The days are long, but the years are short. Never has this seemed more true than for our eleven-year-old son Noah.
While we have focused on loving and caring for our children, we have spent most of Noah’s short life researching, fundraising, collaborating, and searching for a cure. And even as we face the harsh reality that a cure may not come soon enough for Noah, we have plunged ahead with the many changes that life has demanded.
Our home is now center stage to a juggling act of nurses, medications, emergency room visits, and all-night vigils when Noah or Laine can't sleep because of this ruthless brain disease. Today, Noah has a feeding tube, is confined to a wheelchair, and does not talk. We were thrilled in the fall of 2011 when he seemed to communicate, “Yes” and “No” by blinking his eyes. But it didn't last long. It was devastating when he stopped smiling.
Every day is a struggle. We carry Noah back and forth to his wheelchair, fix his feeding tube when he pulls it out, and gently wipe his mouth throughout the day. It is devastating to watch your child lose skills, function, and independence and not be able to do a thing about it. Yet even while Noah’s body deteriorates, his spirit continues to inspire us.
His eyes light up when he sees his sisters, particularly little Laine with whom he has developed a special relationship. His body twitches with glee when we play the movie Cars and Lightning McQueen speaks. And when he has a fit of frustration, he relaxes and looks up at us with trusting love when we hold him in his room.
We don't know exactly what tomorrow will hold, but we have seen glimpses of the unbearable challenges in our future. The disease is ruthless. At this time it is fatal, usually between the ages of eight and 12. Each day is long for our eleven-year-old son, but we treasure it. For the years ahead will be far too short.
Life wasn't always this way. Noah developed normally as an infant and toddler. He talked and giggled, loved watching trains and wrestling with Daddy, and was always on hand to get into a little silly mischief. He loved baseball, and as he grinned under his toddler baseball cap we used to dream about him becoming a pitcher for the Chicago Cubs.
When Noah turned three, we noticed that he exhibited a speech delay and began having tremors. Our first neurologist said that these symptoms were normal for a boy, and that he should grow out of them. Months later, Noah collapsed and stopped breathing. We had no idea what had happened, but the doctors told us it was probably a seizure. At the time, it was the most frightening day of our lives.
A couple of weeks later, Noah had another seizure and was diagnosed with childhood epilepsy. About nine months after the epilepsy diagnosis, an MRI scan revealed some atrophy of his brain, but no cause. We also noticed Noah's speech began regressing, and he had difficulty performing simple activities such as eating with a fork and brushing his teeth.
Duke Children's Hospital Sheds Some Light
After several months, a friend of the family put us in touch with Duke Children's Hospital. Within weeks, Jen, Grandma Jacque, and Noah drove 850 miles to Duke University for a two-week evaluation. The team ran every test imaginable to determine the cause of his seizures.
While at Duke, Noah started the Ketogenic Diet, a special regimen to control his seizures. We were thrilled when Noah responded very well to the diet. His seizures were reduced dramatically and the fog around him seemed to lift. Noah, Jen, and Grandma Jacque returned home after two weeks and Noah was doing great… we were getting our little boy back.
News No Parent Wants To Hear
On March 17, 2009, our lives changed in a way no one could imagine. As we were sitting down for our St. Patrick's Day dinner, we received a call from Noah's neurologist at Duke Children's Hospital. Two of the tests which had been sent to separate labs came back confirming the same diagnosis. Noah was diagnosed with Late Infantile Neuronal Ceroid Lipofuscinosis (LINCL), often referred to as Batten disease. Over the next few hours as we researched the disease, our world quickly crumbled. It was never the same again.
We Will Not Stop Fighting
Today we live in close contact with families around the world who also have children with LINCL-Batten disease. We have found great comfort in our new extended “family,” and rely daily on the amazing outpouring of love, prayers, and support that we have received from our family, friends, community, and supporters around the world.
Noah's Hope, and ours, is that researchers will soon discover a cure for this devastating disease. While we focus on loving and nurturing our children, we continue to fight tirelessly to make Noah’s Hope a reality. Together we can make a difference for the future.
Every childhood disease deserves a cure.
--Jennifer and Tracy VanHoutan (Parents of Noah, Laine, and Emily)