Calling Winnipeg!

Amy Tinsley, the baby from Northern Ireland who underwent life-saving treatment at Children's Hospital a year ago, says hello.

A little over a year ago, baby Amy Tinsley arrived in Winnipeg from Ireland after having been diagnosed with hypophosphatasia, a potentially fatal genetic disorder.

At the time, the prognosis did not look good for Amy. Babies diagnosed with this rare disorder do not normally live more than a year.

Fortunately for Amy, Dr. Cheryl Rockman-Greenberg, head of the Winnipeg Health Region's Pediatric Program, was heading up a new drug trial for the treatment of hypophosphatasia. Under her care, Amy became the first child in the world to receive ENB- 0040, a drug developed by Montreal-based Enobia Pharma.

The drug worked. Today, Amy is back home, just outside of Belfast, getting ready to celebrate her second birthday on February 20.

And what a celebration it will be. Thanks to the medication, Amy is on her way to living a normal, healthy life, according to Amy's mom, Leanne Tinsley.

"How would I describe her progress - one word: phenomenal," says Leanne. "How often does it happen that a child, whose bones and muscles are so weak that she can't sit up or even breathe for herself is then able to stand and furniture-walk with absolutely no medical equipment 12 months later? This drug has given Amy hope of life and has improved her quality of life, fit to match the average child."

Leanne says Amy is now strong enough to stand as long as she wishes but is not yet walking on her own due to difficulty balancing, as a result of her curved spine. "She seems determined to walk, and her pediatrician, physiotherapist and I are all hopeful that this will happen around the time of her second birthday."

Meanwhile, Leanne says, Amy is now strong enough to feed orally and is making up for lost time in learning how to speak. "She has learned enough words to make known her tastes - breakfast, pancake, toast, apple, sweetie, juice, and chocolate."

Rockman-Greenberg, meanwhile, says Amy's progress has been "spectacular."

"Amy looks like she has really come on strong. She is much stronger. She crawls, stands up, takes steps. She's also developmentally catching up."

Children's Hospital is one of only two hospitals in the world authorized to enrol children in the ENB-0040 drug trial. Earlier this month, Rockman- Greenberg launched the second phase of the study in Winnipeg, which is expected to take 24 weeks. Four boys with hypophosphatasia between the ages of 5 and 12 will participate in the study. The boys come from France, Lebanon, British Columbia and northwest Ontario.

Amy's story and the work done by Dr. Rockman-Greenberg and her team was highlighted in the inaugural issue of Wave last May. You can read that story here.

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Wave is published six times a year by the Winnipeg Regional Health Authority in cooperation with the Winnipeg Free Press. It is available at newsstands, hospitals and clinics throughout Winnipeg, as well as McNally Robinson Books.