Jackson took his first trip to Costco the other day. He sat in the cart wide-eyed, an explorer drinking in the lights and color and motion. He paid no attention to the stares, the whispers and pointing fingers.
His mother, Traci Holbrook, could not ignore them. People came up to her and wanted to know why her kid had tubes taped to his face, snaking to an oxygen tank. A few ignored the sticker on his car seat, which asks people not to touch his hands before washing their own, and pawed and poked and cooed at him, marveling at his rock-star-worthy long eyelashes. Well-meaning, maybe, but still. Traci met the stares with glares, told the touchers to back off, tried to answer rude questions with something like patience.
"I'm sure it must seem strange to people, seeing him for the first time," she says. "But the mama bear in me comes out and says, 'Bring it on. That's my child you're pointing at, and I will kick your ass.'"
Eighteen months ago, Traci would have counted herself among the clueless. Heart trouble was something she associated with old people. The idea that her own son could be born so different, at risk of dying from a heart attack or stroke or even a bad case of the flu before he ever saw his first birthday, was unthinkable. But then came the fetal ultrasound, confronting Traci and her husband, Chris, with the hardest decision they had ever faced — followed by her introduction to the unofficial sorority of "heart moms" raising kids with congenital heart defects. It's a world of round-the-clock feeds and meds and oxygen monitoring, tests and operations and baffling setbacks, grueling hospital stays and ferocious hope — a gauntlet of surgeries and aggressive medical interventions that not every child will survive.
The gawkers at Costco have no idea how fragile Jackson Holbrook is. Or how tough. Peel off his top, the one declaring that he's DADDY'S LITTLE MONSTER, and you'll find a Frankensteinish track down his breastbone from having his chest cracked open three times. There's an angry red scar from a lung biopsy across his right side, a port for a feeding tube on the left side of his abdomen. Other marks and bumps dot his torso, souvenirs of past blood draws and drains and catheters. Weighing in at a mere sixteen pounds, he's small for his age, but the doctors at Children's Hospital Colorado call him Samurai Jackson. He's a warrior who's scrapped his way through more life-or-death battles in his first twelve months than you'll see in a Kurosawa double feature.
Despite all the tests and prodding and poking, he remains a surprisingly unfussy baby, with a smile that can blaze up a room. His smile receives frequent mention in the frank, often poignant e-mails Traci sends to a long list of family, friends and supporters, updating them on his progress, as in this report from last May:
Jackson almost died twice in a matter of days. But he didn't give up the fight. The surgeon and the doctors and the nurses did their part to help him, but Jackson also had to step up and do his part.... Every time they knocked him out with drugs, they did it because he was fighting them, telling them he wanted to do it himself, for himself. He is amazing. I am truly in awe of him. Everything that has been done to him, and when he sees us, he still smiles....
Impressed as they might be by the samurai, some staffers at Children's are even more in awe of his mother, who shows up for appointments with detailed notes and questions and has been a vocal champion for Jackson. "Traci is a pretty phenomenal woman," says Dr. Adel Younoszai, director of fetal cardiology at Children's. "She puts some people in the medical system back on their heels a bit. What she feels is what you get. All moms, to some extent, have that kind of intrinsic caring. For her it's a very rich, very deep emotion. She loves that child."
Traci says she's grateful for the support and resources that Children's offers to parents at its Heart Institute, from sleepover rooms to social workers and "child life specialists." Staffers have helped her with logistics and insurance issues and encouraged her to be actively involved in Jackson's treatment. "I am absolutely a member of his medical team," she says, "and I seek out the people who are going to talk to me, not at me."
The Holbrooks have also learned from sharing information with other heart moms and dads in person or online, a vast network of families struggling with daunting medical challenges. Around 40,000 babies are born each year in the United States with a congenital heart defect, or CHD — roughly 1 percent of all live births. It's estimated that nearly a third of CHD babies have some degree of neurological or developmental disability, too, and heart defects remain the leading cause of death in infants. According to the Children's Heart Foundation, twice as many children die from CHD each year than from all forms of pediatric cancer combined.
