Disabled Travelers Guide to the World

In an article in a recent San Francisco Chronicle, there was mention of this website:
http://www.disabledtravelersguide.com/

The Chronicle article says: “It’s worth a look, if only for some of the homey, casual approach to really helpful information.”

A copy of the 2008 book “Disabled Travelers Guide to the World” can be read online and/or downloaded at no charge. (From the home page, click on the blue book image on the left.)

“Letting Go: What should medicine do when it can’t save your life?”

There’s a thought-provoking article in the August 2010 issue of “The New Yorker” magazine on modern medicine being “good at staving off death with aggressive interventions — and bad at knowing when to focus, instead, on improving the days that terminal patients have left.”

Here are some excerpts:

* People have concerns besides simply prolonging their lives. Surveys of patients with terminal illness find that their top priorities include, in addition to avoiding suffering, being with family, having the touch of others, being mentally aware, and not becoming a burden to others. Our system of technological medical care has utterly failed to meet these needs, and the cost of this failure is measured in far more than dollars. The hard question we face, then, is not how we can afford this system’s expense. It is how we can build a health-care system that will actually help dying patients achieve what’s most important to them at the end of their lives.

* In the past few decades, medical science has rendered obsolete centuries of experience, tradition, and language about our mortality, and created a new difficulty for mankind: how to die.

* The difference between standard medical care and hospice is not the difference between treating and doing nothing… The difference was in your priorities. In ordinary medicine, the goal is to extend life. We’ll sacrifice the quality of your existence now—by performing surgery, providing chemotherapy, putting you in intensive care—for the chance of gaining time later. Hospice deploys nurses, doctors, and social workers to help people with a fatal illness have the fullest possible lives right now. That means focussing on objectives like freedom from pain and discomfort, or maintaining mental awareness for as long as possible, or getting out with family once in a while. Hospice and palliative-care specialists aren’t much concerned about whether that makes people’s lives longer or shorter.

* It’s worth pausing to consider what had just happened. Step by step, Sara ended up on a fourth round of chemotherapy, one with a minuscule likelihood of altering the course of her disease and a great likelihood of causing debilitating side effects. An opportunity to prepare for the inevitable was forgone. And it all happened because of an assuredly normal circumstance: a patient and family unready to confront the reality of her disease.

“It has become, in my view, a bit too trendy to regard the acceptance of death as something tantamount to intrinsic dignity,” [Stephen Jay Gould] wrote in his 1985 essay. “Of course I agree with the preacher of Ecclesiastes that there is a time to love and a time to die—and when my skein runs out I hope to face the end calmly and in my own way. For most situations, however, I prefer the more martial view that death is the ultimate enemy—and I find nothing reproachable in those who rage mightily against the dying of the light.”

The fact that we may be shortening or worsening the time we have left hardly seems to register. We imagine that we can wait until the doctors tell us that there is nothing more they can do. But rarely is there nothing more that doctors can do.

Two-thirds of the terminal-cancer patients in the Coping with Cancer study reported having had no discussion with their doctors about their goals for end-of-life care, despite being, on average, just four months from death.

“A family meeting is a procedure, and it requires no less skill than performing an operation,” said Dr. Susan Block.

According to Block, about two-thirds of patients are willing to undergo therapies they don’t want if that is what their loved ones want.

And here’s a link to this terrific article:

http://www.newyorker.com/reporting/2010/08/02/100802fa_fact_gawande?currentPage=all

ANNALS OF MEDICINE
LETTING GO
What should medicine do when it can’t save your life?
by Atul Gawande
The New Yorker
August 2, 2010

Robin

“How can I be sure it’s PSP?”

I received this email this evening. Perhaps others can respond.

“I was diagnosed with PSP in 2006. I am 34 years old. How can I be sure it’s PSP? Well I was in denial for the longest time. I have gone to a second and third opinion. … My speech was the first symptom, I had everyone telling me to go to the doctor to get checked out. I brushed it off like I was just over-tired. When I finally went to a specialist, he told me I would be the perfect candidate for this disease called PSP with the exception of my age. He sent me to an eye specialist and he grabbed a drum like contraption with black and white lines. He spun it and said I had PSP. My balance has been an issue, with constant falls. And I cannot drink water like I used to.”

“Medicine for a disease that has no known cure is tough to find. I was on Sinemet for a while I went up to 12 pills daily. I stopped because it wasn’t having any affect on me anymore. I participated in that study with CoQ10, with no significant change or determination. I am currently taking Amantadine, it seems to help at least with the balance thing.”

“On Caregiving” (by anthropologist caregiver)

Local group member Judy forwarded this article from the current issue of the Harvard Magazine on to me.  It’s adapted from two articles by a Harvard Medical School professor of medical anthropology on the topic of caregiving, published in the UK medical journal The Lancet.  The Harvard professor, Arthur Kleinman, is the author of What Really Matters: Living a Moral Life Amidst Uncertainty and Danger.  He is also the caregiver of his wife with an atypical form of Alzheimer’s Disease.

