Abstract
Abstract
Objective:
Donation after cardiac death (DCD) or donation of organs after removal of life support is an accepted means of organ retrieval that usually occurs in the setting of sudden illness but has not been described in people with progressive neurologic illness. We report DCD in two people with progressive amyotrophic lateral sclerosis (ALS).
Methods:
Case series at an academic medical center of two men with progressive ALS who underwent withdrawal of artificial life support, rapid cardiac death, and subsequent organ donation. The primary outcome was donation of organs in concordance with patient and family wishes.
Results:
Both patients underwent peaceful withdrawal of life support in the presence of family, and multiple organs were donated.
Conclusions:
Patients may legally and ethically refuse life-sustaining care. These patients considered their lives to be more burdensome than beneficial near the end of their lives, both carefully planned the time and circumstance of their deaths, and both fulfilled a long-standing desire to donate their organs. This study describes a potential opportunity for patients with progressive neurologic illness.
Introduction
Amyotrophic lateral sclerosis (ALS) is a progressive neurologic disease 9 in which the motor paralysis occurs while in most cases the patient remains cognitively and emotionally intact, and the most common cause of death is respiratory failure. Patients with ALS increasingly plan for a peaceful death using hospice, and fewer are being admitted to hospitals for end-of-life care. 10 Some patients die at home, 11 and withdrawal of ventilator support can be done at home. 12
As in any medical situation, patients with decision-making capacity may ethically refuse any care they do not desire, even if such refusal hastens their death. We present our experience with two ventilator-dependent patients with advanced ALS who requested to discontinue ventilator support with the added request that they be allowed to donate their organs.
Case Reports
Case 1
A 44-year-old African American male developed ALS at age 20. He grew progressively weaker and required home ventilator support via tracheostomy for 23 years. He had always expressed to his mother, family, and health care team that he wanted to be allowed to die with dignity when he could no longer feed himself. He discussed organ donation with his home care physician, family, and pastor several months before his death. He was found to be not depressed by multiple examiners. His family contacted the organ procurement organization about possible organ donation. After much discussion with his mother, family, pastor, and several re-confirmations of his desire, with documentation, he was admitted to the Virginia Commonwealth University Health System (VCUHS) Palliative Care program. In the operating room, he was given a total of 10 mg midazolam and 12 mg morphine intravenously, titrated to calmness and sedation, as the ventilator was removed. At the standard 5 minute time after cardiac death (no pulse, no electrocardiogram activity) his body was taken to the operating room where the transplant team procured the organs. His family was escorted to the waiting room by the palliative care chaplain as soon as death was declared. Several organs were successfully transplanted. Further contact with his family confirmed that they were grieving appropriately and grateful for the opportunity to donate.
Case 2
A 55-year-old Caucasian American male with familial ALS developed weakness in his upper extremities, speech changes, and difficulty with swallowing and breathing. One year later a tracheostomy and percutaneous endoscopic gastrostomy (PEG) were placed. Another year later the patient had quadriparesis with only trace finger movement, and he communicated through augmentative eye movement devices. His cognition was preserved and he was not depressed. He was enrolled in hospice at home. He expressed his desire for organ donation upon death, which he planned when he felt that his disease was placing too much of a burden on his wife. He had watched his father and brother die of ALS. Three years after diagnosis he expressed his wishes for withdrawal of life support, after two respiratory arrests and subsequent emergency intensive care unit (ICU) admissions. Several weeks after his ICU admissions, his family contacted the organ procurement organization about possible donation.
After much discussion with his wife, pastor, and several re-confirmations of his expressed desire and alternatives, with documentation, he was admitted to the VCUHS Palliative Care program to withdraw life support and donate his organs consistent with his long-standing wishes. In the operating room, he was given a total of 10 mg midazolam and 5 mg morphine intravenously, titrated to calmness and sedation, and the ventilator was stopped. After the standard 5 minute waiting time and tests confirming cardiac death, his body was taken to the operating room. His family was escorted to the waiting room while his wife waited near the operating room, as she wanted to hear directly from the surgeons which organs had been harvested. Several organs were successfully procured. Further contact with his wife by the palliative care team, neurologist, and the organ procurement organization confirmed that she was grieving appropriately.
Reactions of the Health Care Professionals
Case 1: The responsible nurse (BB) stated, “This was one of the most intense and difficult things I have done as a nurse, but ultimately certainly also very rewarding knowing that I helped ensure his comfort and some other people's lives will be improved as a result of organ donation.” The responsible physician (TS) noted, “They had been thinking this through for months, and the patient had prepared a CD of his favorite music to have in the operating room. The extended family was there, as well as the minister, to the last heartbeats. The patient was able to communicate with whispers, say his final good-byes, and re-affirm his wishes.”
