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A.L.S. Lving for today, locked in a paralyzed body  

CHN Editor Comments

I am always interested in articles that pertain to A.L.S., as I have had two friends die from it.  On the onset of being diagnosed with A.L.S., it is not at all uncommon for patients to fear the disease and its accompanying symptoms.  No doubt some will express a desire for assisted suicide.  The reasons for wanting to hasten their death are varied -   fear of the disease being at the top.   A.L.S. is a  progressive, paralyzing disease that will mean loss of certain independence.  Some fear loss of dignity.  While many use the term dignity, I prefer the term 'modesty'. (We don't put doors on bathroom stalls to preserve our dignity, but to protect our modesty and privacy.)  There may be a  fear becoming a burden to loved ones while others may fear being abandoned.  Depression is a common side affect, which hinders one from thinking rationally.  Suicide and assisted suicide may be the first thoughts that come to mind and often the hardest thoughts to rid oneself of especially when one is depressed. (Note in this article how Mr. MacDonald was told he had A.L.S.:

"The initial diagnosis, in February 2002, was delivered by a doctor who detailed the physical losses to come and eventual death in a clipped, clinical monologue that ended with his handing Mr. MacDonald a box of tissues and walking out. "If it weren't for the kids, I'd have gone off a bridge," he says."  

No one wants to suffer so the patient may begin to dwell on death rather than life  as a certain way to end all emotional and physical suffering.  

Unlike accidents which result in the person immediately becoming a quadriplegic - the patient with A.L.S. will lose certain abilities little by little.  Some patients with A.L.S. see this as a positive difference because it gives them a little more time to adapt to the overwhelming physical losses.  

It is crucial to have a team of support to come in and help that person want to will him or her self to live until natural death, or the disease runs its course.  It is a family disease in that, the whole family is affected.  The spouse or partner of the patient with A.L.S., has many needs such as respit, and someone to vent to. Children need to express their fears and hurt too. Such needs will change as time goes on. 

There are rewards for holding on to life. In this article, Dr.Lodish says he looks forward to each new day.  Lodish says because he has held on, "he has been able to see many of life's milestones, including the marriages of two of his three children."  Key to holding on or pressing in, is hope - and being surrounded with loving support.  I know my both my friends were blessed to have the love and support of their spouses and family.  According to their wives, neither ever expressed any desire to have their death hastened.  Just how key hope is, consider this clip from a serious indepth study on A.L.S., published in the New England Journal of Medicine [NEJM]:

We found that hopelessness, but not depression, was associated with a willingness to consider assisted suicide. In contrast, one study of patients with human immunodeficiency virus infection and two studies of patients with cancer found that depression was associated with an interest in hastening death.... 

Major depression is characterized by depressed mood or loss of interest or pleasure in activities. Associated findings include feelings of worthlessness or guilt; thoughts of death or suicide; difficulty concentrating, making decisions, or thinking; and changes in sleep, energy, appetite, and psychomotor activity. 

Hopelessness is a way of thinking in which negative expectations about the future are pervasive. Many depressed patients express hopelessness, but patients may be hopeless without being depressed. In patients with psychiatric disorders, hopelessness is a better predictor of suicidal intent and actual suicide than is depression.. . . Although some persons with ALS in our study expressed hopelessness, many similarly disabled persons did not.  NEJM Volume 339:967-973 Number 14 October 1, 1998 (emphasis mine) 

While much of the New York Times article is encouraging, I find the comments on statistics regarding deaths by assisted suicide to be rather dubious.  Since assisted suicide is illegal in except in Oregon, there is absolutely no accurate way of  determining the number of deaths by assisted suicide in the USA aside from Oregon.  

Dr. Ganzini (Dr. Ganzini was part of the team who conducted the study in the NEJM) claims patients with A.L.S. "are 25 times as likely to die by doctor-assisted suicide as people with other diseases." Dr Ganzini contradicts herself  here.  And Dr. McCluskey said that "at least 90 percent of patients with A.L.S. decided to die when they could no longer breathe on their own, although medical science can extend their lives much longer."  Is  McCluskey saying 90 percent died by suicide or assisted suicide?  Or did some or all of that percentage refuse a breathing tube?  In light of data published in the New England Journal of Medicine, I question the authenticity of these statistics. 

