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Eur Respir J 2006, doi:10.1183/09031936.06.00004906
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ORIGINAL ARTICLE |
Diurnal ventilation via mouthpiece: survival in end-stage Duchenne patients
1 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
* To whom correspondence should be addressed. E-mail: michel.toussaint{at}inkendaal.be.
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Abstract |
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To assess the impact of diurnal mouthpiece ventilation (MIPPV) as the extension of the nocturnal ventilation (NIPPV) in Duchenne muscular dystrophy (DMD).
Forty two DMD patients, aged 15-33 years, normocapnic at night with NIPPV, receiving MIPPV since end-diurnal hypercapnia appeared.
Transcutaneous CO2 tension (TcCO2) 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.
One year, three year, five year and seven year survival rates reached 88%, 77%, 58% and 51% respectively. The mean survival was 31 years. VC stabilized during five years with MIPPV. Symptom scores significantly decreased and TcCO2 normalized during the day (61.5±16.7 to 43.5±5.5 mmHg). No accident and minor side effects were observed in this 184 cumulated patient-years study.
Daytime mouthpiece ventilation is safe, prolongs survival and stabilizes the vital capacity in Duchenne patients. It is recommended on condition that patients are equipped with a self-supporting harness.
Keywords: Duchenne, mouthpiece, neuromuscular, non-invasive, survival, ventilation
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