“FTD talk” fact sheets on PSP and CBD plus BSN’s top resources lists

FTD talk (ftdtalk.org) is a website run by a group of scientists investigating frontotemporal dementia (FTD) at the University College
London Institute of Neurology in the UK.  The site’s focus is on the behavioral variant of FTD.

However, they have one-page fact sheets on PSP and CBD:

“What is corticobasal syndrome?”
www.ftdtalk.org/ftd-factsheets/factsheet-9-what-is-corticobasal-syndrome/

“What is progressive supranuclear palsy?”
www.ftdtalk.org/ftd-factsheets/factsheet-10-what-is-progressive-supranuclear-palsy/

These aren’t my favorite resources on PSP and CBD but there may be some value in a one-pager.

The resources we do like are here:

PSP Top Resources
www.brainsupportnetwork.org/education/progressive-supranuclear-palsy/#resources

CBD Top Resources
www.brainsupportnetwork.org/education/corticobasal-degeneration/#resources

Palliative care discussions in MSA – when to have, what to discuss, etc.

This is an interesting study published in May 2017 about palliative care discussions (or the lack there of) among 22 Canadian patients with multiple system atrophy (MSA) seen at a movement disorder center.  “Palliative care discussions” include diagnosis and symptom management, prognosis, goals of care, and comfort measures.  Interestingly only 16 of the 22 patients discussed these issues with their MDs, according to medical records.  (So maybe not all “discussions” are recorded in patient charts.)

You can find the full article here:  (available at no charge)

https://www.cambridge.org/core/journals/canadian-journal-of-neurological-sciences/article/palliative-care-discussions-in-multiple-system-atrophy-a-retrospective-review/4F1398B9A85ECA7588455DCAD237C6B3/core-reader

(Thanks to long-time friend Vera James for sharing this link with me.)

The authors argue that “Early and effective advance care and end-of-life planning discussions are…essential for effective palliative care in MSA. … [These] discussions … will allow people with MSA to make treatment decisions that better align with their goals of care prior to an emergent situation.”

And the authors argue that advance care planning must taken into account respiratory dysfunction.  “Respiratory dysfunction, including but not limited to stridor, is the leading cause of death for people living with MSA, and it may present acutely with or without prior evidence of stridor.”

Perhaps these discussions are not occurring because stridor and sleep apnea are NOT the most common symptoms seen in MSA?  Both symptoms occur among a minority of patients.  Among the 22 Canadian patients, the most common symptoms were parkinsonism, orthostatic hypotension, GI/GU dysfunction, gait impairment, and ataxia.

But even patients with “modified diets did not have a documented discussion about possible future requirements for artificial feeding.”

However, if you have stridor, your medical team is more likely to discuss advance care planning with you (and document it in the medical record).  “Five of the six patients with stridor had a documented discussion about tracheostomy before one was required for life-sustaining purposes. Of the three patients who required emergent tracheostomy, one had a documented discussion declining tracheostomy in the management of stridor. Nevertheless, this patient was later intubated in intensive care secondary to respiratory failure.”

Of the 22 patients, 6 were deceased.  Though one of those six had a DNR (do-not-resuscitate) order in place, resuscitation was attempted by the care facility where the patient was living.
And only 2 of those six had a healthcare agent listed in the medical record.  Four of the six died in ICU.  Of those four, “discussions surrounding resuscitation and goals of care took place” days or hours before death.

The authors say:  “Our chart review shows clear gaps in the current approach to advance care planning, goals of care and palliative care discussions in patients living with MSA, at least at our centre. Important advance care planning and end-of-life discussions took place very late in the disease trajectory and often at a time when the affected person was no longer able to participate. While our study is limited to a retrospective review of charts at a single centre, it nonetheless shows significant gaps in the provision of care.”