Here’s an excerpt:

…I am writing principally about people like me who give care to loved ones who suffer the infirmities of advanced age, serious disabilities, terminal illnesses, and the devastating consequences of such health catastrophes as stroke or dementia.

Faced with these crises, family and close friends become responsible for assistance with all the mundane, material activities of daily living: dressing, feeding, bathing, toileting, ambulating, communicating, and interfacing with the healthcare system. Caregivers protect the vulnerable and dependent. To use the experience-distorting technical language: they offer cognitive, behavioral, and emotional support. And because caregiving is so tiring, and emotionally draining, effective caregiving requires that caregivers themselves receive practical and emotional support.

But, to use the close experiential language of actually doing it, caregiving is also a defining moral practice. It is a practice of empathic imagination, responsibility, witnessing, and solidarity with those in great need. It is a moral practice that makes caregivers, and at times even the care-receivers, more present and thereby fully human.

Here’s a link to the full article:

harvardmagazine.com/2010/07/on-caregiving

Forum: On Caregiving
A scholar experiences the moral acts that come before—and go beyond—modern medicine.
by Arthur Kleinman
Harvard Magazine

July-August 2010

This is well worth reading!  I’ve copied the full text below of the combined article from Harvard Magazine.

And, if you prefer, you can read the two articles from The Lancet, which are posted on the Harvard Magazine‘s website here:

Catastrophe and caregiving: the failure of medicine as an art
The Lancet
January 5, 2008

harvardmagazine.com/sites/default/files/Lancet_Catastrophe-and-caregiving.pdf

Caregiving: the odyssey of becoming more human
The Lancet
January 24, 2009

harvardmagazine.com/sites/default/files/Lancet_Caregiving.pdf

Robin



Forum: On Caregiving

A scholar experiences the moral acts that come before—and go beyond—modern medicine.
by Arthur Kleinman
Harvard Magazine

July-August 2010

In 1966, as a visiting medical student at a London teaching hospital, I interviewed a husband and wife, in their early twenties, who had recently experienced a truly calamitous health catastrophe. On their wedding night, in their first experience of sexual intercourse, a malformed blood vessel in the husband’s brain burst, leaving him with a disabling paralysis of the right side of his body. Stunned and guilt-ridden, the couple clutched hands and cried silently as they shared their suffering with me. My job was to get the neurological examination right and diagnose where the rupture had taken place. I remember the famous professor, who went over my findings, repeating the neurological examination and putting me through my paces as a budding diagnostician. He never once alluded to the personal tragedy for the sad lovers and the shock to their parents. Finally, I found the courage to tell him that I thought the failure to address what really mattered to them—how to live their lives together from here on—was unacceptable. Surely, it was our medical responsibility to offer them some kind of caregiving and hope for the future. He smiled at me in a surprised and patronizing way; then he said I was right to insist that there was more to this case than the neurological findings. The professor had the patient and his wife brought to the lecture hall where he presided over the teaching rounds, and he gave them as sensitive an interview as I might have hoped for, including empathic suggestions for rehabilitation, family counseling, and social-work assistance.

I have long regarded this experience as iconic of contemporary medicine’s caregiving paradox. The balance between science/technology and art has shifted so far toward the former that the latter is a pale shadow, a fragile remnant of what had for centuries been crucial to the work of the doctor. To prepare for a career of caregiving, medical students and young doctors clearly require something besides scientific and technological training that, even as it enables the physician as a technical expert, risks disabling her or him as a caregiver.

That work can be reasserted when we have the time and desire to do so, as the distinguished neurologist showed me, but it is regularly overlooked and left aside. I recently visited a noted southeast Asian hospital where the doctor-patient relationship for patients returning for follow-up visits lasts one to three minutes—hardly time to ask a few questions, do the quickest physical examination, and write a prescription. Not a place where the art of caregiving is likely to flourish. The residents with whom I spoke got the message. The structure of training and of service delivery discourages and even disables the art.

My own experience of being the primary caregiver for my wife, on account of her neurodegenerative disorder, convinces me yet further that caregiving has much less to do with doctoring than the general public realizes or than medical educators are willing to acknowledge. Caregiving is about skilled nursing, competent social work, rehabilitation efforts of physical and occupational therapists, and the hard physical work of home healthcare aides. Yet, for all the efforts of the helping professions, caregiving is for the most part the preserve of families and intimate friends, and of the afflicted person herself or himself. We struggle with family and close friends to undertake the material acts that sustain us, find practical assistance with the activities of daily living, financial aid, legal and religious advice, emotional support, meaning-making and remaking, and moral solidarity. About these caregiving activities, we know surprisingly little, other than that they come to define the quality of living for millions of sufferers.