Dr. Cicely Saunders, of St. Christophers, in London England, is the founder of the modern palliative care movement. Physicians from many parts of the globe come to St. Christophers to learn more about her hospice and palliative programs which span 4 decades of service. Moreover, having cared for over 300 patients with A.L.S. and having written authoritative articles on A.L.S. would naturally qualify her as being an expert.  In her many articles, Saunders states that once the patient with A.L.S. is made comfortable and has support, assisted suicide no longer is a issue, even with patients who in the beginning insisted they would exercise their autonomy,and at the time of their choosing would seek assisted suicide.  Patients with A.L.S. die peacefully in her Hospice or at home, without their death being hastened.  We need many many more encouraging stories - sans talk of assisted suicide and euthanasia. Let us not forget the brilliant scientist, Stephen Hawking, who has been living with A.L.S. for nearly 40 years.  Visit Stephen Hawking and A.L.S..   

 Cheryl Eckstein

 

Living for Today, Locked in a Paralyzed Body 

 

Gregory MacDonald, paralyzed below the neck but committed to life, and his son, Dylan

By JOHN SCHWARTZ and JAMES ESTRIN  The New York Times

Published: November 7, 2004

Dr. Jules Lodish welcomes visitors to the downstairs bedroom of his Bethesda, Md., home with a robotic greeting that bursts from his computer's speaker.

Ten years of living with amyotrophic lateral sclerosis, or A.L.S., a progressive, paralyzing disease, have stilled nearly every muscle; he types with twitches of his cheek, detected by a sensor clipped to his glasses.

But ask him how he feels about his life, and Dr. Lodish, his eyes expressing the intensity denied to his body, responds: "I still look forward to every day."

A.L.S., or Lou Gehrig's disease, is often described as a kind of living death in which the body goes flaccid while the mind remains intact and acutely aware. The prospect of being trapped in an inert body and being totally dependent on others drives many sufferers to suicide.

When Attorney General John Ashcroft attacked an Oregon law allowing doctor-assisted suicide in 2001 - a case that is still working its ways through the legal system - patients with the disease were among those who supported the law in court.

But while the legal case and much of the national attention has focused on the issue of the right to die, less is known about those patients who want to live, and, like Dr. Lodish, will go to extraordinary lengths to do so.

With adequate medical care, patients often can live for years relatively free of physical pain from the disease itself. "It's more a sort of existential, psychic sort of pain," said Dr. Leo McCluskey, a neurologist in Philadelphia who treats many people with the disease.

As a result, patients and their families are forced, on a daily basis, to take stock of the meaning and quality of their lives and to make repeated decisions about how much is too much.

"With A.L.S., you have a choice about when to stop treatment," letting nature take its course, said Dr. Linda Ganzini, a professor of psychiatry at Oregon Health and Science University in Portland, who has studied patients making end-of-life decisions.

What keeps many patients alive, experts say, is a sense of having unfinished business - perhaps a milestone "like getting the last kid off to college," said Dr. Mellar P. Davis, a professor of hematology and medical oncology at the Cleveland Clinic.

Those patients who do best are those who have insurance that covers nursing and medical care, allowing them to avoid some of the major health risks associated with the disease, and family members who can cover the hours when expert help is unavailable.

Many patients, Dr. Ganzini said, have deep religious beliefs that help sustain them, and they are able, "to find hope in the future, find meaning and tolerate the daily ongoing losses that they are experiencing."

As Dr. Davis put it, "Quality of life becomes a moving target - what was one day an unacceptable quality of life becomes an acceptable quality of life."

Dr. Lodish's body sits limp in a wheelchair and his tongue lolls; a machine breathes for him through a tracheostomy tube in his throat. He lost the ability to talk more than three years ago, he says, then jokes, "but not the ability to be annoying."

At a time when even business executives fall into the shorthand of teenagers' text messages - "r u going 2 the mtg?" - he refuses to cut corners, communicating in eloquent sentences in person and via e-mail.

There are no half measures for Dr. Lodish, a hematologist and oncologist who devised his own intricately detailed treatment regimen. He wrote a 30-page guide for his nurses that sets standards for a sterile environment that go beyond hospital practices, rules that have helped him avoid the infections that kill many patients.

