Miguel Gonçalves

Changing trends in the management of end-stage neuromuscular respiratory muscle failure: recommendations of an international consensus.

ObjectiveRespiratory management of patients with end-stage respiratory muscle failure of neuromuscular disease has evolved from no treatment and inevitable respiratory failure to the use of up to continuous noninvasive intermittent positive pressure ventilatory support (CNVS) to avert respiratory failure and to permit the extubation of “unweanable” patients without tracheostomy. An international panel experienced in CNVS was charged by the 69th Congress of the Mexican Society of Pulmonologists and Thoracic Surgeons to analyze changing respiratory management trends and to make recommendations. DesignNeuromuscular disease respiratory consensuses and reviews were identified from PubMed. Individual respiratory interventions were identified; their importance was established by assessing the quality of evidence-based literature for each one and their patterns of use over time. The panel then determined the evidence-based strength for the efficacy of each intervention and made recommendations for achieving prolonged survival by CNVS. ResultsFifty publications since 1993 were identified. Continuous positive airway pressure, oxygen therapy, bilevel positive airway pressure used at both low and high spans, “air stacking,” manually assisted coughing, low pressure (<35 cm H2O) and high pressure (≥40 cm H2O) mechanically assisted coughing, noninvasive positive pressure ventilation part time (<23 hrs per day) and full time (>23 hrs per day; CNVS), extubation and decannulation of ventilator-dependent patients to CNVS, and oximetry feedback for noninvasive positive pressure ventilation and mechanically assisted coughing were identified. All noted interventions are being used with increasing frequency and were unanimously recommended to achieve prolonged survival by CNVS, with the exception of supplemental oxygen and continuous positive airway pressure, which are being used less and were not recommended for this population. ConclusionsCNVS and extubation of unweanable patients to CNVS are increasingly being used to prolong life while avoiding invasive interfaces.

Lung insufflation capacity in neuromuscular disease.

Bach JR, Mahajan K, Lipa B, Saporito L, Goncalves M, Komaroff E: Lung insufflation capacity in neuromuscular disease. Am J Phys Med Rehabil 2008;87:720–725. Objective:To compare maximal passive lung insufflation capacity (LIC) with lung inflation by air stacking (to maximum insufflation capacity [MIC]) and with vital capacity (VC); to explore relationships between these variables that correlate with glottic function and cough peak flows (CPF); to demonstrate the effect of routine inflation therapy on LIC and MIC; and to determine the relative importance of lung inflation therapy as a function of disease severity. Design:Case series of 282 consecutive neuromuscular disease (NMD) clinic patients 7 yrs and older with VC <70% of the predicted normal value. All cooperative patients meeting these criteria were prescribed thrice-daily air stacking and/or maximal passive lung insufflation to pressures of 40–80 cm H2O, and they underwent measurements of VC, MIC, LIC, and unassisted and assisted CPF on every visit. Results:Means ± standard deviations for VC, MIC, and LIC were 1131 ± 744, 1712 ± 926, and 2069 ± 867 ml, respectively, and, for unassisted and assisted CPF, they were 2.5 ± 2.0 and 4.3 ± 2.2 liters/sec, respectively, with all differences statistically significant (P < 0.001). MIC minus VC correlated inversely with LIC minus MIC (P = 0.01) and, therefore, with glottic function. Both MIC and LIC increased with practice (P < 0.001). Increases in LIC but not MIC over VC were greatest for patients with the lowest VC (P < 0.05). There were no complications of lung mobilization therapy. Conclusions:Passive lung insufflation can distend the lungs of patients with NMD significantly greater than air stacking, particularly when glottic and bulbar-innervated muscle dysfunction is severe. LIC, MIC, and VC measurements permit quantifiable assessment of glottic integrity and, therefore, bulbar-innervated muscle function for patients with NMD. The patients who benefit the most from insufflation therapy are those who have the lowest VC.

Expiratory flow maneuvers in patients with neuromuscular diseases

Bach JR, Gonçalves MR, Páez S, Winck JC, Leitão S, Abreu P: Expiratory flow maneuvers in patients with neuromuscular diseases. Am J Phys Med Rehabil 2006;85:105–111. Objectives:To compare cough peak flows (CPF), peak expiratory flows (PEF), and potentially confounding flows obtained by lip and tongue propulsion (dart flows, DF) for normal subjects and for patients with neuromuscular disease/restrictive pulmonary syndrome and to correlate them with vital capacity and maximum insufflation capacity. Design:A cross-sectional analytic study of 125 stable patients and 52 normal subjects in which CPF, PEF, and DF were measured by peak flow meter and vital capacity and maximum insufflation capacity by spirometer. Results:In normal subjects and in patients, the DF significantly exceeded PEF and CPF (P ≤ 0.001). For normal subjects, PEF and CPF were not significantly different. For patients with neuromuscular disease/restrictive pulmonary syndrome, the CPF significantly exceeded PEF (P < 0.05). No normal subjects but 14 patients had DF lower than CPF. Thirteen of these 14 had the ability to air stack (maximum insufflation capacity greater than vital capacity), indicating greater compromise of mouth and lip than of glottic muscles. For 14 of 88 patients, maximum insufflation capacity values did not exceed vital capacity, mostly because of inability to close the glottis (inability to air stack). Nonetheless, for 11 of these 14 patients, the DF were within a standard deviation of the whole patient group; thus, bulbar-innervated muscle dysfunction was not uniform. CPF and PEF correlated with vital capacity (r = 0.85 and 0.86, respectively), and with maximum insufflation capacity (r = 0.76 and 0.72, respectively). Conclusions:Measurements of CPF, PEF, and DF are useful for assessing bulbar-innervated, inspiratory, and expiratory muscle function. Care must be taken to not confuse them.

