Long-term Noninvasive Mechanical Ventilation Research Articles

Telemedicine for Facio-Scapulo-Humeral Muscular Dystrophy: A multidisciplinary approach to improve quality of life and reduce hospitalization rate?

BackgroundFacio-Scapulo-Humeral Muscular Dystrophy (FSHD) is an autosomal dominant inherited disorder characterized by a variable and asymmetric involvement of facial, trunk, upper and lower extremity muscles. Although respiratory weakness is a relatively unknown feature of FSHD, it is not rare. Telemedicine has been used in a variety of health care fields, but only recently, with the advent of sophisticated technology, its interest among health professionals became evident, even in such diseases. ObjectiveTo demonstrate the telemedicine efficacy in FSHD. MethodsFour siblings affected by a severe form of FSHD, living in a rural area far away from the referral center for neuromuscular diseases, who used a wheelchair, suffered from chronic respiratory failure and were provided with long-term non-invasive mechanical ventilation, received a 6-month period of telemedicine support. This consisted of video conferencing (respiratory physiotherapy, psychological support, neurological and pneumological assessment, nurse-coach supervision) and telemonitoring of cardiorespiratory variables (oxygen saturation, blood pressure, and heart rate). ResultsWe performed 540 video conference sessions per patient, including three daily contacts with short monitoring oximetry measurements, blood pressure, and heart-rate measurements, psychological support, neurological and pneumological assessment, nurse-coach supervision. ConclusionsOur findings indicate that our telemedicine system was user-friendly, efficient for the home treatment of FSHD, and allowed reducing hospital admissions.

Successful tracheostomy decannulation after complete or sensory incomplete cervical spinal cord injury.

Retrospective study. To report the successful tracheostomy decannulation/extubation in cervical spinal cord injury (C-SCI) patients. Tertiary university hospital, pulmonary rehabilitation center. Complete or sensory incomplete C-SCI patients who had received invasive acute phase respiratory management, and succeeded in decannulation/extubation became candidates of this study. Retrospective review was conducted on the transitions of the respiratory status. Sixty-two patients (M: 55, F: 7) were identified. Traumatic etiologies accounted for 93.5%, and mean onset age was 47.6±15.8 years. Sixty patients (96.8%) had undergone tracheostomy and the other two received endotracheal intubation during acute phase. All patients succeeded in decannulation/extubation after employing mechanically assisted coughing and noninvasive mechanical ventilation (NIV). Mean time since tracheostomy to decannulation was 7.0±14.5 months. Of the 60 tracheostomized patients, 12 succeeded in decannulation without applying long-term NIV, 31 switched to continuous NIV after decannulation. Fifteen patients totally weaned off from ventilators after NIV. Two patients who once succeeded in decannulation were re-tracheostomized. For the 31 patients with continuous NIV, mean hours of daily need for ventilatory support had reduced from 15.3±8.0 to 5.7±5.7 h at final follow-ups. Complete or sensory incomplete C-SCI patients, even with high neurological level can be successfully decannulated through NIV and aggressive use of mechanically assisted coughing. Undesirable tracheostomy can be avoided by employing the noninvasive respiratory management.

Obesity might be a good prognosis factor for COPD patients using domiciliary noninvasive mechanical ventilation.

