By Dorothy O’Donnell
My husband Bert of 60 years retired from central Scotland Police in 1991. He had been a police officer for 28 years and was always very active. Bert started working for himself running a successful gardening business until he handed it over to our son who had recently retired from the fire and rescue service.
Retirement for Bert meant he could spend more time on the golf course and enjoy his love of Scottish dance music and playing his much loved fiddle with Stirling Strathspey and Reel Society Orchestra, and various concert parties. Bert had good health over the years and rarely visited a doctor. It was at his accordion and fiddle club that he first became aware something was wrong when he couldn’t keep his bow on the fiddle strings. A visit to his doctor resulted in her prescribing Valium as she thought it was nerves, despite having played on stage for years! The Valium did not help the shakes.
When his speech became distorted, Bert had his second visit to a doctor which resulted in another doctor referring him to a neurologist. An MRI scan of the brain showed deterioration of the cerebellum, an area of the brain that controls coordination, mobility, balance, and speech. Bert’s neurologist pulled no punches when he explained to us what Bert’s condition entailed and the problems it would inevitably bring. Nerve cells in the cerebellum had deteriorated and were dying off. He told us there was no cure and no medication that would help! Quite a brutal diagnosis and hard for us to take.
Because we had been advised that no medication would help, we gave acupuncture a try, but after 16 weeks, we stopped as there was no obvious improvement.
As Bert’s coordination and mobility worsened, we adjusted our thoughts and looked to the future focusing on what he was still able to do rather than focusing on what he was no longer able to do. Because of his lack of balance, in 2016, we parked Bert’s electric bicycle and replaced it with an electric four-wheeled mobility scooter. It was great that he was independent once again (sometimes with me following behind on my electric bike). We managed a further two cruises with Bert using yet another set of wheels, this time a walking frame with a seat on it, which came in handy to carry his dinner plate from the buffet.
COVID-19 came as a shock to all of us, especially for us when a doctor, that had never met Bert, phoned us at home to say that Bert should be put on the DNR (Do No Resuscitate) register, because if he had to be resuscitated, he would not survive! It was a real body blow and extremely upsetting.
Following Bert’s last consultation with his neurologist four months ago, Bert’s condition has now been classified as Multiple Systems Atrophy. He is battling this rare condition with all its limitations, but he continues to laugh a lot. This is usually because I don’t always understand what he’s trying to say and get the missing word wrong! The blame lies with me and my hearing loss! Bert never complains about his condition, and just gets on with what he does so well like sudoku, crosswords, and solitaire on his iPad.
So, our life has changed since MSA arrived but with the continuing help and support from our two wonderful sons and their caring and loving wives, we will continue to deal with this awful illness as best we can.
Bert sends his best wishes to his fellow sufferers here in the UK and with all of you in the USA…Multiple Systems Atrophy has no borders where this disease is concerned.
By Cathy Chapman, MSA Coalition Patient Representative
Summer is a welcomed season after the long winter months. With warmer weather, it is important to protect yourselves from overheating, especially on days that are hot and humid. A common symptom in MSA is heat intolerance. This is caused by the body’s inability to sweat, making it difficult to regulate body temperature.
Ways to reduce body temperature
- Drink plenty of cool water. Drink at least eight glasses (or about two quarts) of water per day and more on hotter days. It is best to avoid drinks with sugar as they can increase dehydration, which increases body heat.
- Stay in a shaded area or indoors where air is cooler.
- Placing a cool, damp cloth on the back of the neck can help to keep body temperature cooler.
- Avoid hot baths or showers
- Wear light, breathable clothing, preferably cotton or linen.
NOTE: Certain medications may interfere with the body’s ability to respond to the hot weather, further complicating the problem. This includes vasoconstrictors such as decongestants, beta blockers for high blood pressure, diuretics, and antidepressants. It is best to check with your pharmacist first, about the medications you are taking.
A more serious complication of overheating is heatstroke. Look out for these symptoms:
- Altered mental state (ex. confusion, agitation, irritability, seizures)
- Nausea and vomiting
- Flushed skin
- Rapid breathing
- Increase in heart rate.
