High atop the tallest horse in the group, Megan Nighbor was so close to Wyoming she could feel it.
She had on summer clothes and her new Durango cowboy boots as a storm quickly moved over Custer, on the western edge of South Dakota.
This was the family’s first trip, in what seemed like forever, that didn’t involve Boston or a hospital. On this journey, everyone got to choose something they wanted to do. And for Megan, riding a horse in the West was her wish.
“One day we go and do it. And it storms, it’s lightning. Started hailing for a couple minutes,” she said, sitting next to family at their house in Dalton. “The horses were slipping because of all the rocks. And I’m in a tank top.”
She wasn’t about to let the cold August rain stop her fun, as Toby the horse moved slowly along.
“It was a really tall horse, and I was like, ‘Watch, I’m going to get the tallest horse,’ and I did,” she said. “(My sister) Michaela and I had like Clydesdales and (my parents) had regular horses.”
Normally with her small frame she gets the small horses, her mom, Sandy, said. And she doesn’t like that.
Megan made the ride almost the entire way, getting picked up by a Jeep with only a short distance left on the trail.
At 13 years old, Megan is like any other teenage girl, twirling blonde locks when she talks about playing in the school band, helping the dance club with routines and loving to be outdoors.
The only difference is Megan, who has progeria, is in the body of someone her grandparents’ age.
But that has never slowed her down. She is always on the go and rides horses whenever she can – once taking a lesson in Tennessee from a cowboy named Ryan who caught her eye.
“Oh, was that in Tennessee?” Megan asked her parents in her high voice as a giant smile came to her face. “I remember him. OH, MY, GOD!”
The skateboard flew down the road with Megan firmly planted on top riding next to her cousin Taylor.
They had found the old boards at a relative’s home, painted them orange, wrapped duct tape around the middle and penned words everywhere.
Her life in Dalton, about 20 minutes outside of Portage in Green Lake County, is as normal as can be. But starting this week, Megan’s story is part of the HBO documentary “Life According to Sam” that follows children with progeria, a rare disease that speeds up the aging process – roughly six times as fast.
When Megan was 6 months old, Sandy and Steve noticed she was falling behind in the growth pattern. Within the next year, it was obvious something was not right.
After visits to doctors, nothing was coming up. That is, until one doctor recalled a picture of the child who looked like Megan. The child had wrinkles, arthritis and no hair. The eyes were young, but the rest of the body wasn’t.
When Sandy called a picture up on a computer screen, the girl looked like Megan. And the word progeria was next to her, along with the fact there is no cure.
There are about 250 children that have progeria (103 who have been identified in 37 countries), and Megan is the only one in Wisconsin.
The family flew to the East Coast for testing that became available after the gene that causes progeria was found in 2003. Megan became a pioneer in finding treatments and a cure. And over the last decade, the drugs have had great results, helping reverse blood vessel changes that cause cardiovascular disease and hardening of the arteries. There’s also improvement in bone strength. While the average person with progeria lives only to 13, some live into their 20s. And there’s no telling how long a person with progeria can live with these new treatments.
“The one thing (Megan) always wanted out of the whole thing is a little hair to grow,” said Steve. “That didn’t happen.”
While medicine side effects have made her sick periodically, Megan doesn’t like to be home, away from her classmates at Markesan Middle School.
“I hate missing school,” she said. “Because I’m always afraid I won’t be able to catch up or I missed the big test.”
It’s common for her to miss 60 days in a school year, but that hasn’t stopped her from making the honor roll. She enjoys school and a little extra space with two lockers, but can’t quite reach the hooks to hang up her backpack. She also has lunch buddies who help out, and she loves to chat the noon hour away with them.
The first couple years of school were scary, Steve said, and they were a little cautious. With a June birthday, they decided to hold her back a year.
“She’s going to be the smallest all the time anyways, but she doesn’t need to be the youngest and smallest,” Steve said.
But the school years have gone well. Megan has friends who help her with things like carrying books, and like most teenagers she loves texting and is on the iPad all the time. She has her favorite classes, art and health, and ones she doesn’t love so much – like math.
“All the social media skills are there,” Steve said. And she has a lot of good friends who treat her like a sister.
“The teachers have been great,” he added. “Some we have gotten to know quite well.”
Steve, who is on the school board, said moving out of the district for another job has never been a consideration with school going so well.
“Everybody that knows Megan, she will put a smile on their face. Everyone loves seeing her,” he said. “And she goes to school, the high-schoolers are like, ‘Hey Megan,’ way down the hall.”
Marching to the beat
Steve sat down to watch the rest of “Life According to Sam” last week when Megan came over and said, “Start it from the beginning.”
“She shed a few tears the first 15 minutes,” Steve said. “How many times in that movie does (Sam’s father) mention 13 years old, that’s the average age of life.’ And there’s no cure. It’s not that Megan didn’t know any of this, but to hear this all again with the doctors, but she handled it well.”
They are the only ones in the Nighbor household who have watched the film, with others not quite ready to relive that.
In the movie, we meet Sam Berns who is a funny, easy-going high school junior who marches in the band, helps others in science and is on a first-name basis with Dave Matthews and his band mates.
He also is turning 17 today.
Sam was thrust into the spotlight when his parents, Drs. Leslie Gordon and Steve Burns, set out to find a treatment and cure for progeria, creating the Progeria Research Foundation. And in the film Sam puts the disease into perspective.
“I didn’t put myself in front of you to have you feel bad for me,” Sam says in the opening moments. “I put myself in front of you to let you know that you don’t need to feel bad for me. I want you to get to know me. This is my life. Progeria is part of it. It’s not a major part.”
