Disagreement About Surgical Intervention in Trisomy 18.

In this case, we explore physician conflict with performing surgery (tracheostomy) for long-term ventilation in a term infant with trisomy 18 and respiratory failure. Experts in neonatal-perinatal medicine, pediatric bioethics, and pediatric palliative care have provided comments on this case. An additional commentary was written by the parent of another infant with trisomy 18, who is also a medical provider (physical therapist).

Disparities and ethical considerations for children with tracheostomies during the COVID-19 pandemic.

The COVID-19 pandemic is exacerbating longstanding challenges facing children with tracheostomies and their families. Myriad ethical concerns arising in the long-term care of children with tracheostomies during the COVID-19 pandemic revolve around inadequate access to care, healthcare resources, and rehabilitation services. Marginalized communities such as those from Black and Hispanic origins face disproportionate chronic illness because of racial and other underlying disparities. In this paper, we describe how these disparities also present challenges to children who are technology-dependent, such as those with tracheostomies and discuss the emerging ethical discourse regarding healthcare and resource access for this population during the pandemic.

The Charlie Gard Case, and the Ethics of Obstructing International Transfer of Seriously Ill Children.

In 2017, the court case over medical treatment of UK infant, Charlie Gard, reached global attention. In this article, I will analyze one of the more distinctive elements of the case. The UK courts concluded that treatment of Charlie Gard was not in his best interests and that it would be permissible to withdraw life-sustaining treatment. However, in addition, the court ruled that Charlie should not be transferred overseas for the treatment that his parents sought, even though specialists in Italy and the US were willing to provide that treatment. Is it ethical to prevent parents from pursuing life-prolonging treatment overseas for their children? If so, when is it ethical to do this? I will outline arguments in defense of obstructing transfer in some situations. I will argue, however, that this is only justified if there is good reason to think that the proposed treatment would cause harm.

To trach or not to trach, that is the question.

Progressive neuromuscular disease requires increasing degrees of respiratory support to sustain life. Each step from intermittent to continuous-and noninvasive to invasive-ventilation requires thoughtful consideration based on the goals of the patient and family, and the inherent benefits and burdens of the treatment. Tracheostomy, in particular, should not be viewed as an inevitable next step when less permanent or invasive methods prove insufficient. Like other modes of respiratory support, tracheostomy may represent a bridge to recovery of pulmonary function, or a stabilizing action in the hope that novel therapies may prove beneficial. In other situations, tracheostomy represents a destination therapy, necessitating consideration of the implications of chronic mechanical ventilation. Institutional, social, and financial considerations may affect decisions related to tracheostomy, as may implicit bias regarding quality of life. The complexity of such care and decisions highlight the need for optimal palliative care throughout the patient's life.

Ethical challenges in tracheostomy-assisted ventilation in amyotrophic lateral sclerosis.

The special nature of amyotrophic lateral sclerosis (ALS) and tracheostomy with invasive ventilation (TIV) leads to challenges that can be difficult in two senses: not only to handle well, but also to discuss with patients and other involved stakeholders. Because of the delicate nature of interpersonal relations and communication in ALS, some of the downsides to TIV may almost take on a nature of taboo, making them difficult to raise for open discussion. Yet these ethical challenges are important to be aware of, not only for health professionals and managers but, arguably, also for patients and next of kin. They are important also for a wider professional and societal debate about whether and to whom TIV should be offered. In this paper we highlight and examine ethical challenges in TIV for ALS, with a special emphasis on those that are hard to discuss openly and that therefore might fail to be addressed. The analysis is structured by the four core principles of healthcare ethics: beneficence, nonmaleficence, respect for patient autonomy, and justice.

How Should Refusal of Tracheostomy as Part of an Adolescent's Perioperative Planned Intubation Be Regarded?

