This is a nice interview with Christine Tissot, who has multiple system atrophy (MSA) and lives in Vermont. Vermont neurologist Dr. Robert Hamill is also interviewed. The article’s author is a friend of Christine’s.
Multiple Systems Atrophy (MSA): The Story Of Christine Tissot
By Suzanne Gillis
No one I know had ever heard of MSA. I bet you haven’t either.
Neither had my friend Christine Tissot. Not until she learned of her own diagnosis two years ago. There is no test for MSA, no known cause or risk factors, no remission, no cure.
Diagnosis can only be done symptomatically and is often confused with other neurological diseases such as Parkinson’s and Multiple Sclerosis or ALS (Amyothropic Lateral Sclerosis, or Lou Gehrig’s disease). Treatment of symptoms requires the expertise of dozens of doctors and healthcare workers from different fields.
Multiple System Atrophy is a rare and rapidly progressive neurodegenerative disorder that is associated with the loss of nerve cells in several parts of the brain that affects all body systems we take for granted. It strikes less than five people in 100,000; their average lifespan from onset is less than eight years.
Symptoms occur and systems decline progressively at varying times, either alone, in combination, or in totality. The effects include: loss of balance, muscle coordination and strength; fainting and dizziness due to sudden severely low blood pressure; speech and swallowing difficulties; sleep problems; impotence; breathing impairment; dry mouth and skin; rigidity; vocal cord paralysis; tremors; and bladder and bowel problems. MSA does not affect memory, intelligence, or cognitive ability.
The disease strikes both men and women in middle age who become totally disabled; it results in death. Clinical definitions and facts do not come close to describing the enormous strain and chaos the disease ravages on the patient, medical providers, caregivers, family and friends. Jobs are lost and families are emotionally stressed and bankrupted. Public awareness is practically nonexistent. Research and funding are scarce.
Christine Tissot, 62, lives with her spouse Jody Crosby in Shelburne, Vermont. She generously consented to be interviewed by Vermont Woman to help educate and bring awareness to the public about MSA; funds are severely needed for research. The interview was conducted in late February at the couple’s home. Christine uses a wheelchair and spoke through a microphone to enhance her voice.
VW: Christine, you immigrated from Marseille, France, to the U.S. in 1979 with your family, including your children, Antoine and Mathilde, both now married and in their thirties. You also have two wonderful grandchildren.
CT: True. We moved to Tacoma, Wa., then to Charlotte, Vt. in 1982, when my children were five and eight. We lived there for 28 years.
VW: Until recently, you worked most of your life as an independent wholesale importer of European fashion footwear. You were also an active skier, kayaker, and hiker. You were living a full and vigorous life and looking forward to retirement. That is, until one beautiful day, four years ago on a back country skiing excursion from Bolton to Stowe, you realized something was terribly wrong.
CT: I have skied in the Alps since an early age. I was very comfortable with the challenges of the Vermont mountains. That day I fell several times. At first, I thought my new skis were defective. I bought them specifically for this event. I returned them and purchased another pair, but the next time I fell again and again, when I should not have. Then I knew the skis were not the problem, it was my body that was not working right. So I made an appointment with a neurologist in November 2008, following up with another neurologist for a second opinion in July 2009. An MRI of the brain and spine proved nothing. Neither doctor was able to make a diagnosis.
VW: What did these neurologists think was wrong?
CT: First they thought perhaps I had MS or Parkinson’s, which I did not. Neither mentioned MSA. However, the MRI did show a slight atrophy of the cerebellum, so one neurologist was convinced the cause of my problems might be from drinking. That was funny because I barely drink alcohol. I think she thought it was because I was French. My symptoms continued to get worse. Finally, in February 2010, I returned to the first neurologist.
VW: You must have been so frustrated and scared at this point.
CT: Yes, it was scary. The neurologist was shocked at how quickly my symptoms had progressed. For the first time, he was able to make the tricky diagnosis of MSA. This was difficult because the disease is very rare. I had certainly never heard of it. He told me to go home and look MSA up on the internet and then call someone else for a second opinion.
VW: Let me get this right! He told you to go home, by yourself, and look MSA up on the internet!
CT: Yes. I got home and told Jody, who also had never until this moment ever heard of MSA. I went to my office, which is in another building, and Jody went into the house to research MSA. Separately, but at the same time, we googled MSA.
VW: What an appalling way to find out. This must have been a horrible time for you both.
CT: It is a day I will never forget, February 8, 2010. Simultaneously, we ran toward one another outside. We were shocked, of course, though somewhat relieved to finally have an answer. Still it was the worst news. We made an appointment with Dr. Hamill, chair of neurology at Fletcher Allen Health Care for a second opinion.
VW: Please describe your meeting with Dr. Hamill.
CT: He confirmed the diagnosis and took the time to explain the disease in great detail. He also said I should attend to my affairs as soon as possible and prepare for the worst. We thought we would still have a few good years left to enjoy. But the disease was quite advanced. I now know I had symptoms years ago.
