To Infinity and Beyond

By Laura Edwards

Daniel skiing at Alpine MeadowsDaniel Kerner always loved the outdoors. He loved to visit the ocean and mountains and feel the warmth of the sun on his face and the wind in his hair. He loved his family and friends. He loved life.

Daniel became an angel on April 12, 2010 – two years ago tomorrow.

I never met Daniel, but my parents and Taylor met him on several occasions, and I’ve shared his story before. Daniel and my sister will always be linked on paper because they formed the bookends of a historic clinical trial in Portland, OR (Daniel came first; Taylor went last). But Daniel’s story means much more than that. And his courage had – and continues to have – an incredible impact on me.

It’s difficult to believe that it’s been two full years since Daniel earned his wings. In a world ruled by Batten disease, it’s easy to lose track of time – and children. So many children are lost (and eventually, all of them; Batten disease is universally fatal). So many children whose hands I’ve held or stories I’ve hugged close to my heart.

It’s amazing how time passes; amazing how the world manages to keep chugging right along after the premature loss of an angel like Daniel – without even missing a beat. But no matter how many anniversaries come and go or how many children we lose, we must never forget. And we must never stop fighting.

I’ll go to infinity and beyond, Daniel – whatever it takes – to beat this monster for angels like you.

I wrote a letter to Daniel on April 13, 2010 – the day after his passing. Read it here.


The Bell Still Rings for T

By Laura Edwards
Taylor and Santa, 2003

Taylor and Santa share a moment in 2003

“At one time most of my friends could hear the bell, but as years passed it fell silent for all of them. Even Sarah found that one Christmas she could no longer hear its sweet sound. Though I’ve grown old the bell still rings for me, as it does for all those who truly believe.” 

-Chris Van Allsburg, The Polar Express

This holiday season is our sixth since doctors discovered that my little sister, Taylor, has infantile Batten disease. This is my 42nd post in 2011. It’s been a packed, whirlwind kind of year – for the rare disease and Batten disease communities…for my family…for me. And now, just 10 days shy of 2012, I’m exhausted; fresh out of inspiration for this blog.

When I’m wracked with writer’s block, like tonight, I’ll often go back and reread some of my five years’ worth of combined posts on my old blog, Transmissions, and this one for inspiration. I’ve found each year of our unexpected journey to be different than its predecessor, and my own words, read through the lens of increased experience, can be striking. But at the same time, little has changed…because after all, we’re still traveling the same cursed road, looking for an exit sign, hoping we’ll find it in time for T.

The following appeared on my original blog, Transmissions, on Dec. 31, 2008.

Reflections

There are about five hours (ten days) remaining, on the east coast at least, in 2008 (2011).

I hate New Year’s resolutions and rarely make them. Few of the ones I’ve ever made have lived to see February, and I say, why do we need a new year as an excuse to make a commitment to something important in our lives? Most New Year’s resolutions are hokey or clichéd, like losing weight. The only diet I ever really stuck to was the one I was forced to undergo six weeks before my wedding, when I realized on the day of my portrait shoot that my mother was a beanpole when she got married, and that I’d better starve myself if I hoped to wear her wedding dress for a full six hours (the hour on the front porch of Cone Manor that day was too much).

2008 (The span from 2008-2011) had its high points, but I’m ready to let it go. I began the year  (four-year period) on a cross-country flight to Portland, Oregon, where my sister was slated to be the sixth and final participant in an experimental study that involved the complete shaving of the full head of hair she loved, the drilling of eight boreholes in her skull and a harrowing seven-hour brain surgery during which my family and I sat huddled in the waiting room of the children’s hospital, our fingers crossed and our hearts in our throats. Taylor’s acceptance into the study was a miracle – the decisive phone call from Portland the month prior left us in happy tears – but her participation in it was the scariest thing any of us had ever experienced. Our time in Oregon, like Taylor’s spot in the trial, was a contradiction. We slept little, and I will never forget the way Taylor looked in the recovery room following her surgery. If I had ever for a moment doubted that my sister was sick, I was sure of it then. On the other hand, we couldn’t all stay in her room around the clock, so my husband and my brother and I had the opportunity to see some of one of the most beautiful areas of our country. As nerve-wracking as that week was, I made some great memories on the road to Mount Hood, in the Columbia River Gorge and on the beaches of coastal Oregon. And on the plane ride home, John and I each said what we had both been thinking: Taylor had likely just been given the best chance of survival that any child with Batten Disease has ever been given.

Though the rest of the year (following four years) unfolded more quickly than I could record it (them), those eight days in Portland will stand still in time forever. I still feel as though someone else lived them in my body as I watched from a distance, invisible and silent to the world around me. These last 12 months (five years and five months) since the diagnosis, I’ve watched the disease take bits and pieces of my sister that it didn’t already have, but I’ve also watched her learn the Braille alphabet and heard her laugh, again and again. I’ve put blood, sweat and tears into fundraising and building awareness – and it has never been easy – but I’ve had the honor of helping start a non-profit named for my sister, and as much as I fret over the talks I give at our events – as shy as ever – I’ve seen the tears in people’s eyes and felt the warmth in their hands and their hugs after I’ve finished. I’ve watched my family continue to bond and rally around this cause, but I’ve been there on the worst days, too – the days the reality of Taylor’s disease hits us hard in the face. I’ve been there on the days that even when it seemed as though it couldn’t get any worse – as we’ve realized once again that Taylor has a disease no child has ever survived – another family member had a heart attack, or suffered a bad fall, or just went through a hard time. On the night one of my mom’s brothers had a heart attack, I asked her other brother what was happening, and he simply replied, “The sky is falling.” And right then, it was. But before the sky crashed down on us, we came together, and put up our hands, and with all of our might, we pushed the sky right back up again to where it belonged. And the next morning, the sun rose in that sky.

