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Miranda - I'm not perfect ... but I sure am special!

My story begins a few days after my birth in British Columbia, when my umbilical cord fell off and then refused to heal. At every diaper change, my parents repeatedly put Band-Aids on my belly button and each time they soaked up blood. After two weeks, almost as if by magic, my wound finally stopped bleeding! The pediatrician asked for a blood test, but nothing out of the ordinary was detected.

During the summer after I turned two, I fell on the cement and busted my knee. I had a big scratch the size of a quarter that took almost a month to heal. Throughout that same summer, I had so many bruises on my legs that I looked like a child who’d been beaten.
Several months later, still worried about all my symptoms, my parents met with another pediatrician. More in-depth blood tests were performed and all the factors were checked to finally arrive at a diagnosis — I had a deficit of factor XIII. Worse, I had a severe deficiency of factor XIII. They found only .05% of factor XIII in my blood. And you know what? I am lucky in my unluckiness; only one in three million people are factor XIII deficient … I always have a playful smile when someone says, “what are the chances that …!”

So, here’s the million-dollar question I’ve heard hundreds of times now in my life: “But what is factor XIII deficiency?”

The short version I tell my friends is this: There are 13 factors necessary for your blood to clot. The 13th factor is like glue that holds your scab in place so the bleeding will stop. Without this 13th factor, the chain breaks down and the bleeding starts up again. So, I get infusions that boost my factor XIII level to protect me from spontaneous bleeding. I find this is a simple way to explain my condition and much easier for people to understand than the clinical version!

During that fateful meeting with the pediatrician, he explained to my parents that it was imperative that I receive infusions of factor XIII concentrate to prevent bleeding in my brain. My parents asked, “what’s the worst-case scenario if we chose not to proceed with treatment?” And the doctor replied, “If she does not have the infusions of factor XIII and is in a car accident, she will never make it to the hospital.” The decision was made instantly: I would start infusions in the coming weeks.

Ever since I was little, my parents made me aware of my health issues and whenever someone would ask me what could happen if I bumped my head, I would respond without missing a beat: “Bleed and die.”

A few months later, a three-year-old in pig-tails, I got my first infusion of factor XIII. I looked at my mom and beamed: “Look mommy, I didn’t cry!”

In the following weeks, my family and I moved to Yellowknife in the Northwest Territories. My new pediatrician, Dr. Sam Wong, had to familiarize himself with my bleeding disorder, not having known about it before. So, it was an educational experience for him. As resources were limited in Yellowknife, and we needed to be closer to a large urban centre, we moved to the South shore of Montreal. This is how I came to meet Dr. Georges-Étienne Rivard, a leader in the field of hematology-oncology in Canada.

After several visits to CHU Sainte-Justine, my parents had to learn to do my infusions, and naturally had a few issues at the beginning. I remember several years ago, after my mom had blown a couple of my veins and was almost in tears, I gently told her, “It’s ok mom, take your time, start again!” It didn’t stress me out or make me angry! Now, I’ll let you in on a little secret — I am trying to stretch out for as long as possible the day when my parents will pass the torch and I will self-infuse twice a week. I know … I should be doing it myself, but it’s my little quiet moment with my mom or my dad and I’m in no rush to take over that job!

I’ve been very lucky since birth to have no complications or internal bleeds and I’m crossing my fingers that it stays that way. On the other hand, any surgery comes with its own precautions, whether it be a simple dental extraction (I’ve had seven teeth pulled) or a major operation like the one I underwent six months ago. I was at Sainte-Justine February 6 of this year, for Dr. Stephen Parent to perform scoliosis surgery and repair my spine 50 degrees. Leading up to my operation, many consultations were held to ensure that everything was in place for “D-day.” It was the first time that Dr. Rivard had a patient with factor XIII deficiency undergoing surgery for scoliosis. He planned ahead and increased my factor XIII dose before and after my procedure to ensure that coagulation and healing went well. I was like his guinea pig!

To be honest this operation was no fun for me … or my parents, or my brother, or for the wonderful nurses who heard me groaning or yelling in pain! My big brother Josh had the hardest time seeing me like this. But it’s all over now. My surgery was extremely successful; I had no complications during or after and now I have an amazing straight back! The cherry on top is I no longer hear my mom saying: “Mimi, stand up straight!”

In short, I am a little bit special … I have factor XIII deficiency, ADHD with hyperactivity, braces (for now), a 45cm scar covering two metal bars and 28 screws in my spine. Even though there are always risks related to my health condition, I don’t let them stop me and I take the necessary precautions. Let’s just say my early years were full of uncertainty and maybe some slightly exaggerated protections, right Mom and Dad?

At the end of the day, I’m a normal teenager with her joys and her struggles. I’ve just finished Secondary 2, I love my boyfriend Marwan, I frequently fight with my brother, I’m a motorcycle enthusiast and I give my parents a little bit of a hard time! I dream of becoming a pathologist and having children. I’m lucky to be surrounded by amazing people and I can’t wait to see what the future has in store!

- August 2017