In the chill hours after she came into this world one short December day, they called Riley Hooper their “Dream Baby.”
While the other infants born around her screamed, filling the hospital hallways with wet, newborn cries, Riley, beautiful daughter of Jeff and Kelly, sweet little sister to brother Jordan and sister Kamryn—who was then still practically a baby herself—took in the world with big, saucer eyes and snuggled in, calm as “Silent Night.”
Soon, they called this baby girl “Smiley Riley.”
And the best probable future flowed out before the Hooper family like a clean, blue cloth. They had three beautiful, healthy children. They had a strong faith. They were blessed beyond measure.
Riley hit all the usual milestones in her first few months. Rolling over. Finding her feet. Sitting up. Crawling. Picking up Cheerios. Her first tooth.
She babbled and cooed. She seemed to say “Duck” and “Cup.” She wore that smile like a halo.
And then everything seemed too still.
“When she was a year old, she was nowhere near walking,” Kelly Hooper recalls. “I thought, ‘She’ll do it. Give her time.’”
Riley took a few steps. But soon it was clear something was wrong.
A visit with Baton Rouge pediatric neurologist Dr. Charlotte Holman confirmed there was some sort of problem.
Two months and several tests later, while other families were hanging stockings and hunkering down for Christmas Eve, Dr. Holman delivered the life-altering diagnosis. Riley tested positive for Rett Syndrome, an incurable neurological condition usually found in females. At age 2, Riley would start to regress.
Among an array of individually varying symptoms, Rett Syndrome often results in a loss of motor skills and speech. Those who live with it usually develop seizures.
Until the 1990s, Rett Syndrome was often misdiagnosed and largely misunderstood by doctors.
Now, they know it is caused by an abnormality in the MECP2 gene. This understanding also gives families and researchers hope that, one day, sooner rather than later, they’ll find a cure.
Last month, the World Rett Syndrome Congress took place in New Orleans. There, researchers and scientists convened to share their knowledge of the syndrome.
Kathryn Kissam of Baton Rouge is the chairman of the International Rett Syndrome Foundation and is passionate about finding a cure.
Dr. Holman says raising awareness of Rett Syndrome, which only recently has been understood by the medical community, helps get the word out so that parents who suspect their child has Rett Syndrome can get a proper diagnosis and treatment.
“Rett Syndrome is a condition with very specific features, such as losing developmental milestones, small head size, hand washing movements and seizures, that can be genetically identified,” she says.
For the Hoopers, Rett Syndrome has meant an entirely new future, vastly different than the one they first envisioned.
This morning, Riley is, as always, smiling. On every wall, there are pictures of her and that radiant grin. Riley as a baby. Then as a toddler. Then blooming into a gorgeous little girl with smooth brown hair, sea-colored eyes and delicate features.
Above the family’s gleaming dining room table, adorned with a vase of brightly colored daisies, the three Hooper kids beam in separate canvas prints, each four times larger than life.
The future may look different, but the family is no less rich.
Sixteen-year-old Jordan is a junior at Walker High. On the quiet side, he’s passionate about sports. Riley, an outdoors girl, is a fixture at his games. Seven-year-old Kamryn still shares a room with Riley and can often be found reading to her sister early in the morning.
“They have a special bond,” Kelly Hooper says.
When friends ask Kamryn what is wrong with Riley, she tells them, “Her brain is sick.”
Riley is five years old. She sleeps in a crib. She crawls from place to place. She wears size 2 clothes and weighs 21 pounds. She’s still in diapers. Through extensive physical therapy, the Hoopers were able to help Riley remember many of the skills she might have lost as Rett Syndrome took hold through her toddler years. That Riley can crawl is a constant relief. That she knows her mom and can hear and clearly enjoy music is bliss.
At Pediatria Health Care, a Baton Rouge school for medically fragile children, Riley has become a member of a thriving community who are all facing medical obstacles. Riley has lots of buddies.