Cachexia is known to be a deteriorating factor for survival of patients with chronic obstructive pulmonary disease (COPD), but data related to obesity are limited. We observed that obese patients with COPD prescribed long-term noninvasive mechanical ventilation (NIMV) had better survival rate compared to nonobese patients. Therefore, we conducted a retrospective observational cohort study. Archives of Thoracic Diseases Training Hospital were sought between 2008 and 2013. All the subjects were prescribed domiciliary NIMV for chronic respiratory failure secondary to COPD. Subjects were grouped according to their body mass index (BMI). The first group consisted of subjects with BMI between 20 and 30 kg/m2, and the second group consisted of subjects with BMI >30 kg/m2. Data obtained at the first month’s visit for the following parameters were recorded: age, sex, comorbid diseases, smoking history, pulmonary function test, 6-minute walk test (6-MWT), and arterial blood gas analysis. Hospital admissions were recorded before and after the domiciliary NIMV usage. Mortality rate was searched from the electronic database. Overall, 118 subjects were enrolled. Thirty-eight subjects had BMI between 20 and 30 kg/m2, while 80 subjects had BMI >30 kg/m2. The mean age was 65.8±9.4 years, and 81% were male. The median follow-up time was 26 months and mortality rates were 32% and 34% for obese and nonobese subjects (P=0.67). Improvement in 6-MWT was protective against mortality. In conclusion, survival of obese patients with COPD using domiciliary NIMV was found to be better than those of nonobese patients, and the improvement in 6-MWT in such patients was found to be related to a better survival.

Nichtinvasive Beatmung beim Patienten mit persistierender Hyperkapnie

Chronic respiratory failure is caused by insufficiency of the inspiratory muscles, i.e. mainly the diaphragm, which represents the so-called "respiratory pump". Insufficiency of the respiratory pump causes hypercapnia. Diseases with chronic hypercapnia are characterized by reduced survival. Mechanical ventilation-mostly applied as noninvasive mechanical ventilation (NIV)-improves ventilation and unloads the inspiratory muscles. Strong evidence supports the use of domiciliary NIV already in mild degrees of chronic respiratory failure caused by neuromuscular diseases, thoracic restrictions and obesity hypoventilation. In these diseases long-term NIV improves both physiological parameters (such as blood gases) and clinical outcome, e.g. exercise capacity, right heart dysfunction, sleep quality, disease-specific aspects of health-related quality of life (HRQL) and survival rate. In contrast, its influence on long-term survival in chronic obstructive pulmonary disease (COPD) patients is not clearly proven. Prescription of home NIV in COPD should therefore be restricted to severe degrees of chronic respiratory failure. Finally, there is an indication for domiciliary NIV in patients after prolonged weaning from mechanical ventilation. This paper elaborates underlying pathophysiology, diseases and how NIV works in chronic hypercapnic respiratory failure.

Deventilation syndrome in severe COPD patients during long-term noninvasive mechanical ventilation: poor sleep pattern, hyperinflation, or silent chronic muscular fatigue?

Deventilation syndrome in severe COPD patients during long-term noninvasive mechanical ventilation: poor sleep pattern, hyperinflation, or silent chronic muscular fatigue?

Noninvasive mechanical ventilation with high pressure strategy remains a “double edged sword”?

O associated with a high backup ventilator rate) may improve adherence, physiological, and subjective outcomes when compared with delivery of high pressure noninvasive mechanical ventilation (without elevated backup respiratory rate). The authors concluded that both strategies are equivalent in all the recorded outcomes, showing thus that driving pressure, but not backup respiratory rate, is essential to gain physiological and clinical benefits in this population when in a chronic stable condition.Despite previous randomized studies showing the potential benefits of long-term noninvasive mechanical ventilation in hypercapnic patients with chronic obstructive pulmonary disease, current research has still not clearly indicated the best strategy to improve the patient’s adherence with treatment.

Chronic respiratory care for neuromuscular diseases in adults

Neuromuscular diseases (NMD) may affect respiratory muscles, leading to respiratory failure. Studies show that long-term noninvasive mechanical ventilation (NIV) improves symptoms, gas exchange, quality of life and survival. NIV improved these parameters in muscular dystrophies and also in patients with amyotrophic lateral sclerosis without severe bulbar dysfunction. NIV should be started at the onset of nocturnal hypoventilation. In selected cases, NIV may be simpler, better accepted by patients and cheaper than invasive mechanical ventilation, but it cannot be used as an alternative. Tracheostomy may be preferred by patients unable to protect their airways and wishing to survive as long as possible, or by ventilator-dependent patients. Glossopharyngeal breathing consists of taking air and propelling it into the lungs. Chest percussions and vibrations can help to mobilise airway secretions but they cannot substitute coughing. Manually assisted coughing requires substantial lung inflation through air stacking or deep lung insufflation, followed by an abdominal thrust with open glottis. The combination of mechanical in-exsufflation with an abdominal thrust is a mechanically assisted cough. In conclusion, recent advances in respiratory care of NMD have improved prognosis and many caregivers have changed from a traditional noninterventional to a more aggressive, supportive approach.

