Mayo Rochester finally reports results from mesenchymal stem cell study in MSA

Finally Mayo Rochester has reported results from its mesenchymal stem cell study of multiple system atrophy.  Local support group member John Yanez-Pastor participated in the study.  This was a phase I/II safety and tolerability study, NOT an efficacy study.  Wolfgang Singer, MD, reported at this week’s annual meeting of the American Academy of Neurology that the 24 probable MSA patients who participated in the very small study reported no serious adverse events with the treatment.

I guess with the phase II aspect of the study, a bit about efficacy could be studied.  Dr. Singer said that the “efficacy of MSCs on slowing multiple system atrophy progression….appeared to be dependent on the dose, and, in the highest dose individuals, had a painful implantation response.”

Slowed disease progression was measured by the Unified MSA Rating Scale.  There was only one point difference between the study group and a “historical placebo group.”  I’m not sure a one point difference really leads to better quality of life.  (Sorry.)  There was no change on on any autonomic scales.

Mayo Rochester is in the late planning stages for a multicenter, double-blind, placebo-controlled phase II/III study.

There’s a four-minute video interview with Dr. Singer here:

link.videoplatform.limelight.com/media/?mediaId=689b6aec47124b42b39095f7863f1cf8&width=630&height=421&playerForm=19ca168687014a7b8cff84fa4c87d03f

And here’s a link to the full article from Clinical Neurology News:

www.mdedge.com/clinicalneurologynews/article/136483/movement-disorders/video-pilot-stem-cell-trial-multiple-system

VIDEO: Pilot stem cell trial for multiple system atrophy shows promising results
Publish date: April 25, 2017
By: Jeff Evans, Clinical Neurology News
At AAN 2017

Robin

Teacher who lost the ability to walk, talk and care for himself gives one last incredible lecture

This sweet article was published last week on the website of ITV, a news station in the UK.  It’s about Paul Norman, a former teacher with multiple system atrophy (MSA), giving “one last incredible lecture” and his son making a film about his dad.  The lecture is at the end of this article. If you go to the webpage, you can view “One Last Lecture” plus some photos of Paul Norman and his family.

Robin

www.itv.com/news/london/2017-03-15/teacher-who-lost-the-ability-to-walk-talk-and-care-for-himself-gives-one-last-incredible-lecture/

Teacher who lost the ability to walk, talk and care for himself gives one last incredible lecture
ITV REPORT
15 March 2017 at 3:31pm

Multiple system atrophy results in parts of the brain and spinal cord gradually becoming more damaged over time. For more information click here.  (http://www.nhs.uk/conditions/multiple-system-atrophy/Pages/Introduction.aspx)

A teacher who lost the ability to walk, talk and care for himself gave one last incredible lecture. Paul Norman, 58, was diagnosed with Multiple System Atrophy in 2012 forcing him to retire from his job as an English teacher.

His son Will, 22, began filming his every day life, eventually taking him back to Billericay School, Essex, where he taught English for 15 years.

Paul delivered an inspiring final assembly to a packed hall of his former students, written on his iPad and played out on a speaker.

He told his audience of his devastating struggle with his condition, even making them laugh with jokes about his “Stephen Hawking” voice.

He passed away ten months later, in April last year, before film and English student Will had finished the documentary ‘One Last Lecture’, featuring the scene.

Much to Will’s regret, his father never got to see the completed film, which

has been watched thousands of times since it was published online last month.

During the emotional lecture, he tells the students:

“I’m doing this partly to educate you about disability. People have a habit of assuming that because the body doesn’t function properly the brain doesn’t either. But in reality the vast majority of disabled people function just the same mentally with all of the same feelings and emotional needs as everyone else.”

“It is frustrating of course being trapped inside a body that doesn’t work and frustrating for the people that care for me. The other day I was moving my hand to scratch my nose and my carer thought I was signalling for my glasses.”

“And recently my mum asked me what I would like to eat from the fridge. I asked for tiramisu and she thought I said tin of soup. It’s very confusing.”

– PAUL NORMAN

And leaving them with some important life lessons, he adds:

“Teaching is really all about learning, and I have not stopped learning due to my disability. In fact some of the things I have learnt I will share with you, because being disabled as I am enables me to gain insight into what really matters in life.”

“When I see the atrocities that people commit I wonder how far human beings can have yet to evolve. War is pointless. There are many good things about this world and most people are good.”

“They should look at the positive side of people rather than the faults. Health is more important than money and the world is still a beautiful place.”

“Love is important and making this film has certainly brought me closer to my son William. Life is a challenge and one should strive to be a better person.”

“Although I am not religious I do believe that you should always treat others as you wish to be treated yourself.”