When he could no longer eat, he did the research to come up with a recipe for the nutrient blend that flows down his feeding tube - even determining that the ingredients were kosher - and he typed the two-page guide to its preparation, twitch by twitch, with a special program on the laptop that helps him to choose whole words or phrases from scrolling lists.

This wire to the world keeps him connected to his family and friends, and allows him to remain an important part of their lives. He continues to provide medical consultation, and now advises patients with A.L.S. and their families on how to organize their own care and use the communication devices he has mastered.

"One irony is with many people I communicate more now than when I was well," Dr. Lodish said.

By holding on, he said, he has been able to see many of life's milestones, including the marriages of two of his three children.

When his older daughter, Elizabeth Lester, became pregnant with the first grandchild, she asked her father to make the official family announcement.

"He still plays the same role for me," she said, "I still consult him on financial matters and other kinds of things."

Dr. Lodish said that his own determination to live comes, in part, from his long experience in treating cancer patients, who often feel that a diagnosis is a death sentence.

"I spent my career getting people to live with their illnesses until they died," he said, "if they weren't cured."

Now, he says, "My illness has validated my approach to my caring for people." His wife, Carolyn, says she and her husband draw comfort from each other and from family jokes that have worn thin with repetition and age.

"We all say he's a better dancer than he used to be," Mrs. Lodish said with a tired smile.

bulletChoosing Death

Not everyone with the disease makes the same choice as Dr. Lodish. Patients with the illness, Dr. Ganzini said, are 25 times as likely to die by doctor-assisted suicide as people with other diseases, she said.

Dr. McCluskey, the neurologist in Philadelphia, said that at least 90 percent of patients with A.L.S. decided to die when they could no longer breathe on their own, although medical science can extend their lives much longer. For many, the tracheostomy surgery to implant a breathing tube marks a dividing line between living naturally and being kept alive artificially, and introduces serious medical complications and new burdens of care on family members.

Without a tracheostomy, patients' lungs stop working on their own. The level of carbon dioxide builds up in the bloodstream and the patient goes to sleep. Morphine is given to ease suffering and anxiety. Within hours - sometimes within minutes - sleep gives way to coma and coma to death.

Once a tracheostomy has been performed, however, a patient who wants to end his life has to make an active decision to turn off the ventilator.

Dr. McCluskey discusses death with every patient, he said, but the conversations begin in earnest when breathing begins to become impaired and the choices are not so hypothetical. After they have come to a decision, he revisits the issue again and again, giving them opportunities to change their minds.

Most patients decide that they want a feeding tube, Dr. McCluskey said, but the vast majority turn down a breathing tube. Most of those who do request mechanical ventilation leave instructions in advance to turn off the machine if they come to the point that all connection to the outside world ends. He calls this the "locked-in state."

Dr. Lodish has not yet reached that point, but he said that when he did, he could imagine deciding that he had had enough of living with his illness.

"It would have to interfere with my ability to relate to people," he said.

The crucial element, Dr. McCluskey said, is choice.

"That's what this whole conversation is about - to empower people," he said, "even though they are so debilitated and sick, they are actually in control."        

Photo(missing): Dr. Jules Lodish is a hemotologist and oncologist living with A.L.S. for 10 years.  He is not retired.

bulletNeeded for Longevity

Dr. John R. Bach, a respiratory expert at University Hospital in Newark, said that more patients might choose to live longer if their doctors knew more about the disease and the best treatments - and about hope.

"Physicians invariably tell these patients that they have a year or two to live and that there's no hope," he said.

Dr. Bach said he encouraged patients to think in terms of the longest possible life with the disease, and he has developed techniques to put off the insertion of a tracheostomy tube as long as possible, including mechanized girdles that mimic breathing, and masks and mouthpieces that keep the air flowing in and out of the lungs.

Speaking of the withdrawal of medical support, Dr. Bach said, "I have nothing against euthanasia" for patients who truly want to die. But, he said, "you should not allow euthanasia for the first two years," because many patients reach an accommodation with the illness.

"Do you know what happiness is?'' Dr. Bach says, citing a survey that showed patients on ventilators tend to rank their level of satisfaction with life at 5.1 out of a possible score of 7; the average person in the same surveys has a score of 5.5. "Happiness is reality divided by expectations."

