Memories, Advice and Virtual Hugs from Judy

Memories, Advice and Virtual Hugs from Judy

By Judy H. Biedenharn, RN, BS

As people get older, they retire from their careers, often downsize their homes and, in general, tend to slow down their activities. I checked off the retirement item over ten years ago and am now working on the other two! To help accomplish this, I am stepping down from some of my volunteer activities – one of which is my active and official involvement with the MSA Coalition as an Emeritus Board member.

Resigning from the Board was a little difficult to do as multiple system atrophy has been a big part of my life for almost 35 years. My husband, Ned’s, symptoms began in ~ 1985 with a diagnosis of “most likely Shy-Drager Syndrome” in 1991. (MRIs and other advanced medical screenings were not routinely done before the turn of the century!). He received validation of the diagnosis from the doctors at Vanderbilt Medical Center in 1995 stating “they had never had a patient admitted for evaluation having a correct diagnosis and on all the correct medications” to address treatable symptoms. However, that being said, they wished us good luck, and told us about the Shy-Drager Support Group, started by the wife of a patient of Dr. David Robertson there at Vanderbilt… and that began my association with the Shy-Drager Support Group (now known today as the MSA Coalition). I was first connected to the group as a caller into the support line. Then after my husband’s death in 2003, I attended my first patient/caregiver support group conference being held that year in my home state of Ohio.

After that conference I was hooked! I loved what the organization stood for: Patient Support and Education… and I have attended every in-person conference for the last 15 years. I joined the Board of Directors a few years later and served as Chair for a few years and then moved to the Emeritus Board.

I have so many good memories after I joined the Coalition:

  • Seeing the Board of Directors grow from 5 members (Dr. David Robertson, Dr. Tom Chelimsky, Vera James and Don Crouse and me) to over a dozen members today.
  • Watching the numbers of patients and their loved ones calling the support line increase (thank you Vera James for ALL of the hours you put in talking to patients and caregivers, researching the internet to get answers to questions, and just being a loving sounding board for those who called in).
  • Seeing the website grow, and grow, and grow (thank you Don) as computers left the business world and became a household item. Patients and their family members were accessing our website, printing off many of the articles that described their individual symptoms, and taking those articles to their hometown physicians, many of whom had never heard of multiple system atrophy. Our website was often the catalyst for the patient getting a correct probable diagnosis. This growth included posting of more and more medical research papers, which brought in more donations, which then enabled us to increase our funding for more research grants. Hopefully that wonderful snowball will keep rolling and get bigger all the time.
  • Somewhere during those years, the Board did increase in size (thank you current Board members) and we added two more pillars to our mission, making our mantra Education-Support-Research-Advocacy, and the name of the organization was officially changed to the MSA Coalition.

But even with all of those good, positive things happening, it is YOU, the patients, family members, and care partners that I have personally talked with on the support line or met over the years—it is YOU who hold a special place in my heart. Watching a patient attend their first annual conference and actually meet another person with multiple system atrophy, and observing the bonding that takes place is a memory that lasts a lifetime.

Great strides have taken place in research and in treatment of symptoms in recent years, but there is still a long way to go. One thing is for sure, you can count on the MSA Coalition to be there every step of the way.

My advice to patients and caregivers is simple:

  • Research your symptoms (the Multiple System Atrophy.org website is the most comprehensive site I know of)
  • Communicate with your doctors
  • Reach out to other family members, friends, and the MSA support line. Communication is best when you all are “on the same page” (or at least in the same book)
  • Be as active as you can for as long as you can (if mobility is a problem, you can even do some exercises in bed!)
  • Consider brain donation at the time of your death. It takes some pre-planning, but is so important for researchers to identify medical problems and determine possible cures for future patients (find out more on our website).

And last, but certainly not least, plan to attend the Annual Patient & Family Conference being held September 30-October2, 2021, featuring excellent physicians and medical professionals from various disciplines who are familiar with multiple system atrophy. Attending is a great way to keep informed and be supported in your struggles with this most difficult disease.

I’m looking forward to seeing you virtually this year… and hope we can have in-person conferences and I can give you a hug in person in 2022!

Hugs and Prayers,

Judy B.

