Every parent’s worst and first nightmare consists of walking into their sleeping child’s nursery to discover they have stopped breathing. There are even several commonly-used techniques aimed at assuaging these fears without waking the child: tickling their feet or placing a finger underneath their nose to make sure they are breathing. But imagine never being able to wake up from that nightmare. Could you live with that fear every day? Zoe’s parents do.
Zoe Colquitt of Dallas is one of just a few hundred children born with congenital central hypoventilation syndrome (CCHS), a disorder of the autonomic nervous system also known as Ondine’s Curse. Many newborns afflicted by this disease don’t live long enough to leave the hospital because the part of their brain regulating automatic respiration fails. They forget to breath while they’re asleep. And, in some extreme cases, the same happens while they’re awake. It is every parent’s worst nightmare.
When Zoe was born, the Colquitt family—mom Shelley, dad Kevin and Zoe’s older sister, Lauren—was thrown into new and frightening territory. Like many being treated for her condition, at 4-months-old Zoe received a tracheotomy and a tube attached to a ventilator was placed in her throat to ensure she was breathing properly.
“When you have a child with any kind of life-altering problem, you have to make a choice about what you’re going to do,” Shelley says. “At the time, we were told Zoe was not going to live beyond a year, that we were young, we could have other children. Maybe it would be best to just turn Zoe [her life support] off. Maybe we should give her up as a ward of the state. Maybe we should put her in a nursing home. They were trying to give us as many get-out-of-jail options as possible.”
The Colquitts took none of them.
“Our decision was no matter how long Zoe lives, Zoe is going to be treated like a child,” Shelley says. “You think that’s an obvious choice, but it’s not because it’s really very hard to treat Zoe like a child. You have to push yourself down to allow your child to be a child. You’re so scared. You’re scared you’re going to kill them. You’re scared that by letting them do and be, they could die, which is true.”
And the Colquitts did just that. A decade after the initial diagnosis, which outlined a future marked by an early death, Zoe hasn’t missed a beat. She loves to play the piano, read books about the fictional exploits of Percy Jackson (who also overcomes congenital disadvantages to reach great heights), eat spaghetti and meatballs, play with her dollhouse and watch Family Ties.
THE DIAGNOSIS
In the first moments following Zoe’s birth, everything seemed fine. She just slept more than most infants and would sometimes turn a pale shade of gray. When doctors discovered she had CCHS, they took her to the hospital’s Neonatal Intesive Care Unit and immediately placed her on life support. Zoe had been fighting to stay alive since the day she was born and even though the prognosis wasn’t positive, the Colquitts fought for every available option to keep her alive for a few more days, weeks or months. When Zoe made it to four months, doctors performed a tracheotomy and Shelley was able to hold her daughter for the first time. Zoe would remain in the hospital for two more months. When she was finally well enough to go home with her parents, she required care 24-hours a day, seven days a week.
Her treatment involved being attached to cumbersome TV-monitor-size machines, a humidifier to keep her lungs moist and two tubes that went straight into her tracheotomy. Before her first birthday, the Colquitts began teaching Zoe to breath on her own through a technique called “sprinting.” Two to four times every day they would wean her from the ventilator and very slowly increase the amount of time she was forced to breath on her own. The point of the excercise being to slowly and sometimes painfully teach the body how to breath on its own. By the time Zoe was 18-months-old, she was only using her machines for 12 hours a night unless she was ill. Sounds almost normal; until you consider most of us breathe 25 to 35 times a minute and Zoe still breathes just 15 times. Shelley is just thankful Zoe was too young to remember those tense moments.
“I was trying to make her as normal a child as I could, to give her a normal childhood,” Shelley says. “It would’ve been a lot easier and a lot safer to keep Zoe on her machines. But I had to let Zoe have a chance.”
When Zoe turned 3-years-old, another life-changing option was dropped in the Colquitt’s lap. They had heard about diaphragm pacers, which are surgically implanted and make the body breathe seemingly on its own. But, the decision to have her undergo a dangerous and experimental surgery was a difficult one for the Colquitts. Zoe developed pneumonia after the surgery and her pacers couldn’t be turned on for six months while she recovered.