Most days, Gregory MacDonald faces his disease with his own blend of black humor and commitment, and says that he wants to live for years to come. Other days he is not so sure.

A.L.S. has ravaged his once-muscular frame. He is unable to move from the neck down. On one of the recent good days, Dr. Bach cheered him with the opinion that if his lung capacity remained stable, Mr. MacDonald could survive without a tracheostomy for another 10 or 15 years.

He was joyful. But an acquaintance who had driven him to see the doctor was incredulous. Mr. MacDonald recalled that she asked, "You want to stay like that for 10 or 15 years?"

"Absolutely!" he responded.

Just a few weeks later, however, came a bad day. He choked on a pill and did not have the strength to cough it up or work it down. The panicky and miserable ordeal lasted for nearly an hour, and he saw a dwindling existence ahead.

"Is life worth it at all costs?" he asked. "If I'm exchanging carbon dioxide, is that the only criterion?"

A school administrator, Mr. MacDonald was an energetic outdoorsman before the disease struck him. He does not fully buy into his doctor's sunny outlook.

"It's a living nightmare, and you can't imagine anything worse," he said. "But from my life experience, I can guarantee you that things can always get worse."

Once an avid guitarist - he played finger-picking country and blues - the first signs of illness came, he said, when "I couldn't play basic things." The initial diagnosis, in February 2002, was delivered by a doctor who detailed the physical losses to come and eventual death in a clipped, clinical monologue that ended with his handing Mr. MacDonald a box of tissues and walking out.

"If it weren't for the kids, I'd have gone off a bridge," he says.

Today he sits in his Tuckahoe, N.Y., apartment on a recliner, a 52-year-old man who can say with a smile, "With the exception of a fatal disease, I'm in almost perfect health."

He punctuates his conversation with a frequent turn of the head to sip air from a mouthpiece.

He says that the good days outnumber the bad days, but that the bad days can be intense. Recently, he won a long dispute with his insurance company, allowing him 12 hours a day of professional nursing assistance instead of the 4 hours a day of the low-skilled care he had before. The difference in his quality of life, he said, has "given me a whole new outlook."

Friends and family, including his girlfriend, Julia Stephenson, and his former wife, Catherine Beazer, help fill in the hours that his insurance does not cover.

"It is in a sense a family disease," Dr. Davis of the Cleveland Clinic said.

Mr. MacDonald is tied into the daily lives of his 13-year-old son, Dylan, and his 11-year-old daughter, Cheyenne, whose custody he shares with Ms. Beazer.

Mr. MacDonald suffers from the litany of medical problems that come with the disease: frozen joints that have to be kept supple through physical therapy, swollen toes that weep blood and can easily become infected. A hand left resting on his leg, over time, can cause agony as gravity forces the fingernail into his skin - a pain that he says can sometimes be worse than the time on a fishing boat when he accidentally slashed his hand with a gutting knife. He still allows himself small treats - a martini in the evening ("Medicinal," he says) and one cigar a week.

When he listens to the songs he once played, it can evoke a physical memory of playing that strikes him unexpectedly.

"A specific tone in a song, a specific cord change can do that - can make me quite weepy," he said.

Before his musical ability drifted away, he recorded a song that he had written for Dylan and Cheyenne. The cassette sits in a safe. 

At the back of Mr. McDonald's mind is a grim calculus of life, death and pensions. He stopped working last spring after completing the school year in a wheelchair, and will retire when his months of previous unused sick days run out in January. If he should die before retirement, his children will receive three years' salary as a death benefit. Over the next three years, the death benefit diminishes and then vanishes.

Mr. McDonald says he wants to be there for his children, but he  wants to be more than a mere "living presence." He wants, he says, a life and a role in their lives that is "not only dignified but meaningful."

"If I can't eat and I can't speak, quite frankly I can't imagine wanting to entertain that for very long," he said.

But he has trouble envisioning dying. "When you have so much to live for, the thought of it becoming unbearable doesn't - it never overshadows the fact that I can't imagine saying goodbye."

bulletMeaning for Others

Finding meaning in life is not only the fundamental challenge for A.L.S. patients, said Dr. Adele Zinberg, a psychiatrist who works with the homeless and who has lived with the disease for seven years.