– PAUL NORMAN

Will started making the film in July 2014 after he visited his father from Billericay, Essex, and found he was wheelchair bound.

He choked on a piece of rice, forcing Will to perform the Heimlich manoeuvre to save his life, and he went back to his university that night and “broke down”.

University of Sussex student Will explained:

“That’s when I decided to start filming, just to capture memories and record how he is now, to hold on to the past.”

– WILL NORMAN

The pair grew close and for the first time Will saw the reality of his dad’s every day struggles. They gathered an assembly of former students and staff, and wrote the speech together.

Final year student Will added:

“It gave him and myself something that we could work on together.”

“He always liked to help me with my homework, and now we had a project to work on together.”

– WILL NORMAN

He completed filming in the summer of 2015, before Paul passed away in April last year, and he completed the movie in mid-January this year.

“One of the most depressing elements of the whole process really is dad never got to see the full finished film. That’s my fault really.”

“He was always asking me when it was going to be ready, but I was always striving for perfection, trying not to let him down with it. There is a feeling of emptiness that lots of people have seen it but he never got to see it.”

“He never got to be the movie star that he wanted to be.”

– WILL NORMAN

Here is the full text of Paul Norman’s lecture.

“Hello everyone. It’s good to see you all.

When William first suggested this to me I was a bit apprehensive because of the changes in me and looking at me is scary.

If I see people slipping away I will understand, however if I see anybody on their phones they will be confiscated.

The last time most of you saw me I was a different person. I now have Multiple System Atrophy, or MSA for short.

It affects every part of me and I mean every part, except my brain.

The worst things are not being able to talk or walk anymore and having to rely completely on other people.

So seeing all of you is very emotional. I cried enough last Christmas when I saw the wonderful video which some of you were in.

I’m doing this partly to educate you about disability.

People have a habit of assuming that because the body doesn’t function properly the brain doesn’t either.

But in reality the vast majority of disabled people function just the same mentally with all of the same feelings and emotional needs as everyone else.

It is frustrating of course being trapped inside a body that doesn’t work and frustrating for the people that care for me.

The other day I was moving my hand to scratch my nose and my carer thought I was signalling for my glasses.

And recently my mum asked me what I would like to eat from the fridge. I asked for tiramisu and she thought I said tin of soup. It’s very confusing.

I have friends and family who visit me regularly and have stood by me.

I also have a wonderful team of carers who go beyond the call of duty to help me and I can’t praise them enough.

I certainly couldn’t do that job if I was able to. They get paid little by society and they do 12 hour shifts.

Imagine 12 hours with me. It’s enough to drive anyone balmy.

I’m not saying they are balmy by the way.

Honestly, I feel like Stephen Hawking – except I have a better voice.

As you can see my sense of humour has not gone, in fact it helps me see the funny side even the moments when it is most difficult.

In fact inappropriate laughter is one feature of MSA, so I’ll laugh when it is most serious. That’s my excuse anyway.

For example I was in a church not long ago and I suddenly started laughing for no reason.

This would have been even more embarrassing had the congregation not known.

It was my uncle Bob’s funeral.

Teaching is really all about learning, and I have not stopped learning due to my disability.

In fact some of the things I have learnt I will share with you, because being disabled as I am enables me to gain insight into what really matters in life.

When I see the atrocities that people commit I wonder how far human beings can have yet to evolve.

War is pointless.

There are many good things about this world and most people are good.

They should look at the positive side of people rather than the faults.

Health is more important than money and the world is still a beautiful place.

Love is important and making this film has certainly brought me closer to my son William.

Life is a challenge and one should strive to be a better person.

Although I am not religious I do believe that you should always treat others as you wish to be treated yourself.

Now enough of this before I turn into John Lennon.

 

Although I can’t talk any more, I can answer you as long as the answer is yes or no.

If for example you ask if it’s nice to see you I can go like that [thumbs up].

Conversely, if you ask me if sitting in a chair all day is much fun, I will go like this [thumbs down].

So if I chat to you later I’m afraid we”ll have to play by those rules, and whatever you do, please don’t ask me two questions at once.

Finally, thank you all for coming. It means a lot to me. I’m sorry it’s a bit short, but at least it’s sweet.”

– PAUL NORMAN

“We Will Go On” Blog by Dan Brooks

My longtime friend Dan Brooks in Riverside reactivated his blog “We Will Go On” in 2016 and moved it to wewillgoon.com.  Dan’s blog has the tagline:

Parkinsonism: Hard to Diagnose.  Harder to live with. 
A blog by a patient with Parkinsonism Plus Syndrome.

In 2006, Dan was diagnosed with multiple system atrophy (MSA), corticobasal syndrome (CBS), and progressive supranuclear palsy (PSP).  As time went on, MSA became the most likely.  It seems that CBS has come back into the picture, however.

