Continuous noninvasive ventilatory support outcomes for patients with neuromuscular disease: a multicenter data collaboration.

BACKGROUND: Typically, patients with progressive neuromuscular disorders (NMDs) develop acute respiratory failure (ARF), are intubated, and when failing spontaneous breathing trials (SBTs) undergo a tracheotomy and receive tracheostomy mechanical ventilation (TMV). However, increasing numbers of patients use nasal noninvasive ventilation (NIV), initially for sleep and this is extended to continuous dependence (CNVS). This can be used as a strategy to assist in successful extubation . We retrospectively reviewed 19 centers offering CNVS and mechanical insufflation-exsufflation (MI-E) as an alternative to TMV. METHODS: Centers with publications or presentations concerning CNVS outcomes data were pooled for amyotrophic lateral sclerosis (ALS), Duchenne muscular dystrophy (DMD), and spinal muscular atrophy type 1 (SMA1). Progression to CNVS dependence without hospitalization, duration of dependence, and extubations and decannulations to CNVS were recorded. Prolongation of life was defined by duration of CNVS dependence without ventilator free breathing ability (VFBA). RESULTS: There were 1623 part time (<23 h/day) NVS users with ALS, DMD, and SMA1 from 19 centers in 16 countries of whom 761 (47%) were CNVS dependent for 2218 patient-years. This included: 335 ALS patients for a mean 1.2 ± 1.0 (range to 8) years each; 385 DMD patients for 5.4 ± 1.6 (range to 29) years; and 41 SMA1 patients for 5.9 ± 1.8 (range to 20) years. Thirty-five DMD and ALS TMV users were decannulated to CNVS and MI-E. At data collection 494 (65%) patients were CNVS dependent but 110 (74 of whom with bulbar ALS), had undergone tracheotomies. CONCLUSIONS: ALS, DMD, and SMA1 patients can become CNVS dependent without requiring hospitalization but CNVS cannot be used indefinitely for many patients with advanced upper motor neuron diseases.

Noninvasive ventilatory support in morbid obesity.

BACKGROUND: In the conventional management of the morbidly obese that normalizes the apnea-hypopnea index (AHI), CO2 levels often remain elevated. METHODS: A retrospective review of morbidly obese patients using volume preset settings up to 1800ml to positive inspiratory pressures (PIPs) of 25-55cm H2O, or pressure control at 25-50cm H2O pressure via noninvasive interfaces up to continuously (CNVS). RESULTS: Twenty-six patients, mean 55.6±14.8 years of age, weight 108-229kg, mean BMI 56.1 (35.5-77)kg/m2, mean AHI 69.0±24.9, depended on up to CNVS for 3 weeks to up to 66 years. There were eleven extubations and seven decannulations to CNVS despite failure to pass spontaneous breathing trials. Thirteen were CNVS dependent for 92.2 patient-years with little to no ventilator free breathing ability (VFBA). Six used NVS from 10 to 23h a day, and others only for sleep. Fifteen patients with cough peak flows (CPF) less than 270L/m had access to mechanical insufflation-exsufflation (MIE) in the peri-extubation/decannulation period and long-term. The daytime end-tidal (Et)CO2 of 14 who were placed on sleep NVS without extubation or decannulation to it decreased from mean EtCO2 61.0±9.3-38.5±3.6mm Hg and AHI normalized to 2.2. Blood gas levels were normal while using NVS/CNVS. Pre-intubation PaCO2 levels, when measured, were as high as 183mm Hg before extubation to CNVS. CONCLUSIONS: Ventilator unweanable morbidly obese patients can be safely extubated/decannulated and maintained indefinitely using up to CNVS rather than resort to tracheotomies.

Noninvasive ventilatory support to reverse weight loss in Duchenne muscular dystrophy: A case series.

This case series of five patients with Duchenne muscular dystrophy demonstrates the nutritional advantages of instituting noninvasive intermittent positive pressure ventilatory support via 15mm angled mouthpieces to relieve tachypnea and provide more time to swallow food safely. In each case weight loss was reversed.

Quantitation of oxygen-induced hypercapnia in respiratory pump failure.

