I was born in December 1963, a little girl, third child, first daughter. It was immediately noticed that my legs and feet were bent the wrong way. After a few months, no change, my parents knew something had to be done. Our family physician referred us to Ottawa General Hospital Orthopedic Department. At six months old my two legs were put into casts, hoping to shape them in the proper direction. After 6 weeks, casts removed, the doctor saw an improvement, however to ensure the bones would continue to develop in the proper direction, the physician requested a brace be made for my feet. White baby booties with a metal bar attached to the souls, toes pointed outward, my Mom would put my feet into the baby shoes at night and place me on my tummy. Now as a Mom, my heart goes out to my Mom in so many ways. Time went by and all was well. No more concerns about my legs!
As I began to walk my Dad noticed a strange thing about it. He saw a discrepancy in my right leg. The femur was deformed, my lower extremity was growing outward and I had a limp. Our family physician referred us to an orthopedic surgeons in Ottawa, Dr. Denis Desjardins, and in Toronto at Sick Kids, Dr. Wally Bobetchko. After several x-rays, many discussions, I was diagnosed with Multiple Enchondromatosis; tumours in the cartilage; Ollier’s disease. X-rays revealed tumours in my right upper distal femur, both lower extremities, two toes on the right foot, and two fingers in the right hand. The information we did have at the time stated that females were prominent to have Ollier’s on the right side, where as in males it was noted to be on both sides of the skeletal and with little information we had, this was helpful. However, amputation was the first suggestion, and one my parents wanted to avoid. As I was getting older, the pain and the deformities were getting worse. Finally, at 4 years old, I somehow knew that Dr. Denis Desjardins, orthopedic specialist at the Ottawa General Hospital was the doctor for me. I told my parents I liked him and he didn’t scare me, which gave my parents some ease.
Dr. Desjardins told us that the ratio for Ollier’s disease was 1 in a million, he had very little information, and no research was at his disposal. My parents were confused, scared, alone and had no one to turn to. The following discussion with my parents and Dr. Desjardins was the amputation of my right leg, from the top of the thigh as it was the most prominent area of tumours in my body. After a few months, thinking, talking, my parents concluded that amputation was not an option. Therefore the discussions turned to surgeries, and by now Dr. Desjardins had acquired some information on the disease, learning a procedure he believed would apply to me. As I was comfortable with Dr. Desjardins, the decision to move forward with surgeries was my best option.
Dr. Desjardins made me feel confident, no fear., and then he explained how the ‘hopeful’ surgeries could save my legs. My lower left leg was not affecting me, and new x-rays did not show any enchondromas, they had unexplainably disappeared. (we now know this to be a different form of Ollier’s). The enchondromas (tumours) in the cartilage of my right femur were manifesting to the bone, therefore causing a delay in growth and development, the bow in my femur was very prominent, and the lower part of my right leg was growing outward, from the knee down it pushed outward, this caused me a lot of pain.
My first surgery was set, I would be four and a half years old, and basically become the guinea pig in the doctors hands. The surgery consisted of opening the right femur along the side of the leg; perform an osteotomy, bone grafts, reconstruction of the femur with alignment of the fibula and tibia. I was in the Ottawa General Hospital, on Bruyere Street, for 12 days. My life changed in those 12 days. There was no children’s hospital yet, therefore your mom or dad could not stay with you overnight. All kinds of emotions ran through me. This was the beginning of independence for me, and helped shape me into the person I am today. I did not know what to expect. I do remember being excited and thought I was going to some sort of a hotel and looking back now it makes me laugh. The windows, they had bars on them! I had to wear their red and white striped pajamas, ( blue and white were for boys). Visiting hours were strictly enforced, the food was what you got and no outside food was allowed. I also remember not being allowed to drink my milk until I was done eating; their logic was to fill up on food, not milk. We also had Nuns doing much of the work, so it was strict at times. I am very thankful for CHEO.
The surgery went very well. Only thing was that my mother was not prepared to see me. I had a body cast, tubes coming out of my leg, IV in both hands. My cast went from my chest, full down my right leg & foot, they wanted me to stay put. This shocked my mom & dad, their little girl, all covered with medical stuff. My mom started to cry, she told me that I hugged her and told her I was just fine, everything would be okay. My mom knew from that moment that she had a fighter, a spunky-fighter she called me, on her hands. The same form of medical procedure took place every year, at one point it became every 9 months until I was 14 years old. Plus I often fractured my femur. Sadly the other big issue was that we were alone in all this. we knew of nobody else with the disease, nor did I have anyone to guide me with any outcome. Aside from my parents support of course, my reach for someone else with the disease was a huge whole in my life.
Things at home were different for us, and I often felt out of place. I was in and out of the hospital all the time, therefore in and out of the house. When I was home recovering I was tutored, and I recall this teacher in particular will never leave my mind, unfortunately for a sad reason and not a joyous one. The teacher was annoyed with me and vice versa. She concluded that slapping me in the face would be the solution. I was so shocked, I began to cry and rang my bell for my mom to come up to my room. A quick phone call, my father came home and fired her right on the spot. Friends accepted me, they were great and I always had a great sense of humor. But the disease brought attention from family, neighbours, friends, they were always concerned and asking about me. The surgeries and recoveries were just so often that it seemed like I was always in a cast. This caused friction between one of my brother’s and me. He is a year and a half older than me. We would get along at times, but mostly he did not want me around. He would end up physically hurting me, at times I was truly afraid. One day, at 12 years of age, (1976 maybe) I had had enough and took some pills. I have no idea what I took, but I certainly remember my mom calling Poison Control, and I drank lots of milk. I ran away from home a few times, then finally I left home the day I turned 16 years old.