VW: Really. What do you mean?
CT: Maybe even nine years ago. I had urinary problems. My gynecologist suggested I might have a neurological problem, maybe MS. That was always in the back of my mind all these years. (Urinary dysfunction is one of the very early signs of MSA.)
VW: Christine, you have been knowingly living with MSA for just over two years. Yet it went undiagnosed for many years. Would you rather have known you had MSA years ago?
CT: No. Nothing could have been done. There was no way of knowing. We did the best we could and we had some good years.
VW: What did you do to prepare for the worst?
CT: Thankfully, my home in Charlotte sold in two days to a very nice family. I was sad to leave it, but the bedrooms and bathroom were on the second floor and it was not possible to live there. We found the perfect condo here in Shelburne. We love it. It works for us. The natural wooded marsh and wetland setting is alive with birds, ducks and other animals and the colors of the sky, trees, grasses and water change throughout the day. Nature gives me relief and serenity.
VT: As a same-sex couple for 13 years, you and Jody were married on May 5, 2010. How did you come to make this decision?
CT: It just felt right. We had lived happily and separately, but suddenly it felt right to marry. I wanted Jody to be a real widow, not just a girlfriend. Jody is my main caretaker. She makes it possible for me to live at home.
VT: Does the status as a legally married Vermont couple help when dealing with medical professionals?
CT: It makes it so much easier on both of us. Before, everyone either assumed or asked if Jody was my caretaker. We were always explaining that she was my partner, but she was so much more. Now we say we are married and that is that.
VT: Are you surprised that you are easily accepted as a couple?
Ct: No one blinks an eye. There are so many medical, business and personal decisions to be made every day. Together we make those decisions. When the time comes, Jody, as my spouse, will make them for me.
VT: How do you manage the financial burden and other paper work?
CT: I have excellent insurance: disability and long-term insurance policies. Once we were married, I was able to get on Jody’s heath insurance plan. I urge everyone to be sure they have good insurance. You never know if or when you may need to use it, if ever. But if you do, having enough insurance is critical and one less thing to worry about. Insurance gives you access to home caregivers, medical equipment and so many other things you need just to get by.
VW: What about health proxies and other legal documents?
CT: Your legal papers must be up to date and clear. An Advanced Health Directive should be registered, with a health proxy, power of attorney and of course, a will. It is best to consult your lawyer to be sure. Don’t put it off.
VW: So you and Jody made many preparations very soon after your meeting with Dr. Hamill in 2010. Then you traveled to France for the last time?
CT. We were in France for a month and visited places where I used to live and spent time with family and friends. I wanted to say goodbye.
VW: When did Atticus come into your lives? (Atticus is a one-year-old Havanese puppy.)
CT: I gave Atticus to Jody for her birthday as an eight-week-old puppy. He is a joy and makes us laugh every day.
VW: You mentioned caregivers. What is a typical week like for you?
CT: MSA has taken a toll on every system in my body. We are lucky to have a wonderful palliative care doctor, Zail Berry, who comes to the house and coordinates all my medical needs. We much appreciate the services of the Visiting Nurses Association, who help with the nursing care, physical and occupational therapy. We have a wonderful hospice volunteer, who gives me a massage once a week. Home Instead, with their skilled caregivers and flexible scheduling, give Jody respite as needed, which allows us to live together at home. Friends have been wonderful in so many ways, from helping us move, to sharing meals, sending flowers and cards, giving puppy care, music, phone calls and visits. One friend comes every Monday for the day, and another for several mornings a week. My children, who live far away, visit and call as often as they can. We could not possibly do this without all the help, love and support from our families and friends.
Jody is retired so she is with me most of the time. The nights are difficult. I sleep sitting up in a hospital bed. Because I cannot move, I need to be turned regularly throughout the night. Caring for me is difficult physically and emotionally and it is intimate. I have a supra pubic catheter, which must be emptied and cleaned two times a day. My wound must be cleaned daily. I am dependant for most everything. I still have use of one arm and hand, but my coordination is poor, so with great difficulty I can eat and use my iPad. But just the smallest thing, like a pen dropping, or just being an inch away, and I cannot reach. It takes Jody two hours every morning to get me up, into the bathroom, showered, dressed and into my wheelchair. I now need someone to be with me all the time.
VW: How has MSA affected your relationship?
CT: Emotionally, it has brought us closer together than we ever imagined. However, the physical and emotional stress has been difficult. For months, Jody tried to do everything herself. It takes a lot to ask for help and have people come into your home. Finally, she was so stressed, sometimes to the point of tears. So we arranged for professional care and reached out to our friends for support. Together they have made a big difference in our lives and they give Jody much needed breaks so she can better run our household and has time to take care of her own needs.
VW: What are your fears?