Here we present a case of a patient in terminal respiratory failure refusing to consent to emergent tracheostomy in the setting of an anticipated difficult intubation. We examine ethical concerns that arise from deviations from the standard of care in the operative setting and the anesthesiologist's sense of culpability. Finally, we will review the ethical arguments and guidelines that support anesthesiologists' participation in palliative operative procedures when limitations on resuscitation are in place.

Should Physicians New to a Case Counsel Patients and Their Families to Change Course at the End of Life?

Although new cancer therapies have changed the prognosis for some patients with advanced malignancies, the potential benefit for an individual patient remains difficult to predict. This uncertainty has impacted goals-of-care discussions for oncology patients during critical illness. Physicians need to have transparent discussions about end-of-life care options that explore different perspectives and acknowledge uncertainty. Considering a case of a new physician's objections to an established care plan that prioritizes comfort measures, we review physician practice variation, clinical momentum, and possible moral objections. We explore how to approach such conflict and discuss whether and when it is appropriate for physicians new to a case to challenge established goals of care.

Traqueostomia no doente crítico na era do consentimento livre e esclarecido / Tracheostomy in critically ill patients in the era of informed consent / Traqueostomía en el paciente crítico en la era del consentimiento libre e informado

Resumo A traqueostomia é procedimento frequentemente realizado em doentes críticos com ventilação mecânica prolongada ou presumidamente prolongada, embora suas indicações, benefícios e riscos sejam controversos. O termo de consentimento livre e esclarecido é necessário para procedimentos cirúrgicos e tem sido amplamente instituído, devendo ser obtido antes da traqueostomia em pacientes críticos. Este artigo faz revisão narrativa das indicações do procedimento e, considerando-o no caso de doentes críticos, aborda a aplicação do termo de consentimento livre e esclarecido. Discutiram-se aspectos teóricos; o que deve constar nos documentos escritos; o que deve ser verbalizado para os doentes e seus familiares, além de outros aspectos práticos. Concluiu-se que os atuais termos de consentimento para traqueostomia em doente crítico não privilegiam a autonomia, pois evitam alocação de recursos para indicação do procedimento.

Abstract Although tracheostomies are often performed in critical patients with prolonged or presumed prolonged mechanical ventilation, the recommendation, benefits and risks of the procedure remain controversial. Informed consent is widely established as a necessary process in surgical procedures and should be obtained prior to the performing of a tracheostomy. The present article provides a narrative review of the process of the medical recommendation of this procedure and, through the use of the tracheostomy in the critical patient, addresses the application of the informed consent term. Theoretical aspects are discussed, such as what should be included in written documents and what should be verbally explained to patients and their families, together with other practical aspects. It was found that the current terms of consent for tracheostomies in critical patients do not prioritize autonomy, as they avoid the allocation of the resources necessary for the recommendation of the procedure.

Resumen La traqueotomía es un procedimiento frecuentemente realizado en pacientes críticos con ventilación mecánica prolongada o presumiblemente prolongada, aunque sus indicaciones, beneficios y riesgos sean controvertidos. El documento de consentimiento libre e informado es necesario para la realización de procedimientos quirúrgicos y ha sido ampliamente instituido, debiendo ser obtenido antes de la traqueostomía en pacientes críticos. El presente artículo hace una revisión narrativa de las indicaciones de este procedimiento y, considerándolo en el caso de pacientes críticos, aborda la aplicación del documento de consentimiento libre e informado. Se discutieron aspectos teóricos; lo que debe constar en los documentos escritos; lo que debe ser verbalizado a los enfermos y a sus familiares, además de otros aspectos prácticos. Se concluye que los actuales documentos de consentimiento para traqueostomía en el paciente crítico no privilegian la autonomía, pues evitan la asignación de recursos para la indicación del procedimiento.

Ethical considerations with the management of congenital central hypoventilation syndrome.

