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Rare disease battled by Tigard family, OHSU researchers

With local foundation's support, university participates in SMA drug trials.


TIMES PHOTO: JAIME VALDEZ - Shawna and Dave Randall sit in the family room with their sons, Cade and Jace, below a picture at their home in Tigard. Shawna and Dave continue to raise money in memory of their youngest son, Cole Parker, who died when he was almost 11 weeks old from a genetic neuromuscular condition called spinal muscular atrophy.Three years, nine months and 29 days ago, Shawna and Dave Randall lost their infant son to a condition neither of them had even heard of before.

Cole Parker Randall was diagnosed with spinal muscular atrophy, a rare genetic disease, in his second month of life. There was no known cure or effective treatment. Cole was 76 days old when he died on Nov. 10, 2012.

“I don’t know how you survive a situation like that without having a tight community,” said Dave Randall.

“I think we were kind of surprised at how many people ... came forward, even when Cole was at home and sick, just with bringing meals and taking our bigger kids out to do things so they weren’t stuck at home, and all those kind of things,” Shawna Randall said.

Since the loss of their infant son, the Randalls have turned their grief toward a goal. In 2014, they started the Cole Parker Randall Foundation to raise money for research into spinal muscular atrophy, as well as to spread awareness of the rare condition and honor their son’s memory. More recently, they established the Cole Parker Randall SMA Research Fund at Doernbecher Children's Hospital, which is associated with the Oregon Health & Science University.

The OHSU connection is meaningful for the Randalls, who live in Tigard. The university is one of just a handful of sites around the country participating in a set of clinical trials testing the efficacy of a drug that could be used to treat spinal muscular atrophy.

“This is a novel drug,” said Dr. Erika Finanger, a pediatric neurologist at Doernbecher who is involved in the trials. “There are no drugs known to treat spinal muscular atrophy whatsoever.”

Finanger said the results of the trials so far have been “very promising.”

“There was a clear improvement in those receiving the active drug, versus those receiving a placebo,” she said.

While Finanger was not involved in Cole’s treatment — he was at Randall Children's Hospital at Legacy Emanuel Medical Center in north Portland — she praised the Randall Foundation for its support of OHSU’s work to find a cure for spinal muscular atrophy. The foundation’s contributions have also helped with what she called “routine care.”

“We also are using some of their support to help look at, for example, bracing for children who have scoliosis,” she explained.

The disease that took Cole’s life was classified as type 1 spinal muscular atrophy. It is the gravest of four types of spinal muscular atrophy that have been identified, with symptoms evident at birth or in early infancy.

“It’s diagnosed by six months, and life expectancy is two years,” Shawna Randall said.

“Two years maximum, depending on the severity of the symptoms and the onset,” Dave Randall added. “Our son Cole lived one day shy of 11 weeks.”

The clinical trials at OHSU are for both type 1 and type 2 spinal muscular atrophy. The latter develops in early childhood and typically renders those with the disorder wheelchair-bound. It can also cause difficulties with breathing and eating. Scoliosis, a distortion of the spine, is a common effect of the disease.

The Randalls said that since Cole’s death, they have met other local families with children living with type 2 spinal muscular atrophy.

“We invite them out to our event as our guests so they can see what kind of support they have and what people are doing locally to try to make a difference,” Dave Randall said.

Spinal muscular atrophy strikes about one in 10,000 people. In order to pass along the genetic disease, both parents must be carriers of the recessive gene that can cause it.

“We had never heard of it,” Dave Randall said. “We didn’t know we were carriers. We didn’t know that.”

Still, spinal muscular atrophy is one of the more common disorders of its type — and medical research and pharmaceutical companies, along with charitable groups such as the Randall Foundation, have now taken aim at the disease.

“If there is a cure, they do develop a cure, then there is reason for ... people to get tested to know if they have it,” Dave Randall said. “If there was something you could actually do about it.”

Finanger said she expects that Ionis Pharmaceuticals, the trial drug’s developer, will take the findings from the clinical trials at OHSU to the U.S. Food and Drug Administration in the hopes of getting approval to market the drug within the next six to 12 months. What happens after that is anyone’s guess, she added.

In the meantime, the Randall Foundation’s work is moving along. After about two years, Dave Randall said, the foundation has raised about $168,000. He said the board is hoping to surpass $250,000 by the three-year mark.

“The biggest check we’ve ever received has been for $3,000,” he added. “So it’s not like we’ve gotten these big $50,000, $25,000 donations. It’s just about community and networking and friends and family supporting us.”

The foundation hosts an annual golf tournament as its major fundraiser, which is coming up on Sept. 17 at The Reserve Vineyards and Golf Club in Aloha. A link to register is available at smilesforsma.org, the foundation's website.

The idea of “smiles” may seem at odds with a fatal disorder like spinal muscular atrophy, but for the Randalls, it is indelibly linked to their memories of Cole.

“We have all sorts of stories with Cole, and one of the things he really taught us — or he taught us a lot of things, I guess — (is) that stress is relative, and pain is relative, and adversity is relative,” Dave Randall said. “From the time he was six weeks old to 11 weeks, he required assisted breathing. He was intubated. He was oxygen-dependent. ... He had a direct feeding line into his stomach. And it didn’t matter what he was going through.”

Time after time, Randall recalled, even through breathing lapses, his feeding tube coming loose and his heart racing as he was raced to the hospital, Cole would “smile huge.”

That memory gives the Randalls strength. And if the work they are doing, the work OHSU is doing and the work that Ionis is doing ultimately leads to a cure for spinal muscular atrophy, then they will all have something to smile about.


By Mark Miller
Assistant Editor
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