Dan recently posted about the confusion over whether he has Parkinson’s Disease, CBS, and MSA.  I’ve copied his post below.

Robin

————————-

www.wewillgoon.com/2017/03/confusion-over-parkinsons-contrasted.html

Confusion Over Parkinson’s Contrasted with CBS and MSA
by Dan Brooks
Saturday, March 4, 2017

I have quite often discussed with some of you in the family, and various friends, about the difference between Parkinson’s Disease and the Atypical Parkinsonian disorders.  I am going to give you a few points to chew on, knowing you are the best ambassadors we have for spreading the word about these rarer forms of Parkinsonism.

Parkinsonism is a condition in which signs and symptoms of Parkinson’s appear in the patient’s disease.  Even though the person may not have Parkinson’s Disease, they have a brain disorder that causes similarly appearing symptoms, including tremors, balance problems, stiffness, walking difficulty and cognitive changes.

Parkinsonism appears with Parkinson’s Plus syndromes such as Multiple System Atrophy, Corticobasal Syndrome and Progressive Supranuclear Palsy.  Since I was first diagnosed in 2006, the neurologist I saw for ten years thought that my condition was one of these three.  As time went on, Multiple System Atrophy became the most likely.  All three of these conditions are determined to be “probable” in life, and are confirmed after death through a brain tissue study.

Even though I have  Parkinsonism it is not Parkinson’s Disease in the simplest form because the disease process in my brain is more involved than in Parkinson’s.  I have Corticobasal Syndrome (CBS), and it is uncertain if it would be alternatively considered Multiple System Atrophy (MSA).  These have overlapping symptoms and are best described as rarer forms of Parkinson’s.

Has much changed?  Not really, except I have more clarity and certainty of the degenerative brain disease that has taken so much from our lives in the Brooks family.  We are fortunate to have this increased clarity because the neurologist I have been seeing of late is a Movement Disorder specialist, which is a doctor of Neurology who specializes in all things Parkinson’s and Parkinson’s-like.   She saw a clear indication in the results of my DaT Scan which demonstrated that I have Parkinson’s Plus, not simply Parkinson’s Disease.

Parkinson’s Plus has long been the understanding of my condition.  I wrote about this in my book, I WILL GO ON: LIVING WITH A MOVEMENT DISORDER.  The confusion arises because the word “Parkinson’s” appears in both descriptions of the diseases.  Technically, they are different in that Parkinson’s Plus is a faster progressing disease and causes more disability sooner.

That is why I was unable to continue driving and had to retire at 51.  I have difficulty walking with coordination and I struggle with choking on food and liquids.  I also have digestive, urinary, heart rate, blood pressure regulation and body temperature issues.  These are not visible to friends and family so what appears to be a better day, could be a day I am having trouble with my blood pressure or having great difficulty coughing after drinking liquids.

I also have very abnormal horizontal eye movements which are caused by a loss of neurons in the area of the brain that controls eye movement.  At times I see double as a result.

I am so glad that you are interested and are trying to grow in your knowledge.  MSA is a disease I have been identified with for 10 years or more.  I have CBS, but if it were to turn out to be MSA at some point, that would be a very similar prognosis. My greatest concerns are pneumonia, breathing constriction, and urinary infections.  I do not have idiopathic Parkinson’s, but I do have a form of Parkinsonism, and much of the research being done for P.D. will have a benefit and weight heavily on the potential for discoveries that relate to PSP, MSA and CBS syndromes.  I will always identify with my fellow patients who have Parkinson’s Disease, and the support groups for Parkinson’s are virtually the only in person, brick and mortar groups we can attend anywhere near the Riverside, CA area. As always, thanks for reading! — Patient-Online

Supranuclear gaze palsy occurs in more than just PSP

“Supranuclear gaze palsy” (SGP) refers to impairment of horizontal gaze and/or vertical gaze.  This symptom denotes “dysfunction in the connections responsible for conducting voluntary gaze commands to the brainstem gaze centers.”

As many of you know, SGP is a classic clinical feature of progressive supranuclear palsy (PSP).  In fact, it is part of the diagnostic criteria for PSP.  However, this symptom is not specific to PSP and can occur in many other neurological disorders, including parkinsonian conditions.

In this Washington University (St. Louis) study, researchers examined the clinical records of 221 parkinsonian patients who had visited the movement disorders clinic and who had donated their brains for research.  [By the way, Brain Support Network has been responsible for over 350 brain donations — quite a bit more than the WashU brain bank.]

