Impediments to Humane Noninvasive Management
Despite the fact that many children with neuromuscular disorders (NMD) can become so weak that they can require continuous ventilatory support, few if any ever require tracheostomy tubes for it. However, with physicians being experienced only in bi-level PAP and invasive management (and the financial incentives favoring the latter), with the fact that patient support organizations like the Parents of Duchenne muscular dystrophy, Congenital Myopathies, Families of SMA, and SMA and ALS Foundations have ignored noninvasive management until recently and continue to fail to recognize definitive management with continuous noninvasive ventilatory support (CNVS), virtually all patients not managed by "Centers of Noninvasive Respiratory Management" will eventually develop acute respiratory failure and get invasive airway tubes. In 2013, Philips-Respironics put mouthpiece NVS mode software into their Trilogy[TM] ventilator. This has generated interest in CNVS at least in Europe where noninvasive management is not financially disincentivized.
Considering the financial incentives for physicians, hospitals, and other institutions for placing invasive airway tubes for tracheostomy mechanical ventilation (TMV) and the fact that healthcare professionals are taught as students that ventilatory support can only be provided via invasive tubes, despite numerous publications that indicate the desirability, quality of life advantages, and preference of ventilator users for noninvasive methods rather than TMV, physicians tend to rationalize to themselves the desirability of traching people to themselves by permitting themselves to think that patients are just as happy with trach tubes as without them (JBCV31,JBCV66,JBCV73,JBCV75,JBCV162). Indeed, because the docs who think this way have no experience in using optimally using NVS and MIE, their patients do in fact become better off with trach tubes. For example, without CNVS including that administered via mouthpieces and MIE used at adequate pressures, their patients would be dyspneic without tracheostomy tubes. In a recent response to a letter to the editor pointing out that myopathy patients do not need tracheostomy tubes for ventilatory support (MC1), the authors wrote, “While we strive to minimize the use of invasive therapies when possible, an invasive interface does not necessarily worsen the perceived quality of life of many patients with ventilator-dependent neuromuscular disease. Our philosophy is to fully inform families of the benefits and potential adverse effects of medical therapies..." without having any experience in using CNVS, MIE, or extubation to CNVS and MIE, "...including the use and interface for assisted ventilation. We strive to respect, rather than judge the decisions families and their providers must make in the most difficult of circumstances," circumstances made more difficult by lack of knowledge and experience with CNVS and MIE" (Impediments1). Thus, besides not having incentive to learn noninvasive management, most physicians do not understand it.
A spinoff of this, especially for children, is the especially unfortunate impediment to noninvasive management due to the advice given to patients and their families by parents who, disdaining the idea of having consented to their children undergoing tracheotomy but being told by all their physicians that there was no alternative, gave in to it and subsequently may feel guilty for having done so. While their children became habituated to the tubes, they never benefitted from optimal noninvasive management and so got to believe that they were better off with them. Once the mind makes a decision, it fabricates rationales to justify it. Since currently the majority of continuously ventilator dependent children have tracheostomy tubes, some parents canvas their respective organizations to give tracheotomies "equal time" in their literature, since convincing others that tracheotomy is warranted supports their rationales and may assuage guilt. For example, the Care of Congenital Myopathies group’s “A Guide for Families” literature, after more comprehensively discussing noninvasive management than any other NMD patient network, notes that tracheostomy “may be necessary in people who need long-term mechanical ventilation and for whom non-invasive methods were not successful..." something that we have not seen in over 1500 patients. "Sometimes a tracheostomy is temporary, such as after a surgery or serious illness..." even though not needed because extubation to CNVS could have been done, "...and can be removed later. Throat muscle weakness and all-day breathing assistance needs are two of the most primary reasons for choosing a tracheostomy over non-invasive ventilation..." when physicians do not offer CNVS and MIE. "The decision to have a tracheostomy or not should be considered carefully by your family and the medical team..." even though no members of it are able to extubate, decanulate, or know how to manage ventilator users noninvasively. "A tracheostomy may be a necessary life-saving procedure for some people with CM..." who have physicians lacking knowledge of noninvasive management.
Once tracheostomy tubes are placed and small children become continuously TMV dependent because of it (JBCV193), 4 out of 5 patients (at least those with ALS) eventually die due to complications of the tube (JBCV63), and hospitalizations and respiratory complications are far more frequent for these people than for people managed noninvasively (JBCV126). No one prefers to have tracheostomy tubes for lung ventilation rather than NVS or CNVS unless they were never correctly managed by NVS (as opposed to bi-level PAP) and MIE (JBCV66). Perhaps one of the worst things about tracheotomy tubes, however, is that once placing a tube, many people who had no throat muscle weakness at all then aspirate airway secretions and food and can require gastrostomy tubes because of it. Then, even if the tube is removed, they many continue to aspirate and have difficulty swallowing for the rest of their lives. Tracheostomy tubes cause dysphagia and aspiration (Impediments2). This can also impede weaning. Unlike NMD patients who almost invariably have at least some dysphagia even before they require NVS, SCI patients do not. Kirshblum et al. found that risk factors for dysphagia were age, anterior cervical spine surgery, and tracheostomy and TMV with the latter upon admission to a rehabilitation facility being the strongest predictor. The patients were aspirating or required a modified diet. While 7.5% of patients with no history of tracheostomy had dysphagia, 20% did whose tubes were removed before admission and 38.3% with tracheostomy tubes on admission had it (Impediments3). In addition, 41.7% (5 of 12) of non-ventilator users with tracheostomies at admission had dysphagia. The incidence of aspiration associated with tracheostomy tubes has been reported to be 7% to 87% and is believed to be due to glottis injury due to preceding intubation, fixing the trachea to the skin, diminished laryngeal movement and uncoordinated closure, loss of protective reflexes and proprioception, esophageal and hypopharyngeal obstruction by tube cuff pressure. Thirty-seven precent of patients with tracheostomies in place on admission were aspirating. Breathing with the tube open was reported to further increase aspiration possibly due to decreased subglottic pressure. Only half of the patients aspirated when the tube was occluded. In two other studies, about 80% of 77 trached ventilator users had abnormal modified Barium swallow exams and over 50% were aspirating (Impediments4,Impediments5).