I lead her across the living room, holding her hand behind my back, so that I can navigate the two of us between chairs, sofas, end tables, over Persian rugs, through the passageway and into the kitchen. I help her find and carefully place herself in a chair, one of four at the oval-shaped oak table. She turns the wrong way, forcing the chair outward; I push her legs around and in, under the table’s edge. The sun streams through the bank of windows. The brightness of the light and its warmth, on a freezing winter’s day, make her smile. She turns toward me. The uneven pupils in Joan Kleinman’s green-brown eyes look above and beyond my head, searching for my face. Gently I turn her head towards me. I grin as she raises her eyebrows in recognition, shakes her long brown hair, and the soft warmth of her sudden happiness lights up her still strikingly beautiful face. “Wonderful!” she whispers. “I’m a Palo Alto, a California, girl. I like it warm.”

I place a fork in her right hand and guide it to the poached egg in the deep bowl. I have already cut up the toast, so that I can help her spear pieces of bread and soak up the yolk. She can’t find the teacup in front of her, so I move her hand next to its handle. The Darjeeling tea glows hot and golden red in the Chinese cup. “Wonderful!” she again whispers.

Later, while I am trying to decide what she should wear, Joan frowns, fussing with her feet. “These nails are too long. And where are my shoes? I need to find my shoes!” She stands before about 18 pairs on a rack, shoes her unseeing brain can’t recognize. “Don’t get agitated,” I interject with foreboding. “Do you want a Zyprexa?”

“No! No pills. Why do I need pills? I’m healthy.”

“Joan, you have Alzheimer’s disease. You’re not healthy. You have a brain disease. A serious problem.” I can barely conceal the frustration in my voice.

“Why did God do this to me? I’ve always been good. I never did anything to cause this. Should I kill myself?” She says it in such a way as to signal to me, as she has before, that this is a statement of pain and a cry for help, not an earnest question to discuss or to make plans. In fact it means the opposite: because, as in the past, she quickly changes tone. “If you love then you can do it! We can live and love.”

“We can do it,” I repeat, each time a little bit more weakly, enduring the unendurable.

And so another morning begins, another day of caregiving and care-receiving between a 69-year-old man and a 70-year-old woman who have lived together passionately and collaboratively for 45 years, absorbed in an intense relationship— intellectual, aesthetic, sexual, emotional, moral. What has made it possible to get even this far are our two adult children, their spouses, my 96-year-old mother, my brother, and our four grandchildren who sometimes take the hand of their often uncomprehending grandmother, because she is standing alone, lost, and lead her back into the protective, enabling circle of our family.

For seven years we have lived through the progressive neurodegenerative disorder that has unspooled the neural networks of Joan’s brain. It originated in the occipital lobes at the far back of the brain. The pathology of undoing has inexorably worked its way forward to the parietal and temporal lobes on the sides of the brain, and finally to the frontal lobes that mount up behind the forehead, through the layers of neurons and nodes of connecting neural nets that structure and retain memories, focus attention, balance emotion with common sense, underwrite judgment, and make possible the ordinariness of reading, writing, telling stories, understanding jokes, recognizing people, orienting oneself in space and in time, but also within emotional and moral coordinates, and, of course, doing things in the world.

This trail of unraveled brain structure and mounting dysfunction is, in physical terms, only one of inches; yet its silent, implacable wrecking creates entirely new conditions for living a life and being with others. Joan has an atypical form of Alzheimer’s disease. She is functionally blind. She cannot find her way in our home, where she has lived since 1982. She often misinterprets those objects she does see, treating a chair as if it were a table or the floor lamp a person. Left unaccompanied, she walks into doors and has banged her legs so hard into low tables she didn’t see that she has caused deep contusions. Once, at our son’s house, she opened a door and fell down a flight of unseen stairs, breaking her pelvis; at the onset of the disease, she ran into the street, where a pick-up truck ran over her right foot.

Joan can’t, on her own, find her way out of the bedroom. Yet, once safely in my hands or those of our trusted home health aide, she can walk effectively. A China scholar who translated and interpreted ancient texts, she can no longer read. A wife and mother whose fierce commitment to the family was its moral backbone, she now struggles to be part of family functions and can sometimes seem impassive and cut off from us. Formerly the primary caregiver for her husband and children, she is now the care-receiver. She may no longer be who she was even seven years ago, but her subjectivity has not so much disappeared—there is much of her personality that is still present—as altered. And that alteration has affected what had been for four decades an all-consuming relationship—our identity and orientation. I still cannot accept to treat her as if she can no longer share the sensibility and narrative we have created, and yet, more and more frequently, she can’t. She is happy much of the time. It is I, the caregiver, who, more often, am sad and despairing.

