients

Acute Neurorespiratory Rehabilitation Unit, Neuromuscular Excellency Centre VUB-Inkendaal and Centre for Home Mechanical Ventilation, Z.H. Inkendaal Rehabilitation Hospital, Brussels, Belgium.

CORRESPONDENCE: M. Toussaint, Acute Neurorespiratory Rehabilitation Unit, Neuromuscular Excellency Centre VUB-Inkendaal and Centre for Home Mechanical Ventilation, Z.H. Inkendaal Rehabilitation Hospital, Inkendaalstraat, 1, B-1602 Vlezenbeek, Brussels, Belgium. Fax: 32 25315301. E-mail: michel.toussaint{at}inkendaal.be

Keywords: Duchenne, mouthpiece, neuromuscular, noninvasive, survival, ventilation

Received: January 13, 2006
Accepted June 16, 2006

The present study aimed to assess the impact of diurnal mouthpiece intermittent positive pressure ventilation (MIPPV) as the extension of the nasal intermittent positive pressure ventilation (NIPPV) in Duchenne muscular dystrophy (DMD). In total, 42 DMD patients aged 15–33 yrs, normocapnic at night with NIPPV and receiving MIPPV since end-diurnal hypercapnia, were studied.

Transcutaneous CO₂ tension (P_t,CO2) was prospectively monitored at the end of the day, before and after MIPPV initiation. Vital capacity (VC), breathing pattern and maximal inspiratory strength were measured. Patients were asked to score the presence (1 point) or absence (0 point) of seven respiratory-linked symptoms before and after MIPPV establishment.

Survival rates reached 88, 77, 58 and 51% after 1, 3, 5 and 7 yrs, respectively. The mean survival rate was 31 yrs. VC stabilised during 5 yrs with MIPPV. Symptom scores significantly decreased and P_t,CO2 normalised during the day (8.17±2.22 to 5.78±0.73 kPa). No accident and minor side-effects were observed in this 184 cumulated patient-yrs study.

In conclusion, daytime mouthpiece ventilation is safe, prolongs survival and stabilises vital capacity in Duchenne muscular dystrophy patients. It is recommended on the condition that patients are equipped with a self-supporting harness.

This article has been cited by other articles:

				P Soudon, M Steens, and M Toussaint A comparison of invasive versus noninvasive full-time mechanical ventilation in Duchenne muscular dystrophy Chronic Respiratory Disease, May 1, 2008; 5(2): 87 - 93. [Abstract] [PDF]

				M Toussaint, P Soudon, and W Kinnear Effect of non-invasive ventilation on respiratory muscle loading and endurance in patients with Duchenne muscular dystrophy Thorax, May 1, 2008; 63(5): 430 - 434. [Abstract] [Full Text] [PDF]

				F. Lofaso, D. Orlikowski, and J-C. Raphael Ventilatory assistance in patients with Duchenne muscular dystrophy. Eur. Respir. J., September 1, 2006; 28(3): 468 - 469. [Full Text] [PDF]