Two Badass Sisters Inspire the Rare Disease World

00:02 I am Suzanne Hoff, 76, here with my good friend Sharon Neumann 66, in my living room in Traverse City, Michigan. Sharon, you are a grief recovery specialist, a former catastrophic injury and wrongful death litigation paralegal, and from 7 years of age have been the loving companion and caregiver for your younger sister, Nancy, who is now 64. At age 5, she was diagnosed with fibrodysplasia ossificans progressiva, or FOP, a rare incurable genetic disease. Since then, you and Nancy have had a challenging, life changing, remarkable journey together. And as individuals, 800 people throughout the world have been diagnosed with this genetic disease. Many more may have it yet undiagnosed. While FOP may progress at various speeds for each person who lives with it, every family member must be deeply affected by the demands of this disaster disease. Sharon, please tell us your story.

01:26 Thank you, Suzanne. It's so nice to be with you and to be asked that question. You're right. I have this very loving relationship with my sister and our journey has been both challenging and profoundly nurturing in so many ways. We were both born in Michigan. We have an older brother who has since passed, was seven years older than I am now. Well, seven years older than me throughout my life until he died at 33. Getting my thoughts in order here. My mother wasn't supposed to be able to have any more children. She had a very terrific pregnancy and delivery with my brother. So I was a surprise and was welcomed joyfully into our growing family soon after my birth. I wasn't quite one when my mother became pregnant with my sister and was soon thereafter in desperate failing health and hospitalized for the bulk of her pregnancy. And my brother and I were sent to live with my father's mother. So I experienced a separation from my mother at the age of one that lasted for five, six months. That was a precursor to what was to come. But Nancy was born and my mother survived after a near death experience. During that pregnancy, neither one of them were expected to live. So we were so happy to be back together as a family. You can imagine how happy we were. And slowly, slowly mom recovered and everything was well and happy. I loved my baby sister. I helped my mom a lot because mom was still weak. So even before Nancy was diagnosed, there was a special bond between us. Dad was an aerospace engineer with Chrysler Corporation in Michigan and had a job offer when we were before school age at a aerospace contractor in Connecticut that was working on the Kennedy space program. So an even greater adventure awaited us. Our family said goodbye to our extended family in Michigan and moved with excited anticipation to Connecticut. We were in Connecticut for a couple of years. I had started school. School. I was in second grade. Nancy was just in kindergarten. My brother was 14, just entering high school. Beautiful neighborhood, lots of kids. We were playing tag in the street that September evening before, right before summer turned to fall. School had already started. But summer nights were hot and sticky, fireflies bouncing around in the air. Streetlights come on, moms call everybody in and then it's over and we're getting ready for bed. You know how the 50s and 60s were if you lived during that time. Well, during that evening when we were playing, Nancy took a fall on the street. She fell hard on her bottom, cried for a little bit and I comforted her. And then we went back to playing. But when we were called into the house, mom looked at us and was just disgusted with all how dirty we had become. We played hard and she brought Nancy into the bathroom first. Because Nancy was little. Ern still needed help with a bath. And she said, sharon, you're next. So you just sit there and don't touch anything. They weren't in the bathroom very long and I heard a blood curdling scream from my mother, from my father. And a moment or two later my parents are rushing out the door with Nancy in a blanket and my dad calls out over his shoulder to my brother, Johnny, you're in charge of Sharon going to the hospital with Nancy. For a long time that's all we knew because they didn't come back during my waking hours. Anyway, what my mother saw that caused her to scream was a grapefruit sized swelling on the back of Nancy's neck. It was clear to her that something was terribly wrong in a very sudden way. They were in the mom and Nancy were in the hospital for over 40 days. My dad had to keep going to work. My 14 year old brother was in charge of me, making sure I got up and went to school and all of that. So during this time of uncertainty and fear for everybody, I was alone a lot. And it wasn't too long before my brother said, well, you know the drill. So I'm seven years old. He says, you can do this on your own now.

07:16 Oh, brothers.