She is a source of great concern to each of us, her family members, about how to best manage her condition. We grieve what we have lost and fear what we know lies ahead. We have each of us gone through feelings of loss, anger, and frustration. We have been marked by a special kind of pain. But we have also experienced a deepening sense of responsibility, gratitude for all that we had lived through together, love, solidarity, and a shared sensibility that we have resisted what is beyond our control and are, individually and collectively, more for it. This is not meant as a self-satisfying summing up—there is no final summary yet and the proper genre is tragedy, as millions who are engaged in these everyday practices know.

Economists configure caregiving as “burden.” Psychologists talk about “coping,” health-services researchers describe social resources and healthcare costs, and physicians conceive it as a clinical skill. Each of these perspectives represents part of the picture. For the medical humanities and interpretive social sciences, caregiving is a foundational component of moral experience. By this I mean that we envision caregiving as an existential quality of what it is to be a human being. We give care as part of the flow of everyday lived values and emotions that make up moral experience. Here collective values and social emotions are as influential as individual ones. Within these local moral worlds—family, network, institution, community—caregiving is one of those things that really matters, but usually not the only thing.

As a scholar, I engage with other medical humanists to understand the dimensions of this moral activity—how it is experienced and organized. In part, I hope it can be better taught. I believe that what doctors need to be helped to master is the art of acknowledging and affirming the patient as a suffering human being; imagining alternative contexts and practices for responding to calamity; and conversing with and supporting patients in desperate situations where the emphasis is on what really matters to the patient and his or her intimates. A program of medical training that makes this happen, however it is innovated, should combine practical experience of caregiving for health catastrophes in homes and institutions, where students actually do those things that families do, with the knowledge that stands behind the art of medicine.

But here, I am writing principally about people like me who give care to loved ones who suffer the infirmities of advanced age, serious disabilities, terminal illnesses, and the devastating consequences of such health catastrophes as stroke or dementia.

Faced with these crises, family and close friends become responsible for assistance with all the mundane, material activities of daily living: dressing, feeding, bathing, toileting, ambulating, communicating, and interfacing with the healthcare system. Caregivers protect the vulnerable and dependent. To use the experience-distorting technical language: they offer cognitive, behavioral, and emotional support. And because caregiving is so tiring, and emotionally draining, effective caregiving requires that caregivers themselves receive practical and emotional support.

But, to use the close experiential language of actually doing it, caregiving is also a defining moral practice. It is a practice of empathic imagination, responsibility, witnessing, and solidarity with those in great need. It is a moral practice that makes caregivers, and at times even the care-receivers, more present and thereby fully human.

If the ancient Chinese perception is right that we are not born fully human, but only become so as we cultivate ourselves and our relations with others—and that we must do so in a threatening world where things often go terribly wrong and where what we are able to control is very limited—then caregiving is one of those relationships and practices of self-cultivation that make us, even as we experience our limits and failures, more human. It completes (not absolutely, but as a kind of burnishing of what we really are—warts and all) our humanity. And if that Chinese perspective is also right (as I believe it is), when it claims that by building our humanity, we humanize the world, then our own ethical cultivation at the very least fosters that of others and holds the potential, through those relationships, of deepening meaning, beauty, and goodness in our experience of the world.

I am not a naive moralist. I’ve had far too much experience of the demands, tensions, and downright failures of caregiving to fall into sentimentality and utopianism. Caregiving is not easy. It consumes time, energy, and financial resources. It sucks out strength and determination. It turns simple ideas of efficacy and hope into big question marks. It can amplify anguish and desperation. It can divide the self. It can bring out family conflicts. It can separate those who care from those who can’t or won’t handle it. It is very difficult. It is also far more complex, uncertain, and unbounded than professional medical and nursing models suggest. I know about the moral core of caregiving not nearly so much from my professional life as a psychiatrist and medical anthropologist, nor principally from the research literature and my own studies, but primarily because of my new life of practice as a primary caregiver.

I learned to be a caregiver by doing it, because I had to do it; it was there to do. I think this is how most people learn to be caregivers, for people who are elderly, disabled, or chronically or terminally ill. But of course this is also how parents, especially mothers, learn to care for children. My point is not so dissimilar to what William James claimed was how we learn to feel emotions: we move, we respond, we act. Our muscles (voluntary and involuntary) move. And so out of practices comes affect. And out of practices comes caregiving. And out of the billions of ordinary acts of caregiving perhaps also comes much of that which, imperceptibly and relentlessly, sustains the world.

We are caregivers because we practice caregiving. It is all the little concrete things I described in caring for my wife that taken together and over time constitute my caregiving, that make me a caregiver. So much depends on those concrete things: the doing, the feeling, the shadings, the symphonic complexity, the inadequacy, the living at every moment and over what can be such a long journey of the incompleteness yet the presence of a caregiver.

