Tuesday, September 13, 2016

The wish to die in ALS

Rabkin J, Goetz R, Murphy JM, Factor-Litvak P, Mitsumoto H; ALS COSMOS Study
Group. Cognitive impairment, behavioral impairment, depression, and wish to die
in an ALS cohort. Neurology. 2016 Aug 5. pii: 10.1212/WNL.0000000000003035. [Epub
ahead of print]

To evaluate relationships among cognitive, behavioral, and psychiatric/psychosocial measures assessed in a multicenter cohort of patients with amyotrophic lateral sclerosis (ALS).
Recently diagnosed patients with definite or probable ALS diagnosis were administered 7 standardized psychiatric/psychosocial measures, including the Patient Health Questionnaire for diagnosis of depression and elicitation of wish to die. The Cognitive Behavioral Screen was used to classify both cognitive and behavioral impairment (emotional-interpersonal function). An ALS version of the Frontal Behavioral Inventory and Mini-Mental State Examination were also administered.
Of 247 patients included, 79 patients (32%) had neither cognitive nor behavioral impairment, 100 (40%) had cognitive impairment, 23 (9%) had behavioral impairment, and 45 (18%) had comorbid cognitive and behavioral decline. Cognitive impairment, when present, was in the mild range for 90% and severe for 10%. Thirty-one patients (12%) had a major or minor depressive disorder (DSM-IV criteria). Cognitive impairment was unrelated to all psychiatric/psychosocial measures. In contrast, patients with behavioral impairment reported more depressive symptoms, greater hopelessness, negative mood, and more negative feedback from spouse or caregiver. A wish to die was unrelated to either cognitive or behavioral impairment.
While we found no association between cognitive impairment and depression or any measure of distress, behavioral impairment was strongly associated with depressive symptoms and diagnoses although seldom addressed by clinicians. Thoughts about ending life were unrelated to either cognitive or behavioral changes, a finding useful to consider in the context of policy debate about physician-assisted death.

In August, a 41-year-old artist with amyotrophic lateral sclerosis (ALS) generated headlines in the mainstream press when she held a two-day gathering of friends and family before taking a fatal combination of medications. She became one of California's first residents to use the state's new law allowing physicians to help terminally ill people end their lives…

In the latest study, known as the the Cohort Study of Oxidative Stress (COSMOS), researchers at multiple institutions sought to determine if there were possible associations between cognitive, behavioral, and psychiatric/psychosocial impairments in ALS patients, and the wish to die…

“Being depressed was not related to having a wish to die,” said the principal study author Hiroshi Mitsumoto, MD, FAAN, the Wesley J. Howe professor of neurology at Columbia University at the Neurological Institute of New York and New York-Presbyterian Hospital/Columbia University Medical Center. Of the 62 patients who expressed a wish to die, only 23 were determined to be clinically depressed…

Still, he noted, the study did find that patients with behavioral impairment showed “considerable and consistent” associations between disease characteristics, and were more likely to have depressive symptoms, lower quality of life and higher stress levels. Patients with behavioral impairment were more likely to to have depressive symptoms, lower levels of positive affect and higher levels of negative affect, and higher mean scores of hopelessness, lower quality of life, and higher stress levels…

In the study, the researchers looked at 247 recently diagnosed ALS patients, 32 percent of whom had neither cognitive nor behavioral impairment. Of those who had cognitive impairment, 90 percent were in the mild to moderate range, and 10 percent had severe cognitive impairment. Twelve percent had a major or minor depressive disorder…

“That way if there are cognitive, behavioral, and psychological issues noted, the patient can be properly treated and appropriate counseling should be given,” he said. “The goal is to take care of the patient and the family, and be aware of these issues, because the wish to die and physician-assisted death issue will come up more and more in the future, so we simply cannot ignore them.”…

Linda Ganzini, MD, MPH, professor of psychiatry and medicine at Oregon Health and Science University in Portland, agreed that loss of autonomy is a primary concern. ALS patients are 30 times more likely to die with physician-assisted death in Oregon, compared to all other deaths, and about 3 percent of ALS patients choose that option…

“For me and people in the field working with ALS patients, this study is reassuring that depression isn't much of a worry as part of their decision-making capacity as much as we have feared,” said Dr. Ganzini.“It's still very important to test patients for their understanding of their options, whether it's about the disease or treatment, and whether they understand the options of palliative measures that might improve their quality of life and make them want to live longer.”

Dr. Katz did not feel that the study findings would necessarily help neurologists respond to requests for physician-assisted death. Patients who were demented and who had severe depression would not be allowed to be part of physician-assisted suicide, he said, because they don't have decision-making capacity, adding that the laws specifically address the role of depression.

