Read an Excerpt
You're Too Cute to Be Disabled
Living with Limb-Girdle Muscular Dystrophy
By Shelley Tudin
iUniverse, Inc.
Copyright © 2012 Shelley Tudin
All right reserved.
ISBN: 978-1-4697-3710-2
Chapter One
Early Childhood
I was raised and still reside in Brantford, Ontario, Canada. It is an average-sized city about one hour southwest of Toronto. Many famous people have come from Brantford, but the two who come to mind are Alexander Graham Bell, inventor of the telephone, and Wayne Gretzky, one of the greatest hockey players of our time.
My childhood was not unusual. My working-class parents raised three children: Michele was the oldest sibling; I, the infamous middle child, was born ten-and-a-half months later in 1964; and my brother, John, was the baby of the family. For those of you who are doing the calculations, my mother went in for her six-week checkup after delivering her first child in September 1963—and learned she was pregnant with me. Maybe that's when my energetic personality and spirited nature began.
As a young child, I was the hyper one and tested my parents whenever I could. Our house on Kensington Avenue was where all the neighbourhood kids hung out. My mother always said that it was such a difficult task getting me to come inside for dinner or bedtime. I would rather wet my pants than take the time to come inside for a bathroom break. Heaven forbid I miss some of the action! Driving to a cottage on summer vacations, Michele sat on one side and Johnny sat on the other, sleeping so calmly. I was the one in the middle and every ten minutes asked, "Are we there yet? How much farther?" I can't remember a time when my parents had private conversations that I didn't hear.
My father used to say, "Nothing gets past this one. Shelley, go to sleep!"
While I wasn't quiet at home, I was a shy child in school. Still, I always volunteered at the end of the school year to help the teacher clean her classroom. I guess I was a people pleaser. I always got good grades; some probably called me the teacher's pet. In first grade, I produced a painting of a tree with falling leaves. I remember my teacher commenting to others that it was rare to see a six-year-old child create a three-dimensional landscape. Maybe this was the start of a budding young artist!
As a small child, I never excelled in sports. I loved to figure skate. My parents even paid for private lessons when I entered into our local skating club's competition. I do remember looking at the results later that day. My name was at the bottom of the list, placing thirtieth out of thirty. I don't know if this was supposed to be character building, but something told me that I was not going to be the next Dorothy Hamill.
Every winter, my dad made the biggest ice rink in the neighbourhood. With their skates and hockey sticks, the neighbour kids headed to our backyard. We always picked captains, who then chose their team members one by one. You probably know where this is going: I was always the last child waiting to be selected and relegated to the net. The boys fired the puck so hard at me that I dove out of the way. The sponge pucks stung when they hit my frozen limbs. One thing I hated more than being last was being in pain. Rejection was tough, but I think I got used to it. I may not have had any athletic ability in my tiny frame, but boy did I try.
I remember those cold, winter evenings when my parents and siblings took turns holding the frigid hose and flooding the rink. Mr. Gretzky, father of the famous hockey player Wayne, lived one block over. He informed my father that using a sprinkler would create the perfect ice surface. Who would have thought flooding a rink with a sprinkler could be so easy, and much warmer? One of us would run outside and move the sprinkler every hour. My mother was concerned the rink was too large because our backyard bordered two large, eight-foot metal clothes line posts cemented into the ground. My mother argued with my father and boldly stated, "John, someone is going to skate into one of those posts and seriously injure himself."
My father replied, "Rosaleen, you worry too much."
One evening, my father played scrimmage hockey with the neighbourhood kids. One of the MacGregor boys passed my father the puck at the edge of the rink. As he skated toward the net on a breakaway, my father suddenly stopped dead as he hit his head on the metal post and fell back on the ice. I am sure he saw stars that evening that were not the real ones that glittered so brilliantly in the dark winter sky. We all watched to see if there was any life or movement from my father lying so still on the ice.
Dad slowly sat up and said, "God, I hate it when Rosaleen is right!"
Everyone laughed hysterically. The boys helped my father stand upright; one of them assisted him off the ice. My father had a wicked headache that night and finally acknowledged that having the biggest skating rink in the neighbourhood was not safe. The following winter, our rink was smaller in size with the edge of the ice ending two feet shy of those metal posts.
The Diagnosis
When I was eleven, my life changed. One day, my father took me to one of my softball games at a park not too far from our home. When it was my turn up to bat, I got a hit and started running to first base. Unbeknownst to me, a young woman sitting beside my father noticed I was having difficulty running as I barely made it to the base. This woman happened to be a nurse. She spoke to my father and asked him to talk to our doctor about the physical difficulties I was having. She definitely thought something was not quite right. My father was a little embarrassed, but he never considered the possibility of the events that were about to follow.