CT: Every day I worry what will be next to go. I am worried about becoming totally immobile, when I will not be able to move or speak. I intend to stop eating and drinking before I get too weak and before I lose my will and still have a bit of control left. I do not want to use up all my financial resources that living longer will require and exhaust the support around me. I take my lessons from my pets and animals. They know when the end is near and they disappear into the woods, or we intervene to help the dying process along.
VW: When will you decide to stop eating and drinking? How will you know it’s time?
CT: Since there is no hope for improvement, when I am in constant pain and miserable; when I can no longer move, or speak or control my bowels, then I will know. It gives me comfort when I have so little control, that the one thing left that I can control is eating and drinking. I have aged 20 years in six months, and feel at the same time like I have gone back to infancy. I think of death as a relief.
VW: The ending of life is so difficult for most people to think or talk about.
CT: Yes. Our culture has difficulty contemplating death.
Life is seldom easy. I am not surprised that this happened to me. Perhaps I am more prepared than others. I accept my destiny. I am not angry. I am not depressed. I am sad. I would have liked to have had more time. I am a grain of sand in the universe, and in that sense what happens to me is not important. What happens to me is important to me. That is why when the time comes, I hope I will have the strength and will to stop eating and drinking. The hardest part will be the first few days. It may take two or three weeks. This is a natural way to die. Pain will be eased by medication. I know I have to make this decision sooner rather than later.
VW: What is your philosophy about life?
CT: Live fully while you can. Live in the moment. Do not worry about things you cannot change. There is no escaping death. It is just a matter of time for each and every one of us.
VW: Thank you, Christine. Do you want to add anything more?
CT: I have had a rich and interesting life. Jody has brought so much joy into my life.
I have two wonderful children and grandchildren I love.
In mid-February, a celebration of Christine’s life was hosted by Jody and Christine for thirty friends, some of whom have known each other for nearly 40 years. Together with Christine, we have shared many joys and some heartache. We are all old enough to have lost loved ones. Usually we celebrate these losses after they are gone. On this occasion, we were able to share stories and memories with Christine present. Mary Provencher sang a song she wrote with Christine, called “Remember,” a beautiful gift from Christine to her friends.
There was not a dry eye in the room.
MSA from a Neurologist’s Perspective
According to Dr. Robert Hamill, “MSA is rare, affecting about 20 to 25 people in our area.” Hamill is Chair of the Department of Neurology at the University of Vermont College of Medicine and Fletcher Allen Health Care in Burlington, Vt.
Describing the experience of his patient Christine Tissot, Hamill said, “MSA is not always recognized unless a patient is referred to a neurologist, as many physicians are unaware of the disorder and patients are called atypical Parkinson’s Disease patients. Thus the prevalence of the MSA disorder may be higher than we know. UVM/FAHC, which serves 750,000 citizens in Vt. and N.H., follows about 15 patients with MSA; others are seen at Dartmouth or Albany.”
The cause of MSA is unknown, but thought to be an abnormality in the protein synuclein. “PD is also a synucleinopathy,” said Dr. Hamill, “and thus MSA and PD appear to be linked to some degree…It is hoped that research findings from studies in PD will provide insights into the cause(s) of MSA.”
Although much is known about PD and related neurological disorders, Dr. Hamill said, “Treatments directed at possible causes [of MSA] have been and are continuing to be tried, but I am unaware that anything has worked so far.
“Research funding in neurological disorders across the board is a huge issue. Limited funding sources are from the National Institutes for Health and the federal budget. Only about 10 to 15 percent of approved grants are actually funded. The focus is on diseases that affect larger populations. Currently, UVM/FAHC is involved in research projects funded by NIH to slow PD; positive results may turn out to be helpful for MSA patients.”
Dr. Hamill went on to note that, “Vanderbilt University, The Mayo Clinic and New York University are currently involved
in clinical care and research directly related to MSA. There are about 130 ongoing clinical trials aimed at improving the symptom complex of the disorder.
“Over thirty years of practicing neurology, I have been struck and awed by the strength of the human spirit when faced with the daunting challenges of the nervous system, the organ system that truly manages our lives. Christine faced MSA head on, has stayed positive and engaged, has never wavered…a remarkable person. I so wish we could do more.”
Though information is sometimes restricted, a resource for locating trials is www.clinicaltrials.gov. Other helpful links include www.msaawareness.org and www.mayoclinic.org/msa.
I have been one of Christine’s many friends, who have been helping her through this transition. In my profession as a hospice nurse, I have cared for several others with similar neurological diseases, although not MSA. As her friend, it is very hard to watch Christine lose her bodily functions each and every day. I have learned so much from both Christine and Jody about determination, courage and resiliency. In that sense, Christine has given me so much more than I feel I have been able to give her. Christine’s son Antoine recently told me that his mother is his hero, who has been teaching him all his life and continues to do so in her dying process. I was deeply moved by his words, so powerful from a son, as so many caregivers are women.
VNA hospice nurse and friend