"A lot of what I do in my work is help people find meaning - everyone needs it, whether it's through their family or their work or some other cause," said Dr. Zinberg, who can operate a powered wheelchair. "Everyone has to feel that their life has meaning."

She says that some patients ask, "How can I talk to you about my problems when you have your own problems?"

She tells them: "Mine seem more obvious. But we all have our own challenges."

Dr. Lodish counsels other people who have suffered life blows. He recalled that a doctor friend recently asked his advice about ways to help a friend who was deeply depressed after a serious automobile accident.

"Much of this boils down to whether or not one can hang on to who one is," he wrote in response. He said that he was still what he had always been: a father, a husband, a friend.

"In fundamental ways, I feel totally unchanged," he said. "Quintessentially, I have found that ambulation, movement, swallowing, eating, talking, breathing, and self care are not me. They are substantial physical losses; but they are not me."

Source http://www.nytimes.com/2004/11/07/health/07A.L.S..html?ex=1100408400&en=c7dab0b510b0b967&ei=5006&partner=ALTAVISTA1

 
bulletMany Als Patients Favor Assisted Suicide 

NEW YORK, Sep 30 (Reuters) -- A majority of patients with the fatal neuromuscular disease amyotrophic lateral sclerosis (ALS, or Lou Gehrig's disease) say they would consider assisted suicide, according to a report in the October 1st issue of The New England Journal of Medicine.

"Many (patients) would request a prescription for a lethal dose of medication well before they intended to use it," report a team of researchers in Portland, Oregon, led by Dr. Linda Ganzini of Oregon Health Sciences University. In 1994, Oregon voters approved the Oregon Death with Dignity Act, clearing the way for the legal use of physician-assisted suicide. Delayed by court challenges, the Act was finally implemented in 1997.

Despite the enormous media coverage surrounding the assisted suicide debate, the Portland researchers point out that the level of acceptance of this option among terminally ill ALS patients "has not been studied."

ALS patients face one of the most bleak prognoses of any patient group, since their incurable neurological illness triggers a gradual, irreversible paralysis ending in death -- usually within 2 to 3 years of diagnosis.

The investigators questioned 100 ALS patients as to their opinions regarding physician-assisted suicide. All of those interviewed had suffered from ALS for an average of nearly 3 years.

According to the survey, "56 patients said they would consider assisted suicide." Most of those patients (44 of 56) said they would request a prescription of a lethal dose of medication from their physician, if such assistance was legally available.

"Only one patient, however, would take the medicine immediately," the researchers point out. "Most of the patients would reserve it for future use. These findings support the notion that some seriously ill persons gain psychological comfort from knowing that taking a lethal dose of medication is an option." The authors found that patients considering assisted suicide were more likely to be male, well-educated, and non-religious, compared with patients rejecting such an option.

In a commentary, Dr. Lewis Rowland of Columbia-Presbyterian Medical Center in New York City, noted that the Oregon state law might prove useless to many late-stage ALS patients. He explained that the law allows for the use of assisted suicide using a lethal dose of oral drugs, with lethal injection being prohibited. However, "all patients with ALS who live long enough will lose the use of their hands," Rowland points out. In these cases, "someone must administer the drug," he said, "but that would be euthanasia, not assisted suicide, and we would be on the slippery slope feared by critics of physician-assisted suicide."

Rowland calls for more research into discovering the cause of ALS, which he calls "the best hope for finding effective treatment," and legislation to expand the availability of palliative care.

This week's issue of the Journal also carries an essay by Carol Poenisch of Northville, Michigan, describing the 1993 assisted suicide of her ALS-stricken mother, Merian Frederick. The suicide, using carbon monoxide gas, was carried out with the help of Dr. Jack Kevorkian.

According to her daughter, Frederick "wanted control over her life at a time when every day was a struggle to regain control over a lost function."

Poenisch describes how, after documenting that assisted suicide was Frederick's wish, and informing family members and her minister, Frederick's son drove her to Kevorkian's apartment in the middle of the night in order to "avoid turning my mother's house into a crime scene." Her son lived out of state, "and we thought it was therefore less likely that he would be prosecuted." The rest of the family went to a motel to avoid the media. At 4 AM, they learned "that everything had gone well." And at 8 AM, their mother's death was the lead story on the television news.