Yet the numbers also indicate a 30 percent drop in the mortality rate of CHD babies over the past decade. A great deal of the credit goes to dramatic medical advances over the past quarter-century that have greatly expanded prenatal detection and surgical options for a staggering array of heart defects; while many of the most complex heart problems can't be truly "cured," pediatric heart surgeons have increasingly refined techniques for re-engineering the system to get blood and oxygen where it's needed. Some of the earliest beneficiaries of these surgeries are now in their thirties and leading healthy lives, without a heart transplant — raising hopes for CHD families worldwide.
Those families may be playing a vital role themselves in the improving survival rate. Like the Holbrooks, many of them are deeply involved in just about every phase of their child's treatment. It's not unusual now for major pediatric cardiology centers such as Children's to connect pregnant women who've just learned that their fetus has a heart condition with parents of other CHD babies, so they can compare notes, confide fears and plan for the future.
"As we see these families communicate in social forums, we get a sense of what other centers are doing," says Stacey Lihn, president of Sisters by Heart, a national organization of mothers of children born with a rare condition known as hypoplastic left-heart syndrome. "We can hook up practitioners who are doing something different, compare and tweak medications, and so on. I believe we're educating the centers as to what's going on elsewhere. We're trying to bridge that gap between families and doctors."
Lihn's group has sent out more than 600 "care packages" to CHD families over the past three years, filled with items such as side-snap onesies that make it easier to dress a baby hooked up to wires and tubes. Lihn was a featured speaker at the 2012 Democratic National Convention, praising the Affordable Care Act for providing a safety net for her own daughter, Zoe — who'd had two open-heart surgeries by the time she was four months old and was facing an insurance "lifetime cap" that would have deprived her of any future coverage. Her activism has been an inspiration to other moms, including Traci Holbrook.
"The culture of medicine is gradually changing, especially in pediatric medicine," says Lihn. "When you get this diagnosis, you feel so helpless. The one thing families can do is educate themselves and play some role in their child's care. That gives you some sense of control. You have to advocate for your child. You may not have an M.D. by your name, but you know your child best."
Since Jackson's birth, the Holbrooks have been through a crash course in cardiology — with additional lessons in patience, crisis management and adjusted expectations. While juggling a marriage, a job as a paralegal, and raising Jackson's older brother, Mason, Traci has learned more than she ever wanted to about pleural effusion, electrolytes and junctional heartbeats, and she's gotten to the point where she can reasonably gauge Jackson's oxygen saturation level by the degree of blue tinge in his complexion. ("Any day with pink toes is a good day," she says.) But she's also learned that there's a great deal she can't control, that comes down to the mysteries of medicine and prayer and the heart of a samurai.
"I hope he'll outlive me, but to be honest, I don't know," she says. "Right now I'm just trying to live and enjoy my children. I want the chaos of a normal life. That's all I want."
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There are more than three dozen types of congenital heart defects. In the vast majority of cases, there's no known cause for the condition, no possible means of prevention or cure. Many families don't even find out their child has heart trouble until after delivery; increasingly, though, CHD is being detected before birth, leaving expectant parents facing a formidable decision.
Younoszai, Jackson's cardiologist, likens a parent's discovery of a fetus with CHD to the shock of the sudden passing of a loved one — it's the death of many dreams of "normal" family life, of what that child was supposed to become. "Nobody expects their child to have high medical needs," he notes. "One of the things I've learned is that it's a mourning process. Every time your life goes through that kind of change, where expectations and reality have completely split, there's a sense of loss, anger and denial."
Before Jackson came along, Chris and Traci Holbrook had other plans of their own. Both had come to Colorado looking for a fresh start, from opposite sides of the West. Clean and sober after youthful bouts with drugs and alcohol, Chris had followed some friends to Denver from Phoenix and found work as a technician for a lawn-service company. Traci had come from Montana in 1998 with her first husband and divorced a couple of years later. The couple met through an online dating service in 2003. Two years later, Chris popped the question during a gondola ride at the Venetian in Las Vegas.
"She thought I was going to say 'I love you' for the first time," he recalls. "But there was no doubt in my mind. I'm hesitant to commit, but once I decide, that's it."
At first, Traci was not in any hurry to have kids. The experience of having Mason, now a robust four-year-old, convinced her that she wanted more children. But in the summer of 2012, 22 weeks into her second pregnancy, the couple's perinatologist told them it was time to change their plans.
