validation of a dynamic evaluation tool in respiratory failure

Conditions: Respiratory Failure

Summary: determing optimal time of ventilator disconnection is a challenge for both acute and chronic neuromuscular disease. in one case it is helpful for weanning from ventilator and in the other to optimize daytime ventilation in the most severe patients. the investigators propose to validate a new non invasive tool for monitoring respiratroy parameters in neuromuscular patients in both acute and chronic conditions.

context: mechanical ventilation transformed the prognosis of neuromuscular diseases whether for acute or chronic diseases. the available tools to estimate the severity of respiratory insufficiency and indicate or follow the efficiency of mechanical ventilation are based on blood gazes explorations, tests respiratory function and sleep evaluation. these tools give either limited information, or are with difficulty applicable to patients heavily handicapped except during hospitalization. in patients under mechanical ventilation, it is sometimes difficult to know the daily optimal time of ventilation. the respiratory events are not generally recognized and are unspecific (headaches, drowsiness, dyspnoea, etc.) or are identified by blood gazes abnormalities (hypercapnia. in acute setting, treble is going to raise the problem of the weaning of the mechanical ventilation and the optimal moment of the extubation without risking a reintubation. these problems are identical and concern the evaluation of the degree of ventilatory autonomy for home care. the development of methods estimating this autonomy is thus fundamental. objectives: the objective of this project is to validate a non-invasive system for measure and acquisition of respiratory parameters, incorporating a transmission system working by a "wireless" technology allowing the remote monitoring of these patients. the secondary objectives are to determine the parameters of success of weaning by using the device in neuromuscular patients intubated for respiratory failure in acute setting and to determine the optimal time of free breathing possibilities in patients ventilated in a chronic way at home. methods: after validation of the device on 10 healthy subjects, continuous acquisition of the ventilatory data by means of the teleresp system compared with measure of the transcutaneous pco2, of sao2 and arterial gazometry at the end of free breath trial in 40 acute and chronic neuromuscular patients. selection criteria: healthy volunteers patients affected by acute neuromuscular pathology and starting weaning from invasive ventilation (guillain barré and myasthenia). patients affected by neuromuscular pathology and home ventilated in a diurnal and night-way by invasive interface or not (respiratory autonomy of at least 1:00 am). number of patients, center: 10 healthy volunteers and 40 patients hospitalized in the intensive care unit or in the home of ventilation unit of the raymond poincaré hospital. total duration of the study: 19 months

Eligibility criteria:
inclusion criteria: healthy volonteers man or woman of more than 18 years realization of a preliminary medical examination patient having signed an informed and written consent major healthy subjects, presenting no chronic pathology or not chronic patients chronic patients man or woman of more than 18 years affected by neuromuscular pathology realization of a preliminary medical examination patient in the stable state at the time of the study for at least 1 month ventilated in a not invasive or invasive way in diurnal and night-period. respiratory autonomy \> 1:00 am patient having signed an informed and written consent acute patients man or woman of more than 18 years affected by syndrome of guillain blocked in aigue phase having required the invasive ventilation or affected by myasthenia generalized with myasthénique crisis having required the invasive ventilation. realization of a preliminary medical examination patient in phase of neurological recovery vital capacity \> 15 ml / kg respiratory autonomy \> 1:00 am patient having signed a lit(enlightened) and written consent exclusion criteria: healthy volonteers subject refusing to participate in the study subject under guardianship or guardianship pregnant or breast-feeding woman chronic patients patient refusing to participate in the study patient under guardianship or guardianship pregnant or breast-feeding woman patients ventilated only at night clinically significant bronchial dimensions(congestion) required by oxygen therapy acute patients patient refusing to participate in the study patient or subject under guardianship or guardianship pregnant or breast-feeding woman clinically significant bronchial dimensions(congestion) required by oxygen therapy fio2 \> 40 % pep \> 5 cms h2o state of shock

Keywords: neuromuscular disease