Comparison of two humidification systems for long-term noninvasive mechanical ventilation

There is no consensus concerning the best system of humidification during long-term noninvasive mechanical ventilation (NIMV). In a technical pilot randomised crossover 12-month study, 16 patients with stable chronic hypercapnic respiratory failure received either heated humidification or heat and moisture exchanger. Compliance with long-term NIMV, airway symptoms, side-effects and number of severe acute pulmonary exacerbations requiring hospitalisation were recorded. Two patients died. Intention-to-treat statistical analysis was performed on 14 patients. No significant differences were observed in compliance with long-term NIMV, but 10 out of 14 patients decided to continue long-term NIMV with heated humidification at the end of the trial. The incidence of side-effects, except for dry throat (significantly more often present using heat and moisture exchanger), hospitalisations and pneumonia were not significantly different. In the present pilot study, the use heated humidification and heat and moisture exchanger showed similar tolerance and side-effects, but a higher number of patients decided to continue long-term noninvasive mechanical ventilation with heated humidification. Further larger studies are required in order to confirm these findings.

Long-term noninvasive mechanical ventilation for children at home: a national survey.

Experience with domiciliary noninvasive mechanical ventilation (NIMV) in children is limited. The aim of this study was to determine the number of patients and categorize the use of domiciliary NIMV in children in France. An anonymous cross-sectional national study was performed, using a postal questionnaire sent to all specialist centers utilizing domiciliary NIMV for chronic respiratory failure. Patients aged <18 years and receiving home NIMV were included in the study. Detailed information was obtained from 102 patients from 15 centers: 4/15 centers cared for 84% of patients; 7% of patients were under 3 years old; 35% were between 4-11 years old; and 58% were older than 12 years. Underlying diagnoses included neuromuscular disease (34%), obstructive sleep apnea (OSA) and/or cranio-facial abnormalities (30%), cystic fibrosis (17%), congenital hypoventilation (9%), scoliosis (8%), and other disorders (2%). NIMV was started because of nocturnal hypoventilation (67%), acute exacerbation (28%), and/or failure to thrive (21%). Volume-targeted ventilation was preferred in restrictive disorders (56%) and central hypoventilation (56%), while pressure support ventilation (PSV) was preferred in cystic fibrosis (71%). Patients with OSA and/or cranio-facial abnormalities were ventilated with continuous positive airway pressure (45%) or bilevel PSV (52%). In conclusion, NIMV is currently used in France for the domiciliary management of children with a variety of conditions causing chronic respiratory failure. However, NIMV in children is still performed on a small scale, and combined with the heterogeneity of the patient population, its application would best be served by centers specializing in the initiation and long-term follow-up of these patients.

Physiologic Evaluation of 4 Weeks of Nocturnal Nasal Positive Pressure Ventilation in Stable Hypercapnic Patients with Chronic Obstructive Pulmonary Disease