————–

Arthur Kleinman is Rabb professor of anthropology in the Faculty of Arts and Sciences, professor of medical anthropology and professor of psychiatry at Harvard Medical School, and Fung director of the Harvard University Asia Center. He is the author of What Really Matters: Living a Moral Life Amidst Uncertainty and Danger. This essay was adapted from two articles written for The Lancet’s Art of Medicine department, “Catastrophe and caregiving: The failure of medicine as art” (371:9606, 22-23; January 5, 2008) and “Caregiving: The odyssey of becoming more human” (373:9660, 292-293; January 24, 2009), available to our readers as PDFs courtesy of The Lancet. The second article contains suggestions for further reading.

 

LA Times article on anticholinergics + a list

Hopefully you all are aware of the potential side effects of anticholinergic medication — particularly important to know about for the elderly and for those with dementia.

There was a good Los Angeles Times article on this topic last week.  See:

www.latimes.com/news/health/la-heb0713-cognitive-impairment-20100713,0,5747679.story

Medicines from class of drugs commonly used by the elderly, including Benadryl and Dramamine, can cause impaired thinking, study finds
Los Angeles Times
Thomas H. Maugh II
4:05 PM PDT, July 13, 2010

Mentioned in the article is a resource that I’ve never heard of before but looks like it would be very promising:  it’s a list of the “anticholinergic cognitive burden” of anticholinergic medications.  You can find it here:

www.indydiscoverynetwork.org/AnticholienrgicCognitiveBurdenScale.html

Anticholinergics block the activity of the neurotransmitter acetylcholine, which is important for cognition.  The most commonly used anticholinergics by the elderly are sleep aids (such as Benadryl, Excedrin PM, Tylenol PM) and incontinence drugs (such as Detrol).  Quite a few antidepressants (Paxil, Elavil) are also anticholinergic.

Robin

 

LA Times article on anticholinergics + a list

Hopefully you all are aware of the potential side effects of anticholinergic medication — particularly important to know about for the elderly and for those with dementia. There was a good Los Angeles Times article on this topic last week. It’s copied below along with a link to the article.

Mentioned in the article is a resource that I’ve never heard of before but looks like it would be very promising: it’s a list of the “anticholinergic cognitive burden” of anticholinergic medications. You can find it here:
http://www.indydiscoverynetwork.org/Ant … Scale.html

http://www.latimes.com/news/health/la-h … 7679.story

Medicines from class of drugs commonly used by the elderly, including Benadryl and Dramamine, can cause impaired thinking, study finds

Thomas H. Maugh II, Los Angeles Times
4:05 PM PDT, July 13, 2010

The family of drugs that includes the over-the-counter medications Benadryl, Dramamine, Excedrin PM, Sominex and Tylenol PM can double the risk of impaired thinking in elderly African Americans, and presumably in Caucasians as well, researchers said Tuesday.

The family, called anticholinergics, blocks the activity of the neurotransmitter acetylchoine, and also includes the prescription drugs Paxil, Detrol, Demerol and Elavil. Most of the anticholinergics are used by the elderly to aid sleep and to relieve bladder leakage problems. The drugs have previously been shown to increase the risk of delirium as well. Delirium is a form of sudden-onset cognitive impairment.

Blacks are already known to be at high risk for cognitive impairment, one reason that researchers focused specifically on them. The team, led by Dr. Malaz Boustani of the Regenstrief Institute at the Indiana University School of Medicine, took advantage of an ongoing study called the Indianapolis Ibadan Dementia Project, whose aim is to compare risk factors for dementia in blacks in Indianapolis and Yoruba in Ibadan, Nigeria. The new study involved 1,652 Indianapolis blacks who did not suffer cognitive impairment when they entered the study. Researchers collected information from participants about their use of the drugs, and also collected medicine bottles from their homes during each assessment. They found that 11% of the subjects used anticholinergics.

After six years of follow-up, the team reported in the journal Neurology, patients taking one anticholinergic were 46% more likely to suffer from cognitive impairment, while those taking two were twice as likely. The risk was somewhat higher in the patients who did not carry what is known as the E4 variant of the gene APOE, which plays a role in the metabolism of triglycerides, a form of cholesterol. That finding was somewhat surprising because APOE E4 increases the risk of Alzheimer’s disease.

“Simply put, we have confirmed that anticholinergics, something as seemingly benign as a medication for inability to get a good night’s sleep or for motion sickness, can cause or worsen cognitive impairment, specifically long-term mild cognitive impairment which involves gradual memory loss,” Boustani said in a statement. “I tell all my [elderly] patients not to take these drugs….Our research efforts will now focus on whether anticholinergic-induced cognitive impairment may be reversible.”

A list of medications with anticholinergic effects can be found here.

Copyright © 2010, The Los Angeles Times

Evaluating swallowing in PSP patients (German research)

This is an interesting German study of dysphagia (swallowing problems) in PSP.

