Mandy Piercey doesn’t know how many times her daughter, Alana, has broken bones in her body. That’s because any effort to keep track, she said, would be an effort in futility and frustration.
“I stopped counting at 178,” she said. “And that was two years ago.”
The Piercey family, who moved to a home in Winston from Redmond at the beginning of 2019, is used to taking things in stride with Alana. The number of surgeries she’s undergone and bone fractures she’s sustained have reached head-spinning proportions.
This past month, Alana underwent a surgery in Portland that straightened her spine, which was once bent at a 48.1-degree angle. Yet she doesn’t feel sorry for herself — she refused to leave a summer-camp event near Sweet Home after a bone break for fear that she wouldn’t be allowed to come back.
It’s all just part of a routine that the 9-year-old Alana has gotten used to.
“She might be the toughest girl, or person, I’ve ever known,” said her dad, Matt Piercey.
Alana Piercey has Osteogenesis Imperfecta, more commonly known as brittle bone disease or OI. It’s a genetic disorder that, according to the U.S. National Library of Medicine, has no cure and causes easy bone breaks and skeletal abnormalities. The organization also noted how rare the disorder is — approximately six to seven people per 100,000 worldwide are affected — with the most common types of the disorder, types I through IV, affecting four to five people per 100,000.
Alana is one of at least two children in Douglas County — Sutherlin 7-year-old Sophia Roberts was diagnosed as an infant — who have been diagnosed with the brittle-bone disorder. Doctors have told Alana and her parents her condition falls between type III and type IV in severity, which can include severe bone deformity and easy bone breaks, poor muscle tone in arms and legs, loose joints, an early loss of hearing, and a blue discoloration of the sclera, or the whites of a person’s eyes. The depth of blue in the sclera, Mandy Piercey said, is a clear indicator of how prone Alana is to a bone fracture — the deeper the blue, the more prone she is.
The knee-jerk reaction to the diagnosis, Mandy Piercey said, would be to shelter their children and keep them out harm’s way in an effort to ensure their safety. But neither Alana’s parents or Candace Roberts — Sophia’s mother — have succumbed to that line of thinking, opting instead to allow them life experiences while preparing, and anticipating, for something to go wrong.
“We could easily just keep her home and not let her go anywhere,” Candace Roberts said. “But what kind of life would that be?”
Mandy Piercey knew exactly what to do when Alana was diagnosed with OI, but not on purpose.
A friend of hers asked her to help research OI, fearing her child might have the disease. A few years later after Alana had turned a year old, she had two fractures, one when her older brother stepped on her and another, a week later after she had healed after being in a cast for six weeks.
Doctors in Redmond couldn’t pinpoint the cause. Not long afterward when Alana’s teeth began to come in, Mandy said, they came in with a bluish-gray tint but would turn yellowish and start chipping not long afterward. Alana’s soft spot on her head also hadn’t filled in.
Multiple doctors and dentists later, Alana was correctly diagnosed with OI and Dentinogenesis Imperfecta, another genetic disorder that causes tooth enamel to weaken, flake and chip off, which in turn greatly accelerates the death of a tooth.
Mandy, who had already studied OI, wasn’t intimidated. Matt Piercey, not knowing what his wife knew.
“She looks at me and says, ‘We can do this,’” he said. “I’m bawling my eyes out when I look at her and say, ‘Do what?’”
To date, Alana Piercey has made dozens of trips to see specialists in Seattle and at Oregon Health and Science University in Portland. Her mother said the only bones in her body that haven’t broken have been her arms and her femur, although doctors had to break her femur to prepare her for one of her many surgeries.
Her most recent major surgery, which took place in September, combated her scoliosis, which had stooped her backbone to a slumped 48.1-degree angle. Matt said the out-of-pocket cost for the back surgery — which he said bonded a titanium rod and 26 screws into her backbone — would have exceeded well over $1 million without financial help.
Her siblings, Mataya, 14, and Christian, 6, often help care for Alana, and the family has a “break box” on hand during family outings for the just-in-case times when, for example, an impromptu splint is needed for a fracture. It’s an invaluable tool that helps the family maintain the normalcy of family outings and togetherness as opposed to taking a more conservative approach to limit her chances of getting hurt.
