Neurology -- Correspondence for Albert et al., 65 (1) 68-74

Correspondence published:

Wish to die in end-stage ALS: Mandaville Gourie-Devi, and Senior Consultant Neurologist and Chairperson of Department of Clinical Neurophysiology, Sir Ganga Ram Hospital, New Delhi (29 June 2006)

Reply from the Author: Steven M. Albert (29 June 2006)

Reply to Kennedy: Steven M. Albert (11 October 2005)

Wish to die in end-stage ALS: Philip R. Kennedy, MD, PhD (11 October 2005)

Wish to die in end-stage ALS 29 June 2006

Mandaville Gourie-Devi,
Emeritus Professor
Institute of Human Behaviour and Allied Sciences,Dilshad Garden, Delhi-110095,India,
and Senior Consultant Neurologist and Chairperson of Department of Clinical Neurophysiology, Sir Ganga Ram Hospital, New Delhi
Send Correspondence to journal:
Re: Wish to die in end-stage ALS

mgouriedevi{at}gmail.com Mandaville Gourie-Devi, et al.

I have been following with great interest the articles, editorial and letters dealing with various issues pertaining to “wish to die in end-stage ALS”. [1-5] Having had a special interest and having been involved in the management of ALS patients in India during the last 30 years, I wish to share my experiences.
Although mine is not a systematic study, I would like to convey the findings here. In the government funded referral institute hospitals, 70 to 80% of ALS patients belong to low and middle economic strata, with monthly income ranging from US$ 50 to 500 per month and no access or means to afford life support systems at home or advanced technologies for communication. Despite these distinct disadvantages and limitations in health care, less than 20% (approximate figure) expressed desire to end their lives, the figure being much less than that reported by Albert et al (43.4%). [1]
One difference may be that our patients were living with their extended families, the majority with their sons, and not in hospice or nursing homes. Albert et al reported that 60% who wished to die and 48% who did not express the wish, used hospice at baseline. [1] This difference was not considered to be significant. Emotional security provided by the family members during the terminal stage of the disease may promote a positive attitude to illness. Strong faith in religion and cultural rituals also led many of our patients to accept the illness and death as God’s will.
Albert et al also reported that patients who expressed the wish to die had less comfort in religion and greater degree of hopelessness. [1] In our experience, those who expressed the wish to die felt that they did not want to add to the financial burden of the caregiver and therefore did not want to live. It would be interesting to examine, if feasible, the issue of “wish to die in end stage ALS” in different populations with socioeconomic and religious diversity and various levels of health care.
References
1.Albert SM, Rabkin JG, Del Bene ML, et al. Wish to die in end-stage ALS. Neurology 2005;65:68-74.
2.Rabkin JG, Albert SM, Del Bene ML, et al. Prevalence of depressive disorders and change over time in late-stage ALS. Neurology 2005;65: 62-67.
3.Quill TE. ALS, depression, and desire for a hastened death: (How) are they related? (Commentary). Neurology 2005; 65: 1.
4.Olney RK, Lomen-Hoerth C. Exit strategies in ALS. An influence of depression or despair? (Editorial). Neurology 2005; 65:9-10.
5.Kennedy PR. Wish to die in end- stage ALS (Letter to the editor). Neurology 2006; 66: 783-784.
Disclosure: The author reports no conflicts of interest.

Reply from the Author 29 June 2006

Steven M. Albert,
University of Pittsburgh
A211 Crabtree Hall, 130 DeSoto St. Pittsburgh, PA 15261 USA
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Re: Reply from the Author

smalbert{at}pitt.edu Steven M. Albert

Dr. Gourie-Devi raises an important point regarding the wish to die in neurodegenerative disease. She suggests that the wish to die will vary according to aspects of culture. These include family involvement as a source of emotional security and religious rituals that give meaning to the end of life. The prevalence of the wish to die among ALS patients may indeed be lower in India, for example, with its different family organization and religious life. We agree that an investigation of cross-cultural variation in the end of life experience would be very valuable.
We would draw attention, however, to one challenge of such research, which is well illustrated in our study of American patients. If we ask, “Have you thought about taking your life or asking your doctor to end your life?”, 43.4% say yes. If we ask “Have you ever seriously discussed taking your life or asking your doctor to end your life?”, only 18.9% say yes. “Thought about” and “seriously discussed” are quite different. A cross-cultural investigation would need to be sensitive to such fine distinctions, as well as challenges in translation and the context of such inquiry.
Disclosure: The author reports no conflicts of interest.