- Headache – can be throbbing in nature.
Seek medical help immediately if you experience any of these symptoms. Until help can arrive, apply cool, wet towels to your neck, arm, and armpit. Always consult with your doctor regarding any symptoms you are experiencing with heat intolerance.
The MSA Coalition is excited to share that after nearly a decade of hard work, the Movement Disorder Society (MDS) Task Force on MSA Criteria Revision has reached an important milestone with its publication of the new Multiple System Atrophy (MSA) diagnostic criteria on April 21, 2022.
Last revised in 2008, the second consensus criteria for the diagnosis of MSA are widely recognized as the reference standard for clinical research but lack sensitivity to diagnose the disease at early stages.
The MDS Taskforce was chaired by Dr. Gregor Wenning (Left), MSA Coalition Board Member, and Dr. Horacio Kaufmann (Right) along with over a dozen other team members and consultants from around the world.
We are proud to share that an unrestricted MSA Coalition grant has allowed for open access of this publication as we believe our community members should have full access to the latest information about this disease.
Access the full publication here.
By: William Underwood, MSA Coalition General Advisory Council
There are many frustrations that come with being a care partner for an MSA patient, but the biggest one for me is how difficult it is to find compatible trials to enroll in. I am the care partner for my wife of 54 years. As of this moment my wife cannot meet the criteria for any trials, and I have spent hundreds of hours searching and contacting trials. After jumping through many hoops, she did get a compassionate care single-use drug Verdiperstat, which showed promise, however it did not slow the progress of the disease.
We are members of a support group of approximately 30 people, and to my knowledge, most have not even tried to enroll in a trial, as they find it too complex and are waiting for the doctor to recommend a miracle cure. What would really be helpful would be to have a database which could have the ability to quickly match patients with the best trials and expedite care, hopefully prolonging the quality of life for many patients.
My wife is 75 years old and was diagnosed with Parkinson’s Disease in late 2016. She is being treated at the U.R. Neurological Clinic, and while they are very helpful, they do not have the time to keep up-to-date on new trials and possibly recommend them to patients, leaving it to care partners to search on their own.
Trying to sort through trials that hold promise is very time-consuming. If you do a search based on unlimited travel and multiple symptoms you come up with hundreds of trials in progress, but most of do not affect my wife’s specific disease progression.
I begin all searches by using the following criteria;
- First, is it first interventional (i.e. participants are assigned to groups that receive one or more intervention/treatment)?
- Next, can I reasonably get to the location?
- Finally, does she match the many inclusions and exclusions? This can be a complex task, reviewing age limitations, conflicts in medications, required washout periods, physical abilities, H&R scale etc.
Another critical consideration is seeing if this trial medication or procedure may exclude you from other trials that may show more promise in the future. This can be a tough decision as you may not have enough information to make this decision. New trials are constantly under development and your personal window of opportunity may be limited due to general health and disease progression, so this consideration is not always easy.
My concern and that of many others is that we will miss a chance to help our loved ones because we do not have the capability to locate the best trials. It takes a long time to review critical trial criteria to see if each trial does apply to you and do a risk/ benefit assessment. Then we must contact the trial team for a consent to release medical records form and then request medical records to be sent in a timely manner for review by the trial team (this usually will require follow-up) and allow them to make an initial decision on your acceptance. This can take several weeks and is a very nerve-racking experience!
I feel that this is a time-consuming clumsy process and would like to suggest that neurology centers use their existing individual patient medical databases and compare this data with the trial criteria in the various trial websites. This would require either a person or a program be trained to go through trials and sort by requirements, but it would save a lot of time for patients and caregivers.
Find more information and resources on Clinical Trials here.
By: Belinda Franklin
There are three typical stages of Multiple System Atrophy (MSA) – Premotor (sexual and urinary dysfunction, sleep disorder, orthostatic hypotension), Possible (Parkinsonian, cerebellar features), and Probable (multidomain autonomic failure, pyramidal signs), but in my case, things were different. First, I had Frontal Executive Dysfunction which led to being misdiagnosed with Early-Onset Frontotemporal Dementia in December 2012. Secondly, recurrent falls and cerebellar issues (such as failing vision in only one eye) were early. Lastly, my case of MSA is not as linear as is typical.
