Spinal muscular atrophy type 1 (SMA-1), also known as Werdnig–Hoffman disease, is a severe neuromuscular disease with an autosomal recessive pattern of inheritance in which degeneration of spinal motor neurons results in progressive and irreversible muscular atrophy. Even though patients can maintain respiratory function in the early months of life, most die within 1 year if they do not receive some type of mechanical ventilatory support.1,2

The decision of initiating or withholding this treatment carries important ethical implications and raises considerable doubts in the health professionals involved in the care of these patients in any capacity (neonatologists, neuropaediatricians, intensive care paediatricians, primary care paediatricians, nursing staff, psychologists etc.).3 A few years ago, the ethical dilemma involved choosing between connecting the patient to an invasive mechanical ventilation system (initially through an endotracheal tube and subsequently by means of tracheotomy) or to limit treatment efforts to comfort measures and terminal sedation. At present, the use of noninvasive ventilation by means of some type of facial interface is a third option.4,5

In cases of children with an SMA-1 diagnosis, the decision-making process should include honest information and take into account the natural history of the disease, the opinions of the family, the long-term respiratory and nutritional support options, and care options that limit life-sustaining support. The healthcare team should be interdisciplinary and include the paediatrician in charge and other professionals acting in coordination. At times, the dilemma arises because patients visit the clinic presenting with respiratory failure and neither the family nor the paediatrician know how care should be approached at that juncture.

In recent years, advanced simulation has been used as an interactive method to aid the practical training of health professionals.6–8 Simulation also allows the analysis of common errors made by participants and the identification of knowledge gaps that need to be remedied.9–11 While simulation can be used to teach providers how to manage situations that pose ethical dilemmas, we do not know of any studies that have analysed this subject.

Our hypothesis was that primary care paediatricians (PCPs) have difficulty handling cases in which the main clinical issue is of an ethical nature, and the aim of our study was to assess the approach used by PCPs in a case that poses an ethical dilemma (infant with SMA-1 presenting with respiratory failure) using a simulated environment.

We generated a scenario of acute respiratory failure in an infant with SMA-1 and included it in the programme of continuing education with advanced simulation for PCPs promoted by the Sociedad Española de Pediatría de Atención Primaria (Spanish Society of Primary Care Paediatrics).8 In the context of the course curriculum, the educational goal of this particular scenario was to train paediatricians to assess not only the clinical and technical aspects of the case, but also its ethical aspects. The advanced simulation system and educational methodology used have been described in previous publications.8–10

Most teams (85%) posed the ethical dilemma to the parents, although only 29% did so of their own accord, with the “parents” broaching the subject in the rest of the scenarios. Eighty-eight percent did not explore whether the parents knew the diagnosis and the short-and long-term prognosis of the disease.

Of all teams, only 11.7% asked the parents about the previous clinical condition of the child and his quality of life. Only two teams (6%) involved the parents in the decision-making process and took into account their opinion, while 23% actively excluded the family from the process.

None of the teams explained the different treatment options that could be considered for a patient with SMA-1 with respiratory failure to the family, nor attempted to contact the physician in charge of the patient to find out what had been previously discussed or the care plan that had been agreed on for situations like the one presented in the simulated scenario.

In the event of respiratory failure, all teams initiated oxygen therapy after 1.7±0.6min. When it came to decisions relating to respiratory support, 94% of the teams started ventilation with a self-inflating bag and facial mask, and 62% proceeded to perform orotracheal intubation followed by ventilation with a self-inflating bag.

As paediatricians, we should have the necessary skills to adequately manage acute conditions that may emerge in clinical practice from a scientific, technical and also ethical standpoint. While education and clinical experience lay the foundation for these skills, it is difficult to learn how to manage certain situations because they rarely present themselves.6,8 Advance simulation is a valuable teaching tool that allows the generation of scenarios in a controlled environment to facilitate acquisition of practical skills by health professionals without posing risks to patients.6,7,11,12 For the most part, advanced medical simulation programmes have focused on technical aspects and in procedures to be performed in emergencies, possibly to address the needs perceived by educators and participants, with a lesser emphasis on non-technical skills.12

The aim of our study was to contribute information on the approach of PCPs to a simulated clinical case in which the main concerns were ethical in nature. To this end, we chose to simulate a case of an infant with SMA-1 presenting with respiratory failure, as this disease is a good model of ethical conflict in decision-making relative to respiratory support.1–4 While there have been attempts to reach an international consensus on the subject, the attitudes of health professionals and families are diverse, so that any of the three possible courses of action (invasive ventilation, noninvasive ventilation and limitation of treatment) may be taken.1,2,5 In specific real-life cases, decision-making is not easy and is usually preceded by a process that includes diagnosis and informing the family about the prognosis of the disease and available treatment options, expected quality of life and technical, psychological and social support options.13,14 In this process, the establishment and communication of a negotiated care plan is one of the objectives that need to be pursued so that every health professional that has to provide care for the patient for any reason knows what needs to be done. Thus, rather than studying the choices made by PCPs in an emergency, our aim was to assess how they approached the ethical challenge of informing the parents, involving them in the decision-making process and implementing the decisions previously agreed on by the healthcare team and the family.

We observed that less than a third of the teams posed the ethical dilemma to the parents of their own accord, with parents otherwise having to bring up the subject with the clinicians. Furthermore, the parents were rarely engaged in the decision-making process either to learn how well informed they were and what their expectations were prior to the clinical deterioration of the child, or to take their opinion into account at the time of the visit. While PCPs may know and provide care for patients with complex conditions, they are not usually primarily responsible for managing the care of these patients nor for making this type of decisions, which are usually the responsibility of specialised colleagues that work in hospital settings. However, we observed that none of the teams asked whether there was a care plan that had been negotiated by the paediatrician in charge and the parents, a piece of information that would be very helpful in decision-making in the event of acute respiratory failure.

On the other hand, when it came to the respiratory care of the patient, all PCP teams administered oxygen therapy correctly and promptly, initiating positive pressure ventilation with self-inflating bags and facial masks followed by endotracheal intubation in 62% of the cases, which demonstrated their ability to quickly deliver appropriate care in instances of acute respiratory failure in infants.

Our study shows that our role as paediatricians requires further theoretical and practical training on the subject of care ethics, shared decision-making and the clinical relationship. The scientific knowledge and technological resources that can be applied to the diagnosis and treatment of our patients keep growing, but it seems that these advances have not been matched by advances in training and resources to address the ethical aspects that arise in the context of the clinical relationship.15

There are some limitations to our study. On one hand, there are the limitations resulting from conducting a study in a simulated and controlled environment, as its results may be biased by the methodology and thus cannot be directly extrapolated to clinical practice. On the other hand, while the chosen scenario is considered a good model of ethical conflict, it has particular connotations that prevent the generalisation of the results. In any case, this limitation would apply regardless of which scenario were chosen.

Since the training programme that incorporated the use of advanced simulation was aimed at PCPs, our results cannot be extrapolated to other groups of paediatricians that work in hospital settings or with subspecialty training. In fact, it would be interesting to reproduce the same study in hospitalists (not only paediatricians but also nursing staff) to learn how they approach this conflict. Although the assessment of the actions taken was systematic and involved the repeated reproduction of audio and video recordings, it was conducted by a single researcher, which may have biased the results.

In conclusion, when presented with a simulated case in which the main clinical issue was of an ethical nature, PCPs had difficulty interacting with the family and involving the family in decision-making. The practical training of paediatricians should include scenarios with ethical dilemmas.

The authors have no conflicts of interest to declare.