Jesús Sancho

Mechanical insufflation-exsufflation vs. tracheal suctioning via tracheostomy tubes for patients with amyotrophic lateral sclerosis: a pilot study.

Sancho J, Servera E, Vergara P, Marín J: Mechanical insufflation-exsufflation vs. tracheal suctioning via tracheostomy tubes for patients with amyotrophic lateral sclerosis: A pilot study. Am J Phys Med Rehabil 2003;82:750–753. Objective To compare the effects of mechanical insufflation-exsufflation vs. suctioning via tracheostomy tubes on respiratory variables for six amyotrophic lateral sclerosis patients. Design In this prospective crossover study, six consecutive patients with amyotrophic lateral sclerosis who required continuous mechanical ventilation via tracheostomy tubes and developed chest infections underwent measurement of pulse oxyhemoglobin saturation (SpO2), peak inspiratory pressure (PIP), mean airway pressure (Pawm), and work of breathing performed by the ventilator (WOBv) at baseline and 5 and 30 min after tracheal suctioning and 5 min after mechanical insufflation-exsufflation. Results The baseline values were 93.50 ± 2.26% for SpO2 in ambient air, 18.50 ± 4.23 cm H2O for PIP, 4.67 ± 1.37 cm H2O for Pawm, and 1.03 ± 0.25 J/liters for WOBv. Only WOBv changed significantly, decreasing after tracheal suctioning (P < 0.05), whereas all variables improved significantly after mechanical insufflation-exsufflation. Conclusion For ventilator-dependent patients with amyotrophic lateral sclerosis, mechanical insufflation-exsufflation via a tracheostomy tube with an inflated cuff may be more effective in eliminating airway secretions than conventional tracheal suctioning.

Alternatives to endotracheal intubation for patients with neuromuscular diseases.

Servera E, Sancho J, Zafra MJ, Catalá A, Vergara P, Marín J: Alternatives to endotracheal intubation for patients with neuromuscular diseases. Am J Phys Med Rehabil 2005;84:851–857. Objective:To evaluate the usefulness of continuous noninvasive mechanical ventilation and mechanical coughing aids to avoid endotracheal intubation and tracheostomy during episodes of acute respiratory failure in patients with neuromuscular disease. Design:We conducted a prospective cohort study at the respiratory medicine ward of a university hospital to study the success rate of the use of continuous noninvasive mechanical ventilation and manually and mechanically (CoughAssist) assisted coughing to avert endotracheal intubation in 24 consecutive episodes of acute respiratory failure for 17 patients with neuromuscular disease. The noninvasive mechanical ventilation and coughing aids were used to reverse decreases in oxyhemoglobin saturation and relieve respiratory distress that occurred despite oxygen therapy and appropriate medication. Noninvasive mechanical ventilation was delivered by volume ventilators (Breas PV 501) alternating nasal/oronasal and oral interfaces. Results:Noninvasive management was successful in averting death and endotracheal intubation in 79.2% of the acute episodes. There were no significant differences in respiratory function between the successfully treated and unsuccessfully treated groups before the current episode. Bulbar dysfunction was the independent risk factor for failure of noninvasive treatment (P < 0.05; odds ratio, 35.99%; 95% confidence interval, 1.71–757.68). Conclusions:Intubation can be avoided for some patients with neuromuscular disease in acute respiratory failure by some combination of noninvasive mechanical ventilation and mechanically assisted coughing. Severe bulbar involvement can limit the effectiveness of noninvasive management. ABBREVIATIONS: ALS: amyotrophic lateral sclerosis; ETI: endotracheal intubation; FEV1: forced expiratory volume in 1 sec; FVC: forced vital capacity; %FVC: percentage predicted FVC; MIC: maximum insufflation capacity; NIV: noninvasive ventilation; NMD: neuromuscular disease; PCF: peak cough flow; PCFMIC: manually assisted PCF; PImax: maximum mouth inspiratory pressure; PEmax: maximum expiratory pressure; SpO2: pulse oxyhemoglobin saturation

Mechanical insufflation-exsufflation: Pressure, volume, and flow relationships and the adequacy of the manufacturer's guidelines