Of the 221 parkinsonian brains in their brain bank, 27 had supranuclear gaze palsy noted in the clinical records.  The confirmed diagnoses of these 27 were:
* progressive supranuclear palsy (9),
* Parkinson’s Disease (10),
* multiple system atrophy (2),
* corticobasal degeneration (2),
* Creutzfeld-Jakob Disease (1), and
* Huntington Disease (1).

The researchers also looked at the 14 brains donated of those with PSP in their brain bank.  Nine of the 14 had clinical evidence of SGP but five did not.

Curiously, their brain bank doesn’t have many dementia with Lewy bodies (DLB) cases because their brain bank has a bias towards movement disorders rather than dementia.

This paragraph about MSA is interesting:

“In a study of oculomotor function in MSA, Anderson and colleagues suggest that the presence of clinically slow saccades, or moderate-to-severe gaze restriction, implies a diagnosis other than MSA. In contrast, our data indicate that SGP can be seen in patients who have subsequent autopsy-confirmation of MSA at a frequency similar to that seen in PD. Cognitive impairment is an exclusion criterion for the diagnosis of multiple system atrophy (MSA), according to the second consensus statement. However, some patients with pathologically confirmed MSA have been reported to have dementia. Cykowski and colleagues have reported that the presence of Lewy body-like inclusions in neocortex in MSA, but not hippocampal alpha-synuclein pathology, was associated with cognitive impairment. We suggest that the association of SGP with MSA in some individuals provides further evidence for cortical pathology.”

The authors point out that other studies show that 90% of those with CBD develop SGP.

SGP is also reported in other disorders such as spinocerebellar degeneration, amyotrophic lateral sclerosis, Whipple disease, and Niemann-Pick disease type C.

I’ve copied the abstract below.

Robin

———————–

Parkinsonism Relat Disord. 2017 Feb 24. [Epub ahead of print]

Pathologic correlates of supranuclear gaze palsy with parkinsonism.
Martin WR, Hartlein J, Racette BA, Cairns N, Perlmutter JS.

Abstract
INTRODUCTION:
Supranuclear gaze palsy (SGP) is a classic clinical feature of progressive supranuclear palsy (PSP) but is not specific for this diagnosis and has been reported to occur in several other neurodegenerative parkinsonian conditions. Our objective was to evaluate the association between SGP and autopsy-proven diagnoses in a large population of patients with parkinsonism referred to a tertiary movement disorders clinic.

METHODS:
We reviewed clinical and autopsy data maintained in an electronic medical record from all patients seen in the Movement Disorders Clinic at Washington University, St. Louis between 1996 and 2015. All patients with parkinsonism from this population who had subsequent autopsy confirmation of diagnosis underwent further analysis.

RESULTS:
221 unique parkinsonian patients had autopsy-proven diagnoses, 27 of whom had SGP documented at some point during their illness. Major diagnoses associated with SGP were: PSP (9 patients), Parkinson disease (PD) (10 patients), multiple system atrophy (2 patients), corticobasal degeneration (2 patients), Creutzfeld-Jakob disease (1 patient) and Huntington disease (1 patient). In none of the diagnostic groups was the age of onset or disease duration significantly different between cases with SGP and those without SGP. In the PD patients, the UPDRS motor score differed significantly between groups (p = 0.01) with the PD/SGP patients having greater motor deficit than those without SGP.

CONCLUSION:
Although a common feature of PSP, SGP is not diagnostic for this condition and can be associated with other neurodegenerative causes of parkinsonism including PD.

Copyright © 2017 Elsevier Ltd. All rights reserved.

PMID: 28256434  (see pubmed.gov for this abstract only)

Predictors of survival in PSP and MSA

The authors of this review article examined 37 studies of progressive supranuclear palsy (PSP) or multiple system atrophy (MSA).  There’s very little that’s new here.

They determined that these factors predicted a short survival time in PSP:

* Richardson’s phenotype
* early dysphagia
* early cognitive symptoms

In MSA, these factors predicted a short survival time:

* severe dysautonomia
* early development of combined autonomic and motor features

I’m not sure why it’s helpful but in both PSP and MSA, survival is predicted by:

* early falls
* Parkinson Plus score (on a rating scale)
* disease severity score (on a rating scale)

One new thing was that an earlier paper indicated that female gender in MSA predicted a shorter survival time.  That was not found in this meta-analysis.

The authors indicate there is “conflicting evidence regarding the prognostic effect of age at onset and stridor.”

The full abstract is copied below.

Robin

—————–

J Neurol Neurosurg Psychiatry. 2017 Mar 1.  [Epub ahead of print]

Predictors of survival in progressive supranuclear palsy and multiple system atrophy: a systematic review and meta-analysis.
Glasmacher SA, Leigh PN, Saha RA.

