When I read that my son might have an aversion to kisses, I pressed my lips to his over and over. When a parent in my support group said her child hated the feeling of wind on his face, I pushed my son on the park swing as high as I could. I had let go of many “normal” expectations for my son, but the notion that he might not delight in something like a breezy day or a kiss from his mommy seemed cruel. There was so little I could control, but these simple pleasures I would fight for with everything I had.
Maximus is born
Dan said everything was going to be OK, and I believed him.
He had just returned from our son’s side in the Neonatal Intensive Care Unit (NICU) at Northside Forsyth Hospital in Cumming. I was in recovery, eagerly waiting to hold my baby, basking in euphoria over the arrival of our firstborn.
We learned we were expecting just six weeks after our wedding. Dan and I were both ecstatic and immediately posted the ultrasound picture on our refrigerator, right next to the photo of us from the night we met. It was taken seven years earlier on my 21st birthday. My sorority sisters from Auburn University had taken me to a bar to celebrate the milestone, and Dan was the bartender. I gave him my number and we dated off and on for a few years until I realized I couldn’t live without him.
In the winter of 2010 he took me back to the same bar where we met and proposed. We married 10 months later and built our new life in a two-story home in Sugar Hill.
When it came to decorating our nursery, we didn’t want to know the gender of our baby ahead of time, so I had options in both pink and blue ready to go.
When I return to that moment in the hospital recovery room, daydreaming about my newborn son, I am overwhelmed with gratitude for Dan. He knew Max was in trouble, but he did not want to rob me of my moment of bliss. He knew that time would come soon enough.
Minutes after Dan gave me his most reassuring nod, a nurse came to talk to me. I expected to hear that my son was doing great and I would hold him soon. Instead, she threw my entire world off its axis. She said my newborn baby was struggling. She used the word tachypneic and explained that meant his breathing was excessively rapid. She said I would check out of the hospital long before he would. I looked at Dan in disbelief and found him glaring at the nurse.
When Dan left to return to our son, I began to hyperventilate. Heavy sobs and a guttural cry unlike any sound I’ve ever made shook my body. The only words I could muster were, “I want my baby.” The recovery nurse could do nothing to calm me. She left my side briefly and I heard her make a call.
“Please let me bring her,” she said. “She is very upset and she needs to see her baby.”
Minutes later, still lying in my recovery bed, nurses pushed me down a long, cold hallway and into an elevator. I was wheeled into the NICU room headfirst, surrounded by a sea of light blue scrubs. Nurses shuffled about busily and I heard a steady beeping noise. Two nurses parted like curtains to reveal Dan, his upper body stretched over an incubator, sobbing. Inside the incubator was our baby boy, his chest rising and falling violently.
“Mrs. Janton, I’m Dr. Miller.”
I heard him a couple times before I had the wherewithal to turn my head. He was looking down at me, his face surrounded by a halo of white light. I watched his mouth move, but all I heard were the murmurs of the nurses, the rhythmic sound of machines and my husband weeping. The doctor repeated himself a few times as I shook my head, unable to grasp reality.
“We don’t know what’s wrong yet,” he said. “He could have pneumonia, we see some features that may indicate a genetic disorder — we really don’t know, but we are very concerned.”
I don’t think I said a word when the doctor finished. I just nodded in shock. It wasn’t until I was being wheeled away that I began to scream. I felt hands holding mine and stroking my forehead, but I don’t remember faces. I was crying too hard to see. Nothing was as it was supposed to be, nothing made sense and there was nothing I could do about it.
On day three in the hospital, the geneticist knocked on my door. Dan and I were in good spirits, completely smitten over our son. We planned to name him simply Max, but Dan began calling him Maximus in the NICU, and we loved the strength of it. The name means “the greatest,” a perfect fit for our little guy.
He was still tethered to multiple machines showing little, if any, improvement, but we made ourselves believe he was just having a rough start. We looked past the tubes, the unique features of his body and the bewildered glances from the medical staff. Our baby boy was perfect and beautiful in our eyes. He was going to be OK, and we did our best to concentrate on that hope.
The geneticist shook her head at our optimism, and we stared at her blankly as she explained our son might have something called Costello syndrome, a diagnosis that only 300 to 400 people have worldwide. Her expression was cheerful as she described the gastrointestinal issues that may plague him throughout his life. Bright lipstick highlighted her wide smile as she told us about the cardiac issues and the increased risk of cancer. Her oddly enthusiastic bedside manner made her news impossible to comprehend.
