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J Clin Neurol
J Clin Neurol
JCN
Journal of Clinical Neurology (Seoul, Korea)
1738-6586
2005-5013
Korean Neurological Association

39227337
10.3988/jcn.2024.0249
Letter to the Editor
Withdrawal of Life-Sustaining Mechanical Ventilation for a Patient With Amyotrophic Lateral Sclerosis in Locked-In Syndrome
https://orcid.org/0000-0001-6444-977X
Choi Seok-Jin ab
https://orcid.org/0000-0001-7473-1082
Yoo Shin Hye cd
https://orcid.org/0000-0002-1626-2721
Lee Sun Young de
https://orcid.org/0000-0001-7525-5313
Sung Jung-Joon bf
a Center for Hospital Medicine, Seoul National University Hospital, Seoul, Korea.
b Department of Neurology, Seoul National University Hospital, Seoul, Korea.
c Center for Palliative Care and Clinical Ethics, Seoul National University Hospital, Seoul, Korea.
d Department of Human Systems Medicine, Seoul National University College of Medicine, Seoul, Korea.
e Public Healthcare Center, Seoul National University Hospital, Seoul, Korea.
f Neuroscience Research Institute, Seoul National University Medical Research Center, Seoul, Korea.
Correspondence: Jung-Joon Sung, MD, PhD. Department of Neurology, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul 03080, Korea. Tel +82-2-2072-1015, Fax +82-2-3668-7831, jjsaint@snu.ac.kr
9 2024
12 8 2024
20 5 537538
27 5 2024
08 7 2024
12 7 2024
Copyright © 2024 Korean Neurological Association
2024
Korean Neurological Association
https://creativecommons.org/licenses/by-nc/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (https://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
National Research Foundation of Korea https://doi.org/10.13039/501100003725 NRF-2018R1A5A2025964
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pmcDear Editor,

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder that results in death by respiratory failure.1 Tracheostomy may be offered to a subset of patients experiencing terminal acute respiratory failure. A nationwide cohort study found that the tracheostomy rate in ALS patients was markedly higher in South Korea (35.3%) than in Western countries.2 Moreover, a recent online survey found that one-third of 42 tracheostomized patients reported that they were first informed about the procedure when they underwent it on an emergency basis (unpublished, Seoul National University Hospital/Korean ALS Association, 2022). Therefore, many patients are provided with an inadequate time to consider whether to undergo tracheostomy, or decisions are made for them by their caregivers when patients are unable to consent. The Act on Decisions on Life-Sustaining Treatment for Patients at the End of Life has been in effect in South Korea since 2018.3 However, there is still no consensus regarding whether tracheostomy for ALS patients should be considered a life-sustaining treatment and when the dying process is considered to have commenced. Here we present a case involving a terminal-stage ALS patient who underwent an emergency tracheostomy contrary to her prior wishes and ultimately died with dignity after the withdrawal of life-sustaining mechanical ventilation.

A 46-year-old female with locked-in syndrome due to ALS had been receiving tracheostomy invasive ventilation at home under the care of her parents for over 2 years. In August 2021 she suffered an unexpected respiratory arrest after her noninvasive positive-pressure ventilation machine became disconnected. Despite her repeatedly expressing a desire for any life-sustaining treatment that would prolong her life to be withheld, there was no legal documentation such as a do-not-resuscitate order. Consequently, she was resuscitated by 1 minute of cardiopulmonary resuscitation and subsequently underwent a tracheostomy following several unsuccessful extubation attempts. Her parents had been caring for her at home with the assistance of a paid caregiver. However, her father had recently died, leaving her mother to care for her alone.

Our home-based care team regularly monitored the overall medical condition of this patient and confirmed that all immediate family members (mother and two older brothers) consented to withdraw life-sustaining mechanical ventilation, respecting her previously expressed wishes. She was admitted to our hospital and the following aspects were addressed: First, her decision-making capacity was determined. No muscle contractions were observed in any extremities, and she was unable to close her eyes. Brainstem reflexes including the corneal and vestibulo-ocular reflexes were impaired, and she could only perform minimal eye tracking. Brain computed tomography revealed severe frontotemporal lobe atrophy. After multiple thorough neurological examinations and discussions, we concluded that her cognitive function was severely impaired to the extent that her responses to simple yes or no questions were unreliable. Second, to ascertain the dying process, two neurologists, one home-based-care physician, and one palliative-care physician concurred that she was in a far-advanced terminal stage and at the end of life. Third, the patient’s intentions were inferred from her consistently asking for life-sustaining treatment to be withheld, which led us to determine that withdrawing mechanical ventilation aligned with her preferences and values, and was also in her best interest. Consequently, intravenous morphine and midazolam were continuously infused for palliative sedation, and the mechanical ventilator was subsequently disconnected. She died peacefully 30 minutes later. The flowchart for withholding or withdrawing life-sustaining treatment is provided in the Supplementary Fig. 1 (in the online-only Data Supplement).

In South Korea, decisions regarding life-sustaining treatment can only be implemented during the dying process.34 Originally intended as protective measures, these regulations may unduly infringe upon the rights of end-of-life patients who are not actively dying, despite the similarities in treatment burdens and care goals between the terminal and dying phases.4 Additionally, the act does not differentiate between withholding and withdrawing treatments.3 Physicians in intensive-care units across Asia report that they frequently withhold life-sustaining treatments, but also that they rarely withdraw them at the end of life. Notably, the proportion of patients opting for treatment withdrawal is significantly lower in South Korea than in other countries.5 It may be debatable whether a patient who has undergone tracheostomy invasive ventilation is in the dying process. However, the tracheostomy was performed on an emergency basis in the present case, which is a common scenario for many Korean ALS patients and was contrary to this patient’s explicit wishes to withhold life-sustaining treatment. Consequently, she remained in a locked-in state being sustained by these measures. We believed that withdrawing mechanical ventilation best honored her wishes.

We suggest that two key conclusions can be drawn from this case: First, counseling on life-sustaining treatments such as tracheostomy should be provided when the disease is in a stable phase, prior to the development of terminal acute respiratory failure. Second, to minimize unnecessary physical and psychological suffering and to improve the palliative care of patients with ALS, it is crucial to engage in advance care planning and end-of-life discussions, including those about life-sustaining treatments.

Acknowledgments

The authors sincerely thank the patient and her family members for participating in this study, as well as the Korean ALS Association for their assistance.

Availability of Data and Material

All data generated or analyzed during the study are included in this published article (and its supplementary information files).

Supplementary Materials

The online-only Data Supplement is available with this article at https://doi.org/10.3988/jcn.2024.0249.

Supplementary Fig. 1

Flowchart for withholding or withdrawing life-sustaining treatment (LST).

Ethics Statement: This study was approved by the Institutional Review Board of Seoul National University Hospital (No. 1904-165-1031). Written informed consent was obtained from the patient for the publication of this study.

Author Contributions: Conceptualization: Seok-Jin Choi, Jung-Joon Sung.

Data curation: Seok-Jin Choi.

Funding acquisition: Jung-Joon Sung.

Investigation: Seok-Jin Choi, Shin Hye Yoo, Sun Young Lee.

Supervision: Jung-Joon Sung.

Validation: all authors.

Writing—original draft: Seok-Jin Choi.

Writing—review & editing: all authors.

Conflicts of Interest: The authors have no potential conflicts of interest to disclose.

Funding Statement: This work was supported by the National Research Foundation of Korea (NRF) grant funded by the Korean government (MSIP) (NRF-2018R1A5A2025964 to J-J Sung).
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