Abstract
OBJECTIVE:
To undertake a systematic review and meta-analysis of studies that investigated prognostic factors and survival in patients with progressive supranuclear palsy (PSP) and multiple system atrophy (MSA).

METHODS:
Publications of at least 10 patients with a likely or confirmed diagnosis of PSP or MSA were eligible for inclusion. Methodological quality was rated using a modified version of the Quality in Prognostic Studies tool. For frequently examined prognostic factors, HRs derived by univariate and multivariate analysis were pooled in separate subgroups; other results were synthesised narratively and HRs could not be reported here.

RESULTS:
Thirty-seven studies presenting findings on 6193 patients (1911 PSP, 4282 MSA) fulfilled the inclusion criteria. We identified the following variables as unfavourable predictors of survival. In PSP, PSP-Richardson’s phenotype (univariate HR 2.53; 95% CI 1.69 to 3.78), early dysphagia and early cognitive symptoms. In MSA, severe dysautonomia and early development of combined autonomic and motor features but not MSA phenotype (multivariate HR 1.22; 95% CI 0.83 to 1.80).In PSP and MSA, survival was predicted by early falls (multivariate HR 2.32; 95% CI 1.94 to 2.77), the Neuroprotection and Natural History in Parkinson Plus Syndromes Parkinson Plus Score and the Clinical Global Impression Disease Severity Score but not sex (multivariate HR 0.93; 95% CI 0.67 to 1.28). There was conflicting evidence regarding the prognostic effect of age at onset and stridor.

CONCLUSION:
Several clinical variables were strongly associated with shorter survival in PSP and MSA. Results on most prognostic factors were consistent across methodologically diverse studies; however, the lack of commonality of prognostic factors investigated is a significant limitation.

© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

PMID: 28250027  (see pubmed.gov for the abstract only)

Low blood pressure standing may hold the key to detecting pre-motor MSA

This is an article posted to the website of NYU’s Dysautonomia Center (dysautonomiacenter.com).  One of their lead researchers, Dr. Horacio Kaufmann, is active in multiple system atrophy (MSA) studies.  In the post, Dr. Kaufmann’s eight-year multi-center study of orthostatic hypotension is described.  The study was recently published in the Annals of Neurology.

Here’s a link to the post:

dysautonomiacenter.com/2017/02/22/low-blood-pressure-standing-may-hold-the-key-to-detecting-pre-motor-msa/

Low blood pressure standing may hold the key to detecting pre-motor MSA
By Dysautonomia Center NYU
February 22, 2017

The researchers discovered the predictors of MSA:

“In a patient with orthostatic hypotension, REM sleep behavior disorder, intact smell and signs that the autonomic nerves outside the brain are mostly spared are key features that suggest an increased risk of developing MSA.”

By “signs that the autonomic nerves outside the brain are mostly spared,” the researchers are referring to “the fact that Parkinson disease destroys the autonomic nerves outside the brain around the heart.”  The researchers noted that “patients that went on to develop MSA had faster heart rates that those that were later diagnosed with Parkinson disease.”

The full article is worth reading.

Robin

 

Five E’s of empowered living with chronic illness

CurePSP hosted a webinar last year that focused on “patient-centered multidisciplinary management” of chronic disorders.  While the webinar was directed to families dealing with progressive supranuclear palsy (PSP), multiple system atrophy (MSA), and corticobasal degeneration (CBD), the concepts apply to all situations, regardless of disorder.  Very little about this webinar was specific to PSP, MSA, or CBD.

The speaker was Becky Dunlop, RN, with Johns Hopkins Parkinson’s Disease and Movement Disorders Center.

You can find an archived recording of the webinar here:

www.youtube.com/watch?v=BWoXJdkkV6I&feature=youtu.be

Your PSP, CBD, MSA Management Plan: Resources and Services
CurePSP Webinar
Speaker:  Becky Dunlop, RN, Johns Hopkins
March 6, 2016

Brain Support Network volunteer Denise Dagan listened to the webinar and said:  “[Becky] really paints a comprehensive picture that there is so much you can do to continue living even after what seems like a catastrophic diagnosis, if you’re willing to learn, and can surround yourself with supportive people.”

As part of her hopeful message, Becky shared five “E’s” of empowered living with chronic illness:

* Education:  learn about a disorder
* Expanding and building your team
* Effective communication:  utilize speech therapy, communication boards, etc.
* Effective coping
* Exploring options:  find a movement disorder specialist, participate in research, etc.

In terms of effective coping, these suggestions were offered:
* psychiatric services, professional counseling, social work services
* stress management
* meditation or exercise
* development of a support network
* support groups:  find or start one
* education programs
* develop and maintain your humor
* pet therapy
* maintain faith and hope
* get your rest
* maintain a healthy perspective
* find beauty in life
* don’t be afraid to ask directions
* recognize and celebrate your role and define the unique you

Denise’s notes about the webinar along with the question-and-answer session are below.  There’s more about PSP in the Q&A than there is in the presentation itself.