Patient Perspective: Living With MSA

Patient Perspective: Living With MSA

By Sharron Mills

Amy was the MSA Coalition’s 20,000th Facebook fan! When we reached out to her to thank her for helping us reach this milestone, she sent us her warm greetings from Australia and told us about her friend Sharron, who has MSA. Amy helped Sharron write her personal story and share some great advice for people with MSA.

Pre-Diagnosis

I’m now in my mid-sixties, but a few years ago I made two significant changes in my life. Firstly, in my mid-fifties, I began a part-time university undergraduate degree, a Bachelor of Arts with a double major in writing and Australian history. Secondly, in my early sixties, I took up Irish dancing. Dancing is where I met Amy, my talented and very patient dance teacher who just happens to be a speech pathologist.

After a couple of years, dancing became harder, not easier, but the ever-patient Amy and her fellow teachers kept encouraging me. At the same time, my typing and handwriting were becoming slower and riddled with mistakes. As unexpected as these challenges were, I thought they – and a few other unexplained symptoms – must be due to my age since I’d never been a sexagenarian before.

A New Normal

However, in October of 2019, I was diagnosed with MSA. By that time, it was no great surprise due to the huge amount of research I’d been doing about my symptoms. Having my suspicions confirmed by a neurologist was still a source of grief because I knew straight away what a catastrophic prognosis I was facing.

When my troubling voice issues and potential swallowing issues made me realize that I would need speech pathology, who else would I go to for recommendations and advice but my friend Amy? As luck would have it, I was referred to palliative care and through them to Speech Pathology and other allied health specialists at the local hospital at around the same time. This didn’t make Amy’s help superfluous, just an additional bonus.

By early 2020, my handwriting and typing had also deteriorated to the point that I couldn’t continue my studies, and my mobility to the point that I couldn’t continue dancing. So now, ironically, I was a writer who couldn’t write and a dancer who couldn’t dance. Because I had come so close to finishing my degree, I did get awarded an Associate Diploma, so my university studies were by no means wasted. Another advantage university gave me was access to the online resources at the university’s library where I did hundreds of hours of research on MSA. You could say this was a case of ‘know your enemy.’

Family and Friends

You might wonder how my family felt about all this. I am close to my three children and eight grandchildren as well as three of my siblings, and it has not been easy for any of them or my husband, Bruce, to come to terms with my diagnosis or my rapid deterioration. In the words of Kath, my eldest daughter, “I never thought I’d be wishing you had Parkinson’s Disease.” Before I was diagnosed, I had been sharing with them little by little, some of my symptoms and doctors appointments without telling them my own suspicions, partly hoping I was wrong and partly preparing them for the bad news that was to come. By the time I told them of my diagnosis it was clear that some of them were already putting two and two together. For instance, my son, Robin, surprised me by already knowing more than my GP did about MSA. Not bad when we are talking about a very rare disease.

Friends have also been affected by my illness. My bestie from high school days recently flew up from Brisbane to Townsville to spend time with me, as did my younger brother and sister-in-law and younger sister who brought our elderly mum with her. They all made me feel very grateful and very loved. On top of that, we live in a village-like semi-rural community, but are fortunate to still have an excellent little medical center within a two minute drive from home. Thus, we have the advantages of a cohesive little isolated village where everyone looks out for each other with the advantages of town health care. There is much to be grateful for.

Coping

It is not unusual to hear people say that they don’t know how I stay so cheerful or that they know they wouldn’t be as happy if they were in my shoes. Initially, I was never sure how to respond because I know that I am fortunate that I am not prone to depression or anxiety and, so far, have not included these among my many symptoms. Sometimes, especially after a long wakeful night, my response was, “You should have seen me at 2am.”

Then I started thinking a bit more deeply about what I actually do to cope and surprised myself with my simple answer, “I just think of something else that makes me happy.” Often that will involve my grandchildren, but, paradoxically, they are also a source of sadness for me because they are saddened by my clear deterioration, and I know there is worse to come.

So I also dwell on the goodness of people – the medical people who do their best for me, family and friends who care about me, total strangers who open doors or pick up things I drop or stop to ask if I need help and social media support pages, like those of the MSA Coalition, where someone is always awake somewhere in the world and ready to listen. There are so many helpers. None of these can cure the pain and discomfort of this disease but they do make it worth staying alive in spite of the pain and discomfort.

