This ethics case study presents a 16-year-old girl Kelly with spinal muscular dystrophy (SMA) type 1 in terminal respiratory failure secondary to pneumonia, who is refusing tracheostomy in the event of unsuccessful intubation. Kelly’s muscle weakness prevented her from properly clearing her lungs ultimately leading to pneumonia and subsequent dyspnea which led to her admission. Although adolescents are not considered fully autonomous and their parents make the ultimate decision about their care, both Kelly’s and her parents’ preferences are the same. The goal of intubation is to help clear the mucous secretions. The anesthesiologist working on this case expresses moral concern about Kelly’s and her parents’ adamant refusal of the tracheostomy as it is the next step in the event intubation fails in order to prevent respiratory failure. The anesthesiologist explains it is “her duty to rescue a patient under her care and by honoring the patient’s wish she could be playing a role in “killing” the patient” (Gentry & Wightman, 2018).
The ethical issue presented in this case is one of Autonomy and Nonmaleficence. Kelly and her parents express that living with a tracheostomy is unacceptable and consistently refused life sustaining procedures like chronic ventilation or tracheostomy during the course of her illness. Kelly also expressed that for her life worth living is one where she is able to speak and not depend on mechanical ventilation which she fears will be her fate with a tracheostomy. On the other hand, the anesthesiologist feels morally conflicted knowing that Kelly’s muscle weakness and contracture may result in a difficult and potentially unsuccessful intubation under sedation, resulting in respiratory depression and death without the possibility of a tracheostomy intervention.
My position in this case is to grant the patient and her family the autonomy to choose what she and her parents ultimately decide after thoroughly addressing the patient’s concerns about the tracheostomy, methods of intubation and discussing any back up plans in the case of failed attempts and Kelly’s impending death. I would ensure Kelly and her parents have capacity and understanding of the possible benefits and consequences of their decisions before the intubation procedure and properly document refusal of any intervention. My reasoning to honor the patient’s autonomy is based off the same idea as a valid do not resuscitate (DNR) order requested by patient or surrogate. Much like advanced care planning with DNR order, careful perioperative discussion is crucial with high-risk patients to ensure the patient’s wishes are respected in the terminal phases of life.
An alternative view point as expressed by the anesthesiologist is based on non-maleficence. Although a tracheostomy in SMA patients comes with a risk of vocal cord damage and potentially permanent ventilation due to unsuccessful tracheostomy reversal (Thavagnanam et al., 2018), if endotracheal intubation fails and Kelly’s death is imminent, a tracheostomy would allow Kelly’s parents to be with Kelly during her terminal phase in life and withdraw respiratory support in a palliative setting. Another viewpoint based on the principle of beneficence is discussing other treatment options with the pulmonologist that could be less risky for Kelly but also benefit her current condition. One such option which can be attempted is fiberoptic nasal intubation which can be done while the patient is awake using topical anesthesia. Fiberoptic nasal intubation is indicated when there is an anticipated risk of difficult endotracheal intubation (Jagannathan & Burjek, 2020) and carries a lower risk of airway compromise or death (Gentry & Wightman, 2018).
In conclusion, this case study brings up a very difficult ethical dilemma for the clinician where they feel morally obliged to rescue the patient to maintain life. However, sometimes these resuscitation attempts can leave the patient in a very debilitated state which the patient wishes to avoid and therefore their resuscitation requests must be respected. As in any ethical conflict, prior to intervention the clinician must address any fears, ambiguity and ensure that the patient and her parents understand the risks and benefits of their choices while ultimately respecting their values and beliefs.
References
- Gentry, K., & Wightman, A. (2018). How Should Refusal of Tracheostomy as Part of an Adolescents Perioperative Planned Intubation Be Regarded? AMA Journal of Ethics, 20(8). doi:10.1001/amajethics.2018.683
- Thavagnanam, S., Chiang, J., Zielinski, D., & Amin, R. (2018) Section 7: Spinal muscular atrophy and home ventilation. Canadian Journal of Respiratory, Critical Care, and Sleep Medicine, 2:sup1, 53-59, DOI: 10.1080/24745332.2018.1494981
- Jagannathan, N., & Burjek, N. (2020, September 04). Management of the difficult airway for pediatric anesthesia. Retrieved from https://www-uptodate-com.
