David Frei opens the packages of syringes, intravenous tubing, sanitizers and bags of drug solution spread out across his dining room table in Kirkwood.
Frei, 53, uses each one step by step until the bag of solution is dripping — about one drop every second, he estimated — from a pole into an IV in his arm.
A small study showed this near-daily treatment could slow his fatal ALS by a third.
The process takes about an hour and 15 minutes. Frei must do it every day for 14 days, then take a break for 10. Then it’s 10 days of treatments, 10 days off — for the rest of his life, however long that may be.
Frei, trying every day to live a full and active life despite losing the strength in his legs, is unsure if it’s worth it, especially with no guarantee it will work.
“It kind of comes down to a trade-off. I extend my life by a third, but if I spend that one-third of the day doing something unpleasant, are you gaining anything?” he said.
Amyotrophic lateral sclerosis — known as ALS or Lou Gehrig’s disease — progresses differently in everyone. Most die within two to five years of being diagnosed. Frei was diagnosed just over a year ago.
Muscles waste away due to the death of the nerve cells that control them, eventually taking away the ability to walk, dress, speak, swallow and breathe. There is no cure.
Radicava is the first new treatment for ALS in 22 years. Study of the drug involved just a small subset — 137 Japanese patients in early stages of the disease who were experiencing rapid decline.
The FDA quickly approved the drug for use in the U.S. in May. Without any research here or evidence that it works in the general ALS population, the federal agency approved the drug for patients at any stage of the disease.
At a cost of $1,000 for each infusion, that has left insurance companies and assistance programs grappling with whom to cover and for how long.
After Frei decided he wanted to try the treatment, he had to wait three months for his insurance to approve his case and figure out payment before finally getting his first infusion Nov. 15.
While advocates lauded the FDA’s quick approval of the drug for patients with so few options, that hope comes with a price that is not just financial. For patients measuring life in months — it’s paid in time.
Frei and his fiancée fast-tracked their wedding date and married in May. An elite athlete and daredevil, he pushes himself to his limit, trying to outsmart and ignore the disease for as long as he can. He has been able to do that with things such as controls for his car and a motorized mountain bike.
“I was pretending like nothing had changed,” Frei said. “I was living life fully, not like a sick person at all.”
But that has become harder over the past few months. He went from needing a walker to a motorized wheelchair. His beloved mountain bike rides are becoming more dangerous.
Trying Radicava gives him a bit of hope.
“I’m afraid if I don’t, I will continue to go downhill kind of quickly,” he said, “and I will be kicking myself for not taking some sort of action.”
Frei fears feeling desperate, however, like he is focusing on the disease instead of life. Worrying about his IV. Worrying whether it will work.
“ALS seems to be a grasping-at-straws kind of disease,” he said. “Grasping at straws is something I very much did not want to do.”
That little shock
Frei is the fifth generation of stained glass artisans who run Emil Frei and Associates, which has decorated the windows of many of the area’s Catholic churches since 1898. The Kirkwood studio has created and restored designs all over the world.
Frei, the youngest of four brothers, prided himself on being the “high-wire guy,” the strong one able to carry big sheets of tempered glass up ladders and scaffolding by himself.
One day early last year, he lost his balance and shattered the glass.
His adventure racing began to suffer, too. Adventure racing is a team sport that involves navigating through unmarked wilderness. It can include mountain biking, running, paddling and climbing and cover distances more than 100 miles.
Frei’s team, sponsored by the Alpine Shop in Kirkwood, was one of the best in the nation. But Frei started to stumble in races. He went from being one of the strongest team members to the weakest.
At first, he thought maybe he needed to train harder or something was off with his thyroid or iron levels. But every test came back fine.
His doctor became suspicious and scheduled an appointment with a specialist. On Oct. 18 last year, his birthday, Frei found out he had a degenerative motor neuron disease. Tests revealed it was ALS.
Frei said he cried a lot. And every time he noticed more weakness, he cried some more. But that got old.
“Every time I could feel any measurable takeaway in my ability, I would go through it again, and I got tired of it,” he said. “To some extent, I’ve gotten used to getting used to it.”
He continued racing on a motorized bike “which is totally cheating,” Frei said, “but they allowed me to continue on with them to enjoy all the social aspects and the racing. The fun was racing friends, not the award.”
He found easier trails for his “dawn patrol” bike rides with his wife, Mary Piper, 52, also an adventure racer. He found biking events that were more about fun and less about training, including stops for beer and hot dogs.
