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Special little girl has one deadly problem

Peter Crnogorac
Northern News Services

Yellowknife (Dec 04/06) - Three-year old Miranda Turcotte knows the ins-and-outs of what could happen to her if she slips and falls. But the little girl is too young to fully appreciate it.

As she spins in a large leather chair at her mother's office at the Commission Scholaire Francophone de Division in Yellowknife on Wednesday, mom, Sophie Turcotte, asks her what would happen if she did hurt herself.

NNSL Photo/graphic

Miranda Turcotte shares a loving moment with mom Sophie. The three-year-old is one of 200 people in the world with a rare blood disorder called Factor XIII. - Peter Crnogorac/NNSL photo

"Bleed and die," Miranda says in a sing-song voice as she finishes another rotation.

The Yellowknife child is special. She is one of about 200 people in the world, and 12 in Canada, to have been diagnosed with Factor XIII, a genetic deficiency of a key blood protein that is crucial to forming blood clots if she were to get a cut.

This form of hemophilia is insidious in that a person with the disorder could develop bleeding of the brain with no provocation, which could result in death within minutes.

Miranda is an outgoing girl. She loves candy, ballet and monkey bars - the latter of which constantly worries her mother.

"We have to watch her but we also want her to have a life," says Sophie.

Factor XIII is considered a genetic disorder, but Sophie and her husband Joel Turcotte have been tested and were found not to be carriers, although they will soon be tested again.

From what the parents know, there is no history of the disorder in either of their families. If it is determined that Miranda did not hereditarily acquire the disorder, she will be that much more unique, even among people with Factor XIII.

"This will mean she has a gene mutation, and will be the first person to have Factor XIII this way," says Sophie.

Miranda's story begins only weeks after she was born. Sophie says that when the stump of the umbilical cord fell from her baby's stomach, Miranda bled non-stop for two-weeks.

"Because the disorder is so rare, doctors had no idea what was wrong with her," Sophie says.

Soon after, Miranda developed terrible bruising on her legs. Sophie and Joel took her to a Surrey, B.C. pediatrician.

They told the doctor about the umbilical cord and bruising incidents and he mentioned the disorder Factor XIII.

Weeks later, when the tests came back, Miranda was diagnosed as the only person in B.C. to have the disorder.

Miranda, her mother, six-year-old brother Joshua, and her RCMP officer dad Joel moved to Yellowknife in June when the officer was transferred from B.C. to the Yellowknife RCMP Detachment.

"We were looking at moving to Nunavut but Miranda must live in a bigger city because she has to be close to a hospital," Sophie explains.

"Hospital staff are aware of Miranda," she says adding that the staff at the Stanton Territorial Hospital have met her daughter many times and have been learning about her condition in case they someday may have to save her life.

Miranda is the only person in the NWT diagnosed with the disorder.

"Her doctor Sam Wong didn't know about the disorder but he's learning now and telling other doctors around the North about it," Sophie says.

Every month, Wong gives Miranda a Factor XIII injection to help with the blood-clotting process.

Since moving to Yellowknife, the Canadian Hemophilia Society has also heard about Miranda.

The three-year-old is now a poster-girl for the society. Sophie encourages people to make donations to the Canadian Hemophilia Society.

"We agreed to do it, because if people donate to the Hemophilia Society, then someday they may find a cure for Miranda and others," she says.

When it's time for Miranda and her mother to leave the school board building, the little girl quickly puts on her winter clothes and excitedly looks out the glass door. Sophie dresses in her coat and mitts and grabs Miranda's hand as the girl begins to open the door. Although she is young and must live with this potentially-deadly disorder for life, it hasn't stopped her from contemplating future career plans.

"I want to be a ballet-girl," she says, as mother and daughter head into the frigid Northern night.