“Dysphagia is…a common feature in PSP occurring in up to 80% of patients. In comparison to Parkinson’s disease (PD), median dysphagia latency and survival time after onset of dysphagia are significantly shorter in PSP. Dysphagia may result in aspiration and subsequent pneumonia, which is the leading cause of death in both disorders. Other complications of swallowing dysfunction are discomfort while eating, difficulties in taking oral medications, weight loss, and dehydratation. Furthermore, swallowing disturbance is associated with increased anxiety and depression and significantly affects the quality of life in parkinsonian syndromes. Therefore, early detection and timely interventions are mandatory to prevent the serious consequences of dysphagia.”

In this study, the FEES test was used: fiberoptic endoscopic evaluation of swallowing. (The R in FEES(R) is “registered trademark.”) The FEES test includes a flexible fiberoptic endoscope in put down the nose, providing a view of the swallowing structures.

Eighteen PSP patients and 15 advanced stage PD (Parkinson’s Disease) patients were given this test. Seven of the PSP patients were tested after they had been given levodopa (Sinemet is a brand name). “Two PSP patients showed relevant improvement of swallowing function after L-dopa challenge.” One patient was able to return to normal food and the other patient had a nasogastric tube removed.

“Aspiration events with at least one food consistency occurred in nearly 30% of PSP patients.”

“In early PSP patients, swallowing dysfunction was solely characterized by liquid leakage with the risk of predeglutitive aspiration during the oral phase of swallowing. … More than half of these patients did not recognize their disability. These observations may suggest that liquid leakage resulting from a disturbed oral swallowing phase is another early and under diagnosed ancillary clinical attribute in PSP differentiating this condition from PD.”

“Endoscopic dysphagia severity in PSP correlated positively with disease duration, clinical disability, and cognitive impairment. … There were no significant differences between the PSP and PD patients.”

This finding was interesting: “No correlation was found with dysarthria severity.” I would’ve thought that dysarthria severity and dysphagia severity were highly correlated.

And this finding was also interesting: “Significant pharyngeal saliva pooling was observed in 4 PSP patients.” It has been shown that “pharyngeal saliva pooling strongly predicts severe dysphagia and substantially increases the risk for the development of aspiration pneumonia.”

“Chin tuck-maneuver, hard swallow, and modification of food consistency were identified as the most effective therapeutic interventions.” Two examples of food consistency modification are restriction to pureed food or restriction to soft solid food. “[In] certain PSP patients with delayed swallowing reflex [a] hard swallow may not be an appropriate maneuver, because it potentially further increases the duration of pharyngeal response.”

The researchers had something to say about PEG feeding tubes. “Although several severely affected PSP patients participated in our study, only two subjects required percutaneous endoscopic gastrostomy. In our opinion, a percutaneous endoscopic gastrostomy should be considered in PSP patients when there is endoscopic or videofluoroscopic evidence that all food consistencies are aspirated without any objective improvement by L-dopa challenge and/or nonpharmacological therapeutic interventions.”

Robin

Movement Disorders. 2010 Mar 29. [Epub ahead of print]

Endoscopic characteristics and levodopa responsiveness of swallowing function in progressive supranuclear palsy.

Warnecke T, Oelenberg S, Teismann I, Hamacher C, Lohmann H, Ringelstein EB, Dziewas R.
Department of Neurology, University Hospital of Münster, Münster, Germany.

Abstract
Dysphagia is a frequent and early symptom in progressive supranuclear palsy (PSP) predisposing patients to aspiration pneumonia. Fiberoptic endoscopic evaluation of swallowing (FEES(R)) has emerged as a valuable apparative tool for objective evaluation of neurogenic dysphagia. This is the first study using FEES(R) to investigate the nature of swallowing impairment in PSP.

Eighteen consecutive PSP patients (mean age 69.7 +/- 9.0 years) were included. The salient findings of FEES(R) in PSP patients were compared with those of 15 patients with Parkinson’s disease (PD). In 7 PSP patients, a standardized FEES(R) protocol was performed to explore levodopa (L-dopa) responsiveness of dysphagia.

Most frequent abnormalities detected by FEES(R) were bolus leakage, delayed swallowing reflex, and residues in valleculae and piriformes. Aspiration events with at least one food consistency occurred in nearly 30% of PSP patients. Significant pharyngeal saliva pooling was observed in 4 PSP patients.

We found no difference of salient endoscopic findings between PSP and PD patients.

Endoscopic dysphagia severity in PSP correlated positively with disease duration, clinical disability, and cognitive impairment. No correlation was found with dysarthria severity. In early PSP patients, swallowing dysfunction was solely characterized by liquid leakage with the risk of predeglutitive aspiration during the oral phase of swallowing.

Two PSP patients showed relevant improvement of swallowing function after L-dopa challenge.

Chin tuck-maneuver, hard swallow, and modification of food consistency were identified as the most effective therapeutic interventions.

In conclusion, FEES(R) assessment can deliver important findings for the diagnosis and refined therapy of dysphagia in PSP patients.

(c) 2010 Movement Disorder Society.

