Family Comes First

Riley was born in 1992. Much like everyone else, his story began earlier—interwoven with his family before him. Riley’s mom, Nina, grew up nestled in the Rocky Mountains of Butte, Montana in a family of five. Growing up, her brother had a hard time walking upstairs. When her mom asked around about it, many people told her, “He’s five-years-old, he just wants attention.” But she didn’t buy that. From Salt Lake City to Seattle, her mom looked everywhere to find medical counsel. Finally, at 11-years-old, Nina’s oldest brother was diagnosed at Mayo Clinic with spinal muscular atrophy (SMA), a genetic disease that impacts the nervous and muscular systems of the body. When her other brother displayed symptoms at 12-years-old, they knew what they were dealing with.

After receiving genetic testing at 18-years-old, Nina learned that the chances of her passing SMA onto her children were slim. A couple of years later, when Riley came along and things weren’t quite right, Nina couldn’t help but wonder: Was it possible he could have a neuromuscular disease like her brothers?

It was at Riley’s three year check-up that her question was answered. Their general physician sent them to a neurologist who first suggested Duchenne Muscular Dystrophy (DMD). Riley received a biopsy at Children’s Hospital Colorado and the results confirmed his DMD diagnosis. Nina recalls, “We were told that he would be in a wheelchair by the time he’s ten and dead by the time he’s 17-years-old, so we should go home and take care of him and love him.”

Connecting to Support Networks

Early on, Riley’s family found Parent Project Muscular Dystrophy (PPMD) and attended their conferences. They needed to understand how to get Riley the best care and understand the latest research. “We knew Montana didn’t have the resources,” Nina says, “We had to go where the care was.”

After only a few years, Riley was diagnosed with mild cardiomyopathy. Luckily, during one of the PPMD conferences, Dr. Linda Cripe, recommended a doctor within driving distance. This meant Riley could attend regular doctor visits and have access to medications, including ACE inhibitors and beta blockers.

Alongside treatment in their home state, Riley and his parents would eventually make annual trips to Cincinnati Children’s for check-ups. By that time, they had a complete muscular dystrophy care team. All of the doctors, nurses, and care providers knew about DMD and how best to treat it. With these support systems in place, Riley maintained a stable condition for years.

Unfortunately, when he was 14 years old, Riley experienced a dramatic health shift, or what Nina calls a progression. The family was visiting New York City in December and all of a sudden, his complexion turned gray; his energy dropped. He had to be carried everywhere. Many families navigating DMD assume these symptoms are related to muscular dystrophy, but sometimes, they stem from the heart.

Pursuing Life-Saving Surgery

Come January, Ron took Riley to a check-up appointment. Nina remembers the phone call from their doctor that followed:

“[The doctor] said, ‘Listen, your husband is driving your son back and I don’t want him to get in a wreck. Riley needs a heart transplant. There’s nothing I can do here. We don’t have the facilities in Montana. I can make sure that he’s comfortable, but he will die if he stays here.”

When they arrived home, Nina shared the news and they immediately contacted their Cincinnati Children’s care team. Two days later, Riley was on an emergency medical flight to Cincinnati. Soon after he arrived, Riley’s cardiologist, Dr. Linda Cripe, confirmed what the family was worried about. Riley’s Heart was failing and his only hope was for a heart transplant. The family only had a few days to decide what to do.

The Cincinnati Children’s care team helped connect them with large children’s hospitals near them. At that time, none of those hospitals had experience with heart transplants for muscular dystrophy and did not feel comfortable moving forward with transplant. Nina recalls, “All we wanted was a chance. Within a week, we left everything behind to go to Cincinnati. People were there to take care of our life in Montana while we were away. We needed to be with him and we were choosing life. We chose life the whole time.”

By February, they were living in an apartment in Cincinnati and going to the hospital for weekly check-ins with the heart transplant team. While they waited for a heart, Riley’s symptoms worsened. Then they finally got the call–Riley could be taken in for a transplant. With full faith in their medical team, Riley’s parents focused on supporting Riley through the surgery. Now 16 years later, March 31st marks the day of the successful transplant.

Adjusting to Post-Surgery Life

Riley was in Cincinnati from February until August of 2007 when he had the transplant, going back to the hospital once a week for monitoring. Nina speaks of that time: “Our care team was like a family to us–the Parent Project too.” Even so, nine months is a long time to be away from home, so they were eager to return to Montana. Even after they got back home to Montana, they still returned to Cincinnati for check-ups once each month, then every three months, then six months, until they became annual visits.

To this day, quality DMD care is still a challenge to find, though they’ve found it can be more important to find medical providers that are willing to learn. “Physicians tend to know a little bit about a lot, but they don’t necessarily know a lot about one thing,” Nina says, “It’s all about finding someone who will listen to your needs—someone you can trust.” Their current primary care physician fits the bill. For Riley’s other care needs, they rely on the contacts they’ve made through Cincinnati Children’s and Parent Project.

Throughout his journey with Duchenne and against all odds, Riley has lived an active lifestyle. When he was younger, he drove a car, which his family was told he never would. He rode a bike. He’s tried skiing on mountains and water. He walked at his High School graduation. “You name it, Riley’s done it,” Nina laughs, “We had a trampoline in our backyard, for God’s sake. [Most importantly], we never told him he couldn’t do things.” Riley’s parents continue to focus on what he can do instead of what people say he can’t do.

The family eventually moved to Las Vegas from 2013–2017, and Riley went to Nevada State College. He did everything he could to keep walking, even using a scooter to go to class. Riley graduated with a degree in criminal justice and a minor in psychology. Riley continued to walk until he was 22 years old, and then transitioned into the wheelchair. Nina was still physically transferring him from the wheelchair into his favorite recliner because with her help he was still taking steps to get there. Eventually, after taking a fall and breaking his femur, while Nina was transferring him, he stopped walking altogether. Riley fought it until the very end. His toughness and strength kept him mobile for all those years. He still has great lung function and only needs to be on an oxygen ventilator at night. His family moved back to Montana in 2017.

He now works part-time for the Montana Department of Justice. Riley spends his weekends going to breweries and sporting events with his family. They just took a trip to Las Vegas to celebrate his 30th birthday and 16 year heart transplant anniversary. To other families navigating DMD, Nina stresses, “You can find people, and you can get help. [DMD] does not have to be a death sentence. Riley’s still considered terminal, but he’s never been terminal to us.” There is HOPE! There’s always HOPE!