An ultrasound had revealed that the fetus had both of the primary vessels carrying blood from the heart, the pulmonary artery and the aorta, on the right side — a condition known as double outlet right ventricle, or DORV. In a normal heart, the aorta is on the left side; the unusual configuration was complicated by the fact that the left chamber wasn't receiving blood flow and hadn't developed properly. In other words, Jackson had only half a functioning heart.
DORV is incredibly rare, the Holbrooks were told. It accounts for less than 1 percent of all heart defects. But the approach to treatment has much in common with other kinds of "single-ventricle" defects, all of which impede the heart's ability to efficiently deliver oxygen-depleted blood to the lungs, receive oxygen-rich blood in return, and pump it to the rest of the body.
The doctors at Children's Hospital Colorado, which sees twenty to thirty single-ventricle cases a year, confirmed the diagnosis. They told the Holbrooks that they were looking at three options. One, they could terminate the pregnancy — a decision that had to be made in the next two weeks in order to avoid the complexities of a late-term abortion. Two, they could have Jackson and allow the doctors at Children's to try to rearrange the plumbing, through a series of three surgeries designed to improve the damaged heart's efficiency.
The third option was to deliver the baby but not attempt any form of medical intervention beyond what the doctors called "comfort care." It was likely Jackson would die within a month. Both parents rejected that option from the start, but wavered between the other two.
"We're both pro-choice," Chris says. "We definitely battled with the question of quality of life. Whether this was the best thing for him and for us. Whether it would be selfish or a favor to him not to go ahead with the pregnancy."
"Those were two of the hardest weeks," Traci says. "I actually was the one who was more for not going forward with the pregnancy."
There was no easy answer. Traci read that the survival rate for single-ventricle kids was 98 percent, "but what they don't tell you is that 'survival' is defined as living for thirty days after hospital discharge," she says. "Twenty percent of them don't make it to kindergarten."
Traci underwent an amniocentesis to see if there was a possibility of Down syndrome or some other chromosomal disorder, but the tests indicated no abnormality beyond the bad ticker. Chris knew that their lives would be better on paper, financially and otherwise, if they chose abortion — but he worried that Traci might never recover emotionally from the decision. They talked to parents of other cardiac kids, some of whom were now in their teens or twenties, playing soccer and going to college.
The deadline arrived. Traci had already made an appointment at the abortion clinic. But in the course of a two-hour phone conversation with her mother, a nurse, she decided she was going to have this baby.
"What it came down to, at the end of the day, is that I couldn't take my child's life," she says. "If God did it, that would be God's choice. I would do my best to respect it. But I couldn't do it."
The last few weeks of her pregnancy flew by in a blur. "It was hard to feel fine physically and be a disaster emotionally," she says.
On October 11, 2012, she paused at the threshold of the Children's delivery room, where she was scheduled for a C-section. A team of doctors was poised to swoop in and help if things didn't go as expected. She thought about how she was taking her son from a safe place inside her to an unsafe world.
The delivery was uneventful. She got to hold Jackson for two hours without any tubes or wires on him. Then they took him away from her, to the cardiac ICU.
********
The good news from the outset was that Jackson's aorta, while oddly placed, was functional; in many single-ventricle conditions, a crimped or underdeveloped aorta can be one of the most serious obstacles to getting blood to the body.
The bad news arrived when Jackson was five days old and about to undergo his first major heart surgery. Traci had noticed that Jackson's ears had looked a little funny at birth, but that had seemed like the least of his worries. On the fifth day a stream of specialists inspected her baby and offered one alarming opinion after another. An ophthalmologist studied his eyes and declared that he had at least one coloboma, a missing notch of tissue that could seriously affect his vision. An audiologist tested his hearing and suggested he might be deaf. A speech therapist advised against having him drink by mouth because he might choke, due to airway issues.
A genetic counselor reviewed the tests and informed the Holbrooks that Jackson appeared to be exhibiting the classic signs of CHARGE syndrome, a genetic abnormality that can vary greatly in severity from one child to the next — and that had failed to show up in the genetic testing done before Jackson was born. The acronym refers to a complex of symptoms involving sight, hearing, physical development, a blockage in the passages that connect nose to throat, and, yes, heart defects.