Becky referred to the WeMove organization.  This organization hasn’t been in business for several years so I deleted that reference.

Robin

===================================

Denise’s Notes

Your PSP, CBD, MSA Management Plan: Resources and Services
CurePSP Webinar
Speaker:  Becky Dunlop, RN, Johns Hopkins Parkinson’s Disease and Movement Disorders Center
March 6, 2016

Learning Objectives:
* Comprehend the need for patient centered multidisciplinary management of PDP, CBD, and MSA
* Identify available resources for individuals living with PSP, CBD, MSA and their families

Patient Centered Care:
* All these individuals strive to identify and meet the needs of the patient
* The Institute of Medicine (2001).  Crossing the quality chasm: A new health system for the 21st century.
* “Providing care based on patient’s needs and expectations is the key attribute of quality care.”

Bergeson & Dean Commentary on Patient Centered Care in JAMA (2006)
* Ensures access and continuity (access to psychiatry, PT, and other services, and continuity among and between service is ensured)
* Provides opportunities for patient and family participation (key take-home point of this presentation)
* Supports self management
* Coordinates care between settings

Individualized therapy involves not only the pharmacological, but also the appropriate use of allied health professionals, assistive technologies, educational and support resources along the chronic illness continuum.

The Es of Empowered Living with PSP, CBS, MSA.  Identify strategies in each of these areas that will help patients and families.
* Education
* Expanding & building your Team
* Effective Communication
* Effective Coping
* Exploring Options

Education:
Knowledge is Power
Lay Education / CurePSP offers
* Network of education and support groups
* Numerous publications
* Web resources
* Conferences and Symposia

Highlights of Lay Educational Resources
* Webinars on specific topics such as incontinence, aphasia, advance directives
* Resources like:
– CurePSP
– National Institute for Neurological Disorders and Stroke http://www.ninds.nih.gov
– Lee Silverman Voice Treatment website

Expanding and Building Your Team:
* Lay people (family, friends, disease community/support group, church)
* Healthcare professionals including:
– Primary Care Physician
– Neurologist, especially a movement disorder specialist
– Urologist
– Cardiologist (orthostatic hypotension)
– Ophthalmologist (double vision, difficulty focusing)
– Psychiatrist (depression, anxiety)

Visual Disturbances (Importance of Ophthalmologist)
* Dry eyes (natural tears and lubricants)
* Difficulty looking down (prismatic lens)
* Difficulty with eye movements, focusing
* Double vision (covering one side of glasses with tape)
* Sensitivity to light (sunglasses or tinted glasses)
* Blepharospasm (Botox around the eye muscle)

Psychiatric Issues (Importance of Psychiatrist, although PCP could help with some of these as well)
* Depression
* Inappropriate laughter or crying
* Impulse control problems (difficulty realizing limitations caused by disease so trying to do what they’ve always done may risk falling)

Role of Allied Team Members:
* Physical Therapy
* Speech Therapy
* Occupational Therapy (managing home safety, managing patient’s ability to participate in every day activities safely)
* Nursing
* Social Workers

Physical Therapy
* Maintain mobility
* Prevent falls
* Suggest walker and wheelchair as appropriate
* Instruct in safe transfer and walking
* Instruct caregiver to maintain caregiver health

Speech Therapy:
* Communication boards (www.givinggreetings.com/olderadults.html)
* Encourage patient to speak slowly
* Allow adequate time for response
* Prevent social isolation

Speech Therapy / Swallowing Issues:
* Place the chin in a downward or neutral position to close off the airway during swallowing
* Learn the Heimlich maneuver for use in the event of choking. (All family members.)
* Have suction equipment available for clearing the airway at some point in the disease.
* Thickening agents for liquids
* Consideration of a feeding tube (Verdun, 2000)

Example of a communication chart.  The person having communication difficulties just has to point.  [Editor’s note:  see webinar]

Occupational Therapy:
* Home modifications
* Home safety

Dietician:
* Unanticipated weight loss
* Maintaining a healthy weight
* Management of constipation

Developing your Personal Support Network:
* Educate family and friends
* Identify resources thru church or social networks
* Devise a plan by identifying needs
* Ask for help
* Consider creating an online care network.  www.caringbridge.org is a central place to keep family & friends up to date and ask for help.