After a visit with our family doctor, he arranged two referrals, one to an allergy specialist and one to an orthopaedic surgeon. A few weeks passed before my mother took me to our local allergy specialist for my appointment. I remember the drive to the doctor's office and my comment to my mother: "I hope I'm allergic to smoke. Dad and you will have to quit smoking."
The doctor called my mom into his office after the allergy test was over. I assumed I had some serious allergies that he wanted to discuss privately with my mom. Unfortunately, I wasn't diagnosed as allergic to smoke, but something else that none of us could have foreseen happened.
My mother left his office with a worried look on her face. What I didn't know at the time was that Dr. Patel noticed a tiny, eleven-year-old girl who got up from a chair in his waiting room—a little girl who put her hand on her leg to push herself up. He also noticed a strange, waddling gait to my walk. Later that week on Dr. Patel's advice, my mother took me to another doctor for a blood test. I remember seeing the orthopaedic surgeon. He had commented about my knee caps being turned in. At that time, the doctors believed I may have swivelled hips. Later in life, after speaking to my mother, she informed me that Dr. Patel asked if our family had a history of muscular dystrophy. My mother was shocked and told him that there was no history of this disease in our family.
I don't remember the length of time that passed after the blood test before my parents took me to Sick Children's Hospital in Toronto. The blood work showed raised creatine kinase (CK) levels that suggested there may be a problem in the muscles. CK is a muscle enzyme, which is released into the bloodstream at high levels when there is muscle fibre damage. The doctors ran a series of tests, including a muscle biopsy. In October 1975, at the young age of eleven, I was diagnosed with limb-girdle muscular dystrophy.
We didn't talk about our trip to Toronto; however, I do remember how I felt when I entered the hospital room. As the doctor left the room, my mom and dad had tears in their eyes. It was quiet in that hospital room—too quiet. I can't imagine how parents feel when their child has been diagnosed with a life-changing disease. Our family physician thought it best that my parents keep this information from me so it wouldn't upset or scare me. This seems pretty archaic by today's standards, but my parents took the physician's advice and sheltered me from my diagnosis. Life continued as normal.
One day in the fall of 1976, when I was in seventh grade and twelve years old, I came home from school upset. Some of the kids in class were talking about my disability. The gym teacher who taught grade seven took it upon herself to talk to the class about me and commented to the class that I had been recently diagnosed with muscular dystrophy. My friends knew what I had before I did. My mother was very upset and called the school principal. She was angry that my parents were not asked before the teacher spoke to the class. This became one of many occasions that people in my life informed me about my condition before my parents spoke to me.
My mother and I took the train for my yearly checkup to the Muscular Dystrophy Clinic at Sick Children's Hospital in Toronto. I can't remember a time when I wasn't anxious as the train approached Union Station in Toronto. I would tell my mother, "I am not staying in the hospital; I don't care what the doctors have to say!" This must have been so difficult for my mother to hear.
I remember when I started walking on my tippy toes. My calf muscles were so strong, overpowering the shin muscles, which caused the tendons around my ankles to tighten. It was virtually impossible to step on my heels. The doctors at Sick Children's Hospital suggested I have surgery to lengthen the tendons to relieve the pain I felt while trying to walk. This would also allow me to return to somewhat of a normal pattern of walking in which my heels could hit the ground before my toes. My parents consented to the operation. I did not have any aspirations of becoming a ballerina, so I agreed—not that I was given a choice.
I spent a week in the hospital. Two knee-high walking casts were put on my legs after the surgical technique of lengthening the Achilles tendon. I remember returning home from the hospital four days before Christmas 1977. I hated that hospital, but I do remember my grandma and grandpa visiting me. They bought me the cutest Raggedy Ann doll, which was later used in one of my watercolour paintings. Eight weeks after surgery, the casts were removed. I experienced a new sensation when my tiny heels hit the floor.
Prior to this procedure, the simple task of walking was painful. Compare a tendon to a rubber band that stretches to its full capacity. In my case, that rubber band or tendon expanded; however, it could not relax to its original size to allow elasticity or proper movement of my feet. The pain I once felt when walking had disappeared. Since the diagnosis of LGMD, this was the first time in my life when I wasn't ridiculed by other children. Non-disabled children can be cruel to young individuals who have physical limitations. I wanted to be able to walk similar to other children—free from embarrassment and humiliation. This surgical procedure is not often performed today. The doctors now feel it best to let children with this disease walk however need be without causing interruption and further weakness to the muscles during recuperation.