07:17 Yeah, yeah, they're great. And we had a great relationship and I felt comfortable with that. But inside I was experiencing a lot of fear and anxiety. I realized going to school and that manifested itself in not knowing. All of a sudden everybody's just gone out of my life. The people who care about me and that I love are Just not there. I'm in the same house, I'm going to the same school. But I found the activity in the classroom overwhelming. I couldn't concentrate. I just could hardly stand to be in the classroom. And no one was talking to me about anything that was going on in the family. I was still. So I started not going to school. We had the privilege of living abutted to some property where there was a lake and places to wander. And I spent my time there until it was discovered I wasn't going to school. So eventually mom and Nancy were brought home with a diagnosis, a misdiagnosis of a terminal cancer. And we celebrated Christmas way before Christmas. I prayed my little heart out that Nancy wouldn't die. And had a lot of spiritual experiences during that time, I feel. And there came a time when I felt that. And she agreed, because we talked about it together, that she wasn't going to die. And we don't know what our parents are all crying about. And we became bonded and we shared a room. In fact, we shared the same bed. So when she came home from the hospital severely disabled, even after that short period of time, tumors all over her back, I would talk with her and help her fall asleep. And there was no pain medication. The pain was excruciating. And she found comfort in my laying hands on the places that were achy. And we enjoyed this wonderful, warm, healing, electrical connection when we did that. And that's something we continued for many years and we eventually found. My mother became quite panicked and hopeful, both panicked and hopeful at the same time. A lot of complicated emotions going on. When she realized that Nancy wasn't dying, that we were still playing and having fun and adapting to the disability. And I was helping her be comfortable and being her little guardian angel at school and everywhere else. Mom began the search for someone who knew what was really going on. No one locally knew anything. We ended up going to Yale Medical School, where someone there and an expert there referred us to Doctors Hospital in New York City. And it's there that I was present when Nancy and my mom were also being evaluated and counseled by two doctors who had traveled all over the world. And they recognized what she had, and we finally had the right answer. And at that time it ultimately became known as fop.

10:56 Did you have any support from extended family, neighbors? Who was there for you?

11:05 No one, to be honest. We had moved from Michigan to Connecticut. Our relatives were all far away. I don't think they understood exactly what was going on. My mother, I think, was on the phone quite A bit with her two sisters, but no one asked me how I was. And the rule of the day back then was to not necessarily burden the children with the truth, which would have been actually very helpful. The neighbors, we hadn't been living there very long, had a couple of friends, school friends who stayed close, neighborhood kids, but there wasn't a tremendous amount of support initially anyway.

11:59 And then came the diagnosis which changed your life.

12:03 Yes. Yeah. Yeah. FOP Fibrodysplasia sifakans progressiva is an ultra rare disease that results in bone growth in connective tissues, muscles, tendons, ligaments, places where bone doesn't normally grow. It turns one organism or organ in the body system into bone, locking the patient's body into a frozen position. Some people are more affected than others, but it's progressive during childhood and then stabilizes into adulthood. Absent trauma, the disease quiets down. But nearly all of the patients with FOP experience joints that are locked up, including the jaw, all the major joints. And the most affected people, like my sister, could be propped in a corner and still be in that corner years from now, unless someone were there to help them move. That's how limited the mobility can be. It's very painful while the disease is active and very, very disabling. So in the advanced stages of the disease, two and three people are needed to provide care. In the minimal affected, you know, one person could provide the supportive assistance. Yes.

14:04 In the course of this, and it has been diagnosed as the genetic disease disease, did it really concern your family? Are you going to get it? Is anyone else in the family going to develop it yet? Although it hadn't shown up, apparently.

14:25 I had a fear that I would develop fop, and with every bump or bruise, I waited and watched. No one ever informed me otherwise that it wasn't possible. There wasn't a lot known about it. In fact, we never met another person until late in our 20s. We were very alone with this. So my parents marriage suffered as a result of the life changes. Their marriage did not survive. Without going into all the details, it just eroded over time. There were other contributing factors, but FOP is like an atomic bomb that drops in your family and you keep trying to put the pieces back together. They never fit back together quite the same way. And without extensive supports and the knowledge that so many now receive today, I can't imagine families not buckling under the experience that we had. But through it all, Nancy and I stayed close. And when it came time for me to go off to university like I had planned, like my brother had done before me. I reassured her that once I was settled, she could come live with me. We have to. But we had to figure out how to get from here to there. And that never really happened. We were never really separated because my father told us, told me privately, that if you leave, I'm putting her in a home because there'll be no one to take care of her. And your mother? Because my mother was also dealing with some very serious health problems. So I had to rethink my plan. I was devastated, but the reality was now full force in front of me. I was to be Nancy's caregiver for the rest of my life, or she would be in a home. And that was not a place where she would survive any kind of care setting like that, because her care needs were so specific. So we made a pact and went forward.

