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Photos of Grante Robert Nikolaus “Nikko” Elmore and Bryson Hankins reveal similar boyish good looks — tousled, dark-haired curly locks.

Bryson seems to sense the outpouring of love from Nikko’s mom, Carolann, greeting her each time with an exuberant hug.

After all, Elmore recently helped give the 5-year-old the race car-themed birthday party of a lifetime in the detached three-car garage at her Concord home. It is where her son’s tyke-sized 49er jersey is framed.

The jersey is embossed with his nickname “Granite” for the toughness and tenacity Nikko showed throughout a more than two-year, fatal bout with Pompe disease, a rare genetic neuromuscular disorder, diagnosed in 2001, when he was just over 6 months old.

“Nikko never cried. He was the weakest baby and the strongest,” says Elmore, referring to how Pompe disease precludes the body from processing glycogen, causing protein build up in vital organs and muscles. “We tried to make everyday like a party.”

And at Bryson’s party, guests arrived in their electric cars, joining the red and yellow plastic push car that Nikko could never ride, and into which the children placed gifts that they were donating to UCSF Benioff Oakland Children’s Hospital in memory of a boy they never met.

“It’s the party I didn’t get to give my little boy,” Elmore says.

Bryson’s mom, Tammy, says, “Carolann thought it would be healing to see what she’d envisioned.”

Elmore’s thank you notes to the parents were yet another opportunity for her to increase awareness of Pompe, its early signs — missing key milestones such as the inability to feed, roll over, respiratory complications and an enlarged heart — and the need for its inclusion in the uniform newborn screening panel, so that the infant can reap the full benefits of enzyme infusions.

Currently, a handful of states have approved the test, however, California is not yet among them.

“Think of how many lives he has saved,” Elmore poses, referring to Nikko’s being part of a fledgling experimental group with weekly infusions of the missing enzyme.

“She’s so committed in spite of losing her own child. She’s been an ambassador for Pompe so selflessly,” says Dr. Priya Kishnani, a professor of pediatrics in the medical genetics division at Duke University Medical Center, where Nikko participated in the second clinical trial back in 2003.

“There is no question she has a lot to be proud of,” she adds. “Nikko taught us a lot.”

The enzyme replacement therapy, Myozyme, was approved by the Food and Drug Administration in 2006, and is being produced in large scale by the biotech firm Genzyme.

Kishnani reported further advancements, including clinical trials that are “targeting the skeletal muscles more efficiently.”

In 2009, scientists discovered means to “significantly reduce” or even prevent a possible immunological response to the treatment, which also improves its efficacy.

And, she noted, they are awaiting approval for clinical trials that would “go in and correct at the level of the (contributing) DNA.”

“There’s that vision of gene therapy as a good option for treatment,” Kishnani adds.

While continuing to advocate for the successful treatment for others, Elmore has lingering unanswered thoughts about her son’s prognosis had he received the infusions at birth.

“We’ll never know how he would’ve fared,” she says.

Nikko’s toddler-age bedroom décor remains, with the rocking chair where he sat and a wall sign that reads: DREAM. Elmore created a space there for her crafts and textiles that she donates, ever giving back.

“I come in here and feel his spirit,” she says.

 

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1 thought on “In Son’s Memory, Mom Pushes For Awareness for Pompe”

  1. Bill Figaro says:

    Thank you for this story as I lost a son to Pompe’s disease in 1985. Keep. Up the fight!

    Bill Figaro
    Bill@thegrowinc.com

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