“He may always have a funny look about him,” she said of my child, the baby I had dreamed of meeting since I was a little girl.
Dan’s face, permanently tan from the golf course, was now white and his uncharacteristic silence scared me.
The doctor offered to show us a website with more information, but we declined. She left us some notes about the condition, and we promptly tucked them away where no visitors could see them. We did not want speculation or questions from others.
Blood was drawn to test for three genetic syndromes, and we did not allow ourselves to entertain any of them. We were told the test results could take months to receive. We spent that time being optimistic and prayerful. We brought Max home when he was 6 weeks old, along with a nasogastric feeding tube, a cardiac monitor, a feeding pump and a variety pack of syringes, none of which had I read about in my bedside copy of “What to Expect When You’re Expecting.”
Max could not eat by mouth. We made every effort with both breastfeeding and bottle-feeding during his NICU stay, but it never clicked. We continued to try to feed him by mouth at home, but he received his food and medicine through a tube in his nose. An IV pole, equipped with a feeding pump, stood cold and sterile next to Max’s soft, white bassinet in our bedroom. We were eager to put him in his baby blue nursery with the white crib and cozy beadboard ceiling, but his overnight care required we keep him nearby.
Plagued with severe gastric reflux, Max could not handle much volume, so his round-the-clock feedings went at a snail’s pace, sometimes as slow as one ounce per hour. I pumped breast milk every three hours and Dan and I took turns cleaning Max’s pump bag just as often. Max vomited often, sometimes 20 times a day, which resulted in ER trips and hospitalizations a few times. Before leaving the house with him, we learned to pack a half dozen blankets in lieu of a burp cloth and mastered grace under fire while others watched in horror. Dan and I have cleaned up more vomit than a janitor in a fraternity house.
Max was 3 months old when we got the news. It was Oct. 17, our wedding anniversary. The geneticist’s office called to say the results were in, but they couldn’t be released over the phone and they couldn’t see us in the office that day. I knew Max’s pediatrician would have a copy of the results, so I called his office and scheduled a sick visit for that afternoon.
I had given in over the previous weeks and researched the syndrome online. I saw pictures of children with Costello syndrome and recognized features — the curled ears, thick eyebrows and deep creases in the palms of his hands and bottoms of his feet. I read about feeding struggles that mirrored Max’s and prayed it was just a coincidence. Despite all my efforts to stay optimistic, I saw proof. I never admitted it to Dan, afraid if I said it out loud it might be true.
“Are you sure you want to hear the results without your husband here,” Dr. Vigil asked as I sat before him, clutching Max on my lap. I had tried to call Dan before the appointment, but he was at work and I couldn’t reach him. It was already the afternoon and I knew the doctor’s office would probably close before Dan was off for the day. I couldn’t risk that chance.
“We’ve waited long enough,” I said. “I need to know now.”
“The test came back positive, Mrs. Janton. Max has Costello syndrome. I am so sorry.”
No website, no instinct, no geneticist could have prepared me. In one fell swoop, every ounce of hope I had mustered over the past three months was taken. Everything I had envisioned for my child, for my family, disappeared. I wept silently as fear spread through my body like a disease. I tightened my hold on Max, afraid if I let go I would lose him forever — a feeling I haven’t shaken to this day.
I waited until Dan came home from work that evening to tell him. He was sitting on the couch, nuzzling Max when I sat in front of him and placed my hand on his knee. I don’t remember exactly what I said or how I said it, but I remember Dan didn’t say a word. He held Max against his chest and closed his eyes for what felt like an eternity.
Later that evening I decided to go ahead and give Dan his anniversary gift. It was a custom-made sign I had designed and ordered when I was just three months pregnant. I walked out of our bedroom and, without saying a word, held it up for him to see: a weathered piece of wood, painted with a lyric from our wedding song, a fortuitous message: You and me together, we can do anything.
Our new normal
Costello syndrome is caused by a gene mutation, characterized by gastrointestinal problems, delayed physical and mental abilities and a weakened immune system. People with this condition have relatively short stature and may have reduced growth hormone levels. Heart conditions are prevalent, as are unusually flexible joints, weakened muscle tone, tight Achilles tendons and vision problems. In most cases, babies with Costello syndrome are born large, then their growth slows drastically.