PubMed ID#: 20629130 (see pubmed.gov for this abstract only)

Visual symptoms in PSP and practical ways for helping a patient

Dr. Richard Armstrong has written extensively on visual impairment in PSP and other parkinsonism syndromes.  His latest article for optometrists is a good general review of PSP, followed by a review of the visual signs that can be used to identify PSP, and a comparison between these PSP visual symptoms and those of other movement disorders, especially Parkinson’s Disease (PD).

Dr. Armstrong states:

Although primarily a neurological disorder, patients with PSP may develop a variety of visual signs and symptoms that could be useful in differential diagnosis. In addition, a PSP patient may develop visual problems during the course of the disease and the eye-care practitioner can contribute to the management of these problems.

He examines these many “ocular aspects,” including eyelid mobility (such as apraxia of lid opening, blepharospasm), fixation, ophthalmoplegia (such as vertical supranuclear gaze palsy), saccades, smooth pursuit movements, nystagmus (including square wave jerks), and reading.

Here is Dr. Armstrong’s list of practical ways an optometrist can help a PSP patient:

  • “PSP patients tend to fall more readily, resulting in a sedentary life style, while vertical gaze palsy also leads to difficulties with reading and eating. New devices are being tested in which refraction positive moveable prism glasses are placed in front of the eyes to help patients with these problems.”
  • “PSP patients often complain of dry eyes due to decreased blinking and artificial tears can be used to help this problem.”
  • “Patients with difficulties in opening their eyelids may have ‘lid crutches’ fitted to spectacle frames that can hold the lids open.”
  • “[Blepharospasm] has been treated successfully with botulinum toxin A…”
  • “PSP patients have a particular difficulty with vertical saccades and in suppressing fixation, both of which may contribute to vertical gaze palsy. Deficits in gaze control may … increase the risk of trips or falls while climbing stairs. Balance training in combination with eye movement and visual awareness exercises have been shown to improve gaze control in PSP patients. Patients can be referred to physiotherapists or occupational therapists.”
  • “Some patients with PSP may have been treated with L-dopa… Some patients treated with L-dopa exhibit a worsening of apraxia of eye opening as a result, a condition that is often alleviated when the drug is discontinued. By contrast, treatment with apomorphine may reduce apraxia of eye opening.”
  • “[Visual] problems that cannot be corrected, such as oculomotor apraxia or visual field defects, should be brought to the attention of carers. As a consequence of such information, carers can make accommodations for these problems, for example, by careful positioning of objects.”

I’ve copied the abstract below along with a link to the full article, which is available at no charge online.

Robin


Full article

Abstract

Clinical and Experimental Optometry. 2010 Jul 12.
Visual signs and symptoms of progressive supranuclear palsy.
Armstrong RA.
Vision Sciences, Aston University, Birmingham, UK

Progressive supranuclear palsy is a rare, degenerative brain disorder and the second most common syndrome in which the patient exhibits ‘parkinsonism’, that is, a variety of symptoms involving problems with movement. General symptoms include difficulties with gait and balance; the patient walking clumsily and often falling backwards. The syndrome can be difficult to diagnose and visual signs and symptoms can help to separate it from closely related movement disorders such as Parkinson’s disease, multiple system atrophy, dementia with Lewy bodies and corticobasal degeneration. A combination of the presence of vertical supranuclear gaze palsy, fixation instability, lid retraction, blepharospasm and apraxia of eyelid opening and closing may be useful visual signs in the identification of progressive supranuclear palsy. As primary eye-care practitioners, optometrists should be able to identify the visual problems of patients with this disorder and be expected to work with patients and their carers to manage their visual welfare.

PubMed ID#: 20629667

“Encouraging Comfort Care” publication and checklist for families

I read about a new publication titled “Encouraging Comfort Care.” Comfort care is the same thing as palliative care, where the focus is on the patient’s physical and mental comfort, rather than treatments to restore a person to good health. The publication is from the Alzheimer’s Association’s Greater Illinois Chapter so focuses on comfort care for dementia patients living in care facilities. But I think everyone — whether dealing with dementia or not, and whether caring for someone in a facility or not — will find value in this 21-page care planning booklet. 

Here’s a link to “Encouraging Comfort Care”: 

http://www.alzheimers-illinois.org/pti/downloads/Encouraging%20Comfort%20Care_SINGLE.pdf

According to the table of contents, these topics are covered: 
What is comfort care? 
Facts about dementia 
How the brain and body change over time 
Dementia and residential care facilities 
Comfort care in action 
Medical decisions you may face 
What does research tell us? 
Who decides? 
How to create meaningful and enjoyable visits 
Eating can be comforting too 
When is it time for hospice care? 
Active dying 
Checklist for encouraging comfort care 
Resources and references 

I’ve copied below excerpts from a “checklist for encouraging comfort care.”