I’m not just anxious about 2009 (2012)…I’m terrified. I’m not lost on the fact that Taylor has a degenerative disease, and that until she is cured, she will only continue to decline until there is nothing left of the sister I love. In between my nightmares, though, are the good dreams – the ones where the clouds have parted, and the shades over her eyes have been lifted, and she can see once again, and the disease eating away at her has been banished forever. I know that for every good day, there may be a bad one, and vice versa. Tomorrow we may feel lost, but the next day we may only BELIEVE. The harder it rains one day, the brighter the sun shines the next. I can’t live thinking there won’t be more good news, lots of good news. I have to see the good in the bad. I have to BELIEVE that we will find the cure. And I do.

So bring it on, 2009 (2012). I know there is good to be had in your days. I know there will be tears and frustrations. I know there will be setbacks, but there will also be progress. We have nowhere to go but up. And I’m going to keep on jumping.

Nearly three years after writing that post – true to my word – I’m still jumping. My landings aren’t always that soft, and the bell doesn’t ring quite as clearly as it once did, but we are still here, fighting, believing. And so the bell still rings for T.

Happy holidays to all of our supporters. Thank you for helping us believe.


Notes From Germany, Day Four

By Laura Edwards

I just read my mom’s latest CaringBridge entry and pondered how to summarize her words here for my last Notes from Germany post before realizing I could never tell the story as well as she has. The conference is over, and Mom now has only to make her way back over the Atlantic and home to Charlotte. Usually, the words on this page are mine. The ones that follow now are hers. 

Good morning!  Well, it is already Sunday morning in Hamburg, Germany – early evening in Charlotte.  I have been in Hamburg since Wednesday morning for the International NCL (Batten disease) Congress.  The Congress is held every two years and alternates between the US and Europe.  I have met and talked with researchers from the US, UK, Belgium, Finland, Japan, Denmark, Germany…during the first lunch meeting, the woman sitting next to me was from Moscow.  We struggled with language but managed to communicate just the same. 
 
There is a good deal of interesting research happening, but nothing that is close to being our miracle.  I admit, it has been a difficult week to keep a smile on my face. Then, I pick myself up, paste on that smile and take myself right back out there.  Don’t misunderstand – there is good work being done, and it needs to keep going.  It is just not happening within the next year, and I want help NOW.  

There are several research projects specificall
y for INCL (T’s form of the disease) that need funding or continued funding.  Most importantly, Dr. Hofmann (Taylor’s Tale has funded her work for the past two years) will need a third year of funding.  She was the second presenter at this conference and announced that she is ready for preclinical trials.  She has made the enzyme and must now determine a delivery method to the brain.  You can’t imagine the joy and pride it gave me to hear that information…and the gratitude I feel for our friends and family who have made it possible for Taylor’s Tale to provide the support.

During the final session of this conference,  I heard the first report from the Portland trial.  The presenter arrived yesterday and was genuinely shocked to find that Child Number Four’s dad and I were attending this conference.  “I didn’t think parents would come this far.”  I wanted to say, “Come on, I’ll go to the ends of the earth for this child. I did the trial, isn’t that proof enough?”  He shared the data with us over breakfast before the public unveiling.  There was nothing shocking.  He was able to share, however, that there is absolute proof that the treatment T and the other five children received shows great promise.  Unfortunately, they were able to get that proof because of the death of one of the children.  Signing on to the trial was one of the most frightening times of my life.  I have no regrets.  I don’t think it is the answer at this time, but I believe it has helped in some ways and given us important time. This research must continue.  There are so many people to thank for the opportunity we had to take part in the trial – the sponsor, the team at the hospital in Portland, our friends and family who kept their hearts wrapped around us, and of course, the families of the five children who bravely went before Taylor.  I have always felt that my family expanded to include the other trial families the day T had her surgery.  It is a bond that will always exist.

I hear that Hamburg is a beautiful city.  I didn’t come as a tourist – this was a mission.  I’ve seen nothing more than the subway station (my luggage took an extended visit in London as I headed on to Hamburg, so I decided to save taxi fare and took public transportation), the University and my hotel.  This was a free afternoon, so I grabbed my book and headed down for tea.  I stayed in my corner of the cafe for three hours – the most relaxed I’ve been in months. You might be wondering why I didn’t walk around the block.  It is COLD, and I brought spring clothes.  People are wearing wool coats and sweaters!

Taylor had a big week while I’ve been here.  She finished up the fifth grade.  Laura sent the cutest picture of T and her friends at the moving up ceremony.  Thank goodness for the Internet!  Also, Jim and T went back to Duke and left with a prescription for a new drug therapy that we want to try.  The hope is that it will help provide stability while we wait for that miracle.  We’ll keep doing all that we can to keep our girl as healthy as possible while the researchers do their work.

The dad and granddad of a newly diagnosed five-year-old boy, Noah, attended the conference.  The sadness, fear, frustration and yes, anger, is so apparent.  My heart just ached for them.  Those feelings don’t go away with time; you just learn to control them a bit.  Please support Taylor’s Tale and our research fund.  There is good science in the works, but it will need support to get to the clinic…I know that it can get there!  Noah, Taylor and so many others are counting on us.

I look forward to getting back to Charlotte tomorrow night. As always, thanks for helping me believe.  Miracles happen every day.

Love, Sharon


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.