Fanciulli, Alessandra, and Gregor K Wenning. “Multiple-system atrophy.” The New England journal of medicine vol. 372,14 (2015): 1375-6. doi:10.1056/NEJMc1501657
I have been through these mobility stages many times. I have severe autonomic dysfunction which was as depicted right on time in year two. My infections from dysautonomia averaged more than five hospitalizations per year over the next five years.
Each time I was hospitalized for these infections, I lost more mobility. Physical therapy helped me in the early years to regain my strength within a few months of these hospitalizations, but it took a long time before I could go home. I had to go to an in-patient Skilled Nursing Facility (SNF) to receive three-hour PT and OT six days per week.
I could be temporarily released to my husband on Sundays for no more than three hours. This course of 1:1 rehab took nine weeks just to regain enough strength to meet the goal of being able to safely transfer to a wheelchair for a discharge to home. Then I had another six months of combined in-home and outpatient physical therapy before I felt comfortable using my walker short distances within my home. Finally, I used my manual wheelchair permanently to get from the bed to the toilet to the sink. After three dangerous falls out of the wheelchair onto the bathroom tile, I am not allowed out of bed without assistance anymore.
In late year six, an emergency tracheostomy was required due to a paralyzed diaphragm, low lung function and vocal cords that closed automatically upon exhalation, resulting in an impaired airway.
I have seen nine neuro-specialists repeatedly over the years and in each specialty, we reached a point where the targeted therapy was only working for my symptom relief. There was really nothing more to be done. I now only venture to a clinical setting to see the pulmonologist who performs my trach changes every few months and cultures the growth of a nasty bacterial infection I contracted in-hospital post-tracheostomy.
In-home hospice has been a blessing. I have an aide five days each week. The daytime RN comes twice weekly or daily depending on whether I have an active infection or other injury. Hospice treats infections with antibiotics over a longer course of time. They bring any required equipment such as X-Ray machines to me.
Although I am now bedridden and cannot speak, I am relieved to know I will never see the inside of a hospital room again I wanted to be clear that if you see the typical MSA stages of what will be happening and when, it may not apply to YOUR case of Multiple System Atrophy.
While I’m still with you, I wanted to share some items* that I have found to be particularly useful as a patient:
- Grabbers were a must and I had them all over the house early on because my balance was so bad, I’d end up on the floor with whatever I was trying to retrieve.
- A swivel board was helpful for getting in and out of the car when I was a passenger. I also have the Able Life Car Assist Grab Bar that is portable and attaches to the door latch while you are entering and exiting.
- The WaterPik Dual Action Brush/Floss helped when I became too uncoordinated to use regular floss and my grip was slipping on my electric toothbrush. On bad days, my hospice aide can use it to keep my teeth clean. I’ve received glowing reports from my dentist on my most recent exams.
- A toilet bidet attachment fits just under the toilet seat with controls on the side. I have limited range of motion in both arms so getting thoroughly clean after bowel movements was next to impossible. The bidet has prolonged my personal dignity in that situation where it has been lost in so many other areas of personal care.
- I have by my bed a three-tier cart.
- The top is a solid surface where I keep my laptop, tissues, and a clear lazy-Susan with 4 compartments. One compartment holds all medication bottles. Other compartments hold pens, dry erase markers, stamps, a magnifying glass, nail clippers/nail files, hand sanitizer, eye glass cleaner wipes, wrist BP monitor and finger pulse oximeter and anything else I use on a regular basis.
- Underneath there are two wire shelves. The first is level with my bed so all equipment (urine collection canister, suction machine, vertical power outlet tower, and nebulizer) is on that shelf within easy reach. The bottom shelf holds all the supplies (suction catheters, trach ties, 4×4 split gauze, etc.) in individual clear containers.