16:59 And so through elementary school, middle school, high school, and then into college. Do you look back now and say, well, this really did affect my friendships, my social activities and my dreams.

17:17 That's a powerful question.

17:19 How did you feel about all of that?

17:23 There is a complicated kaleidoscope of emotions surrounding all that. Yes, my independent hopes, dreams, interests were highly curtailed. I was invisibly disabled from pursuing all that.

17:47 Say that.

17:48 Explain that. Explain that. I was invisibly disabled. So if someone were to meet me without knowing the situation, meet me independent of Nancy, they wouldn't know to look at me, that I had any kind of disadvantage or disability whatsoever. But when you make a commitment to care for someone with a catastrophic disability and you love them and you're thinking about their needs all the time, you also experience the disease and you are disabled. Maybe not in the same physical way, but you're disabled from being able to live out your own independent hopes, dreams, interests, curiosities about life. Interestingly, though, throughout this experience, I have been able to live my dreams just in a different way than I would have ever thought possible, maybe even in an even more magical, beautiful, life widening way. So at this point in my life, I'm very grateful. But going back and looking back, all the challenges, everything from being bullied in school and teased and taunted and pushing back on those people to protect my sister, and having to fight our way into every opportunity that we eventually enjoyed, meeting those challenges and making a decision that we would meet those challenges was the best thing we could have done.

19:54 Well, that's an enormously challenging pathway to develop a mature character.

20:03 I would agree. If I could wave a magic wand, it would have been different.

20:09 And do you Think it was and is your bond with Nancy that made that possible for you? Well, as something within you that said, yes, the bond with Nancy and something.

20:25 Within you, the bond with Nancy and something inside of me that I haven't ever been able to describe. My sense of purpose in life. Even before this diagnosis visited Nancy, my basic personality is to be helpful and be involved and stand up for the little guy. I was born ready, I think, for this. And there was evidence in that. Shortly before my brother drowned, he took the other path. There was nine years difference between he, Nancy, and seven years between the two of us. He had seven years with my parents. They supported him through college. He came to me one day shortly before he died, and we had a conversation on the subject of how was I able to do this. And also framed with his regret that he didn't help more because he chose to run.

21:33 Not unusual.

21:35 No, it's not unusual. And my parents didn't stop him. I said go because their plan was that I would stay. He regretted that and I offered him that it was okay. So we were able to have that conversation before he drowned unexpectedly in a flash flood recently. You know, fast forwarding. Well, I'll go back a little bit. So there was another big change in our lives that I was unprepared for, frankly. We met a researcher at the NIH through a series of handwritten letters. I discovered him in a little tiny article in the newspaper while we were living in Petoskey. And I recognized that, oh, my gosh, this researcher at the National Institutes of Health is studying fop. I don't know why our local paper put it in there, but I'm glad they did and I'm glad I read the paper that day. I wrote him a long letter and said, please get in touch. And he did. And that changed our. That was the beginning of a big change in our lives.

22:56 The turning point. Yes, perhaps.

22:59 Yes. So what happened at that time was eventually we had a personal meeting with him. We were connected with a handful of other patients that he was working with.

23:16 How old were you then?

23:18 I was late 20s.

23:25 So that put.