Background: The long-term daily use of noninvasive mechanical ventilation (NIMV) to treat chronic respiratory failure in chronic obstructive pulmonary disease (COPD) patients is not widely recommended, partly because of a lack of clear clinical results and partly because the physiological mechanisms by which the daily application of NIMV would be helpful in these patients have not yet been clarified. Objectives: We designed a physiological study in order to assess the effects of supervised long-term NIMV on gas exchange, respiratory muscle function, pulmonary mechanics and to ascertain the possible effect of the treatment in responders and nonresponders. Methods: Fourteen consecutive inpatients with stable hypercapnic COPD (pH = 7.37 ± 0.01; PaCO₂ = 56.73 ± 6.48 mm Hg) underwent 4 weeks of nocturnal NIMV delivered with a bilevel ventilator ‘physiologically’ set to reduce tidal transdiaphragmatic pressure (Pdi) by at least 50% and the amount of dynamic intrinsic positive end-expiratory pressure by 70%. Various measurements were compared with those obtained in a control group of consecutive patients with comparable baseline characteristics who refused NIMV and underwent breathing exercises for the same period of time. Results: By the end of the 4 weeks NIMV had induced a slight but significant (p < 0.01) reduction in resting PaCO₂ (53.78 ± 5.64 mm Hg) associated with a decrease in the pressure time product of the diaphragm per minute (from 172 ± 60 to 136 ± 61 cm H₂O/l/s; p < 0.05). This latter value was primarily due to a significant shortening of the inspiratory duty cycle, while Pdi and lung mechanics were not modified. Eight of the 13 NIMV-treated patients (1 dropped out for nonmedical reasons) had a clear reduction in PaCO₂ (>3 mm Hg or >5% from enrollment) and were classified as responders. The acute reduction in PaCO₂ during the first trial with NIMV resulted to be a strong index of the final response. The subgroup of responders had a significantly increased maximal Pdi (from 41 ± 19 to 49 ± 23 cm H₂O, p < 0.05) and an enhanced ability of the ventilatory pump to clear CO₂ (9.7 ± 3.4 vs. 7.2 ± 2.9 cm H₂O × s/min; p < 0.01). No significant changes were observed in the control group. Conclusions: These results suggest that in a remarkable and identifyable proportion of patients with stable hypercapnic COPD, nocturnal NIMV may decrease resting PaCO₂, reraising the role of chronically supporting the respiratory pump.

Is It Safe for Patients With Chronic Hypercapnic Respiratory Failure Undergoing Home Noninvasive Ventilation To Discontinue Ventilation Briefly?

A brief discontinuation (< 1 week) of long-term ventilation may be necessary in patients who are not totally ventilator-dependent in cases of technical problems, intolerable nasal irritation, upper airway congestion, or travel. We examined the incidence, timing, and causes of possible clinical deterioration after a brief withdrawal of ventilation in patients with chronic respiratory failure (CRF) who were well-established on long-term noninvasive mechanical ventilation (NIMV). Prospective clinical study. Eleven inpatients in clinically stable condition (COPD, 6 patients; and restrictive thoracic disease [RTD], 5 patients) who had severe CRF (PaCO(2), > 50 mm Hg) and had been receiving NIMV for (mean +/- SD) 19.3 +/- 5.3 months were enrolled. Arterial blood gas (ABG) levels, maximal inspiratory pressure (PImax), breathing pattern, dyspnea rating, and life symptoms (measured by a questionnaire) were recorded daily after NIMV withdrawal for 6 days or until the patients showed clinical and/or ABG level deterioration. Pulmonary function tests were performed and neuromuscular drive was measured at the beginning and the end of the study. Five of the 11 patients (45.4%) [COPD, 3 patients; and RTD, 2 patients] were reconnected to a ventilator before the scheduled time because of ABG level deterioration. Despite these changes, none of the patients reported severe worsening of symptoms or other medical complications. The patients whose ABG levels worsened had statistically significant decreases in tidal volume and PImax, suggesting that the development of alveolar hypoventilation was related to respiratory muscle weakness. A brief discontinuation of NIMV in patients who were affected by chronic hypercapnic respiratory failure and were well-established on NIMV is associated with a relatively high incidence of ABG level worsening due to the development of alveolar hypoventilation. If NIMV must be briefly interrupted for clinical reasons, the patient should be monitored closely for abrupt worsening, and prompt technical intervention should be provided if a ventilator fails.