This is part of a weekly series introducing readers to individuals passionate about our Mid-Valley community.
For the past two years, Cody and Jordan Medeiros have been in a race against time to save their son.
Lincoln Medeiros, 4, is the only child in Oregon with SPG50, according to his family, a devastating, ultra-rare neurodegenerative disorder that affects fewer than 100 children worldwide.
There is a cure.
But the treatment, a gene therapy called Melpida, is still in development, and trials to bring it to market are not funded.
In 2023 the couple, who live outside Turner, joined with four other families across the country to raise the $1.15 million needed to launch a Phase III clinical trial, which will provide the treatment to four children.
As they’ve held garage sales and auctions, made and sold tamales, and put on a spaghetti feed, among other fundraisers, they’ve also watched the disorder begin claiming parts of their son’s life.
Already, Lincoln is beginning to lose his ability to walk.
“It gets worse with time. Ultimately, he’ll end up being in a wheelchair by about age 10,” Jordan Medeiros said. “It advances to quadriplegia by around age 20.”
Last month, the families reached their goal, and the clinical trial is set to go forward.
It will take place at UT Southwestern Medical Center in Dallas, Texas, and the first child will be treated in December. The next children will be selected month by month.
However, while Lincoln and the other three children whose families raised the funding qualify for the trial, there is no guarantee they will be chosen.
“We know what the eligibility criteria are and we know Lincoln meets them, but we don’t know how they’re actually selecting kids,” Jordan Medeiros said.
So, the group is raising money for an additional child to get treatment. So far, they’ve raised $199,000 of the $230,000 needed.
“Of course we all agreed to do it because there are four doses and five of us fundraising,” Jordan Medeiros said. “We hope that all five of our kids get it.”
“It’s gut-wrenching to watch your child’s disease progressively take away his skills knowing that a treatment exists to help him,” she said.
A devastating discovery
The couple found out Lincoln had a brain abnormality while Jordan was pregnant.
A 20-week anatomy scan identified the abnormality, called ventriculomegaly, but did not identify a cause. Standard genetic testing came back normal.
They were told Lincoln had an 8% chance of having a severe cognitive impairment.
When he was six months old, doctors told the couple that Lincoln had microcephaly, or a small head size. As he grew, he began falling behind his peers, Jordan Medeiros said.
Additional tests did not identify the cause, so in 2023 the couple decided to pursue further genetic testing.
The results were devastating.
SPG50, or Spastic Paraplegia 50, is an inherited neurodegenerative disease. Symptoms include impaired or absent speech, developmental delays, muscle weakness and motor difficulties, and a small head size.
“Cody and I have a mutation in the same exact gene,” Jordan Medeiros said. “And Lincoln inherited both Cody’s and my mutations.”
Their two other children, daughters Kennedy, 2, and Gracelynn, 10 months, do not have the disorder.
Parents fund research and trials
The couple immediately began doing research, and discovered that the parents of the only child with SPG50 in Canada had raised more than $3 million to try to find a cure for their son’s disorder.
With the help of scientists around the world, Georgia and Terry Pirovolakis developed the gene therapy, and it was given to their son, Michael, in 2022.
The therapy aims to replace the faulty gene with a functional one. It consists of a single injection of a virus into the spine, and then a period where the child will receive immunosuppressants in a controlled environment.
The treatment won’t reverse damage already done to their bodies, but it will stop the disease from getting worse.
If the clinical trial is successful, data evaluation will take a few years.
Then, the drug can move to market so any child diagnosed with SPG50 will have access to it and insurance will cover it, Jordan Medeiras said. If it’s successful, then other rare diseases can use the model to move forward with gene therapies.
“There’s like 10,000 rare diseases. Most of them don’t have a treatment path at all, because there’s just no profit to be made out if it,” Jordan Medeiras said. “We’ve been really grateful. The odds are kind of stacked against us, but we’ve had some really amazing families fundraising.”
“It’s daunting. It really consumes all our efforts,” Cody Medeiras said. “But the bigger point for me is a reminder that we can do hard things and we don’t have to do them alone.”
How to help
To learn more, or to contribute toward the cost of the clinical trial, visit https://www.lincolnscorner.org/
If you have an idea for someone we should profile for this series, please email Statesman Journal editor Jonathan Williams at [email protected]
Tracy Loew covers the environment at the Statesman Journal. Send comments, questions and tips:[email protected] or 503-399-6779. Follow her on Twitter at@Tracy_Loew
