TULLAHOMA, Tenn. (WTVF) — Ryan Schoonover loves to play baseball.
He played three years of Tee ball with a team, and now, he and his dad David spend time playing in the backyard as often as they can.
But Ryan, 11, has Duchenne Muscular Dystrophy, which is a rare genetic disorder that slowly deteriorates his muscles.
"When he got diagnosed we weren't sure if he'd ever be able to play, but being on this medicine he got to play three years of tee ball," David said. "He really enjoyed that. He can still swing that bat from his wheelchair, so we play a little bit different of a game, but we still have fun."
The medicine David references is called Viltolarsen, or brand name Viltepso. It significantly slows the process of Ryan's DMD. Unfortunately, David says Ryan has been without it for over a month, and he is running out of options to get it back.
TennCare is denying coverage of the $23,000 medicine based on the fact that Ryan can no longer walk, which David says is a vast underestimation of what the FDA-approved treatment does for Ryan.
"It really is frustrating because it shows they have no idea what this medicine actually does," David said. "It's not a walking drug. It affects every muscle cell in his body, and the heart and lung muscles are obviously the most important. Those are the ones that cause the early deaths for Duchenne boys. That's the biggest thing we're trying to fight against."
What is Duchenne's Muscular Dystrophy?
Ryan's body doesn't produce enough dystrophin, which means his muscle cells can’t heal and grow stronger. As his muscles get damaged over time, they are replaced with scar tissue and fatty tissue instead of strengthening.
“If you picture a chain of like 70 blocks, Ryan’s missing 45 to 52,” David said. “What this treatment does is it hooks 44 to 53, and the body goes ahead and produces a shortened strand of that protein (dystrophin).”
This means that instead of getting no natural dystrophin, Ryan will receive some. The treatment tricks his body into producing it by connecting the exons together that are normally not able to connect because of the gene mutation. It isn’t a cure, but it means his overall quality of life is better for a longer period of time.
“Whatever we gotta do, we’ll do it,” David said.
Ryan has had DMD since 2016, when he was only 3 years old.
Thanks to his dad's involvement with the Jett Foundation, Ryan was part of a first-of-its-kind clinical trial meant to slow DMD, which led to the exon-skipping therapy he now uses. He was the last boy out of 15 picked to be part of the lengthy and time consuming clinical trial.
“The first two years of the trial we had to travel to St. Louis, Missouri. We were living in Murfreesboro at the time, so a little over five hours both ways. We went there every week for two years,” David said.
During the trial, he went through two muscle biopsies where they cut muscle tissue out of each of his biceps — one at the beginning and then one after 25 weeks. He had blood drawn before medicine, during medicine and after medicine before being able to go back home each day for the first three years.
“He went through so much during all that. That’s what’s so heartbreaking to me is to have somebody take that away from him when he worked so hard for it,” David said.
Four years later, the FDA approved the new version of exon-skipping therapy, and Ryan was able to receive the treatment at home. But this is where the insurance problems started.
An expensive treatment and new therapy means a complicated relationship with insurance
The medicine Ryan needs costs around $15,000 per infusion, and infusions are required once a week. The bigger Ryan gets, the bigger the infusion, meaning the latest costs $23,000 per week.
“Insurance companies are not too thrilled about paying for it,” David said.
David’s first employer wouldn’t cover the treatment, so TennCare covered his infusions for that year. Then, David got a new employer a few months later and they began paying for the treatments through Cigna. But when David’s employer changed insurance, the new insurance wouldn’t cover the cost, so David again turned to Tenncare. They said they could no longer cover the medicine for Ryan based on the fact that he can no longer walk.
His dad's employee insurance plan denied the treatment for the same reason — and upheld the decision to deny it in two different appeals. He was also denied coverage from Tenncare, had an emergency appeal hearing to fight it, and was denied again.
Tenncare argument: United Healthcare (David's Managed Care organization) says the medicine is "not medically necessary" and doesn't meet their criteria — "submission of records confirming that the patient is ambulatory with out needing an assistive device." Ryan can't walk, so he can't receive the medicine.
Ryan's argument (through David): The medicine is FDA-approved and therefore must be covered under the comprehensive child health mandate. The claim that Ryan can't walk and therefore does not satisfy criteria violates federal law because it denies coverage based on Ryan's disability.
“This is a major problem in our healthcare system," Kelley Maynard, founder of the Little Hercules Foundation, said.
Her nonprofit helps kids with these rare diseases get the treatment and care they need.
“This is the perfect example of a payer not understanding a disease progression and not reaching out. And they can reach out. We would explain it to them," Maynard continued. "I wish these payers would engage with the rare disease community without making decisions that are harmful to patients."
She says only 5 percent of rare disease patients have access to treatment, and since they are not population based diseases, insurance companies don’t want to pay for them because they are more expensive.
Ryan's doctor argued that "continued denial of coverage will be detrimental to Ryan and will ultimately result in a more rapid decline in his motor and pulmonary function."
Maynard says if Ryan's doctor — the one who fully understands his disease — says the treatment is medically necessary, then he should be receiving it.
David is holding onto the little victories with Ryan, and continues to look for new therapies his son can be involved in. The Tennessee Justice Center has filed a complaint with Tenncare stating Ryan’s civil rights have been violated by using his disability as an excuse not to treat him, according to David. He also told me that currently because his income increased, he is at risk of losing all his coverage, which pays for his son's in-home nurse and other medical necessities.
TennCare told me they don't comment on pending litigation.
In the meantime, if you want to help Ryan, you can donate to the family’s GoFundMe for his treatments.
Have you experienced something similar to this, or other healthcare issues? Reach out to me at hannah.urban@newschannel5.com.
Fostering Hope provides Christmas for kids in foster care. I'm delighted to see Fostering Hope expand this year to expand their reach to now include kids in Foster care in metro AND foster kids in East TN hard hit by Helene.
-Bree Smith
