This McNeil family photo was taken in 2008, the year the kids were diagnosed with MPS III (from left to right: Oliver, Shannon, Waverly, Matt).
Northern Virginia resident Waverly McNeil was 4 years old when she was diagnosed with a genetic, degenerative disorder present in one out of every 24,000 births: Sanfilippo Syndrome, or MPS III.
Kids with MPS III--short for mucopolysaccharidosis--lack an enzyme necessary for breaking down natural sugars in the body.
As Waverly's father, Matt, explains, "It's essentially like a city if the sanitation workers went on strike. There's nothing to take the garbage out so it just piles up." And it starts to damage cells and the brain in stages.
In Stage One, kids seem happy and healthy, but they'll become more hyperactive and experience restless sleeping.
In Stage Two, these symptoms increase, accompanied by memory loss. As Matt recalls, when Waverly hit this stage, "she started to forget things, like how to use silverware and feeding herself. And she started to have a lot of accidents, so she was semi-toilet-trained for a while and then we put her back in diapers."
Waverly is now 8, but she has regressed to the cognitive equivalent of a 1-year-old. And as she moves to Stage Three, Matt says, "it's just slowing down. She's completely stopped talking, singing. She can still walk with assistance; she's in a wheelchair, though, pretty much for her own safety."
Eventually, she won't be able to walk, chew or swallow, "and typically what happens is kids with MPS III will usually contract pneumonia from aspiration, so some fluids will get in their lungs, they'll get an infection."
And, until a cure is found, there is no Stage Four.
Storytelling the struggles
Waverly isn't the only McNeil with MPS III. Both Matt and his wife, Shannon, carry a recessive gene for the disorder, so their chances of conceiving kids with MPS3 was 1-in-4. And Waverly's five-year-old brother, Oliver, was diagnosed when he was 16 months.
Oliver doesn't talk, though he still can walk, but Matt says at this point his son's cognitive ability is like that of a 1-and-a-half-year-old.
"A kid asked me, 'So is he a big kid or a baby?,' Matt recalls. "And I said, 'Well? Sort of both!'"
But Oliver's heading more toward the latter, now that he's in the second stage of MPS III. His development has pretty much peaked, "and now we're starting to see that he's coming down," Matt says. "He's sort of gained all the new skills he'll ever gain."
What's interesting, though, is because Oliver was diagnosed at an earlier age than Waverly was, in a way, Matt and Shannon were a bit more prepared.
"We knew that everything he was picking up he was going to lose at some point," Matt explains. "With Waverly, we had no idea. So our expectations were, here's this little girl that we're raising to be an adult, and it was much harder at every first that we had with her - every first regression, that we realized, oh, she's not doing this anymore, that anymore. With Oliver, we always knew it was coming, and we knew about when it was coming."
But that preparation didn't necessarily make it easier to cope. In fact, Matt says after his children's diagnoses in 2008, he fell apart.
"I tried to be stiff-upper-lip about it," he says. "And I think it was just more me sort of shutting down a little bit, retreating."
So, he began to write. He'd been dabbling in writing since college, though he never finished anything. Yet each night, after putting the kids to bed, he'd head to his computer and write and write, "and the more I came back to writing, I was slowly sort of lifting out of the funk I had slid in to."
Eventually, he had a young-adult novel on his hands. The Strange Tale of Ben Beesley is about a fly that sets out to save his fellow flies, Waverly and Oliver, from the debilitating venom of some malicious poison spiders.
Matt admits the book is definitely somewhat of an allegorical autobiography.
"I mean, for the kids it's complete sort of action-adventure book about insects," he says. "But it was very much our memoir of my story with our kids."
Only in this story, Waverly and Oliver get cured.
"When I allowed myself to write a happy ending, I think that's when I started to realize that the book could be useful to us to try to get to it in real life," Matt says.
That's why all proceeds from the book's sales will go to the National MPS Society, to help fund research for what Matt calls "an orphan disease."
"There aren't that many people afflicted by it," he says. "So it doesn't get a lot of attention from researchers. So the more families who are affected can do to raise money to find research that'll lead to some day a cure, but at least a treatment that gives them a fighting chance, I mean, that's what we had to do."
But for now, Matt and Shannon relish the small joys they experience with their children, and take things day by day - always hoping, that someday, a cure will be found.
The Strange Tale of Ben Beesley comes out May 15, 2012, on International MPS Awareness Day.
[Music: "Winnie the Pooh Theme" by Disney Studio Chorus from Winnie the Pooh]
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