That is when I met my ex-husband Kevin. We lived in the same high-rise, my family on the 25th floor, he and his roommates on the 21st floor. There was a party for Kevin’s 18th birthday and a friend and I attended. I knew then and there, as soon as I saw him, he was going to be the one. We moved in together 3 weeks later, it was 1980. We married in 1983 and our son Jason was born later that year. We loved to travel, I loved being a mom, and we were very happy. In 1986 our daughter Ashlee was born. We were so proud, and they were the best little children ever. I had a few incidences, one surgery and a battle with the pain. Like many others, Kevin did not see me as being disabled, or with a handicap. I was always spunky, always went beyond my limit and never gave up. I was always willing to try something new and rarely had any fear. That often got me in trouble or I ended up with a fractured bone. I also had my failed moments, drinking to forget about my childhood, and not dealing with the pain and anger inside of me. Sadly this affected our marriage, Kevin and I divorced in 1991.
I finally found others! A day I will never forget. I have ALWAYS wanted to meet someone else with Ollier’s disease. I found a group on Facebook and from that day forward I felt like I was home! I was so alone for so many years. There is still no how or why we were afflicted with this disease, and there is no cure. It is very rare, but the ratio has changed. It is now 1 in 100,000. Support, guidance, understanding and acceptance are all part of the process when diagnosed. I offer this via my support group and my counselling services. In the 1960’s, the mention of tumours/cancer was somewhat new, therefore often leading to amputation and/or death. My parents were facing a huge lifelong decision for me. Hence me being a guinea pig, something that I am proud of to this day. The surgical procedures are now being conducted on others. Surgery is the only way to deal with Ollier’s, which includes leg or arm lengthening procedure(s), bone grafting, and bone reconstruction.
Dr. Desjardins was dedicated to helping me, he researched and went to conferences in Switzerland and Russia in order to save my leg and learn about the disease. He then compiled a team from the University of Ottawa and it all began. As stated my 1st surgery was in 1967, and I had to prepare myself. I agreed to listen to the nurses and doctors, and be a good patient. I knew part of this meant me having to get better after the surgery. Being away from home, becoming independent, no family with me, it felt strange. I was in the hospital for just over 2 weeks, then went back home to recover in my own bed, with my family! All seemed to come along well, Dr. Desjardins and the research team felt confident with the outcome, I learned to adapt to the body cast and the restrictions it gave me. My follow-up appointment showed improvement. Then 3 weeks later, another check-up, revealed my femur was bowing, way too much, the tumours were more prominent. No explanation as to why, plus my leg was not growing properly, the lower part from the knee down was going outward, at a rapid speed. Surgery number 2 was being scheduled for June 1969. This time I was more aware of the situation, I knew more about the hospital, and what was ahead of me once home to recover. It was very difficult for me to stay put in bed, and being in a body cast was a definite challenge. So alone in my room one day, I cannot remember how, but I managed to roll to the edge of my bed, get up and start to walk….kind of like the way Frankenstein walks. I was told to get back in my bed, repeatedly. Unsure, but happy to see me relentless, my mom called Dr. Desjardins as the foot of my cast was wearing out. He was stumped as to how I could have gotten out of the bed ? Let alone walk ?? Back to the plaster room we went. In order to keep me put, or try, the cast now went half way down my left leg. A plaster bar went across from one leg to the other, this would ease the lifting me up for my Dad, he was my ‘carry-guy’ and I was his ‘carry-on’, lol. My future body cast would be the same.
The surgical procedures occurred every 9 to 12 months until I was nearly 14 years old. Along with all this came independence, being away from home and family. I did meet a lot of people and the hospital staff got to know me quite well, they gave me a home away from home. Many other incidents occurred between surgeries, life and all the stuff that comes with it, then other developments, medical issues, arose due to the Ollier’s.
I fractured my right femur, compound fracture, when I was 11 years old. We lived in Lauzon, Quebec, Canada and the orthopaedic team at the hospital had never heard of the disease. Consulting with Dr. Desjardins in Ottawa, it was agreed to put my right leg in traction. It took a total of 3 surgeries and 3 and a half months in the hospital, unable to get out of my bed or the room. Being in the hospital at a young age became lonely, especially a night time. Not being part of my family was a struggle. I learned to be independent at a very young age. The Hotel Dieu de Levis Hospital was run by Nuns. Unfortunately I had some of the saddest and most difficult times at that hospital. Mainly because I was little girl, they had no understanding of the disease, or of my being spunky, and we were English. Although bilingual, French and English, I was forbidden to speak English if a nurse of Nun was in my hospital room. I ended with bed sores, rudeness, and lots of backlash. I was gifted with wonderful things, and board games….My parents were told it was too much for a little girl and that the things should be brought home. Especially the tea cup my Grandfather sent me from England. It was precious, with my month of birth on it, and I loved it. My parents had to take it home, as the Nuns said I was that I was too young to appreciate such a gift. WOW, although raised as a Catholic, my beliefs began to change.
Please note that I am a very happy person, I have raised 3 amazing children. I have 2 beautiful, happy and generous granddaughters, and mostly, I am proud and honoured to support and advocate for Ollier RAREsie Warriors, Maffucci RAREsies, and RAREsies globally. We all deserve a voice, we all need a place to express ourselves, and we all deserve compassion.
Stay Strong, Never Give Up!
Keep Smiling,
Joanne