PURPOSE: To determine the likelihood that clinicians know carbon dioxide levels before administering supplemental oxygen to patients with neuromuscular disorders, to quantitate the effect of oxygen therapy on carbon dioxide retention, and to explore hypercapnia contributing to the need to intubate and use of continuous noninvasive ventilatory support to avert it. BASIC PROCEDURES: A retrospective chart review for patients with neuromuscular disorders intubated or having intubation averted by using continuous noninvasive ventilatory support with carbon dioxide known pre- and during oxygen administration. MAIN FINDINGS: For only 2 of 316 patients who were intubated did clinicians know carbon dioxide levels prior to administering oxygen. For four cases, intubation was averted by continuous noninvasive ventilatory support and mechanical insufflation-exsufflation despite severe hypercapnia and acidosis. After initiating oxygen therapy, patients' carbon dioxide partial pressures increased 52.1±42.0mmHg in over as little as 20min. PRINCIPAL CONCLUSIONS: Clinicians should attempt to use continuous noninvasive ventilatory support and mechanical insufflation-exsufflation rather than supplemental oxygen to normalize blood gases for neuromuscular ventilatory failure and should be prepared to intubate hypercapnic patients for whom oxygen is administered.

Open circuit mouthpiece ventilation: Concise clinical review.

In 2013 new "mouthpiece ventilation" modes are being introduced to commercially available portable ventilators. Despite this, there is little knowledge of how to use noninvasive intermittent positive pressure ventilation (NIV) as opposed to bi-level positive airway pressure (PAP) and both have almost exclusively been reported to have been used via nasal or oro-nasal interfaces rather than via a simple mouthpiece. Non-invasive ventilation is often reported as failing because of airway secretion encumbrance, because of hypercapnia due to inadequate bi-level PAP settings, or poor interface tolerance. The latter can be caused by factors such as excessive pressure on the face from poor fit, excessive oral air leak, anxiety, claustrophobia, and patient-ventilator dys-synchrony. Thus, the interface plays a crucial role in tolerance and effectiveness. Interfaces that cover the nose and/or nose and mouth (oro-nasal) are the most commonly used but are more likely to cause skin breakdown and claustrophobia. Most associated drawbacks can be avoided by using mouthpiece NIV. Open-circuit mouthpiece NIV is being used by large populations in some centers for daytime ventilatory support and complements nocturnal NIV via "mask" interfaces for nocturnal ventilatory support. Mouthpiece NIV is also being used for sleep with the mouthpiece fixed in place by a lip-covering flange. Small 15 and 22mm angled mouthpieces and straw-type mouthpieces are the most commonly used. NIV via mouthpiece is being used as an effective alternative to ventilatory support via tracheostomy tube (TMV) and is associated with a reduced risk of pneumonias and other respiratory complications. Its use facilitates "air-stacking" to improve cough, speech, and pulmonary compliance, all of which better maintain quality of life for patients with neuromuscular diseases (NMDs) than the invasive alternatives. Considering these benefits and the new availability of mouthpiece ventilator modes, wider knowledge of this technique is now warranted. This review highlights the indications, techniques, advantages and disadvantages of mouthpiece NIV.

Physical medicine respiratory muscle aids to avert respiratory complications of pediatric chest wall and vertebral deformity and muscle dysfunction.

The purpose of this article was to describe the use of noninvasive inspiratory and expiratory muscle aids to prevent chest wall deformities including pectus excavatum, to prevent respiratory complications of vertebral surgery, to prevent acute and long-term ventilatory insufficiency and failure in children with paralytic disorders who develop these deformities, and to permit the extubation and tracheostomy tube decanulation of "unweanable" patients. Noninvasive airway pressure aids can provide up to continuous ventilator support for patients with little or no vital capacity and can provide for effective cough flows for patients with severely dysfunctional expiratory muscles. An April 2010 consensus of clinicians from 20 centers in 14 countries reported over 1500 spinal muscular atrophy type 1 (SMA1), Duchenne muscular dystrophy (DMD), and amyotrophic lateral sclerosis (ALS) patients who survived using continuous ventilatory support without tracheostomy tubes. Four of the centers routinely extubated unweanable DMD patients so that none of their over 250 such patients has undergone tracheotomy.