The film follows Gordon’s first drug trial with 28 children, one of whom was Megan.
“We’ve been involved since ’03,” said Steve. “Megan has been a first for a lot of things.”
She was the first to start the drug trial in 2007, only four years after the gene was found that causes progeria. And she was one of the first to receive a new drug.
The filming took place over three years and shows the difficulties Gordon and her sister Audrey, a doctor who helps run the foundation, had trying to get the first drug approved by the FDA.
The drug lonafarnib reverses blood vessel changes that cause cardiovascular disease at such an early age. And the results were stunning -- and may help all of us as we age. Gordon says in the film that they are not sure this is the Fountain of Youth they are after, but the drug could help people with heart disease in the future.
“Visually, we see that (the drug) was doing some things. When Megan was 6 or 7, you could see some things starting to show. She had a lot of hip problems and some other bone problems. She hasn’t had problems (get any worse) since the first year (of the trial).”
“Life According to Sam” also will be watched by Megan’s classmates this week in school.
“Mom was like, ‘Are you sure you want to do this? You really want everyone to see this?” Megan said. “And I think this will help some of these kids understand better. Like why I have to do all this.”
Megan played with her guinea pig on the kitchen table, and petted her furry head.
She wasn’t doing so well when they got her, but Megan helped nurse her back to health with goat’s milk.
“We said a special prayer, didn’t we,” Sandy said. “So God would give her a little special help.”
The guinea pig had big feet, so Megan asked what was another name for Big Foot.
“And they said yeti or sasquatch,” Megan said. “So I’m going to name her Yeti.”
Animals are a big part of the Nighbor family: with two dogs, and Megan’s horses that are kept at an aunt’s home about 5 miles away.
Four years ago, Megan started asking for a puppy, and that was the only thing she had on her Christmas list. She was told to be at an address to see Santa then, and thought only one thing.
“Am I in trouble,” she said. She picked out a dog and named her Daisy Sue, who doesn’t leave Megan’s side when she is sick some days.
When she was first diagnosed with progeria, the family had to travel to the Boston Children’s Hospital every four months for a week, with the foundation raising money for these treatments.
The whole family tried to go each time, but that wasn’t always possible. Progeria is something they all have been going through. And when they had a few free moments, they even got to take in some sites -- like a rocky whale watching tour on a boat that left almost everyone on board vomiting, except Megan.
“Every time we go back out there, I ask everyone, ‘You sure you don’t want to go on a whale ride?’” Steve said with a laugh.
While the medication is helping, Megan must take several drugs, including a cholesterol drug and a growth hormone shot each day. “That’s a lot of stuff in a 32 pound body,” Steve said. “But she’s stretching upward. She is still growing.”
She’s in good shape, flexible and active, he said.
With each trip, they meet other parents and children with progeria, and are often paired together for the drug trials.
“All these kids are amazing,” Steve said. “Kids with affliction always seem to be above the average, amazing kids. I think that’s their way of compensating what is wrong with them.”
The family trips over the years were always medical related, or to help raise money for progeria research.
Steve said Dr. Gordon is trying to get another drug approved by the Food and Drug Administration. While the process is slow, the drug looks to be something that can be successful in treatment. The family is not scheduled to go back until August of next year.
“Scott, Audrey and Leslie, I don’t know where we would be without them,” Steve said. “What they have done in that (10-year) time frame is basically unheard of. But I’m sure for Leslie, it’s not fast enough.”
On to Wyoming
There are 29 days before Michaela Nighbor turns 16 and can get her driver’s license. And she’s counting every minute. And Josh, Megan’s oldest brother, lives not too far away.
The family goes about their lives not letting progeria slow them down. And neither does Megan. This summer she was at a friend’s house, or vice versa, all the time for sleepovers.
“Megan’s got a steady group of friends. Every weekend someone is here,” Sandy said.
“She gets tired, but they will be up in their room coloring, watching videos, just giggling. She’s even got some boys that want to come over.”
And with that, Megan gave her mom the 13-year-old, don’t-embarrass-me look. She even did the boyfriend thing, but broke that off.
While Michaela plays three sports, Megan can be found helping her practice at home, throwing up a volleyball for her to set, or guarding her playing basketball. And she tries to be there for most of the games.
“Mom was texting Michaela. You better tell the girls that Megan was sick last night and throwing up. And she’s coming (to the volleyball tournament) because she doesn’t want to miss it,” Megan said.
And the team won that tournament.
With sports, Megan is always asking her parents why she can’t be involved. “And then she says, ‘We don’t want you to get hurt. But you don’t know because you don’t let me try,” Megan said.
“Yeah, Mom’s usually more worried than Megan,” Steve said of sports.
“That’s what mom’s do,” Sandy said.
With each trip to Boston, they see the benefit in the drugs. And that makes the trip and a shot everyday worth everything, Steve said.
“I say, Megan, it’s up to you. Do you want to keep doing all this,” Steve said. “She says, ‘What choice do I have?’ The hope we have is through them. That’s what we have.”
Since they don’t have to go back to Boston anytime soon, Megan wants to head to Wyoming for their next trip, a place she’s always wanted to go because that’s where the biggest and best horse ranches are -- and where the cowboys live.
When she was in South Dakota, her dad never told her they were only a few miles away – knowing she would want to point her horse in that direction.
“We’re going back there,” Megan said looking at her dad. “We were right there. We got back, and he says, ‘You know how close we were to Wyoming?”
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