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.

Notes from Germany, Day Two

By Laura Edwards
Mom sent me her latest update around 2 a.m. her time after her very full first day in Germany. She talked with several families, including one “new” one – a father (whose son was diagnosed just a few weeks ago) and his father-in-law. The little boy’s father and I have already been in touch via email. There is another family in Hamburg who, like us, started a new non-profit organization to fight the disease, and yet another father whose son, like Taylor, was in the Portland trial.
I accompanied Mom to the last NCL Congress when it was in Rochester, NY, two years ago. It is a very high-level, very technical conference. Few families attend, because the presentations are clearly geared toward experts. I think I finally began to truly understand how much our lives had changed forever when I, an English major, sat with my mom, a music major, in a hotel banquet hall far from home, listening to a scientist from Washington University School of Medicine discuss the systemic and metabolic abnormalities associated with infantile neuronal ceroid lipofuscinosis (INCL).
That’s part of the battle, though. And thanks to BDSRA and Google and the birth and continued development of Taylor’s Tale and the reality of living with this disease each and every day, we understand so much more than we did two years ago. And we are not the only ones whose understanding is developing, becoming fuller, stronger. Progress is being made.
Dr. Sandy Hofmann, whose INCL project Taylor’s Tale has funded (through BDSRA) since that same summer of 2007, was on the schedule today and announced that she is ready for preclinical testing. That’s exciting news! Mom caught up with her briefly afterward and planned to talk with her in more detail later, so I hope to share more insight into this next phase – and how we can help make it happen – in tomorrow night’s post.
Dr. Robert Steiner, lead investigator for the historic trial of which Taylor was the sixth and final participant, arrived in Hamburg today. Results from the Phase I trial will be presented on Saturday – a moment I know my mom both craves and fears, if only for the reason that it is so intensely personal for us.
Tomorrow’s program includes topics such as “A Study of CLN3 Function in Mouse Brain Endothelial Cells” and “Mechanisms of Neurodegeneration in Late Infantile Neuronal Ceroid Lipofuscinosis CLN6.” The music major will be there.

The Quilt

By Laura Edwards

Mom is back from San Diego; she arrived on the red eye this morning. I have many things to report from her journey out west and will do so in time. Tonight, though, I just want to share some reflections from her time with Daniel Kerner, who is nine years old and has late infantile NCL, and Daniel’s mother, Joanna. Together, Daniel and Taylor form the bookends of the historic clinical trial in Portland, OR. Daniel was number one; Taylor was the sixth and final participant. Joanna and my mom have talked with one another countless times across cyberspace and telephone wires, forever connected by their children’s at once great hope and great sacrifice that was their experience in Portland, but they had never met in person. Since the day I was first contacted by another family stricken by Batten disease, as ours is, I have struggled to put words to the relationship that is forged between us all, even if the road between us stretches thousands of miles, as in the case of the Kings and the Kerners. However, Joanna’s most recent CaringBridge journal post recounting the time she and Daniel spent with my mom described it beautifully, so I emailed Joanna and asked her if she would share her words with me. She said yes, so I’ll share them with you now:

“Meeting Sharon King, Taylor’s mom, for the first time was immediately comfortable and welcoming. There was no way that I would not bring Daniel to meet Sharon. We exchanged gifts for the kids; Taylor sent Daniel a UNC baseball cap signed by the Women’s Basketball coach Sylvia Hatchell, and Daniel gave Taylor his newly edited Braille book entitled Love. Someday, Daniel and Taylor will have an opportunity to meet in person. Until then, they will have a coast-to-coast friendship through their moms. There is a special bond the moms share that is on a level deeper than any peer friendships. We are woven together into a different cloth of life that creates a strong and compassionate quilt, assembled painfully through heartbreak and upheaval, grief and acceptance, strength and perseverance. A quilt, we hope, big and strong enough to smother the dragon and deliver our children back to their childhood dreams.”

As I write these words from my living room on the East Coast, Daniel is on the West Coast, probably sharing the company of his parents and his older brother and sister, perhaps eating dinner with them, no doubt making happy memories (because they, with the exception of our constant search for the cure, are the central purpose of every day we are given). Thousands of miles away, I am searching for answers, in the world out there and within my heart. I am fighting for Taylor. I am fighting for Daniel.