Effects of mechanical insufflation-exsufflation in preventing respiratory failure after extubation: a randomized controlled trial

IntroductionWeaning protocols that include noninvasive ventilation (NIV) decrease re-intubation rates and ICU length of stay. However, impaired airway clearance is associated with NIV failure. Mechanical insufflation-exsufflation (MI-E) has been proven to be very effective in patients receiving NIV. We aimed to assess the efficacy of MI-E as part of an extubation protocol.MethodPatients with mechanical ventilation (MV) for more than 48 hours with specific inclusion criteria, who successfully tolerated a spontaneous breathing trial (SBT), were randomly allocated before extubation, either for (A) a conventional extubation protocol (control group), or (B) the MI-E extubation protocol (study group). During the postextubation period (48 hours), group A patients received standard medical treatment (SMT), including NIV in case of specific indications, whereas group B received the same postextubation approach plus three daily sessions of mechanical in-exsufflation (MI-E). Reintubation rates, ICU length of stay, and NIV failure rates were analyzed.ResultsSeventy-five patients (26 women) with a mean age of 61.8 ± 17.3 years were randomized to a control group (n = 40; mean SAPS II, 47.8 ± 17.7) and to a study group (n = 35; mean SAPS II, 45.0 ± 15.0). MV time before enrollment was 9.4 ± 4.8 and 10.5 ± 4.1 days for the control and the study group, respectively. In the 48 hours after extubation, 20 control patients (50%) and 14 study patients (40%) used NIV. Study group patients had a significant lower reintubation rate than did controls; six patients (17%) versus 19 patients (48%), P < 0.05; respectively, and a significantly lower time under MV; 17.8 ± 6.4 versus 11.7 ± 3.5 days; P < 0.05; respectively. Considering only the subgroup of patients that used NIV, the reintubation rates related to NIV failure were significantly lower in the study group when compared with controls; two patients (6%) versus 13 (33%); P < 0.05, respectively. Mean ICU length of stay after extubation was significantly lower in the study group when compared with controls (3.1 ± 2.5 versus 9.8 ± 6.7 days; P < 0.05). No differences were found in the total ICU length of stay.ConclusionInclusion of MI-E may reduce reintubation rates with consequent reduction in postextubation ICU length of stay. This technique seems to be efficient in improving the efficacy of NIV in this patient population.

Indications and Compliance of Home Mechanical Insufflation-Exsufflation in Patients with Neuromuscular Diseases

Introduction: Neuromuscular disease (NMD) patients frequently have impaired cough. Mechanical insufflation-exsufflation (MI-E) has proven efficacy in improving airway clearance, however data related to its long-term home use is lacking. The purpose of this study was to describe indications, safety and compliance of home MI-E in NMD patients. Methods: Four years observational analysis of 21 NMD patients on home MI-E. Diagnosis included bulbar and non-bulbar Amyotrophic Lateral Sclerosis (ALS) and other NMD. Median age was 58 years. Only cooperative patients with unassisted baseline Peak Cough Flow (PCF) < 270 L/min were included. All patients were under continuous mechanical ventilation (6 by tracheostomy). Pulmonary function before initiation of MI-E (median): FVC = 0.81 L, MIP = 28 cmH2O, MEP = 22 cmH2O and PCF = 60 L/min. MI-E was performed by previously trained non-professional caregivers, with an on-call support of a trained health care professional. Patients had pulse oximetry monitorization and applied MI-E whenever SpO2 < 95 %. Median follow-up was 12 months (3-41 months). Results: Ten patients (9 ALS) used MI-E daily. Eleven patients used MI-E intermittently, during exacerbations, and in 8 patients early application of MI-E (guided by oximetry feed-back) avoided hospitalization. All tracheostomized patients used MI-E daily and more times a day than patients under NIV. Four patients (3 bulbar ALS), were hospitalized due to secretion encumbrance. MI-E was well-tolerated and there were no complications. In general, caregivers considered MI-E effective. During this period, 4 patients died, related to disease progression. Conclusions: Home MI-E is well tolerated, effective and safe if used by well trained caregivers. MI-E should be considered as a complement to mechanical ventilation.