Congenital central hypoventilation syndrome (CCHS) is a well-recognized disorder of the autonomic nervous system caused by mutations in the PHOX2B gene. The most characteristic feature is failure of ventilatory control, resulting in the need for respiratory support while asleep, and in some cases when awake also. Most cases present in infancy or early childhood. Technological advances allow patients with mild to moderate phenotypesto receive adequate support by non-invasive ventilation (NIV), or diaphragm pacing (or combination of the two) avoiding the need for long-term ventilation by tracheostomy. Daytime functioning of patients with CCHS who require sleep-time ventilation only is expected to be good, with some additional surveillance to ensure they don't accidentally fall asleep without respiratory support available. Some children with CCHS have other complications, such as Hirschprung's disease, learning difficulties, and cardiac arrhythmias (leading in some instances to heart block and the requirement for a pacemaker). In a few cases, patients can develop neurogenic malignancies. Parents bear a significant burden for the care of their child with CCHS including provision of NIV at home, close monitoring, and regular surveillance for complications. Information about patients with CCHS comes from databases in the United States and Europe, but these don't include infants or children for whom ventilator support was not offered. In this paper we use a case study to explore the ethical issues of provision of treatment, or non-treatment, of children with CCHS.

Ethical issues in laryngology: tracheal stenting as palliative care.

OBJECTIVES/HYPOTHESIS: To improve understanding of aspects of end-of-life care that may not be intuitive to the otolaryngology community. DATA SOURCES AND REVIEW METHODS: A comprehensive review of the literature was performed by searching Medline, Embase, and Google Scholar databases. Primary manuscripts' bibliographies were reviewed to identify any nonindexed references. Prospective consultation by means of one-on-one interviews was sought from nonotolaryngology key stakeholders in the areas of hospice nursing care and patient advocacy in order to identify pertinent issues. RESULTS: We identified over 1,000 articles published from 1965 to 2013 on the topic of tracheal stents, as well as over 40,000 on hospice/end-of-life care. Three articles focusing specifically on palliative care and airway stenting were identified, of which three were case reports and none were definitive reviews. There are a number of significant issues and concepts unique to hospice care. These are likely unfamiliar to all except for head and neck oncology-specialized otolaryngologists. An example is that hospice care focuses on quality of life rather than prolongation of life (such as curative surgery). Patients with nonoperable tracheal obstruction from malignancy face an unpleasant demise from suffocation. For those patients, stenting can relieve suffering by restoring airway patency. CONCLUSIONS: Airway stenting can be a valid palliative care option, even for terminal patients receiving hospice care, when performed to relieve airway obstruction and improve quality of life. End-of-life ethics is an underdeveloped area of otolaryngology that should be explored.

Is tracheostomy still an option in amyotrophic lateral sclerosis? Reflections of a multidisciplinary work group.

QUESTION UNDER STUDY: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease with a poor prognosis. Survival and quality of life of ALS patients have improved through the implementation of multidisciplinary approaches, the use of percutaneous gastrostomy and of noninvasive (NIV) or invasive ventilation. The question of whether or not to propose invasive ventilation (by tracheostomy: TPPV) to ALS patients remains a matter of debate. METHODS: The study reviews the medical literature, the practice in three Swiss and two large French ALS expert centres and reports the results of a workgroup on invasive ventilation in ALS. RESULTS: Improved management of secretions and use of different interfaces allows NIV to be used 24-hours-a-day for prolonged periods, thus avoiding TPPV in many cases. TPPV is frequently initiated in emergency situations with lack of prior informed consent. TPPV appears associated with a lesser quality of life and a higher risk of institutionalisation than NIV. The high burden placed on caregivers who manage ALS patients is a major problem with a clear impact on their quality of life. CONCLUSIONS: Current practice in Switzerland and France tends to discourage the use of TPPV in ALS. Fear of a "locked-in syndrome", the high burden placed on caregivers, and unmasking cognitive disorders occurring in the evolution of ALS are some of the caveats when considering TPPV. Most decisions about TPPV are taken in emergency situations in the absence of advance directives. One exception is that of young motivated patients with predominantly bulbar disease who "fail" NIV.