When the pneumonia finally passed, the battery-operated pacers were switched on and found to be in perfect working order. Sure, the technology could malfunction … and it does. Shelley says Zoe has had almost seven near-death lapses like the one last year, where she passed out for no apparent reason while eating lunch at home. Shelley has resuscitated Zoe in the back of the family suburban, on the staircase and even at the dinner table. Zoe also sleeps with sensors so sensitive that Shelley knows when she turns over in her sleep. But, generally, she lives a life as normal as any other 10-year-old. Her parents try—and oftentimes succeed—in treating her that way, thankful for every breath she takes.
“I learned very quickly with Zoe that birthdays are not just about parties and balloons and cake,” Shelley says. “They’re about surviving one more year. About proving the doctors wrong. Remembering that life is very precious, and it’s very uncertain. It doesn’t matter what’s wrong with you or if there’s anything wrong with you. That’s the way birthdays are done here at my house.”
LIVING WITH GRIEF
Throughout her career, Dr. Sylvia Gearing, psychologist and founder of Gearing Up in Plano, has guided parents through the gut-wrenching journey of raising children with life-threatening conditions like CCHS.
“There is certainly a grief that the life with this child and life course as you had envisioned will not unfold as you thought,” Gearing says. “There’s a loss and there’s grief over that. Perhaps more importantly, the parents are in for a traumatic situation that will be ongoing. If they do not address that, recognize what is happening psychologically and then find a way to deal with it, they don’t go on to create what we call post-traumatic growth.”
Post-traumatic growth is a fairly recent concept in psychology. It refers not only to surviving such traumas but thriving through them—taking the tragedy and sadness and converting them into something significant and life changing. Parents going through serious medical conditions with their children need to be resilient and game-ready every day while at the same time going through a tremendous amount of stress, Gearing says.
“Most people get through stuff, and they get through stuff with the help of their friends and their family and their faith and all the other good things that make them emotionally competent,” Gearing says. “Moms really take a hit emotionally. They often are the primary caretaker and are on the front line with these kids. Women are built for emotions. And the expectation is that they must be strong for the child. If they learn how to handle these feelings, they’ll be way ahead of the game.”
By “handle,” Gearing doesn’t necessarily mean therapy, although that would be ideal. Simply understanding that as a parent you are psychologically impacted by your child’s illness is enough.
Dr. Jessie Gruman agrees. Gruman has become an expert in devastating illnesses—both by fate and by choice. Gruman, president of the Center for Advancing Health in Washington, D.C., has been diagnosed and treated for four different types of cancer. Based on her personal experiences and interviews with more than 200 people, she wrote AfterShock: What to Do when the Doctor Gives You—or Someone You Love—a Devastating Diagnosis.
In Gruman’s experience, such a diagnosis sets up a series of common external demands—diagnosis, search for doctors and treatments, decisions about treatment, then treatment itself. Sometimes patients get stalled in one stage; sometimes things go so quickly it’s difficult to keep up.
“Each person, each story, each struggle is unique,” Gruman says. “I am constantly amazed by people’s resilience, stubbornness and creativity in finding their way forward.”
Today, Zoe’s parents continue to fight for her future. They’re currently lobbying the U.S. Food and Drug Administration (FDA) to allow Zoe to be treated with Desogestrel, a controversial birth-control pill French researchers believe is a possible cure for CCHS. While the drug has not been approved by the FDA, the Colquitts are hoping Zoe will be the first in the United States to try it when she’s old enough (and because they can’t afford to travel to France for the treatment).
Why do they keep pushing the envelope when it comes to their daughter’s treatment? Maybe it’s because their daughter lives on the verge of dying with each breathless moment and yet doesn’t seem to have an ounce of quit in her.
“I will help find and let Zoe be a part of medical trials to find a cure and help give a voice to every child who suffers with CCHS,” Shelley says. “I thought I had a train wreck. I thought I had a child who was going to die. I had to come to grips with the fact that she might die because I knew it was a big possibility. But I had to see the potential in Zoe.”
And they continue to fight for her potential, because that’s what parents do for their children.