He visits the stained glass studio where he jokes, “Lunch is my main job.”
What helps him cope, he reveals, is imagining himself dead.
“It’s not a pleasant place to go. You give yourself that little shock ...,” he said. “And then the next day when I ride my electric bike or get together with friends and drink beer, that all is just as good as ever, and you appreciate it more.”
The hope light
Mary Riggs is the director of care services for the ALS Association St. Louis Regional Chapter, providing education and support for more than 100 counties in Missouri and Illinois.
One recent day, Riggs drove nearly 400 miles to see families with questions about Radicava. She had stops in the Illinois towns of Effingham, Mattoon, Charleston and Altamont.
They had questions about finding the nearest infusion center if they aren’t able to do it home, how the drug is administered, insurance coverage and delays, she said.
So far, Riggs said, regional patients are having to pay out-of-pocket costs of anywhere from $40 per 10-day treatment cycle to up to $2,400. Some patients in rural areas are having to travel 45 minutes from their home to get an infusion.
Riggs said many wonder about the trade-offs.
“They want to continue living as long as possible. And what I mean by that is not living by avoiding death, but living as participating in their life,” Riggs said. “They want to be able to continue doing things that feed their souls, so how can they put this into their lives every day … How can they integrate that into living?”
But families also feel empowered, she said. She sees hope in their eyes for the first time.
“For so long there hasn’t really been much they can do,” she said. “The exciting part for me, who has been working with patients for 18 years, is that I see the ‘hope light’ there.”
Dr. Timothy Miller, neurology professor at Washington University School of Medicine, says discovering new drugs to treat ALS is difficult because the disease is so variable in how it progresses.
The variability makes drugs difficult to study, because it is hard to prove if they are working or not. What causes ALS — which about 20,000 Americans are living with — is also a mystery in most cases.
In about 10 percent of cases, a genetic abnormality is the cause. Miller is leading studies in those cases, and many potential therapies are on the horizon, he said, which could also lead to discoveries that impact the disease more broadly. Other research includes studying the blood and spinal fluid for changes that could guide therapies.
“We are really pushing toward finding something that will really slow this down or stop the progression of ALS,” Miller said. “That is the goal.”
My favorite things
As biking became more difficult for Frei, he took up a friend’s invitation to race go-karts that reach 60 mph at the 11-turn half-mile course at Gateway Motorsports Park. He actually wins most nights.
“I moved on to something I’m kick-ass at,” Frei said.
He created special blocks he attaches to his shoes to help work the gas and brake. “Each friend grabs a foot and puts these homemade shoe things on my feet,” he said.
The night also usually involves post-race beers at McGurk’s in Soulard.
While camaraderie has always been an important part of Frei’s athletic endeavors, it has become increasingly so. “I’ve learned, don’t pass up those kinds of opportunities,” he said.
But competition, his wife says, is something that keeps the fire going in Frei. It’s not about winning, Piper explained. He’s never bitter when he loses.
“He pushes himself as hard as he can possibly push himself, knowing he doesn’t always win,” she said. “If he puts it all there and does his best, win or lose, it’s the effort that is most attractive to him.”
She joked, “I guess we’re going to have to go to card games at some point.”
Piper understands his concerns with trying Radicava. “We’re still living a pretty full and joyful life as far as engaging in as many activities that he can do and enjoy,” she said, “and he doesn’t not want to be able to do those things.”
They have new pursuits, Piper said. They bought tickets to the St. Louis Speaker Series. They are seeing more concerts.
“We always wanted to do those things,” she said, “but we never had time because we were always running or riding, and now we get to do those things together.”
She appreciates every moment they have talking and pedaling their bikes. She loves making blueberry pancakes in the morning and lingering over coffee and the newspaper, and sitting on the porch watching the sunset while drinking a glass of wine and holding hands.
“Just being together,” she said, “is one of my favorite things.”
Frei says he just wants to live as ordinary a life as possible. He’s not yet sure how his daily infusions fit into that. He writes a blog, which he’s found to be a comforting way to share his thoughts. On Oct. 12, almost a year after his diagnosis, he wrote:
“I don’t want to live a disease. I don’t want to have anything to do with this disease. I just want to live my life. It just so happens that my life consists of learning to get along by inventing new strategies to do ordinary things.
“Just do the best that you can and get on with it.”
Seconds after the last drops of a recent infusion, a friend knocked on his door at 10 a.m. Frei zipped up a jacket, and they left for a 12-mile mountain bike ride on the trails above Creve Coeur Lake.