Reply to Kennedy 11 October 2005

Steven M. Albert,
University of Pittsburgh
A211 Crabtree Hall, Graduate School of Public Health, 130 DeSoto St., Pittsburgh, PA 15261
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Re: Reply to Kennedy

smalbert{at}pitt.edu Steven M. Albert

Dr. Kennedy describes engineering advances that allow locked-in patients to communicate after tracheostomy and long-term mechanical ventilation (LTMV). He asks if ALS patients would be less likely to reject LTMV if they knew they could continue to communicate. He also raises the question of whether ALS should be considered “fatal” given the availability of LTMV and advanced communication devices.
Our study did not directly address these issues. However, inquiries with our sample of ALS patients suggest that loss of communication is not the dominant factor in LTMV decisions. Patients who choose LTMV do so because they are committed to living despite the serious disability associated with the disease, and because they consider LTMV a way to stave off death until a cure for the disease becomes available. Reduction of disability post-LTMV through enhanced communication devices (and other prosthetic technologies) is desirable but would not likely alter LTMV decisions.
Given the availability of LTMV, is ALS no longer fatal? This is a semantic issue, as one could also ask if patients dependent on dialysis have “fatal” kidney disease. The more critical question is whether patients consider living with the disease acceptable, given use of such supportive technologies. Half of the LTMV patients in our sample reported red lines that would lead them to end ventilator care. These included dementia, impoverishment of families, nursing home admission, and loss of vision, in addition to loss of the ability to communicate. This suggests that ventilator care and use of prosthetic technologies, even “if freely available,” may not compensate fully for the losses associated with disease progression.
Rather than ask whether a disease should be considered fatal or not, we suggest that clinicians confer with patients to consider what degree of disability they (and their families) find tolerable. For patients willing to undergo LTMV, they might also consider limited trials, where the option to end LTMV may also be considered a legitimate choice.
Disclosure: The author reports no conflict of interest.

Wish to die in end-stage ALS 11 October 2005

Philip R. Kennedy, MD, PhD,
Community Neurological Clinic
3540 Duluth Park Lane, Suite 120, Duluth, Georgia 30096
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Re: Wish to die in end-stage ALS

phlkennedy{at}neuralsignals.com Philip R. Kennedy, MD, PhD

I would like to add to the recent group of articles and the editorial discussing end-of-life issues in ALS patients. I attend to many ALS patients and also supply them with communication technologies through my company Neural Signals Inc.
I am not making the following remarks with any ulterior motives, even though there is a potential conflict of interest. We can supply communication technologies to ALS patients even after they are fully locked-in and fully paralyzed on a ventilator. We have non-invasive external devices that use muscle activities (as long as there is any residual EMG activity), eye movements (provided they have reasonably quick eye movements) and EEG that will allow them to give ‘yes’ or ‘no’ answers to a caretaker’s questions.
We can say to any patient with ALS or any other locked-in condition that they will always be able to communicate provided they remain cognitively intact.
This changes the equation when people are making decisions about ventilators. We all agree that without a ventilator, ALS is a fatal disease. However, it should not be presented as a fatal disease because ventilators in this country are freely available. The ability to communicate, even when locked-in, will perhaps change ALS patients’ attitudes toward the decision about whether or not to take a ventilator.
Approximately 100 years ago, before the advent of antibiotics, pneumonia was considered a fatal disease and certainly it can still be, but we do not tell the patients it is a fatal disease. So why is it now standard practice to tell ALS patients they have a fatal disease? Why not tell them that they have a ventilator-dependent disease. I realize that most of them will choose not to be on a ventilator and prefer to die. However, if the know they can always communicate, then this may give them pause in this decision.
Disclosure: The author is the owner of Neural Signals, Inc.