“Those who feel strongly that physician-assisted death is a bad idea will find reasons to either refute what the data provide in this article or will say it's irrelevant because physician participation in death is never justifiable within the purview of professional responsibility,” said Dr. Russell, chairman of the Ethics, Law and Humanities, a joint committee of the AAN, American Neurological Association and the Child Neurology Society. “For the physicians who endorse participation in hastened death when lawful and when terminally ill patients request it, it will perhaps make them feel slightly better about their participation because there has been an unanswered question about whether depression interferes with a person's decision-making capacity.”



  1. In early July, Betsy Davis emailed her closest friends and relatives to invite them to a two-day party, telling them: "These circumstances are unlike any party you have attended before, requiring emotional stamina, centeredness and openness."

    And just one rule: No crying in front of her.

    The 41-year-old artist with ALS, or Lou Gehrig's disease, held the gathering to say goodbye before becoming one of the first Californians to take a lethal dose of drugs under the state's new doctor-assisted suicide law for the terminally ill.

    "For me and everyone who was invited, it was very challenging to consider, but there was no question that we would be there for her," said Niels Alpert, a cinematographer from New York City.

    "The idea to go and spend a beautiful weekend that culminates in their suicide — that is not a normal thing, not a normal, everyday occurrence. In the background of the lovely fun, smiles and laughter that we had that weekend was the knowledge of what was coming."

    Davis worked out a detailed schedule for the gathering on the weekend of July 23-24, including the precise hour she planned to slip into a coma, and shared her plans with her guests in the invitation.

    More than 30 people came to the party at a home with a wraparound porch in the picturesque Southern California mountain town of Ojai, flying in from New York, Chicago and across California.

    One woman brought a cello. A man played a harmonica. There were cocktails, pizza from her favorite local joint, and a screening in her room of one of her favorite movies, "The Dance of Reality," based on the life of a Chilean film director.

    As the weekend drew to a close, her friends kissed her goodbye, gathered for a photo and left, and Davis was wheeled out to a canopy bed on a hillside, where she took a combination of morphine, pentobarbital and chloral hydrate prescribed by her doctor.

    Kelly Davis said she loved her sister's idea for the gathering, which Betsy Davis referred to as a "rebirth."...

    "Dear rebirth participants you're all very brave for sending me off on my journey," she wrote in her invitation. "There are no rules. Wear what you want, speak your mind, dance, hop, chant, sing, pray, but do not cry in front of me. OK, one rule."...

    At one point, she invited friends to her room to try on the clothes she had picked out for them. They modeled the outfits to laughter. Guests were also invited to take a "Betsy souvenir" — a painting, beauty product or other memento. Her sister had placed sticky notes on the items, explaining each one's significance.

    Wearing a Japanese kimono she bought on a bucket-list trip she took after being diagnosed in 2013, she looked out at her last sunset and took the drugs at 6:45 p.m. with her caretaker, her doctor, her massage therapist and her sister by her side. Four hours later, she died.

    Friends said it was the final performance for the artist, who once drew pictures on a stage with whipped cream.

    "What Betsy did gave her the most beautiful death that any person could ever wish for," Alpert said. "By taking charge, she turned her departure into a work of art."


  2. The doctor spelled out for them the entire course of the disease: first his limbs would become paralyzed, to be followed by the muscles of his neck, esophagus, and tongue. "The day will come," he told Rahamim, "when a fly will land on your nose and you won't be able to brush it off. You will become dependent on other people for everything." And in the final stage, his lungs would stop working. "You have three to five years to live."

    That was 12 years ago. Three of the doctors who attended on him have since died, but Rahamim Melamed-Cohen, while completely paralyzed, is still going strong. Since the onset of his illness, he has written seven books, the latest by means of a computer that types by his eye movements. Until a year ago, when he could still speak clearly, he gave lectures on educational methodology to students in his living room. He maintains a voluminous email correspondence with readers who look to him for encouragement and wisdom. He prays thrice daily and attends synagogue every Shabbat. And he and his wife go out regularly, to the theater, to weddings, and to restaurants, although Rahamim himself no longer eats except through a feeding tube to his stomach. As Elisheva explains, "Although he doesn't eat, he sits with us." His company is obviously worth the effort.

    http://www.aish.com/sp/pg/48960166.html (also referenced in the comment from 5/10/15 to Quality of Life 3/24/15)

    See: https://childnervoussystem.blogspot.com/2015/05/triumph-over-adversity.html?showComment=1431296152620#c8660440624827456479

  3. A searing segment of the film, "Heroes Against Their Will," shows Dr. Melamed-Cohen debating Dr. Noam Reches, the chairman of the Israel Medical Ethics Committee and a leading proponent of euthanasia, who himself has "pulled the plug" on request. Dr. Reches looks at the wheelchair-bound Dr. Melamed-Cohen, with the respirator tube connected to the tracheotomy in his neck, and says, "You can't feed yourself. You can't hug the people you love... If I were in your position, I'd want out."

