Raife Manjarrez is a gangly 14-year-old boy, preoccupied by his cellphone and whatever you’ve got to snack on, so long as it’s not dark chocolate.
To be fair, it’s the end of May, four days into summer break. He’s surely got better things to do than sit in his grandmother’s living room with his mom, talking — or not — to a reporter about his experience with the degenerative eye disease that almost forced his late uncle, Olympic bobsledding great Steven Holcomb, into early, medical retirement.
Raife’s glasses have thick, smudged lenses and slightly crooked frames, the mechanical casualty of chronically itchy eyes that are a hallmark of keratoconus, a progressive weakening and bulging of the cornea that corrupts how the eye focuses light.
“He rubs and rubs and doesn’t take the glasses off to rub his eyes, so his glasses get all bent,” said Raife’s mom, Megan Holcomb. “I don’t even know how he sees through them, they’re so fingerprinted.”
“I got hit by a door at a friend’s house. I don’t rub my eyes that much anymore,” said Raife, whose lifestyle — trampolines, wrestling, being 14 — is not conducive to the maintenance of pristine specs.
Contact lenses aren’t an option, due to the condition’s effect on the shape and fragility of Raife’s corneas. A competitive wrestler, he keeps his glasses on until the moment before he walks onto the mat; mom’s there to hand them back as soon as he steps off.
It’s not like he’s got a choice.
In five years, Raife has gone from being a third-grader who didn’t wear glasses to an up-and-coming Cheyenne Mountain freshman who cannot function without them. Uncorrected, vision in his right eye is 20/200, meaning he can read at 20 feet what those with normal vision can discern at 200. His left eye is 20/400.
“It’s to the point that he can’t see anything without his glasses,” Megan said. “Could you do anything without them?”
“I could walk,” Raife said.
Probably into a wall, though.
Raife’s vision was fine throughout early childhood, but by fourth grade he was having trouble keeping up with classroom lessons because he couldn’t see the board.
“Every six months or so after that he would have trouble seeing again and need a new prescription,” Megan said. “There have been a few times we’ve been in and it’s ‘Woah, his prescription is that far off? Again?’”
At Raife’s most recent appointment in March, Megan had to fight back tears as, with his glasses on, he squinted at the chart and stumbled over letters he’d seen clearly last time they were in.
The disease that had nearly derailed her brother’s legendary athletic career, and life, was coming for her son with a vengeance.
Family stalked by blindness
For generations, the specter of blindness has stalked the family of three-time Olympic medalist Steven Holcomb.
His mother, Jean Schaefer, remembers her grandmother’s advice about always returning every item in the home to the same place “so that when you are blind you’ll know where they are,” said Schaefer, of Colorado Springs.
Today, she knows that specter’s name: Keratoconus. In severe cases, visual acuity can’t be fully restored even with glasses or contact lenses.
The condition is estimated to affect one in every 2,000 people, but numbers are likely much higher.
“The other day, I counted up six people in the family — including me and Steven — with keratoconus,” said Schaefer, adding that there likely are other relatives she’s unaware of, who may not know themselves.
Treatment typically, historically, involves a cornea transplant, with doctors preferring to wait until a patient reaches an age where the benefits of such surgery outweigh the downsides — both the medical risks and the lifestyle adjustments, and sacrifices, required to maintain long-term eye health. That usually happens around middle age, when the cornea can begin to stabilize to a certain degree on its own. Until then, it continues to weaken and bulge out, causing increasing blurring, distortion and greater challenges in navigating day to day life.
Eventually, “there’s a dramatic reduction of what you can see,” said Los Angeles-based keratoconus expert Dr. Brian Boxer Wachler, who pioneered a nonsurgical way to stop the progression of the disease. “Keratoconus is like being in a fun house, but it’s not any fun at all. Treatment is the only option if they don’t want to end up losing sight.”
The sooner it’s diagnosed, and addressed, the better. When and if keratoconus manifests, and the pace at which it progresses, however, can vary greatly even among close relatives. And though the technology to scan for telltale signs is widely available — and most likely on hand in your local eye care clinic — such topographical screenings still are not a routine part of most childhood eye exams.
Because of the family’s history and familiarity with the disease, however, keratoconus was identified early in Raife.
“We knew at that point that it could be hereditary,” Megan said. “And Steven was, like, you need to have him checked for it. So we did.”
Teammates’ lives at stake
Keratoconus came for Steven Holcomb as his star was rising on the competitive bobsled circuit, after Lasik to correct his nearsightedness triggered a rapid worsening in his vision ahead of the 2000-01 season.
“My eyesight kept getting worse by the month,” wrote Steven in his 2012 autobiography, “But Now I See: My Journey from Blindness to Olympic Gold.” He decided to keep the condition to himself, get progressively stronger contacts and “tell everybody about the disease when I finally retired.”
But as the driver of a 1,400-pound bullet, barreling down a switchback course at more than 90 mph, he knew his teammates’ lives were in his hands. The secret ultimately became too much. Steven revealed the diagnosis and was facing forced retirement when the U.S. Olympic Committee referred him to Jeanne Derber, a Colorado Springs provider and the optometric consultant for the Olympic Training Center since 1988.
“He was extremely anxious and very concerned about his future in sports,” Derber said. “The corneal ectasia caused by the Lasik had caused the keratoconus to worsen, and every doctor he’d talked to had told him he needed a cornea transplant.”
