Taylor’s Story Featured in Friday FITspiration Series

By Laura Edwards

Head over to www.runladylike.com, where Jesica – an ordinary runner on a mission to find her extraordinary – shares her “uncensored and often unladylike adventures of running and triathlon training.” Today, she featured Taylor’s incredible story of courage and my plan to run a half marathon blindfolded in her honor on her “Friday FITspiration” series.

I’m grateful to Jesica for sharing our story in such a beautiful way. I’m grateful we connected, and I can’t wait to continue following her running adventures. Later today, she’ll start a journey of her own as she takes part in the famous Hood to Coast race in Oregon, a state that enchanted me with its beauty when I visited it during the week of Taylor’s surgery 11 months before that first 5K in 2008. Best of luck to Jesica, and happy trails to all of my running readers! Don’t forget, you can join the Taylor’s Tale team at Thunder Road on Nov. 16. We’d love to have you!

Read the story

When the Eruption is Over

By Laura Edwards

It is not easy to paint a picture of Batten disease for people who have never seen it before. If you wish to paint with a large brush, you can tell them that children born with Batten disease never survive it; that it is total destruction; like a molten wave of lava and volcanic rock and ash.

Batten disease is not easy for most people to understand, but everyone can see that Taylor is blind. The destruction reached her eyes first. They are still beautiful and rare, the color of the caramel inside a Milky Way bar and framed by impossibly long lashes. But they lost their sparkle long ago.

Mom and Taylor at Crater LakeFive years ago this past January, a clinical trial coordinator brought my sister’s honey blonde hair to us in a Ziploc bag as we waited in a chilly waiting room in Oregon, thousands of miles from our home on the East Coast. Down the hall, a surgeon drilled eight holes into my sister’s skull and gave her hope. Not life, but hope.

For the next several years, my parents and Taylor made frequent return trips to Oregon. Once, they rented a car and drove south to Crater Lake, one of our country’s pristine natural wonders.

More than half a century ago, Freeman Tilden, said to be the grandfather of park interpretation, wrote a text, “Interpreting our Heritage,” that is still used to educate rangers today. One of the essays holds special meaning for us.

The essay, “That Elderly Schoolma’am Nature,” tells the story of a park naturalist meeting a man just inside the rim of Crater Lake. The naturalist can sense that something is different about the visitor from the moment he sees him but only comes to the determination that he is blind after noticing the man’s very dark glasses and putting all of the clues together.

And then, the visitor asks the naturalist to describe the lake to him. But how do you describe one of the world’s most stunning lakes to a man who cannot see?

The naturalist asks the man to take off his gloves, so that he can take his hands and move them around the crater model and describe its shape and depth and skyline and the curious, cone-shaped island in the middle. But how do you describe the blue of its water – a blue that has no equal – to someone who has not seen blue in many long years?

The visitor remembers the blue of the sky from his childhood. The blue of Crater Lake is nothing like the blue of the sky. But in his mind and his heart, he experiences the wonders of Crater Lake more fully than the naturalist could have ever imagined. And as he walks away, the naturalist realizes that the visitor “had extended his power of seeing – which was an achievement beyond price,” and that “We are all of us somewhat blind, even those who believe their eyesight is faultless.”

I love so many things about this story. My dad, who shares my love of national parks, gave a copy of it to Mom and me several years ago, and I’ve held onto it ever since. And my wise mother, who now leads Taylor’s Tale into an exciting future of new partnerships and boundless possibilities, shared Tilden’s essay with me again this week, urging me to apply the story toward our journey in our fight against Batten disease.

“We are all of us somewhat blind, even those who believe their eyesight is faultless.”

Because, as Mom reminded me, we embarked on this journey with our eyes focused directly on beating Batten disease. Our experience, though, has afforded us peripheral vision. We now understand the connection between all rare diseases – so many of which do not have a single approved treatment – and the millions battling for their lives. And just as the park naturalist and the blind man learned from one another, we, as fellow fighters and advocates, can learn from each other and support each other – and by doing so, we can become more efficient and effective. We will never reach our goals if we fight our battles in our own disease silos.

There is a very rare disorder called giant axonal neuropathy, or GAN. It is an inherited, recessive disease that first appears in early childhood. It results in nerve death and quadriplegia, and it is always fatal. The incidence is unknown, but it probably affects fewer than 100 people in the world.