    Dr. Melamed-Cohen responds, "These are the most beautiful and happiest years of my life."

    "Some other ALS patients when they were fully conscious asked to end their lives," Dr. Reches continues. "They didn't want to reach your situation of complete dependence on others."

    Dr. Melamed-Cohen gazes at Dr. Reches and declares, "Believe me, my life is no less interesting than yours."

    All I could think of when viewing this scene was: He may not be able to hug people, but few of us with two functional arms actually embrace others with as much love and caring as Rahamim Melamed-Cohen.


    See: https://childnervoussystem.blogspot.com/2015/03/quality-of-life.html?showComment=1431295730823#c8493107757283249049

  4. I remember when I was finished my first round of tests. The neurologist gave me his preliminary findings "you have bulbar ALS". I had no idea what it meant. He explained the severity of it to me and said he was going to refer me to an ALS specialist.

    I was there alone. Walking out of his office into the empty hallway I broke down in a fit of bitter tears. When I composed myself I headed out of the building. The first thing I witnessed stepping out, was a young man falling to the ground having a seizure. I ran to help him.

    It dawned upon me that there is still much purpose for me. I decided that regardless of the outcome of any future "tests" I would remain positive and find ways to fill life with meaning and purpose.

    This has turned my life and the lives of those around me happier and by far more fulfilling.

    We all suffer hardships and pain, it's what we do with them that makes the difference, "This is the Torah of the Metzorah".


    See: https://childnervoussystem.blogspot.com/2015/05/triumph-over-adversity.html?showComment=1431617658913#c912397699770123724

  5. Rabbi Yitzi Hurwitz is a young man with ALS (see Triumph over adversity 5/3/15 comment 5/14/15): It is already several years since Hashem chose to give me and my family tremendous hardships. For me it is physically paralyzing, for my wonderful wife, Dina, it can at times be emotionally and mentally paralyzing. My children are also subjected to an unwanted roller-coaster ride. I wish and pray all the time that I will be cured. But I am grateful to Hashem for the positive that came from our experience. Dina and I have been blessed with an outpouring of love from so many and our writings and Dina's talks have been uplifting people all over. All this would not have been possible without the darkness we experienced, as it brought to the fore love and abilities we never knew we had. How can we not be grateful.

    With all this said, we all have had enough darkness in our lives. Now it is time for Moshiach to come and for the darkness to end. Let the light shine uninterrupted in our lives.


    See: https://childnervoussystem.blogspot.com/2016/01/triumph-over-adversity-2.html?showComment=1453336299938#c5307845136266627846

    Regarding Rabbi Yitzi Hurwitz (see January 20 comment above and Triumph over adversity 5/3/15 comment 5/14/15)


    See: https://childnervoussystem.blogspot.com/2016/01/triumph-over-adversity-2.html?showComment=1459448449126#c789168220191724973

  6. Abrahao A, Downar J, Pinto H, Dupré N, Izenberg A, Kingston W, Korngut L, O'Connell C, Petrescu N, Shoesmith C, Tandon A, Vargas-Santos AB, Zinman L. Physician-assisted death: A Canada-wide survey of ALS health care providers. Neurology. 2016 Sep 13;87(11):1152-60.

    To survey amyotrophic lateral sclerosis (ALS) health care providers to determine attitudes regarding physician-assisted death (PAD) after the Supreme Court of Canada (SCC) invalidated the Criminal Code provisions that prohibit PAD in February 2015.
    We conducted a Canada-wide survey of physicians and allied health professionals (AHP) involved in the care of patients with ALS on their opinions regarding (1) the SCC ruling, (2) their willingness to participate in PAD, and (3) the PAD implementation process for patients with ALS.
    We received 231 responses from ALS health care providers representing all 15 academic ALS centers in Canada, with an overall response rate for invited participants of 74%. The majority of physicians and AHP agreed with the SCC ruling and believed that patients with moderate and severe stage ALS should have access to PAD; however, most physicians would not provide a lethal prescription or injection to an eligible patient. They preferred the patient obtain a second opinion to confirm eligibility, have a psychiatric assessment, and then be referred to a third party to administer PAD. The majority of respondents felt unprepared for the initiation of this program and favored the development of PAD training modules and guidelines.
    ALS health care providers support the SCC decision and the majority believe PAD should be available to patients with moderate to severe ALS with physical or emotional suffering. However, few clinicians are willing to directly provide PAD and additional training and guidelines are required before implementation in Canada.