When Max was about five months old, I noticed something peculiar about his eyes. They danced about constantly, and we were referred to an ophthalmologist who diagnosed him with Opsoclonus Myoclonus. It’s a neurological disorder that causes the eyes to make erratic movements and is often associated with Neuroblastoma, one of two cancers that are prevalent in people who have Costello syndrome.
It was Christmastime and in between Max’s first trip to Santa and decorating the tree with “Baby’s First Christmas” ornaments, we spent the month taking him from scan to scan. His oncologist was convinced Max had Neuroblastoma, and we lived in a daze until he was proved wrong. We were so relieved, but our fear didn’t go away. We have learned it never leaves, it only hibernates.
I joined an online support group when Max was six months old. Because Costello syndrome is so rare, I depend on the advice of fellow parents just as much, if not more, than that of our medical team. The worldwide support group communicates mostly through emails, and I quickly learned that staying informed is a double-edged sword.
So many stories have made me smile, and I’ve received advice that’s helped immensely, from choosing Max’s feeding tube brand to trying new medications for various issues. But some emails have left me devastated. Stories of cancer diagnoses, risky surgeries and long recoveries, stories about rude comments from strangers and, worst of all, stories of loss.
Recently I woke to read that a 16-year-old with Costello syndrome died from a heart attack, completely out of the blue. I instantly dropped my phone, clung to Dan and wept over a boy I never met.
Max is now 4 years old. He wears braces on his feet, he eats through a gastrostomy tube in his stomach and he is a teeny little guy. He learned to walk soon after he turned 3, and he recently learned to climb the stairs in our house. Multiple times a day, he plops in our laps with a stack full of books, and he loves to chatter about his day in filibuster fashion.
A beautiful, outgoing boy with brown curls and a smile full of shiny white teeth, Max is the mayor of everywhere we go. You can ask anyone from Kroger to the post office and they’ll confirm that he is known as the boy who says “hello.” He loves to greet people and he loves to wave at cars as they pass by. He is often the loudest kid in our music class, singing his “bum bum bums” with the beloved Miss Mary, and clapping for his friends when it’s their turn. We have music playing in our home all the time. Max, like his daddy, is a big fan of Mumford & Sons (or “band,” as he calls them) and their songs always lead to dance parties in the living room.
Among a slew of other specialists, Max sees an oncologist three times a year to be screened for tumors. Cancerous and noncancerous tumors are not uncommon with Costello syndrome, so a regimen of scans, bloodwork and urinalysis are recommended, especially in younger patients. He also sees a cardiologist two to three times a year. Those appointments are blunt interruptions to the happy-go-lucky days we strive to lead. We sit with doctors behind closed doors and have discussions that no parent wants to endure.
Max has hypertrophic cardiomyopathy, which is a thickening of the cardiac muscle. It is the disease best known for claiming the lives of young athletes. Max’s condition has worsened in the past year, but he is not symptomatic. We have been told that it can strike without warning. He is susceptible to having a cardiac episode any time, a possibility that paralyzes me with fear and makes me dread the rare occasions when I’m away from him.
After Max was born, I returned to work at my marketing job for a while. One of Max’s nurses from the NICU became our nanny and caretaker three days a week. It was a perfect arrangement until she had to resign. We researched all our options from private nurses to special needs day cares and were overwhelmed by the costs. We realized the most practical choice was for me to quit my job and stay home with Max. It was a tough decision, since I was the insurance carrier and Dan had just started his own sales business, but it made the most sense.
The benefit is I’m now able to take Max to more therapy sessions every week. We are constantly working to improve his feeding ability, his speech, his motor skills and physical capabilities. He generally meets his goals at a slow pace, but the anticipation only makes the celebration greater. Everything from when he puts his hands out to break a fall to tasting ice cream without gagging is celebrated in our house. Dan and I are both quick to get glassy-eyed when Max says “I love you” or when he applauds himself for shooting the ball in his toddler-sized basketball goal. His enthusiasm is infectious, constantly leaving us to shake our heads, wondering how we got so lucky.