Kelly Hooper can easily tote Riley on her hip. Though Riley fills the halls with squeals and even occasional laughter, she doesn’t speak. Her slender fingers curl inward and don’t grasp at paintbrushes or splay open for high fives.
Kamryn’s starting to realize that Riley might not ever get better. Still, she doesn’t give up. She keeps asking, “When is Riley going to talk to us?”
“You can kill yourself with the ‘if-only,’” Kelly Hooper says.
In darker times, right after the diagnosis, Hooper admits that she would list all the things Riley might never do. She might never get married. She might never have children.
Then it dawned on Hooper that she loves Riley, as she loves all her children, not for what Riley will do. Not because of this future or that future or another one. She loves Riley pure, simple and white-hot.
This is the only Riley that has ever graced the Earth. She will carry Riley. She will feed Riley. She will be Riley’s voice. She will shelter her forever. She will look into the pool of her own heart in amazement as it widens and deepens to hold the vastness of this particular, unique life that is her youngest daughter.
“When we got the diagnosis, initially, it was pretty devastating,” Jeff Hooper says. “Then you kind of sit back and you look at the situation and what are you going to do? You have to make the best of it.
“You make a new reality,” Kelly Hooper says.
Reality. It’s what gives life texture and meaning, those moments, hours, days, weeks and months of ordinary, chock-full of pet peeves and aches and pains, both physical and emotional.
Ask the Hoopers, and they quickly focus on the blessings. But that doesn’t mean the situation is not unbearably difficult at times.
Not all of the family’s difficulties stem from the Rett Syndrome diagnosis.
The Hoopers are in the midst of divorce proceedings.
Jeff and Kelly have been able to pick through the situation as a unified force. They recognize they are both parents. They’ve pledged to work together raising all their children. When it comes to Riley, that’s a process that will probably keep them knit together, for better or for worse, for the rest of their lives, regardless of legal definitions.
“When Kelly and I talked about how we were going to handle this and telling the kids about it, we talked about, ‘This is what we’ll tell Jordan. This is how we are going to put it to Kamryn.’ But you don’t really know what to do with Riley,” Jeff Hooper says. “I wonder if Riley knows what’s going on.”
A person with Rett Syndrome feels a bit like being a bird that’s trapped within an immobile cage of bones and muscles that won’t move, even when they try to think them into doing so. Recent developments in technology have given some who live with Rett Syndrome a constant, streaming way to tell the world what it is like.
Minnesota blogger Karly Wahlin, 27, has Rett Syndrome. She learned how to read by looking closely while her sister and mom read to her. When she was nine, she was given a little typewriter. For the first time, she was able to to describe for her family what she was going through.
“I was so afraid,” Wahlin wrote on her blog Inspired By Love at spiritdances.wordpress.com. “I knew that something wasn’t right in my body because I tried so hard to pick things up, and feed myself or say words, and I couldn’t. It got worse when I was old enough to see other kids younger than me who could do those things. I felt terrified inside because I couldn’t find a way out.”
Wahlin is no longer stuck. And she’s no longer known as just a Rett sufferer, but a poet, a musician, a documentary filmmaker and a blogger with 94,000 hits.
“If girls with Rett are allowed to learn, and are given reassurance on the days they are struggling, they can have a great life, too,” Wahlin writes.
Watching Riley learn how to press “Yes” and “No” on an iPad sparks hope among the Hoopers that she, too, will be able to tell them what she’s thinking and feeling and what she wants to do with her life.
“I really believe that Riley is not going to suffer from this for her whole life,” Jeff Hooper says. “The way things are progressing, we are going to be able to maybe have conversations with her or we’ll get to watch her run around, or maybe I’ll get to walk her down the aisle one day.”
In the meantime, the Hoopers have become Riley’s students in many ways. She has taught them just how patient they really are, and how massive their ability to give truly is.
“Before I had a special needs child, I’d be like, ‘They’re so strong. I’m not strong. I could never do that,’” Kelly Hooper says. “But then you do. You’re a lot stronger than you think.”
Click here to find out how one local woman is working to find a cure for Rett Syndrome.