23:27 Yeah, let's see, my firstborn daughter was 2 or 3. So, yeah, in my mid to late 20s as a result of that and getting. There's more of the story. If people want to Google Nancy Sando or Sharon Neumann they'll find more of the story online somewhere. But we ended up meeting our first other family that had also been coping with FOP alone. They happened to live in St. Joe, Michigan, and they came up to visit us. And the moment Nancy and Andy's eyes met, they'd found their soulmate. They were married before the end of that year. And a story that's detailed in the New York Times archives if anybody's interested. But what came as an outcome of that was the need to begin more independent lives from each other. To this point in time, Nancy and I had always shared a house. We owned the house together. We had to go through a separation of our lives because it had just become too complicated. And the beginning of our our still loving and close but independent lives began. The world was a different place for me. I made a move to Traverse City, Michigan, with my daughters 60 miles away, so I could still be close to Nancy, but not be the person on call and start building my life. I had a lot of work to do because I had not contemplated that I would have an independent life. It was gritty work. It was expensive both in time and money and energy, but I knew how important it was going to be for me and my daughters. I found out that the world away from Nancy was less kind. All through those years when we were together, once we were out of the school system and into just regular life and church life, that people were kind and caring and supportive and helpful away from Nancy, I found that the world's a little more bumpy and I had to find out how I fit. And through the advocacy work I did with a couple of different law firms around the country, all the cases that we worked on, helping my girls find their way to success in their growing up years, and eventually marrying my current husband, who's just a gift in my life and experiencing a life without the day to day physical challenges of living with a rare disease. But still the emotional loving bond with Nancy.

26:37 You then evidently had a good confidence in Nancy progressing her branch of life in the family, and that must have been very comforting for you.

26:52 It was. She was a very successful young woman and businesswoman. The first half of our life there wasn't such a thing as the Internet. We had the good fortune of connecting with an incredible worker with the State Department of Rehabilitation who helped us work together to devise a higher education plan for Nancy that brought her away from home, which was very scary with other caregivers, a very scary experience for all of us. But she came out of that a shining star computer programmer. And through connections I had with my legal work and the community respect for us. Her first big project was developing the database centered record keeping for the Michigan Court of Appeals cases, which had previously not been managed in a computer system. So she had that under her belt and was doing very well for quite a while.

28:09 Both of you were quite accomplished. And still to this day, are you practicing your preferred pathway? And she is still practicing hers.

28:23 Nancy is in the later stages of the disease and she's recently retired from most all the work that she does. She had to give up her business when she was severely injured in a transportation accident. But that gave rise to the founding of the International FOP Association.

28:46 Now, the next remarkable thing that you two teamed up to bring about, please tell us about that.

28:54 Well, thanks to Dr. Zaslav, connecting us with other families, a small group of them decided they wanted to start a more formal organization, a pen pal organization.

29:10 This was without your input or suggestions, a little.

29:13 Well, that was there. Their thought that that's what it would be. But my advice was solicited and I felt that there could be more, that we could help the researchers build the community so that there could be more. Okay. So I conducted my own natural history study of the disease of 44 patients that came to my attention and had information for and gifted that to the research community. While Nancy and the pen pal club were growing, the organization and parents became involved. And there was a small number of families, including ours, that put everything they had in various ways into building the international organization, which has grown into a lifetime of work for me and so many others. But my direction has been organizing other rare disease communities, often in concert with universities and National Institutes of Health and other global organizations. For instance, the leukodystrophies, transverse myelitis, many, many other rare diseases. And now I focus on in these quieter years of my life of winding down a little bit and reflection. I'm still working with a number of rare disease organizations, including the ifopa. And I'm on board of advisors of this brand new effort to identify patients globally with an assortment of rare diseases called Tin Soldiers Global. I'm on that board of advisors. They're doing amazing work. But at this time in my life, I'm working on the grief recovery component of this. Reviewing life, coming to peace with what you've experienced, and living in a place of true gratitude and love, something that Nancy and I have been doing for a long time. And sharing that process and gift to others who are interested in finding that peace and gratitude about whatever their life experience has been. I really love what I do and I love my sister. I only wish she and I had a little more time.

31:45 Well, what a remarkable story, Sharon, and a beautiful story. And a profound story of all the good that's going to come of this change in your life. As a youngster, we never imagine a.

31:59 Head indeed wonder of it do not know, but it has been wonder, a wonder. Closing comment on that would be I came across as I have time to sort through journals, my high school journal, and in there I said, I don't know exactly what I'm going to be able to do or what I want to do in my adult life, but I definitely don't want to be bored. I haven't been bored a minute.