Case 2: The responsible physician (AB) said, “He was still able to communicate with us as we were withdrawing the ventilator …. This was comforting to the family and likely of immeasurable value to the patient. He asked his family to play his chosen music, and indicated when he was ready to wean the vent. He even joked to us that we should make sure the surgeons get all the organs that they can. Titration of benzodiazepines and opioids was challenging, as the typical markers we use for dyspnea were absent, and we weaned the ventilator by making sure the midazolam (Versed™) was already given. The patient and family were both aware and gave permission for us to do this (again, socially very different from any other extubation, though medically not so much). He was able to tell me that organ donation was something that he had been thinking about for years and years, even prior to his diagnosis of ALS.…Establishing this relationship prior to the day of ventilator liberation may have eased some of the concerns of the wife and patient, and when the day came at least there was a somewhat familiar face.”
Discussion
DCD has become an accepted medical procedure that expands the donor pool of life-saving organs including kidneys, the liver, corneas, and other tissues. At the current time there are more than 100,000 patients waiting for solid organ transplants. Two patients with ALS who were near the end of their life elected to withdraw life support and participate in DCD. The standard protocol of the United Network for Organ Sharing (UNOS, http://www.unos.org/about/index.php) was followed in that the Virginia organ procurement organization (http://www.LifeNet.org/mission.php) personnel initiated all discussions about organ donation. The palliative care team was involved to ensure comfort 13 and provide support to the patient and family during the ventilator withdrawal process. 14 Both patients experienced cardiac death within 5 minutes of ventilator withdrawal and were able to donate several organs. The process occurred with some measure of comfort, and surviving families were grateful for the opportunity to donate organs that saved other lives.
We have learned important lessons from these patients and their families. First, we have learned that organ donation is desired by at least some of the 5000 ALS patients and their families just as with other patients as a means of extending life to others. Second, we have learned that DCD in such cases is not easy, as the process seems different than acute trauma deaths, although the legal, ethical, and medical issues are the same. Many of these patients have compelling life stories, are still able to communicate, and may bond immediately with the palliative care staff, which is a change from comatose trauma patients. We have routinely conducted “debriefing” sessions 15 after DCD cases to explore our own feelings and improve the process. As our chaplain (TF) stated, “In debriefing this case with the organ procurement organization personnel and the VCUHS organ donation chaplains, one of the existential points that came up both times was that the patients were choosing their time of death with an extraordinary degree of precision. We all know we are going to die, but we do not know and cannot control when. Furthermore, there is a level of autonomy conveyed to the ALS patients here that goes beyond directing medical care. They are directing something over which almost none of us have autonomous control. Powerful stuff.”
Third, we have learned that DCD requires more planning for an admission, which must occur when the person is paradoxically well enough to undergo organ donation yet is near death. Because these patients are usually alert and able to communicate, we always have enough midazolam and morphine immediately available in the operating room to ensure comfort. The opioid requirements used are similar to those used to ensure comfort in other patients dying at home with ALS. 16 Finally, we have learned that, as with all DCD cases, the process requires agreement of all involved, with meticulous documentation. This includes the absolute confirmation of a fatal neurologic illness, screening for and treatment of depression, and recognition that some patients have depression, pseudo-dementia, or dementia as part of their ALS complex. 17 Table 1 lists some of the important issues to consider.
DCD, donation after cardiac death.
We have heard from other ALS patients that organ donation is an important option for them. We are very much aware that this could be perceived differently by others not involved in the lives and care of these patients, and that reasons to donate organs are always complex. 18 Others might see it as euthanasia or physician-assisted suicide, not valuing a life lived with disability, or raise other ethical issues of justice and fairness that have been explored since the beginning of DCD. Our practice is very different from euthanasia or physician-assisted suicide, which is chosen by about 17% of ALS patients in the Netherlands where it is legal. 19 Many of those patients die with deep conscious sedation induced as part of the physician-assisted suicide, whereas our patients chose to withdraw unwanted life support and received modest doses of midazolam and morphine only to ensure comfort. It is critical to note that the palliative care team in both these cases was informed of the patient's long-standing wishes to undergo DCD after the decision had been made by the patient and family to withdraw ventilator support, and that the discussion about organ donation was facilitated by the organ procurement team and not the palliative care team.
In summary, DCD can be successfully performed in patients with ALS with multiple life-saving organs transplanted to others. This might be the choice of other patients similar to those cases are discussed here, if they were aware of the option. The process requires meticulous planning and often more active symptom relief during the course of withdrawing ventilator support than with nonalert patients.
Acknowledgments
The authors wish to thank ethicists Joann Bodurtha, M.D., M.P.H, and Laura Siminoff, Ph.D., and public health advocate A. J. B. Smith, M.Sc. Public Health, for excellent suggestions on earlier versions of the manuscript.
Footnotes
Author Disclosure Statement
No competing financial interests exist.