Gómez-Merino E, Sancho J, Marín J, Servera E, Blasco ML, Belda JF, Castro C, Bach JR: Mechanical insufflation-exsufflation: Pressure, volume, and flow relationships and the adequacy of the manufacturer’s guidelines. Am J Phys Med Rehabil 2002:81;579–583. Objective Pulmonary complications of neuromuscular disease can be averted by increasing peak cough flows with the use of a forced exsufflation device. The purpose of this study was to examine the pressure, volume, and flow relationships for a range of settings generated by this device, and compare them with clinically efficacious values and the manufacturer’s guidelines. Methods The In-exsufflator was connected to a standard lung model. The resulting forced deflation volumes, flows, and pressures were averaged over 10 cycles at each setting. Results The set insufflation pressures significantly correlated with the generated insufflation pressures and volumes and the exsufflation volumes and flows. Increasing set insufflation time significantly increased generated insufflation pressures, flows, and volumes and exsufflation volumes. Increasing set exsufflation time did not significantly increase generated exsufflation flows. At set pressures of 40 to −40 cm H2O, insufflation time of 3 sec, and exsufflation time of 2 sec, the exsufflation flow was 4.09 l/sec. A plateau insufflation volume of 3.8 l was reached after 4.9 sec of insufflation. Conclusions In-exsufflator performance was very consistent. Its clinical effectiveness can be explained by its generation of exsufflation flows >2.7 l/sec. Increasing insufflation times more than exsufflation times is more important for optimal function. Current manufacturer use guidelines may not yield optimal exsufflation flows.

Tos y enfermedades neuromusculares. Manejo no invasivo de las secreciones respiratorias

Los problemas respiratorios son la primera causa de muerte en algunas enfermedades neuromusculares (ENM), y tanto los propios enfermos como sus cuidadores son conscientes de su importancia. Por ello resulta paradójico que, hasta hace muy poco tiempo, aspectos capitales de las ENM hayan sido tan infravalorados por los especialistas médicos, incluyendo los servicios de neumología. Por fortuna, en la actualidad se aprecia una sensibilización progresiva tanto en el entorno de la neurología como en las unidades de cuidados respiratorios, que en muchos casos proporcionan ya un nivel adecuado de atención. Lamentablemente, los problemas respiratorios de las ENM todavía se consideran una cuestión “emergente” en los sistemas de salud pública y reciben pocos recursos específicos. Esta situación genera una clara discriminación de los pacientes con ENM frente a otros enfermos cuyo manejo es también difícil y costoso, pero que ya disponen de un reconocimiento sociosanitario bien establecido. Para cubrir las actuales carencias, las asociaciones de enfermos y los neumólogos implicados en su tratamiento deben plantearse como objetivo inmediato conseguir la puesta en marcha de unidades especializadas en ENM, unidades que ya gozan de una larga tradición en otros países.

Noninvasive respiratory muscle aids during PEG placement in ALS patients with severe ventilatory impairment

UNLABELLED Although no clear recommendations are given about when percutaneous endoscopic gastrostomy (PEG) should be placed in amyotrophic lateral sclerosis (ALS) patients, some experts underline the risk of respiratory complications when patients had severe ventilatory muscle impairment (SVMI). AIM To evaluate the efficacy of noninvasive ventilation (NIV) and mechanically assisted cough (MAC) to avoid respiratory complications related to PEG placement in ALS patients with SVMI. MATERIAL AND METHODS Prospective study including ALS patients who had chosen to have PEG placement timed by swallowing dysfunction with the aid of NIV and MAC if needed. PEG was carried out under volume-cycled NIV through a nasal mask. MAC was applied prior to and at the end of the procedure. RESULTS Thirty ALS patients (60.43±12.03years) were included. Prior to PEG placement: BMI 25.0±4.6kg/m(2), ALSRFS-R 19.5±5.0, Norris bulbar sub-score 15.1±6.6, %FVC 35.9±18.1%, PCF 2.3±1.2L/s, PImax -35.6±24.6cmH(2)O, and PEmax 40.5±23.9cmH(2)O. Three patients had PEG placement under tracheotomy ventilation because NIV SpO(2) was below 88%. No patient died during the procedure nor did any have respiratory complications. Survival at 1month was 100%. CONCLUSION Respiratory support provided by volume-cycled NIV and MAC permits successful PEG placement in most ALS patients with SVMI.