Max started a pre-K program this year. Sending him to school was an incredibly difficult decision because of health concerns, but we never doubted how happy it would make him. When I drop him off with his teacher twice a week, he throws up a hand as he says “bye, mama,” and he walks to his classroom, hollering “hello” to everyone along the way. What I would give for such confidence.
Life full of magic
This year, Max became a big brother. He showers Baby Duke with hugs and kisses, holds his hand (or his hair) and makes him laugh. And he brings him diapers when he cries. “Baby” is the first word out of his mouth every morning.
Every day I pray for strength to be the mother Max deserves. Loving him is the easy part, keeping my worries at bay is the struggle. Thoughts about health concerns, insurance issues and the future can be all-consuming, preying on my mind in the quiet of the night. I pray he won’t feel left behind when Duke surpasses him in size and ability. I worry about his feelings getting hurt by a stare or a comment. I concentrate on ways to instill confidence. Dan and I work on his manners, holding him to the same standards as we would any other child. We want him to be polite and respectful, and we want to teach him to ignore rude people. I, personally, am still working on the latter.
I have been able to temper much of my anxiety through faith. Ironically, Max’s diagnosis is what made me question God the most and also what drew me closest to him. Motherhood is my sanctuary. It’s the godliest service I’ve ever known, and the connection I feel to my children is nothing short of divine.
After Max was born, I did not get to hold him in the NICU until he was 12 hours old. It was 2 a.m. Dan wheeled me into Max’s room and the nurse said I could only hold him for a few minutes. His breathing was still erratic and he needed as much rest as possible, she said. She draped the wires over my shoulder and placed him in my arms. I cried happy tears, grateful to be reunited with the little life I’d carried for nine months. The nurse watched the monitor, then looked back at me.
“His heart rate is slowing,” she said. “He knows he’s in his mama’s arms.”
That’s right, I thought. He is where he belongs. I knew I would make life better for him. I was determined.
I could not have fathomed, however, all the ways he would make life better for me. The baby boy who was deemed “failure to thrive” is a flourishing toddler, filling our days with hope, perspective and magic.
Nearly every night, weather permitting, Dan and I take the boys for a walk in our neighborhood. We know it’s time to go when Max comes to us saying “stroll,” nodding persuasively. The baby usually dozes on his half of the side-by-side stroller, while Max, on his side, chatters about everything in creation.
Dan and I use this time to share our days with each other, all the highs, all the lows. And we often talk about the future. We dream up vacations, we talk about career goals, and we contemplate all the ways we can make life amazing for our boys. Sometimes our discussions collide with our fears, but Dan says everything is going to be OK and I believe him.
“Go” is one of Max’s favorite words. In addition to his nightly stroll, he loves to toddle around a putting green with his daddy, and he delights in every errand we run during the day. In the car he smiles and claps when I roll his window down and he squeals “more” when the wind hits his face.
Every night I squeeze Max tight as I pace his bedroom floor and pray aloud. He clasps his hands while I bow my head to give thanks for his precious life and to ask God to heal his heart and keep him safe. And every night, after I say “amen,” Max echoes with a “naynen” and kisses me right on the lips.
ABOUT THE STORY
Keri Janton’s story about her son Max was the first runner up in our Personal Journeys Writing Contest last month. I had the pleasure of working with Keri a decade ago, when she briefly worked as a staff writer at the AJC, back when she and Dan were just dating. I was impressed with how thoughtful and thorough she was as a writer, and was sorry to see her leave the business for a job in public relations. It’s gratifying all these years later to see what a wonderful writer she has become. But more importantly, it’s a privilege to share her deeply personal story about a very special little boy.
Suzanne Van Atten
Personal Journeys editor
ABOUT THE WRITER
Keri Janton is a freelance writer and family photographer. Her favorite job, however, is being a stay-at-home mom to her two young sons, whom she is raising in Sugar Hill with her husband, Dan. She loves to write children’s books in her spare time and journals about family life in a private blog that she’s maintained for nearly four years.
ABOUT THE PHOTOGRAPHER
Hyosub Shin was born and raised in South Korea. Inspired by the work of National Geographic photographers, he came to the United States to study photography and joined the AJC photo staff in 2007. Past assignments include the Georgia Legislative session, Atlanta Dream’s Eastern Conference title game, the Atlanta Air Show and the Atlanta Braves’ National League Division Series.
Scroll down for more photos of Max and his family.