- I have a medical table at end of my hospital bed for meals and this is where I keep my “joy basket.” On days when I am having trouble finding my one piece of joy, I reach in the basket and blindly pull out one of the multitude of cards I’ve received over the years, and re-read the cards and hand-written well-wishes.
- Although I resisted it for years, I did finally need the hospital bed with rails for two reasons – REM Behavior Disorder (RBD) caused me to fall out of bed while asleep many times, and I needed a 45-degree incline to breathe more easily day and night. I finally got the bed through hospice and paid OOP for a Purple mattress topper.
I hope I have helped all of you along your journey. I would like to share the chorus from my favorite singer/songwriter Paul Thorn’s single “That’s Life,” a collection of words his mother would say to him when he called her. “Wipe that tear out of your eye. Behind the clouds the sun still shines. You’re in my prayers every night. Enjoy the journey, that’s life.”
* The MSA Coalition is not sponsoring the products linked above or receiving any profit or proceeds from purchased items. The included links are meant as a resource for our patients and care partners, and we encourage you to do your own research to find products that work best for you.
By: Shelby Bowling
My mother lost her fight with MSA on December 24, 2021. Although her journey was tough, the fight this woman had against this disease is truly inspiring. Her symptoms began a few years back. Nothing crazy at first: a few falls, first hospital visits, and her voice got quiet. December 2019 is when we finally got in to see a neurologist, and she was told she had Parkinson’s disease.
Now if you know my mother, this was devastating to her. For her entire life, she has been on the go. A runner, wife, mother, nana, and great grandmother. Slowing down wasn’t anything she intended on doing. At the beginning of that year, she won a 5k race! Who would have thought we would be where we are today?
After her Parkinson’s diagnosis and she started medication for tremors, I just knew something wasn’t right. I was queen of Google trying to find answers. Researching doctors and treatments. She was declining so quickly, and I felt so helpless. Finally, we found a new neurologist. He is the first person to explain MSA to us and how the diagnosis of this disease works. Falls quickly became an everyday thing along with hospital stays, physical therapy, and medication after medication. She was losing weight, but I saw that glimmer of hope she had that it would improve.
Once we were told she had MSA and that she was declining rapidly, she told me, “No more hospitals. No more doctors.” She chose to do hospice care at home, so our family decided to do all the things that made her happy. My sister and I cared for her 24/7. Sometimes she hated this; she never liked being dependent on anyone.
But all in all, I could see how truly happy she was. She was home with her son, daughters, and great grandchildren (probably her favorite part). We watched Clemson football games every Saturday. We went to the state fair, the zoo, and went Black Friday shopping. You name it and she wanted to be there!
As the disease came to an end, I really saw the devastation it could have on someone: the pain that could barely be controlled, the anxiety, the anger that her life was being cut short. We learned to adapt to make her feel loved. My daughter and nephew would crawl up in the hospital bed we had at home for her, and she would light up. I would sit and tell her all the things going on, play music for her, and just tell her how much we all loved her.
She eventually became confined to her bed. Breathing troubles arose. I knew something was wrong, and this was when hospice told me death was coming soon. I was devastated. My mom, the person that became my best friend, would be leaving soon.
That last week was full of family and friends letting her know how much they loved her. My sister and I did all the medications and just tried to keep her comfortable. I can’t count the number of times I crawled up in her hospital bed with her and fell asleep. We were all tired because we wanted every minute with her to last. Her last breath was gut wrenching, but shortly after, I realized that she was no longer in pain.
MSA has changed my life forever. Loving someone and caring for someone with this disease will change the way you look at things. The amount of doctors that were completely unfamiliar with this disease was terrifying. I truly believe if more people were aware of MSA my mother would have had a different outcome.
I hope sharing this story with you all encourages you to help spread awareness.
Because now her fight is my fight.
Shelby Bowling was the winner of The MSA Coalition’s MSA Awareness Month Essay Contest in 2022. The story she shares was an inspiration to our community members and helped spread awareness of this rare disease. The Coalition is here to support patients and family members like Shelby and her mother, and we continue to advocate for comprehensive research, better treatment, and faster diagnosis for MSA!