Undeterred by these odds, the family of a little girl named Hannah decided to fight GAN head-on. In  2008, Lori and Matt Sames founded Hannah’s Hope Fund in their daughter’s honor. That same year, Hannah’s Hope began funding gene therapy for GAN at the University of North Carolina Gene Therapy Center under Dr. Steven Gray. The clinical trial is expected to begin later this year.

Two months ago, Taylor’s Tale and five partners announced funding for gene therapy for infantile and late infantile Batten disease at UNC, also under Dr. Gray. Our project is following in the footsteps of the GAN work that is on the brink of clinical trial. And Lori’s fight for her daughter could very well help lead to a treatment for kids like my sister. Dr. Gray plans to use the same gene vector and methods to treat Batten disease. And if the GAN trial is successful, we will, in Lori’s words, “move like wildfire to apply this to the lives of children with Batten.”

“…move like wildfire to apply this to the lives of children with Batten.”

Together, we can reach our goals. There are so many of us in the rare disease community, and there is strength in numbers. That is an advantage, but it is also the saddest thing of all. There are TOO MANY of us. We’ll change that when we play to our strength by finding treatments for people like Hannah and Taylor.

I think about how the landscape in central Oregon must have looked on the day of the volcanic eruption that created the most beautiful lake in the world. I know that many dedicated people, from the team at UNC to those working to ensure that their science is supported, will move like wildfire to outrun the death and destruction of diseases like Batten and GAN.

And every night, when another day’s work is done, I dream about how beautiful the lake can be when the eruption is over.

Unexpected Angels

By Laura Edwards

A few weeks ago, Taylor and my parents flew about 3,000 miles from our hometown of Charlotte, NC, to Portland, OR, the misty city guarded by towering evergreens where my sister had brain surgery in a hospital on a hill five years ago.

My sister isn’t the same chatty, bouncy girl whose golden locks we received in a Ziploc bag in the family waiting room moments after they wheeled her back for surgery on that cold, gray morning in January 2008. The cross-country trips to Oregon for follow-up care have grown more difficult with each passing year.

For this, their last scheduled visit of the five-year follow-up study, my family had a connecting flight in Phoenix, AZ. My parents had several large carry-on bags to manage in addition to my sister. They had just minutes to get to their connecting flight, scheduled to leave from a terminal on the opposite end of the airport. Mom later told me that as they struggled to make it with their bags and my sister, who can’t see or run, she couldn’t help but think that airports are difficult to navigate for people with disabilities and too much luggage.

Then, a man in a shirt and tie discovered their dilemma. Also from Charlotte, he was on his way to a business meeting. His flight left from a different terminal, but he carried several of my family’s bags and walked with them all the way to their gate. On the long journey, they learned that he has physical therapists and occupational therapists in the family and believes in people who help others overcome injuries and disabilities; but through all the frenzy, they never got his name. Mom told me that if he hadn’t helped them, they would have missed their connecting flight to Portland.

Montego BayWhen my parents finally got on the plane and inched down the aisle towards their seats, they realized that they’d have to maneuver my sister over an elderly woman seated alone. A grandmotherly type, she had a “Pooh bear” shape, chocolate skin and silver hair, and the eyes behind her thick glasses looked kind. She told Mom that after selling Avon makeup for 32 years, she’d finally won a trip to Jamaica. She didn’t have a person in the world, but she’d been waiting for that trip, so she booked her flight and her room on Montego Bay. When she went to apply for her first-ever passport, she told the lady behind the counter, “I’m going to die before I can use this again, so when I come home, can I get a partial refund?” After she told her story, she got quiet for a while. But later, Mom glanced over and saw the woman holding Taylor’s hand in hers. She looked up, and her eyes met Mom’s, and she told Mom she’d pray for her daughter.

The woman on the plane wore her Avon name tag for her first trip to the Caribbean; Mom told me she can picture it, but for the life of her, she can’t remember her name.

Fighting a monster like Batten disease day in and day out makes it easy to get caught up in your own problems. It makes it easy to miss the accidental moments and the unexpected angels.

But at the end of the day, aren’t those life’s most beautiful things?

Aren’t they all any of us can really hope to have – whether or not we’re facing a terminal disease?

When Time Stood Still

By Laura Edwards

Four years ago today, my sister, Taylor, became just the sixth person in the history of the world to undergo a neural stem cell transplant.

I will never forget the first time we got “the call” from the West Coast, informing us that Taylor had been accepted into the groundbreaking clinical trial. Nor will I forget the call that followed shortly afterward, informing us that the sponsor of the study had changed its mind.