A handful of specialists were performing an alternative treatment that could stop the advance of the disease through cornea-collagen crosslinking using riboflavin, a B-complex vitamin that, when activated by ultraviolet light, could be used to create natural rebar-like reinforcements for the collagen fibers that form the structure of the cornea, making them both thicker and stronger. The technique being used, however, required scraping the cornea, which risked infection and scarring.
In 2003, Boxer Wachler developed a noninvasive version that could be done in about 30 minutes, with a one day recovery time. He said his procedure originally was met with skepticism by the medical establishment when he unveiled it at a TEDx talk.
“I was expecting a standing ovation and got just the opposite,” he said. “I had doctors coming up to me after, saying ‘I don’t believe your data’ and, ‘just keep doing cornea transplants.’ ”
Saving Holcomb’s career
Cornea transplant surgery wasn’t an option for Steven Holcomb if he wanted to keep pursuing his dreams.
“A cornea transplant would have put him out of the sport. On top of the six months of recovery for each eye, he wouldn’t have been able to withstand the trauma of being in a bobsled,” Boxer Wachler said. “It’s like being a peanut in a bag and someone’s violently shaking the bag. It’s brutal. That’s why they can only take two, three runs max a day, because of the trauma.”
Steven underwent Boxer Wachler’s procedure in 2008. It revived his Olympic aspirations and pulled him from a near-fatal depression that had taken root in the darkness as his sight dimmed. Two years later, the then 29-year-old led Team USA to its first gold in the four-man bobsled event in more than 60 years at the 2010 Vancouver Winter Games. He then went on to earn bronze in the two- and four-man bobsled events at the 2014 Sochi Games.
“When Steven won gold, this ripple got sent throughout the world … basically the procedure had a gold medal behind it. That silenced most of the critics at that point,” said Boxer Wachler, who says his procedure has an overall success rate of 99.3 percent. “It’s sort of a twisted thing. I saved Steven’s career, but in a way he saved mine, too.”
Now, it was time to save others.
Steven joined “Dr. Brian” in promoting the procedure, and keratoconus awareness, on TV shows such as “Dr. Phil,” and “The Doctors.” He wove it into interviews about his Olympic feats, at every opportunity.
“He recognized what it had done for him, and he now had a platform to help other people,” said Boxer Wachler, who renamed the procedure to honor his patient, and friend.
When Steven learned his nephew also suffered from keratoconus, the two talked about what undergoing the Holcomb C3-R was like.
“Raife was scared, and wanted to make sure he wasn’t going to go blind,” Megan said.
Steven told his nephew not to worry; the procedure was painless and quick, and if Raife wanted, he’d be by his side at Boxer Wachler’s clinic when he had it done.
The first time Raife met the eye doctor who would save his sight was at Steven’s funeral.
In May 2017, as he prepared to compete in his fourth Olympic games, in PyeongChang, 37-year-old Steven Holcomb died unexpectedly in his sleep at the Olympic Training Center in Lake Placid, N.Y. A toxicology report released by USA Bobsled and Skeleton the following month found a “fatal combination” of alcohol and prescription sleeping pills in his system.
Though Steven had battled depression before, his family believes his death was accidental.
He had overcome his greatest physical challenge — his vision — was deep into training and entering his final Olympics as a top medal contender. He had plans, including a surprise visit to his mom in the Springs, where he lived during the off-season.
He’d also made a promise to his nephew.
Holcomb C3-R succeeds
In late June, Raife and Megan traveled from Colorado Springs to Los Angeles, to the offices of Dr. Boxer Wachler, where Raife underwent the revolutionary procedure that bears his uncle’s name.
“It was shocking how fast he was in and out,” said Megan, who during the three-day trip also joined her son and Dr. Brian for a taping of “The Doctors” that will air on CBS this fall.
“They just numbed my eyes with drops and painted this stuff on them,” Raife said. “It wasn’t bad.”
Uncle Steven was right.
“We were hoping to walk out of there not needing glasses at all, like Steven did, but that didn’t happen,” Megan said. “Now we’re here for the next step, to see if he can get contacts. I just hope his prescription hasn’t changed again.”
In mid-August, Raife and Megan were at Jeanne Derber’s office for a contact lens fitting, now that Raife’s corneas had been reinforced and the keratoconus arrested.
In the exam room, the technician helped Raife insert the dime-sized lenses in his eyes.
How do they feel? she asked.
“OK. Funny,” said Raife, blinking heavily as Derber killed the lights and began the eye test.
Megan watched with bated breath as he began reading from the chart.
When he completed the 20/25 line, with only one mistake, she let out a cheer.
“C’mon, Raife,” Megan said, trying to coax a smile, a laugh, a response, from her stoical son. “This is the moment you’ve been waiting for. You’re allowed to be happy.”
Raife didn’t cave then, but as soon as he got a chance he fired off a quick text to his two best friends, to let them know the exciting news.
As they left Derber’s office that day, it was the first time in years that Raife had walked so far without a window of plastic between him and the world.
Without his glasses, the ghost of a mustache riding his upper lip seemed more pronounced.
“Everything looks weird,” he said.
“You’ll get used to it,” Megan said.
With the keratoconus addressed, she hopes her son can now focus on other challenges — being a high school freshman, being 14, growing up.
“How do your eyes feel now?” she asked, once they’d reached the parking lot, handing over her keys so her son could get in the car while she finished her conversation.
Raife shrugged and jiggled the keys, contemplating.
“Can I drive?” he finally asked, the corners of his mouth trembling as he fought the urge to smile, and lost.