Effective Communication

Effective Coping

Some Resources to aide Coping:
* Psychiatric Services (caregivers sometimes need an objective, trained person for our own needs)
* Professional Counseling ( “ )
* Social Work Services ( “ )
* Stress management (to improve quality of life)
* Meditation or exercise (stress busters)
* Development of a support network (list everyone who is there to help you, and call upon them, even if its just one meal weekly)
* Support groups & education programs (learn from each other)

Develop & Maintain your Humor
She used to hand out Sunsweet Prunes because constipation is a common issue.  Her motto was, “We keep you moving.”

Pet Therapy

Maintain Faith & Hope
Identify your faith community
Have hope knowing there are tools out there to help you.  Don’t lose sight of that.

Get your Rest
It supports your stamina.

Find or Start a Support Group

Maintain a Healthy Perspective
When you’re living with a chronic disease, take time to look up and experience the larger world to gain perspective.

Find Beauty in Life
View the sea or mountains, buy yourself some flowers, etc.

Don’t be afraid to Ask Directions
If you don’t stop asking, people will be available to help and guide you.

Recognize and Celebrate your Role and Define the Unique YOU

Caregiver Health is VITAL to the health of the patient !
The first rule of caregiving is to take care of yourself.  So explore all these options…

Exploring Options:
* Movement Disorder Specialists over a neurologist
* Participation in research
* Other specialists (urinary frequency, call a Urologist / unmanaged constipation, call a GI / depression not well treated, modify meds.)
* Occupational Therapy Assessments/Driving Assessments/ Home Safety & Fall Prevention
* Caregiver Resources (National Caregiver Resources, CurePSP)
* Assistive Devices
* Consider Palliative Care and Hospice

Walking aides
U-Step walker has red laser line between back wheels to prevent freezing.
laser cane
www.parkinsonshop.com/

Home environment.  Get an OT consult.
* Considerations for safety:
– Grab bars in bathroom (by toilet, tub, shower, sink)
– Railings on stairs/steps
– Adequate lighting
– Eliminate fall hazards:  Throw rugs, toys, clutter
– Entrance ramp

Gizmos and Gadgets
– Shoehorn with a long handle (medical supply stores)
– Bedrail that slides under mattress and helps get out and reposition themselves in bed.  (Must extend 3-4 feet under mattress for safety.)
– Swivel Seat (getting in/out of a car.  Plastic bags can work just as well on fabric car seats.)
– Ursec Urinal (this is a travel variety.  Good for preventing spills.)

More Gizmos and Gadgets
www.mtsmedicalsupply.com/pages/parkinsons-products.cfm
www.activeforever.com/flyers/Movement_Disorder_Catalog.pdf
Rollator (consult w/a PT before purchasing one.  Having a seat can be good on long outings.)
Stand Ease (helps one to stand from a low seat)
Turn Ease & Car Ease (help get in/out of car or bed.  Silk sheets and/or PJs can make it easier to reposition in bed)
Sock Aide (helps you put on a sock independently)

Complementary Therapies provide enjoyment and an outlet with a person living with a long term disorder.  Improves quality of life.
* Music therapy
* Art therapy
* Therapeutic horticulture
* Aromatherapy
* Animal-assisted therapy
* Spiritual care
* Massage therapy
* Healing touch
* Acupuncture

Hope
That elusive spirit in the heart of man,
With it, desires and fears will withstand.
When present and believed within the soul,
The thread of hope will keep you whole.
by Becky Dunlop, RN (2009)

Q&A
What county, state or federal agencies can help?
Contact your local Area Agency on Aging.  They are charged with helping individuals navigate the healthcare system when they are 60 years +.  If younger approach the state disability services agency.  Nurses and social workers at those agencies can provide direction.

Differences between Parkinson’s & PSP?
Main difference is Parkinson’s being a chronic progressive disease with good medicines for symptoms for many years so people are able to live full lives.  Advanced Parkinson’s disease is similar to PSP.  With PSP an individual will progress chronically and movement becomes worse because there are no medicines to reduce symptoms.  Falls and immobility increase over time.  In advanced Parkinson’s medicines don’t work as well because of brain cell loss.

As PSP progresses is it normal for the patient to become increasingly confused in unfamiliar surrounds outside the home?  What can a caregiver do to lessen the impact on the patient?
Yes, that is normal.  What a caregiver can do is create a routine and repeat verbally and in writing to the patient what’s going on.  Mainly, let them know what you’re going to do at the beginning of the day.  This may limit some of their anxiety and may help them be the best they can be.  Remember these individuals are not dealing with the same neurologic capacities we are as healthy people, therefore any change in routine is anxiety provoking and a disruption to them.  Anything you can do to aid and eliminate that will help them.