Indicaciones y cumplimiento con la insuflación-exuflación mecánica domiciliaria en pacientes con enfermedades neuromusculares

INTRODUCTION Neuromuscular disease (NMD) patients frequently have impaired cough. Mechanical insufflation-exsufflation (MI-E) has proven efficacy in improving airway clearance, however data related to its long-term home use is lacking. The purpose of this study was to describe indications, safety and compliance of home MI-E in NMD patients. METHODS Four years observational analysis of 21 NMD patients on home MI-E. Diagnosis included bulbar and non-bulbar Amyotrophic Lateral Sclerosis (ALS) and other NMD. Median age was 58 years. Only cooperative patients with unassisted baseline Peak Cough Flow (PCF) <270 L/min were included. All patients were under continuous mechanical ventilation (6 by tracheostomy). Pulmonary function before initiation of MI-E (median): FVC=0.81 L, MIP=28cmH(2)O, MEP=22 cmH(2)O and PCF=60 L/min. MI-E was performed by previously trained non-professional caregivers, with an on-call support of a trained health care professional. Patients had pulse oximetry monitorization and applied MI-E whenever SpO(2)<95%. Median follow-up was 12 months (3-41 months). RESULTS Ten patients (9 ALS) used MI-E daily. Eleven patients used MI-E intermittently, during exacerbations, and in 8 patients early application of MI-E (guided by oximetry feed-back) avoided hospitalization. All tracheostomized patients used MI-E daily and more times a day than patients under NIV. Four patients (3 bulbar ALS), were hospitalized due to secretion encumbrance. MI-E was well-tolerated and there were no complications. In general, caregivers considered MI-E effective. During this period, 4 patients died, related to disease progression. CONCLUSIONS Home MI-E is well tolerated, effective and safe if used by well trained caregivers. MI-E should be considered as a complement to mechanical ventilation.

Continuous Noninvasive Ventilation Delivered by a Novel Total Face Mask: A Case Series Report

Noninvasive ventilation (NIV) has been widely used to decrease the complications associated with tracheal intubation in mechanically ventilated patients. However, nasal ulcerations may occur when conventional masks are used for continuous ventilation. A total face mask, which has no contact with the more sensitive areas of the face, is a possible option. We describe 3 patients with acute respiratory failure due to amyotrophic lateral sclerosis, who developed nasal bridge skin necrosis during continuous NIV, and one patient with post-extubation respiratory failure due to a high spinal cord injury, who had facial trauma with contraindication for conventional mask use. The total face mask was very well tolerated by all the patients, and permitted safe and efficient continuous NIV for several days until the acute respiratory failure episode resolved. None of the patients required endotracheal intubation during the acute episode.

Electrophrenic pacing and decannulation for high-level spinal cord injury: A case series

Abstract Background In 1997, guidelines were developed for the management of high-level ventilator-dependent patients with spinal cord injury who had little or no ventilator-free breathing ability (VFBA). This article describes the three categories of patients, the decannulation criteria, and the successful decannulation of four patients with no VFBA and electrophrenic/diaphragm pacing, using these criteria. Method Case series. Conclusion Lack of VFBA in patients with high-level spinal cord injury does not mandate tracheostomy or electrophrenic/diaphragm pacing.

Tracheostomy tubes are not needed for Duchenne muscular dystrophy

To the Editors: Respiratory failure is the main cause of death in patients with Duchenne muscular dystrophy (DMD). Some respiratory management paradigms recommend tracheostomy for ventilator-dependent DMD patients. Prolongation of survival by years and, in many cases, decades using continuous mechanical noninvasive ventilation (NIV) without tracheostomy has been reported 1. Toussaint et al. 2 reported their experience on the use of NIV along with assisted coughing to prolong life and avoid tracheostomy for patients with DMD. However, the commentary by Lofaso et al. 3 on this outstanding work was uninformed. The commentators failed to present the outcomes of continuous NIV from other centres 4–6 and they have, apparently, no experience in continuous long-term NIV. These authors seem to be unaware that more than 250 long-term (mostly 24-h dependent) NIV users whose main interface was a simple mouthpiece during the day and a mouthpiece with a plastic phalange during sleep have …

H1N1 infection and acute respiratory failure: Can we give non-invasive ventilation a chance?

Abstract In 2009, a novel H1N1 Influenza virus has emerged and on June 11 the World Health Organization declared it as pandemic. It may cause acute respiratory failure ranging from severe Acute Respiratory Distress Syndrome to exacerbations of airflow limitation. Non-invasive ventilation is now considered first-line intervention for different causes of acute respiratory failure and may be considered in the context of H1N1 pandemic. Although infection control issues have been arisen, non-invasive ventilation was effective and safe during the Severe Acute Respiratory Syndrome in Asia. It is reasonable to recommend non-invasive ventilation in H1N1-related exacerbations of chronic respiratory diseases, especially in negative-pressure wards. Treatment of early Acute Respiratory Distress Syndrome associated with H1N1 using non-invasive ventilation could be tried rapidly identifying those who fail without delaying endotracheal intubation. Considering the high demand for critical care beds during the pandemic, non-invasive ventilation may have a role in reducing the estimated load.