"Again, we had the feeling that we were criminals who hadn't committed a crime," writes Poenisch.

Of Kevorkian, she writes, "my mother really needed his help, legal or not, and we are thankful he was there."

Poenisch now believes "that many of the hardest aspects of (the suicide) would have been eliminated if the choice she made had been legal." She is a founder of Merian's Friends, a group focused on legalizing assisted suicide in the state of Michigan.

SOURCE: The New England Journal of Medicine 1998;339:967-973, 987-989, 996-998. 

Source of Many Als Patients Favor Assisted Suicide http://www.personalmd.com/news/a1998093012.shtml

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Many with Lou Gehrig's Seek Doctor's Help to Die

Reuters Health
By Alison McCook
Wednesday, May 22, 2002 NEW YORK (Reuters Health) - In the Netherlands,
one in five patients with amyotrophic lateral sclerosis (ALS), often called Lou Gehrig's disease, chooses to die by euthanasia or physician-assisted suicide, Dutch researchers report.

Physician-assisted suicide allows a doctor, under certain conditions, to prescribe drugs that enable a critically ill patient to take his or her own life when desired. In euthanasia, the physician actually administers the lethal drugs. Euthanasia and assisted suicide are currently illegal in the Netherlands, but the acts are not punishable if performed by a doctor under strict guidelines.

And both require "the patient's explicit request," study author Dr. Leonard H. van den Berg, of University Medical Center Utrecht, told Reuters Health.

He added that understanding how many ALS patients choose to end their lives, and at what point in their disease they elect to do so, may help physicians to better treat these patients during their last days of life.

ALS is an invariably fatal disease that attacks the nerve cells that control voluntary muscles. Gradually, muscles weaken and waste, and patients develop difficulty speaking, swallowing and breathing. About one half of patients die within 3 years of their first symptoms.

In the new study, researchers based their estimate of the rate of assisted suicide and euthanasia among ALS patients on surveys of 203 physicians regarding 203 patients' end-of-life decisions.

The investigators found that 17% of patients--a total of 35--chose euthanasia and died that way. An additional six patients, or 3% of the total number, died by physician-assisted suicide. Patients who chose physician-assisted suicide tended to end their lives "somewhat" earlier in the course of the disease than those who chose euthanasia, the report indicates.

However, van den Berg said that all patients who ended their lives did so during the last stages of their disease. In fact, he and his team noted that most patients who died by euthanasia likely shortened their lives by less than one month. They estimate that most of the six patients provided physician-assisted suicide would have lived more than a month longer.

Patients' decisions to end their own lives did not seem to relate to their level of care, education or income, nor to any specific characteristics of the disease, the investigators write in the May 23rd issue of The New England Journal of Medicine. However, ALS patients who opted for euthanasia tended to have more disability than those who died of other causes.

"Patients with ALS may consider having control over their death especially important, since their inability to work, engage in pleasurable activities, care for themselves and communicate constitutes a formidable loss of autonomy; having control over dying could be a means of limiting the sense of loss of self," the authors write.

In addition, ALS patients who said religion was an important part of their lives were less likely than others to choose euthanasia or physician-assisted suicide.

Van den Berg said that suicide rates among ALS patients in other countries may match--and possibly exceed--what is seen in the Netherlands.

In an accompanying editorial, Dr. Linda Ganzini of the Department of Veteran Affairs Medical Center in Portland, Oregon, and Dr. Susan Block of the Dana-Farber Cancer Institute in Boston, Massachusetts, write that researchers should further investigate and address the reasons why ALS patients choose to end their own lives.

Improved end-of-life care, the editorialists note, will help ease the suffering of ALS patients during their last days and may help them face their death with less fear.

"Not only do such efforts decrease interest in euthanasia," they write, "but they affirm patients' humanity."

SOURCE: The New England Journal of Medicine 2002;346:1638-1644, 1663-1665.

Source of article http://www.focusonals.com/many_with_lou_gehrig's_seek_doctor's_help_to_die.htm

 

Other pages of interest:  Stephen Hawking and A.L.S.

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