At a family gathering, I walked over to my mother and boldly asked, "Mom, am I going to die?" I only vaguely remember this and am not sure who was around. My mother must have been talking to her sisters and was shocked that I asked this question. I do know that my aunts and other family members cried as they spoke about this later. I know they felt for me and understood how terrified I must have been to come out and ask this scary question at the tender age of thirteen.
I remember hearing things like "I understand you will be in a wheelchair before you are twenty. You have that disease that the kids on the Jerry Lewis telethon have. Those kids die very young."
Talk about being scared to death. It wasn't until I was seventeen years old that I realized I didn't have one of the serious forms of muscular dystrophy; I wasn't going to die young.
For those of you who are not familiar with muscular dystrophy, the type I was diagnosed with is defined as:
Limb-girdle muscular dystrophy (LGMD) Type 2 is a diverse group of disorders affecting the voluntary muscles, mainly around the pelvic (hip) and shoulder regions. Occasionally, the cardiac (heart) and respiratory (breathing) muscles may be involved.... The onset of LGMD may involve the pelvis, the shoulder area, or both. Early symptoms can include difficulty walking, running, and rising from the floor. Usually, and eventually, affected individuals will find it hard to climb stairs, stand up from a squatting position, and walk. Weak shoulder muscles can make it difficult to raise arms above the head, hold the arms outstretched, or carry heavy objects. The brain, the intellect, and the senses are not impaired.
My two cousins Patty and Susan hosted a muscular dystrophy summer carnival in their backyard to help raise money for the Canadian Muscular Dystrophy Telethon, which aired on television every Labour Day. My uncle Gary made an appearance in his fireman uniform as he was our local platoon chief in Brantford. I remember having fun at these carnivals but never realized my cousins were hosting these carnivals to help raise money for a disease that I had.
Those Awkward Teenage Years
Approximately four years later, when I was fifteen years old, my parents and I went to Sick Children's Hospital for my checkup. On this visit, the doctors questioned my parents about my sister and brother. They wanted to know if my siblings had any physical difficulties or changes. They suggested that the entire family come to the hospital for a series of blood work. Four months after this appointment, the entire family travelled to the hospital to have blood tests completed. Weeks later, my sister Michele, age sixteen, and my baby brother, John, age nine, were diagnosed with the same form of muscular dystrophy.
Approximately 90 percent of LGMD is inherited as an autosomal recessive disorder. For someone to have one of these conditions, they have to have two faulty copies of the gene responsible. All of our genes come in pairs, one from our mother and one from our father. If someone has an autosomal recessive type of LGMD both of their parents must be carriers.... These parents will, together, have a 1 in 4 chance of having another baby with LGMD.
The geneticists explained that all three of my parents' children were passed a double dose of the same defective recessive gene. I believe the odds with all children affected are very slim (due to the 1 in 4 chance). We may be one of the only families in Canada where all the children have been affected with LGMD. No other immediate member of our family has been diagnosed with limb-girdle muscular dystrophy.
Exercise can help those with LGMD keep their muscles strong and limber. It is important, however, that they do not overexert themselves to the point of fatigue. During my middle teenage years, I joined a local swim club and swam three times a week. Swimming is the best exercise for those living with a neuromuscular disease. Swimming exercises all of the muscles. I also competed in the games for the physically disabled. I never won any of the swimming competitions, but it was a great experience meeting other disabled individuals from all over the world. I never felt different when I attended these competitions. For the first time in my teenage years, I felt accepted.
High school was tough for me. Trying to fit in with peers isn't easy as a young teenager. Having a physical disability made it even more difficult. Walking up and down stairs was becoming difficult for me to manoeuvre. The school I attended did not have an elevator until my senior year, so they allowed me to arrange my schedule so that all of my classes were upstairs during the first semester and downstairs during the second semester. I remember the embarrassment I felt as I struggled to get out of the school desk. Other students would stare at me as I experienced these difficulties.
I had to leave class early to get to my locker and my next class before the crowds of students entered the hallway. Many times, I didn't make my destination before students came storming out. Like a herd of elephants, the crowd of students knocked me over. Sometimes I got so frustrated. Being the gutsy person I was, I would say, "At least you could pick up my books since you never off ered to help me up."
(Continues...)
Excerpted from You're Too Cute to Be Disabled by Shelley Tudin Copyright © 2012 by Shelley Tudin. Excerpted by permission of iUniverse, Inc.. All rights reserved. No part of this excerpt may be reproduced or reprinted without permission in writing from the publisher.
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