Here are a few of the resources listed: 

Caring Connections is a program of the National Hospice and Palliative Care Organization aimed at mproving care at the end of life. Contact at (800) 658-8898 or www.Caringinfo.org 

National Hospice and Palliative Care Organization is the largest nonprofit membership organization representing hospice and palliative care programs and professionals in the United States. It offers a wealth of information about end of-life-care and referrals to local hospices. Contact at (800) 658-8898 or www.nhpco.org 

National Health Care Decisions Day is an initiative to encourage people to express their wishes regarding healthcare and for providers and facilities to respect those wishes, whatever they may be. For information about advance directives, contact www.nationalhealthcaredecisionsday.org 

National Long-Term Care Ombudsman Resource Center can direct you to professional and volunteer advocates for residents of nursing homes, board and care homes and assisted living facilities. Ombudsmen provide information about what to do to get quality care and are trained to resolve problems and assist with complaints. Contact (800) 677-1116 or www.ltcombudsman.org 

Center to Advance Palliative Care provides clear, comprehensive palliative care information for people coping with serious, complex illnesses. Contact www.getpalliativecare.org

Robin

The “checklist for encouraging comfort care” is intended as prompt for discussion “with the facility’s staff, other health care providers, or relatives and friends.” Here are some items on the checklist:
_____ Staff know your loved one’s preferences for food, drink, clothing, bathing, etc. 
_____ Staff routinely anticipate needs such as hunger, boredom, toileting and fatigue. 
_____ Pain is evaluated daily and relief is provided with medications and non-drug measures. 
_____ Psychotropic drugs are administered only with your permission. 
_____ Staff consistently interact with your loved one in a calm, kind manner. 
_____ Staff use language that promotes your loved one’s dignity and individuality. 
_____ Staff tap your loved one’s remaining abilities and strengths whenever possible. 
_____ Based on your loved one’s wishes and goals of care, you, the physician, and staff have discussed if and when hospitalization should be considered. 
_____ Based on your loved one’s wishes and goals of care, a decision has been made if cardiopulmonary resuscitation (CPR) should/should not be initiated. 
_____ Based on your loved one’s wishes and goals of care, you have discussed with the physician and staff if oral or intravenous antibiotics should/should not be initiated. 
_____ Staff know your preferences/decisions about hospice care. 
_____ Staff routinely engage your loved one in one-to-one activities involving the five senses. 
_____ Your loved one’s spiritual needs and practices are addressed. 
_____ Funeral arrangements are completed and communicated to staff. 
_____ Ways of caring for yourself are practiced on a daily basis. 

Neuropathology of PSP variants (Mayo research)

This is an important review article from our friends at the Mayo Clinic.  Researchers examine the differences in distribution of tau pathology and differences in biochemical composition of tau pathology in at least two of the variants, as compared to “classic” or typical progressive supranuclear palsy (PSP).

They find that:

  • “In variants of PSP presenting with focal cortical syndromes, such as frontotemporal dementia, corticobasal syndrome and apraxia of speech, there is greater cortical pathology than in typical PSP.”
  • “In variants of PSP presenting with levodopa-responsive Parkinsonism, as well as pure akinesia and gait failure, there is less cortical pathology and more severe degeneration in the cardinal nuclei – globus pallidus, subthalamic nucleus and substantia nigra – than in typical PSP.”

The full abstract is copied below.

Robin

——————————

Current Opinion in Neurology. 2010 Aug;23(4):394-400.

Neuropathology of variants of progressive supranuclear palsy.

Dickson DW, Ahmed Z, Algom AA, Tsuboi Y, Josephs KA.

Abstract
PURPOSE OF REVIEW:
Neurodegenerative tauopathies, of which progressive supranuclear palsy (PSP) is one of the most common, are clinically heterogeneous, reflecting differences in distribution and biochemical composition of tau pathology. This review highlights the range of clinical and pathologic presentations of PSP and its variants.

RECENT FINDINGS:
Progressive supranuclear palsy is a 4R tauopathy with neuronal and glial tau-immunoreactive lesions in neuroanatomically specific nuclei in the basal ganglia, diencephalon, brainstem and cerebellum, with restricted involvement of the neocortex. Hierarchical cluster analyses of clinical and pathologic features of PSP indicate that there are distinct clinicopathologic variants of PSP. In variants of PSP presenting with focal cortical syndromes, such as frontotemporal dementia, corticobasal syndrome and apraxia of speech, there is greater cortical pathology than in typical PSP. In variants of PSP presenting with levodopa-responsive Parkinsonism, as well as pure akinesia and gait failure, there is less cortical pathology and more severe degeneration in the cardinal nuclei – globus pallidus, subthalamic nucleus and substantia nigra – than in typical PSP.

SUMMARY:
Clinical variants in PSP reflect varying anatomical distribution of tau pathology, but they share histopathologic, biochemical and genetic features with typical PSP. The basis for anatomical selective vulnerability in PSP and its variants remains to be determined.

PubMed ID#: 20610990 (see pubmed.gov for this abstract – available free of charge)