“Matt and I live by a pretty interesting philosophy,” Mandy said. “We would rather have a happy, broken kid who gets to enjoy every aspect of life than have a kid who is completely miserable and never gets to enjoy life. She goes four-wheeling. We’ve taken her snowmobiling. We’ve taken her hiking. We’ve taken her to the sand dunes (near Reedsport).
“One time when we went rafting, I slipped and fell (while carrying) her and broke her leg,” she continued. “I looked at her and said to her we have two choices. I told her, ‘We can stop, go home and you can sit on the couch and be miserable, or we can still float the river.’ We floated the river all day.”
The family moved from Redmond earlier this year, with Matt working as a stay-at-home dad and Mandy working as a department supervisor at Lowe’s in Roseburg. Much of Alana’s care and medical expenses are provided through Shriner’s Hospital out of Portland, which Matt said “has been a Godsend.”
Meanwhile, the friend of Mandy’s who asked for help researching OI prior to Alana’s birth didn’t have to go through what the Piercey family did. That child was never diagnosed with OI.
“I don’t know why it happened like that, if it was an act of God or something,” Mandy said. “It was amazing how much being prepared helped us.”
Unlike Alana, however, Sophia Roberts is very mobile, though small in stature. She turned 7 years old on Friday, which is a stark contrast to what doctors told Candace and Sophia’s father, Dustin, upon their child’s initial diagnosis as an infant.
“We were told that a lot of babies who are like her don’t make it past age 2, so the best thing we could do was live our life and prepare for the inevitable,” Candace said.
Sophia’s diagnosis came not long after she was born at Sacred Heart in Eugene. Doctors, Candace said, noticed her bright-blue tint to her sclera on both eyes and her legs, which bowed into a wishbone shape. Her head is also shaped in what Dustin and Candace affectionately refer to as a “strawberry shape.”
Sophia also has had a remarkably-low eight bone fractures, which included a three-year span without a bone break that ended with three fractures in three months this year. Much of that initial good fortune, Candace said, came after her initial diagnosis.
Sophia was prescribed on pamidronate, a medicine that alters bone formation and breakdown in the body. It can slow bone loss and help prevent bone fractures, and the Roberts’ insurance, which at the time the Oregon Health Plan, accepted it even though other providers considered the drug experimental, Candace said. Sophia also had surgery to alter and straighten her legs, which have growth rods in them to prevent additional breaks while accommodating for growth.
That’s helped Sophia become more independent than many children with her condition. She has a wheelchair, but it stays at East Elementary School in Sutherlin during the week and comes home during the weekend. She’s able to hike on her own, and her parents said each of her bone fractures have come through contact, not simple movements like some children given an identical diagnosis. Just in case, Candace is there to help her as a stay-at-home mother, while Dustin is an E5 in the Oregon National Guard.
Now, after celebrating their daughter’s seventh birthday, Dustin and Candace couldn’t be happier with how far she’s come.
”I was terrified when I heard this,” she said. “There were a lot of stages of denial and depression, a lot of us saying ‘Why me?’ Now look at her. Look at where she’s at today.”
Although the Piercey family moved to Winston in January, they knew the Roberts family from previous trips to Camp Attitude, a camp for special-needs children near Sweet Home. Mandy Piercey reached out to Candace Roberts prior to the move to ask about Douglas County, the area around it and everything it had to offer.
It’s common, Candace said, for parents who have children with OI to reach out to one another for support and advice. She said an extended support group for families, whether it’s for advice or something else.
Those have helped map out promising futures for Alana Piercey and Sophia Roberts in spite of the terrifying diagnosis each of their families was given years ago.
“When (Alana is) older and has a boyfriend or decides to bring a boy home, I guess I’ll get my gun collection out to show him,” Matt Piercey joked.
“Sophia told me she wants to play sports. Maybe soccer,” Candace Roberts said. “I’m like, ‘You know, how about chess club instead?’”
In each case, both families are thankful that’s even a possibility.
Jon Mitchell is a page designer, photographer and writer for The News-Review. He can be reached at 541-957-4214, or by email at firstname.lastname@example.org. Or follow him on Twitter @byJonMitchell.