My husband can hear sound, but does not always understand what is being said.  Communication has become extremely difficult.  Is there anything to aid with this situation?
When someone can’t sort out verbal communication, it is very challenging.  Keep it simple.  Keep it slow.  Repeat yourself.  Give him time to process.  In many of these neurodegenative disorders people have bradyphrenia (slow thinking).  Their processing ability is much slower than a healthy person’s.  They may feel as though they understand what’s going on around the, but can’t get it out because what’s going on around them is happening so quickly.  Anything we can do to slow it down, simplify it, repeat, and give them time to reflect, will give them time to help that communication.

What palliative care services can be helpful to PSP patients?
Palliative care services may be engaging a home care agency that offers palliative care.  Identifying a depression may be helpful.  Identifying changes in vision.  Sometimes when a person has excessive saliva we provide medication to dry that and help them be more comfortable.  The whole aim is not to cure, but to keep the person as comfortable as possible, given the circumstances.

Will a discussion of facing the end worsen the depression of the patient?
Depends on the patient.  Many people want to know what lies ahead.  Even people with dementia, people can be concerned.  Reassuring someone and telling them the truth, but that everyone is there to help them is a comfort.  Facing the end may help the individual and allay their anxiety.  It may help them build their coping skills, knowing what to expect.

My PCP is treating my urinary frequency.  Should I see a urologist?
Sure.  A specialist may see something the PCP doesn’t know about.  Two heads are better than one.

Looking for a support group?
Contact CurePSP even for world-wide locations.

When should you consider palliative care?
Now.

Short podcast on Parkinson’s Plus conditions with Dr. Golbe

This is a worthwhile 28-minute podcast about Parkinson’s Plus conditions, including progressive supranuclear palsy (PSP), multiple system atrophy (MSA), vascular parkinsonism, corticobasal degeneration (CBD), and dementia with Lewy bodies (DLB).

soundcloud.com/world-parkinson-coalition/07-parkinsons-plus-conditions

Parkinson’s Plus Conditions
Podcast by World Parkinson Coalition
Speaker:  Lawrence I. Golbe, MD, movement disorder specialist

Brain Support Network Treasurer Phil Myers listened to the podcast and reports: “This podcast was done a year ago in 2016 as preparation for the World Parkinson’s Congress in Portland.  This is good coverage of all variations of Parkinson’s Plus. It compares each of them to Parkinson’s. It does talk about the lack of direct treatment but does discuss symptom treatments. And it talks about the difficulty of diagnosis.”

I’ve shared an outline of the podcast and a few notes below in case you want to forward to your disorder of interest.

Robin

—————————

1:00 – Dr. Golbe begins a general overview of Parkinson’s Plus conditions

1:45 – PSP discussion begins

5:00 – terrific question and answer on why levodopa doesn’t work in PSP, MSA, etc.

6:00 – MSA discussion begins

8:00 – discussion of dementia in MSA

8:20 – is MSA a prion disease?  He can’t comment on any one study in this format.  He says many diseases are prion-like in that there is a toxic chain reaction.  “Can’t catch MSA.”  Key distinction is that in CJD, the key protein is prion.  Prion protein can be transmitted more easily than other proteins.

11:00 – treatment of MSA symptoms

11:52 – discussion of vascular parkinsonism, which isn’t a neurodegenerative disease.

13:43 – CBD discussion begins.  One-tenth as rare as PSP or MSA.  Those conditions are one-twenthieth as prevalent as Parkinson’s.  Life expectancy similar to PSP and MSA:  between 5 and 10 years, typically between 7 and 8 years.  Lots more tau in CBD than in PSP.

15:10 – MSA pathology

15:40 – DLB discussion begins.  Spontaneous fluctuations that happen in DLB don’t happen in PD.  Levodopa isn’t quite as effective in DLB than in PD.  There’s is a problem that levodopa can cause hallucinations in DLB.

18:00 – general discussion.  There is long-term hope via a double-pronged attack.  First, stopping the protein aggregation or misfolding for a group of diseases.  Second, focusing on each individual disease.

We are further along in PSP given the focus on Alzheimer’s – both are tauopathies.

CBD is more difficult to do research on — because it’s rare and because diagnostic accuracy is very poor (50%).

21:00 – general discussion.  Neglected because they are rare, hard to diagnose, and no biomarkers.  FDA can given these diseases the status of an “orphan disease.”

22:45 – general discussion about how to help these families.  First, accurate diagnosis is important.  Sometimes people are given fruitless tests on the way to a diagnosis.  Second, symptoms can be treated.  Palliative management of symptoms.  Referrals can be made.  Third, at academic centers, treatment trials or research projects are available.  These can give patients the feeling of “fighting back” to help future generations.

25:00 – Dr. Golbe stops speaking.  Podcast continues with comments from hosts Dave Iverson and Jon Palfreman.