The Importance of Hydration

The Importance of Hydration

By Cathy Chapman, Patient Representative, the MSA Coalition

Staying hydrated is particularly important for MSA patients because of the autonomic dysfunction with temperature intolerance and orthostatic hypotension.

Here are some helpful guidelines for adequate hydration from the MSA Coalition web site:

  • Drink at least 8 glasses, about 2 quarts, of water per day – more in warm weather or if you exercise vigorously – to ensure proper hydration of the bowel contents.
  • A convenient way to keep track of how much water you drink is to measure out your daily allotment in the morning and use that as your drinking water throughout the day.
  • Warm liquids will promote peristalsis, while cold liquids may inhibit proper bowel function and cause uncomfortable muscle cramping.
  • Coffee, tea, and some herbal teas act as diuretics, drawing water out of your system that can lead to dehydration. To avoid the dehydrating effects of these beverages, drink decaffeinated coffees and teas. Alternatively, you can offset their diuretic effects by consuming extra water.

TIP: Flavoring plain water with slices of lemon, orange or fresh mint adds variety.

Remembering the “Partner” in Care Partner

Remembering the “Partner” in Care Partner

By Elaine Douglas, The MSA Coalition Board of Directors

So much to do. So much our loved one can’t do. We now do the cooking, the shopping, the planning, communicating with family and friends. And pretty soon, we’re doing it all.

We’ve forgotten that we’re a “partner” and there’s another person in this relationship.

Sure, there are some things that we have to take on. But we don’t have to take over. In fact, doing everything can intensify our partner’s feelings of uselessness and deepen the depression that MSA tends to bring on.

How do we maintain our loved ones’ independence and morale by providing them with the right amount of support to optimize their functioning?

That’s the question posed in 4 Ways a Caregiver Can Encourage a Loved One’s Independence*, an article on AARP’s Family Caregiver Website.

For example, even if your partner can’t run to the grocery store, they may be able to order groceries online – or even dictate a list for you. Perhaps they can decide the menu for a family dinner, describe how they made their signature dish, call family members, make lists using Echo or recording function on a phone, help you plan for an outing, choose plants for the garden, make decisions about how to spend the day.

Feeling that they are contributing to the household and the family, even when their abilities are limited, can make a huge difference for your partner.

Remember: You may be able to do things quicker and even better. So what?

The MSA Trust said it well:

Your aim should be to help the person living with MSA maintain their quality of life and independence as best as possible.**

You are partners. Face MSA together!

———————

* https://www.aarp.org/caregiving/life-balance/info-2017/encourage-independence-bjj.html

** https://www.msatrust.org.uk/wp-content/uploads/2015/11/FS820-Guide-for-Care-Workers-V1.0.pdf

Update from the MSA Coalition’s Scientific Advisory Board Chairman

Update from the MSA Coalition’s Scientific Advisory Board Chairman

By Prof. Dr. Gregor K. Wenning, MD, PhD

MSA Diagnostic Criteria Revision

A major effort to revise the diagnostic criteria for multiple system atrophy is nearing completion. A taskforce was convened to consider deficiencies in the current criteria which had not been revised since 2008. The global community of movement disorder specialist neurologists had the opportunity to give their input in a survey with 470 replies received from 78 countries. The final consensus conference chaired by myself and Dr. Horacio Kaufmann was held virtually April 30 and May 1. The next step is for the writing committee to draft a paper to be submitted for publication in the Movement Disorders Journal this summer. Once published, the new criteria will provide a clear blueprint for neurology practitioners to diagnose MSA with more certainty. It will also help it to be recognized earlier so that patients may be considered in clinical research trials.

Update on MSA Coalition Research Program

The MSA Coalition issued a request for research grant applications that were due in mid-April. A total of 21 short research proposals (letters of intent) have been received and are currently under review by the Scientific Advisory Board (SAB). Nearly $1 Million in funding is being requested for these 1-year “SEED” projects. Those judged most promising will be invited to submit a more detailed project plan which will undergo further review over the summer. Seed grants allow researchers to perform small studies to test a hypothesis and gather more data before applying to government sponsored programs for larger multi-year funding support. The role of the SAB is to advise the MSA Coalition Board of Directors on the scientific relevance of proposed projects. The Board of Directors makes the final funding decisions. The seed grant program has been very successful with 42 projects funded since 2013 for a total of $2 Million.