Non-invasive ventilation effectiveness and the effect of ventilatory mode on survival in ALS patients

Abstract Non-invasive ventilation (NIV) prolongs survival in amyotrophic lateral sclerosis (ALS), but there are no data with which to compare the effectiveness of the different ventilator modes – volume (Vol-NIV) or pressure-cycled (Pres-NIV) ventilation – in ALS. We aimed to determine whether the ventilatory mode has an effect on ventilation effectiveness and survival of ALS patients using NIV. We used a retrospective study that included all ALS patients for whom NIV was indicated in two referral units: one using Vol-NIV and the other using Pres-NIV. Demographic, functional and nocturnal gas exchange parameters at NIV initiation were recorded. Eighty-two ALS patients ventilated using Pres-NIV and 62 using Vol-NIV were included. No differences were found in survival from NIV initiation between Vol-NIV (median 15.00 (7.48–22.41) months) and Pres-NIV (median 15.00 (10.25–19.75) months, p = 0.533) patients. Effective NIV was achieved in 72.41% Vol-NIV patients and in 48.78% Pres-NIV patients (p < 0.001). Ventilator mode (OR 12.066 (4.251–32.270), p < 0.001) and severity of bulbar dysfunction (OR 1.07 (1.011–1.133), p = 0.02) were the variables correlated with effective NIV. In conclusion, although Vol-NIV provides more effective ventilation, Vol-NIV and Pres-NIV present similar survival in ALS. Effectiveness of NIV is related to the severity of bulbar dysfunction.

Prolonging survival in amyotrophic lateral sclerosis: efficacy of noninvasive ventilation and uncuffed tracheostomy tubes.

Sancho J, Servera E, Bañuls P, Marín J: Prolonging survival in amyotrophic lateral sclerosis. Objective:To assess the efficacy of noninvasive ventilatory support and intermittent positive pressure ventilation via uncuffed tracheostomy tubes (uTIPPV) to prolong survival in amyotrophic lateral sclerosis. Design:Survival was prolonged by continuous noninvasive ventilatory support or TIPPV dependence. Once noninvasive ventilatory support was no longer adequate for six noninvasive ventilatory support users, they and 22 others underwent tracheotomy within 1 mo of measurement of spirometry (forced vital capacity and forced volume expired in 1 sec), peak cough flows, maximum insufflation capacity, manually assisted peak cough flows, and mechanically assisted peak cough flows. Glottic function was estimated by maximum insufflation capacity, FVC difference, and bulbar-innervated muscle function by Norris scale bulbar-innervated muscle subscore. Cuffless tubes were replaced by cuffed ones when hypoventilation developed despite increasing uTIPPV volumes. Results:The survival of 22 patients was prolonged by continuous noninvasive ventilatory support dependence for 7.8 ± 8.1 mos (range, 1–36 mos; median, 5 mos) after 13.9 ± 11.2 mos (range, 2–36 mos) of part-time noninvasive ventilatory support. Six of these and 22 others underwent tracheotomy and initially used uTIPPV effectively. For ten of the 28 (35.7%) patients, the tubes had to be replaced by cuffed ones after 5.7 ± 7.8 mos with the other 18 still using tracheostomy intermittent positive pressure ventilation volumes via uncuffed tubes for 20.2 ± 17.6 mos at data collection. Pretracheotomy FVC was significantly lower in the ten patients who eventually required cuffed tubes (0.70 ± 0.44 vs. 1.06 ± 0.43 L, P < 0.05). The bulbar-innervated muscle was significantly lower when the uTIPPV users required cuffed TIPPV than when beginning uTIPPV (Norris scale bulbar-innervated muscle subscore, 3.20 ± 1.30 vs. 8.00 ± 3.20, P < 0.05), respectively. Conclusions:Noninvasive and tracheostomy IPPV via cuffless tubes can prolong survival for patients with amyotrophic lateral sclerosis until excessive air leak and hypoventilation necessitate a cuffed tube.

