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In progeria, the spirit is willing, but the body is not

Zach Pickard has had a rough, sometimes physically painful, year.

But because he is Zach, a tiny person with an outsize personality, a quick wit and the unflagging devotion of a new dog who is bigger than he is, you wouldn’t know it.

Some people enjoy bits and pieces of this world. Zach loves everything, all the time: magnets, making “slime”, Nintendo, Impractical Jokers on TV, YouTube and KFC, with which his mother says he is obsessed.

His tiny voice, like that of a doll, is most often roiling with laughter as he delights in the latest verbal or practical joke he has engineered.

At 10, Zach is one of only 144 children in the world with the rapid ageing disease progeria.

He recently chose to have a seven-hour surgery to repair his damaged hip socket. He had been unable to walk for months, and he was restricted to a wheelchair.

For a child who has had to adjust to a slew of limitations in his life – tiny stature, stiff joints, baldness and prominent veins – being in a wheelchair all the time was too much.

Zach’s parents knew that two other children with progeria had gone through the surgery proposed for Zach. It’s called VDRO, for varus derotation osteotomy.

It tips the ball of the femur into the hip socket, where it is stabilised with plates and screws to hold the bone in the proper position.

Before the surgery, Zach’s mum introduced him to a friend of hers who was in a wheelchair. The friend and Zach discussed the limitations of wheelchair living.

Then his parents, Brandon and Tina Pickard, left the decision up to their son: Would he have surgery? Would he adapt to the wheelchair instead?

Zach chose surgery. The operation was Sept 20, 2017, at Cincinnati Children’s Hospital, United States. The Pickards called the seven hours excruciating.

Occasionally, someone in the operating room would call the waiting room to let them know how Zach was doing, but the rest of the time, his parents sat with their fears: What if the surgeons didn’t have enough skin to stitch up?

Because they are so tiny and lack reserves of body fat, children with progeria don’t have as much skin surface as other children, making grafting a near impossibility.

“We were really thinking he was going to be a grumpy patient, but he turned out great,” Tina said.

While in a brace post-surgery, Zach gained a much-needed pound and a half. He is 41 inches (104cm) tall and 32 pounds (14.5kg) now.

The Pickards hope that this year, Zach will be able to resume participation in a progeria drug trial in Boston that is exploring drugs that can add months to the lives of progeria patients.

One in four to eight million newborns have the gene mutation that causes progeria.

The mutated gene causes a collection of symptoms that resemble premature ageing. The average life expectancy is 13 years.

The Pickards have invested in beating that number, becoming active fundraisers for the Progeria Research Foundation.

Just after Zach’s surgery, Carmen the dog arrived. She joins Zach’s guinea pig, K.C. Pickard.

Carmen barks at strangers, but is a friendly, mellow animal, rubbing her face against Zach to ask for head pats.

Zach rolls on the floor to be closer to Carmen, who weighs about as much as he does. In the manner of the best child-pet friends, Carmen is fascinated by everything Zach does.

Zach has been out of school and instructed at home so he doesn’t fall behind in his last year of elementary school before moving on to sixth grade and middle school.

To return to school, he has to be able to independently go to the restroom. He has “land” therapy and “aqua” therapy weekly.

The Pickards have one word for their son’s decision to have surgery, which they repeat to Zach: Brave.

For Zach, it’s a joke. What does “brave” mean?

“It means being brave,” he says, breaking into peals of giggles.

Asked whether the string of magnets he’s working on is a collar for Carmen, he grimaces.

He casts the side-eye of a soon-to-be middle school student, then glances at his big dog and the relatively short band of magnets: “She’s not a Pomeranian, you know.”

Here’s how much strength resides in that tiny body: Zach walks across the Pickard family room aided by a metal walker. He pauses, flexes his arms on the walker and lifts his feet from the floor.–star2.com

 

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