In recent years the MSA Coalition has begun to develop a “CORE” research program as a complement to their successful seed grant program. Core grants are given to more robust collaborative multi-year projects that will help answer key questions about the disease. The “Core G – Genomics” project is now underway and is being coordinated by Dr. Dana Vuzman who will make use of the Clinical Genome Analysis Platform developed at Harvard Medical School. Although we know that MSA is not typically passed from parent to child there are actually clues that scientists can look for in the genetic makeup of those who develop MSA versus those who do not. A large genetic dataset with many thousands of MSA cases is needed however. Because MSA is a rare disease only small collections of isolated genetic samples are available around the globe. The MSA Coalition recognized that research groups were not working together in an organized fashion to combine their data and from that realization the Core G project was conceived. This project brings together a group of global collaborators who all agree to share their data as they look for common genetic traits in a centralized MSA genetic database which will contain thousands of samples. Dr. Vuzman and colleagues are currently working on building the needed infrastructure to enable the data to be collected and queried.

A second core project, “Core E – Environmental factors” is being overseen by epidemiologist Dr. Beate Ritz at UCLA and will look into environmental and genetic risk factors for MSA. This project is being conducted in collaboration with two established studies in Parkinson’s disease at UCLA. Since we know that MSA is most often misdiagnosed as Parkinson’s disease, this project is proposing to create an algorithm for more accurate identification of patients with MSA from Electronic Health Records. These patients can be contacted in future studies to better understand MSA onset, health care needs, and progression. A second component of this project aims to identify and collect pilot data from patients with MSA in a community heavily exposed to pesticides in the San Joaquin Valley in California. For this effort, neurologists are being contacted to identify and enroll people with MSA (as well as healthy control subjects) for interviews and collection of saliva and stool samples.

International MSA Congress 2021

For two days in late February, the 7th International MSA Congress was hosted virtually by Professor Shoji Tsuji at the University of Tokyo. Normally a much anticipated in-person event, this meeting takes place every 2-3 years and gives scientists with special interest in MSA the opportunity to network and update each other on the latest findings as well as educate young scientists and medical professionals about MSA. Although it was forced to be held virtually due to Covid-19, this year’s meeting was organized in a hybrid model to allow for a combination of livestreamed lectures (including very lively Q&A sessions) as well as on-demand presentations and poster sessions. The meeting was well attended by a global audience and has grown considerably over the last decade, due in large part to the support of the MSA Coalition. This year it was noted that well over a dozen speakers or poster presenters had received past support from the MSA Coalition through their research grant program allowing them to carry out and report on much needed studies. As an outcome of this meeting a special supplementary issue of the journal Cerebellum is being planned for publication later this year.

Clinical Trial Update

The MSA Coalition receives a lot of inquiries about ongoing or upcoming clinical trials. The Verdiperstat trial is still in phase 3 but no longer accepting any new patients. It’s important to remember that until every person enrolled has completed their trial no results good or bad are made available either to the drug company, the study investigators or to the public. We understand some patients may be sharing their impressions, however it is important to note that this is a double blind study. New phase 2 trials for ATH434 and Anle138b are currently being planned. Nothing can be announced until the drug companies make these details public. The phase 3 trial of Ampreloxetine (Sequoia study) for the treatment of neurogenic orthostatic hypotension symptoms commonly experienced by those with MSA is still accepting new patients. More details about these and other trials are available on the MSA Coalition’s treatment pipeline page.

Prof. Gregor K. Wenning, MD, PhD is a movement disorder specialist neurologist and a neurobiologist credited with generating the very first MSA mouse model during his PhD studies at the University of London. He is also the editor of the only medical textbook on Multiple System Atrophy. Prof. Wenning is the director of the Dysautonomia Center and chair of the Division for Clinical Neurobiology at Innsbruck Medical University, Innsbruck, Austria. He serves as chair of the Movement Disorder Society MSA Study Group (MoDiMSA) and is co-founder of the European MSA Study Group. He leads a consortium of 28 MSA centers in Europe and Israel, with the goal of driving forward accurate diagnostic markers and translational therapy research. Combining clinical and basic research, Prof. Wenning’s goal is to find a cure for people affected by multiple system atrophy. He has served on the MSA Coalition’s Scientific Advisory Board since 2012 and as chair since 2019.