Non-invasive management of an acute chest infection for a patient with ALS.

We describe a man diagnosed with non-bulbar amyotrophic lateral sclerosis (ALS) who uses 24-h non-invasive ventilator at home, and assisted cough through the use of the mechanical insufflation-exsufflation (MAC) device (CoughAssist, J.H. Emerson). This was essential for the removal of bronchial secretions in order to provide successful non-invasive management (and indeed less suffering for the patient) during an acute respiratory tract infection with hypoxemia and failure of manually assisted cough.

Predictors of Need for Noninvasive Ventilation During Respiratory Tract Infections in Medically Stable, Non-Ventilated Subjects With Amyotrophic Lateral Sclerosis

BACKGROUND: Acute lower respiratory infections can impair muscle strength in patients with amyotrophic lateral sclerosis (ALS). When associated with an increase in load on the respiratory system, this situation may precipitate hypercapnic respiratory failure in non-ventilated patients with ALS. The aim of this study was to determine whether a clinical or functional parameter can predict the need for noninvasive ventilation (NIV) during an acute respiratory infection for medically stable, non-ventilated patients with ALS. METHODS: This was a prospective study involving all non-ventilated subjects with ALS admitted due to an acute respiratory infection to a respiratory care unit from a tertiary hospital. RESULTS: Thirty-two non-ventilated subjects with ALS were admitted to our respiratory care unit due to an acute respiratory infection: 60.72 ± 10.54 y, 13 males, 23 with spinal onset, FVC of 1.58 ± 0.83 L, FVC of 56.21 ± 23.15% of predicted, peak cough flow of 3.41 ± 1.77 L/s, maximum insufflation capacity of 1.87 ± 0.94 L, revised Amyotrophic Lateral Sclerosis Functional Rating Scale score of 22.80 ± 8.83, and Norris bulbar score of 23.48 ± 12.14. Fifteen subjects required NIV during the episode. Logistic regression analysis showed that the only predictors of need for NIV were percent-of-predicted FVC (odds ratio of 1.06, 95% CI 1.01–1.11, P = .02) and peak cough flow (odds ratio of 2.57, 95% CI 1.18–5.59, P = .02). CONCLUSIONS: In medically stable, non-ventilated patients with ALS, measurement of percent-of-predicted FVC and peak cough flow can predict the need for NIV during an acute lower respiratory tract infection.

Bulbar impairment score predicts noninvasive volume-cycled ventilation failure during an acute lower respiratory tract infection in ALS

UNLABELLED Amyotrophic lateral sclerosis (ALS) patients can suffer episodes of lower respiratory tract infections (LRTI) leading to an acute respiratory failure (ARF) requiring noninvasive ventilation (NIV). AIM To determine whether clinical or functional parameters can predict noninvasive management failure during LRTI causing ARF in ALS. MATERIAL AND METHOD A prospective study involving all ALS patients with ARF requiring NIV in a Respiratory Care Unit. NIV was provided with volume-cycled ventilators. RESULTS 63 ALS patients were included (APACHE II: 14.93±3.56, Norris bulbar subscore (NBS): 18.78±9.68, ALSFRS-R: 19.90±6.98, %FVC: 40.01±18.07%, MIC: 1.62±0.74L, PCF 2.51±1.15L/s, PImax -34.90±19.44cmH2O, PEmax 51.20±28.84cmH2O). In 73.0% of patients NIV was successful in averting death or endotracheal intubation. Differences were found between the success and failure in the NBS (22.08±6.15 vs 8.66±3.39, p<0.001), ALSFRS (22.08±6.11 vs 12.71±4.39, p<0.001), PCFMI-E (3.85±0.77 vs 2.81±0.91L/s, p=0.007) and ALS onset (spinal/bulbar 33/13 vs 7/10, p=0.03). The predictor of NIV failure was the NBS (OR 0.53, 95% CI 0.31-0.92, p 0.002) with a cut-off point of 12 (S 0.93; E 0.97; PPV 0.76; NPV 0.97). CONCLUSIONS NBS can predict noninvasive management failure during LRTI in ALS.