Just two spots remained in the study. Taylor, whose estimated two percent PPT1 enzyme production delayed the onset of the infantile Batten disease fatally encoded in her genes and shocked the study team with her ability to not only walk and talk but also attend school, dance to music, answer the phone, learn Braille and maintain a sense of humor sharp enough to keep adults on their toes, was just too healthy for the phase one safety trial.

We all but gave up on spots five and six. Banking on Taylor’s minuscule enzyme production to buy us some extra time but knowing it wouldn’t save her forever, my parents searched every square inch of the medical world as they looked for possible miracles.

Then, out of the blue, another call came. And just a few short weeks later, in early January 2008, we boarded a plane for Portland, OR.

Mom lived in a Residence Inn within sight of the hospital for seven long weeks that winter while Taylor recovered, but the seven days I spent in Portland for the actual surgery felt like seven weeks. Throughout my entire life, I have never experienced anything quite like that week. Time stood still, and I felt as though I was watching another family that merely looked like mine, but wasn’t really mine. Still, there are a few singular moments that stand out to me – moments that inspired feelings so real, they are the only way I can be sure the week actually happened – the images of which will be forever burned into my memory.

Around 5:30 a.m., a few hours before Taylor’s surgery, I walked up behind her in our hotel suite. She sat watching (yes, at that time, seeing) her portable DVD player – a picture of normalcy framed by chaos. The first thought that popped into my head was how beautiful her hair looked. About two hours later, all of it would be shaved off in the operating room.

Not long after they’d wheeled Taylor back to the OR, the study coordinator walked out to the family waiting area and placed a Ziploc bag containing Taylor’s hair in my mother’s hands. It sounds like such a benign, forgettable thing, but it was one of the most powerful moments I have ever witnessed.

Following the surgery, we stood outside clear sliding doors as the nursing staff tried to place Taylor’s PICC line. Still drugged, Taylor nonetheless wasn’t in the mood to be poked and prodded. We felt badly for the nursing staff, but watching her give them a hard time also caused me to smile for the first time that day; seeing some of that spunk meant we had our Taylor back.

One night, we sent Mom and Dad back to the hotel so they could get some real sleep. Around 2:30 or 3, Taylor started crying; nothing we did consoled her. We called the night nurse, who arrived immediately, pulled up a chair and held Taylor’s hand until she stopped crying, her eyelids flickered, and she drifted into exhausted slumber. I will never forget that nurse. She had long, dark hair pulled back into a loose ponytail, was originally from eastern Washington and gave my little sister a night of peace.

Taylor at hospitalFour days after the surgery, Taylor’s care team told us she could go back to the hotel. I never imagined a little girl could be so happy after all she’d been through. We bundled her up in her cheetah print coat, pink hat, scarf, and boots and took her and her stuffed unicorn – her snuggle buddy for those difficult few days – on the wheelchair ride of her life. And as we exited the hospital, Taylor inhaled a deep breath of cold, damp air and smiled, as if to tell the world, I just made history. What else you got?

Years have passed since that singular moment in my sister’s short life, and still, Taylor continues to ask the world that same question. She’s taken a couple more hits along the way. She can’t see those movies on her portable DVD player anymore. She’s not doing Braille. But she still has one hell of a sense of humor.

I wish I could say that surgery in Portland saved Taylor’s life. Some people, in fact, actually think it did. They’ll ask me how she’s doing, and when I tell them, they’ll say, “But she had that surgery…”

That’s when I tell them what a phase one safety trial is. And, as if that isn’t enough of a knock, I’ll remind them that Batten disease is a monster.

leaving the hospital

When we received that deciding phone call in late 2007, Portland was the best shot Taylor had. We were desperate. We didn’t know if it would work, but we also didn’t know if we would ever get another shot. And when Taylor was finally accepted, we called it a gift. But if you want to know what I really thought then – and believe more than ever today – it’s this: Taylor, and the five other children in the trial, gave the greatest gifts of all. They endured an incredibly invasive brain surgery and intensive recovery and gave the gift of hope to millions with Parkinson’s disease, Alzheimer’s disease and other far more prevalent brain disorders who will very likely someday benefit. I will never forget that week I spent with my little sister in Portland, OR. And I don’t want the world – or the company that sponsored a particular phase one clinical trial – to ever forget the grand sacrifice of six very sick children.