The threshold moment: ethical tensions surrounding decision making on tracheostomy for patients in the intensive care unit.

With the aging of the general population and the ability of intensivists to support patients using ventilator support, tracheostomy has become a vital tool in the medical management of critically ill patients. While much of the medical literature on tracheostomy has focused on the optimal timing of and indications for performing this procedure, little is written on the ethical tensions that can revolve around decisions by patients, surrogates, and physicians on its use.This article will elucidate the ethical dilemmas that can arise surrounding the use of tracheostomy in critically ill patients and how ethics consultants and committees can approach these cases to allow resolution.

Tracheotomy and children with spinal muscular atrophy type 1: ethical considerations in the French context.

Spinal muscular atrophy (SMA) type 1 is a genetic neuromuscular disease in children that leads to degeneration of spinal cord motor neurons. This sometimes results in severe muscular paralysis requiring mechanical ventilation to sustain the child's life. The onset of SMA type 1, the most severe form of the disease, is during the first year of life. These children become severely paralysed, but retain their intellectual capacity. Ethical concerns arise when mechanical ventilation becomes necessary for survival. When professionals assess the resulting life for the child and family, they sometimes fear it will result in unreasonably excessive care. The aim of this article is to present an analysis of ethical arguments that could support or oppose the provision of invasive ventilation in this population. This examination is particularly relevant as France is one of the few countries performing tracheotomies and mechanical ventilation for this condition.

International survey of physician recommendation for tracheostomy for Spinal Muscular Atrophy Type I.

The ethics of invasive mechanical ventilation for children with the neurodegenerative disease Spinal Muscular Atrophy Type I (SMA I) is highly debated, and wide variability in clinical outcomes exists internationally. We conducted this international survey to identify physician characteristics associated with recommendation for tracheostomy and ventilation for SMA I. A cross-sectional online survey was distributed to 1,772 pediatric pulmonologists and pediatric intensivists from online membership directories of American Thoracic Society, American College of Chest Physicians, and European Respiratory Society. Questions explored physician demographics, attitudes and experience with SMA and end-of-life care, knowledge of consensus guidelines, and recommendations for respiratory care of SMA I. A logistic regression model assessed the independent effects of physician variables on the recommendation for invasive ventilation for SMA I. A total of 367 (21%) physicians completed the survey; 82% were pediatric pulmonologists; and 16% pediatric intensivists. Seventy percent of respondents were from the U.S. Fifty percent of physicians were aware of SMA consensus guidelines. Physicians from Commonwealth countries (U.K., Canada, Australia, etc.) were less likely to recommend tracheostomy/ventilation than U.S. physicians (7% vs. 25%, P = 0.005). Logistic regression modeling identified years of experience, pediatric pulmonology specialty, agreement with a pro-life statement, and recommendation for non-invasive ventilation as predictive of recommendation for long-term invasive ventilation for SMA I. In the largest international survey on this topic, we identified regional differences in physician recommendation for invasive ventilation for children with SMA I. Our data demonstrate a need for increased awareness of consensus guidelines and further dialog about the physician role in variability of care for children with SMA I.

[Non invasive ventilation in the ICU for the neuromuscular patient: ethical issues]. / Ventilation non invasive et insuffisance respiratoire chronique d'origine neuromusculaire: implications pour l'intensiviste. Aspects ethiques.

Non-invasive mechanical ventilation in patients with chronic neuromuscular disorders is an effective tool for treating dyspnea or sleep disturbances often observed in such patients. So, NIV has to be considered as a palliative treatment and it must systematically be offered to these patients. Mechanical ventilation, non-invasive or invasive (via a trachesotomy) have to be considered systematically with patients and families at an earlier stages of these diseases in order to design a strategy in case of acute respiratory failure. In a second parent paper of this issue, we discuss the medico-legal implications of mechanical ventilation in neuromuscular failure, particularly the end-of-life aspects.