To remedy this situation, the authors propose a framework for palliative care discussions in MSA:

“In the early stages of MSA, it is important to determine who the person with MSA would like to have present for important healthcare discussions in general, and then establish each patient’s readiness to discuss end-of-life decisions. Since diagnosis of MSA is difficult, there may be a period of ambiguity regarding the diagnosis, and the patient might not yet be receptive to hearing about the disease trajectory and his/her prognosis. Nevertheless, even without a determinate diagnosis, identifying personal supports and discussing personal values will help to inform and simplify future discussions regarding end-of-life care, when appropriate. Information regarding diagnosis, expected disease trajectory, symptom management and prognosis should be disclosed to the person with MSA and his/her family or an appointed decision maker, where possible. Physicians should initiate and document the results of important discussions, after assessing the patient’s readiness for such discussions. These include the identification of a substitute decision maker or power of attorney for healthcare, the presence of advance care plans, preferences regarding artificial nutrition and hydration, including their expected benefits or the lack thereof, end-of-life care wishes and preferred place of death. While tracheostomy may extend the length of life, it does not, in our view, extend or improve the quality of life. Therefore, it is important to discuss tracheostomy and to document the patient’s wishes surrounding tracheostomy before an unexpected emergent event.”

The authors expect the neurologist to be active participants in the palliative care process.  Neurologists can also make referrals to more specialized palliative care services.

“For some people, a referral [to palliative care] at the time of diagnosis might be appropriate, whereas for other people it may be more appropriate to initiate a referral once further severe symptoms develop, including dysphagia, stridor, sleep-disordered breathing, severe orthostatic blood pressure changes, increasing falls or other symptoms of distress.”

“Cognition in PD and LBD” – Presentation by Stanford MD (Nov 2018)

I stumbled across the slides from a presentation given by Kathleen Poston, MD, movement disorder specialist, Stanford, at a conference for laypeople in November 2018. The presentation is about “cognition in Parkinson’s Disease and Lewy body dementia.”

The slides I like the most are the two slides on the lefthand side of page three of the PDF. I’m constantly talking to caregivers about the balancing act between motor symptoms, cognitive symptoms, Parkinson’s medications, dementia medications, and psychosis medications.

http://med.stanford.edu/content/dam/sm/adrc/documents/2018-ADRC-kathleen-poston.pdf

Robin

“Little Wished-for Deaths” (beautiful caregiving story)

This is a beautiful story about a woman who cared for her 90-plus year old grandfather with Parkinson’s Disease and dementia.

Excerpt:

“Because grief, like death, doesn’t adhere to our constructs. The wished-for deaths of ailing loved ones doesn’t make them any less loved. It only means we hoped for an end to suffering, on both accounts. And deaths that are supposed to be small can sometimes feel big. … The funeral commemorated a life that spanned nearly a century, putting those four years into a birds-eye perspective. They were sometimes burdensome, yes, and sometimes beautiful, but only a small portion of a rich and varied life: of his and mine both.”

The full article is here:

www.nytimes.com/2019/10/11/well/family/little-wished-for-deaths.html

Little Wished-for Deaths
by Mary Pembleton
October 11, 2019
New York Times

Robin

“The Soul of Care: The Moral Education of a Husband and a Doctor” (book review)

This is a review of a new book “The Soul of Care: The Moral Education of a Husband and a Doctor” by Arthur Kleinman, MD, a psychiatrist and anthropologist at Harvard.  It is about the “stressful, harrowing, depressing, and rewarding” aspects of caregiving for a spouse with early-onset Alzheimer’s Disease.

Excerpt from this article:

In his new book, [the author] chronicles the harrowing decade he spent caring for his wife, Joan, after she was diagnosed with early-onset Alzheimer’s disease in her 50s.  He describes the process of navigating a health-care system that seemed to offer plenty of clinical care but little compassionate care as she declined from a lively, witty Sinologist to a woman unable to see, dress herself or recognize loved ones. In moving detail, Kleinman tells the story of a husband trying to honor, in the best way he could, his wife’s ultimate wish: “I will not die without dignity.”

You can read the full article here:

www.washingtonpost.com/health/caregiving-for-a-sick-loved-one-can-be-stressful-harrowing-depressing–and-rewarding/2019/09/27/dbb41cf4-c918-11e9-a4f3-c081a126de70_story.html

Health
Perspective
Caregiving for a sick loved one can be stressful, harrowing, depressing — and rewarding
By Dhruv Khullar
September 30, 2019 at 6:00 a.m. PDT
Washington Post

Robin