Traci couldn't believe it. She was profoundly grateful when a sympathetic nurse chased all the yammering experts from the room. In an e-mail to friends, she wrote:
I've never felt so battered and beaten. That awful day in the CICU when they just kept coming at us with bad news, and then we had to hand him over for heart surgery, was one of the worst days of my life.But the Holbrooks had no time to brood on what this new diagnosis might mean — not with his first surgery looming. The procedure consists of placing a band around the pulmonary artery to limit the amount of blood flow to the lungs. In a heart where the "red" (oxygen-rich) and "blue" blood mix together in the same chamber, the circulation to the lungs tends to be the path of least resistance, and too much blood headed in that direction can damage vessels there, a common problem in CHD cases.
The surgery went well. After three weeks, the Holbrooks were allowed to take Jackson home. It was clear by then that several of the most disturbing possibilities raised by the CHARGE diagnosis hadn't materialized. The coloboma didn't seem to be seriously affecting his vision. He could hear out of his right ear. His parents rejoiced at every modest sign of progress.
"They told us he might be blind, he might be deaf," Chris says. "He's neither. I'm looking forward to him sitting up, not what he's going to do when he graduates Harvard."
The most serious problem appeared to be with his airways. Fed with a bottle, he seemed inclined to aspirate into his lungs. He flunked a "swallow study" and was soon put on a feeding tube, much to Traci's frustration. From an e-mail last February:
It is killing me not to be able to feed my child. It's the basic job of a mom to feed her baby, and I can't. I mean, sure, I shove food in his G tube every 3 hours, round the clock. But it's not the same, and I am struggling with it.
Yet for a few weeks, things settled into a kind of routine. An exhausting routine, certainly, with feedings and meds that had to be administered in the middle of the night, looking after Mason as well, and scrambling to get things ready for the home nurse who helped monitor the baby during the day so that Jackson's parents could keep their jobs. (Chris has private health-insurance coverage through his employer that pays for some of Jackson's care, and the Holbrooks pay additional premiums for Medicaid coverage for Jackson.) Jackson slept a great deal and seemed to be improving. It was a strangely calm and isolated time that Traci treasured all the more because she dreaded what was coming next.
This past spring, just as he turned six months, Jackson returned to Children's for his second heart surgery. This time he was undergoing a Glenn shunt, also known as a "partial Fontan" operation. The operation involves connecting the superior vena cava, which carries venous blood from the arms and head, to the pulmonary artery — essentially diverting that "blue" blood so that it flows directly to the lungs to receive oxygen rather than being pumped there from the heart. That increases the efficiency of the heart and boosts oxygen levels by reducing the amount of blue blood mixing with red in the single heart chamber. Down the line, when the child is older and stronger, a third operation (the "full Fontan") creates a similar diversion of blue blood from the lower part of the body to the lungs, completely separating the red and blue circulations, as in a normal heart.
The typical recovery period for a Glenn, as heart moms call it, is seven to ten days. Jackson's initial response was so encouraging that his parents were hoping to take him home on the eighth day. But then his oxygen levels crashed, and the doctors didn't seem to know what was wrong. They put Jackson on a ventilator. Early on a Friday morning, heart surgeon David Campbell called Traci and asked her how far away she was from Children's. Her heart sank.
It's not his heart, Campbell assured her. The incision in his sternum was infected down to the bone. They were going to have to open up his chest for the second time in two weeks and clean it out.
The next few days were agonizing. From an April 25 heart-mom e-mail: Thursday has been one hell of a roller coaster ride. We came in this morning at about 8 am, and Jackson was looking great.... By 8:30 it was clear something was very very wrong and by 9 am there were no less than 20 people in Jackson's room. His oxygen sats [saturation levels] dropped into the upper 20s and low 30s, and they were unable to bring them up.... There was talk of putting him on ECMO, which is a type of life support to allow his heart and lungs to rest and heal.
Traci couldn't follow everything that was going on, but she knew about ECMO, shorthand for extracorporeal membrane oxygenation technology. That would be like turning your child completely over to a machine; she had seen CHD babies go on the machine and never come off it. At the height of the crisis, a young resident came out to update Traci on what the doctors were doing, using the usual medical jargon. It was too much for Traci.
"I don't fucking understand a word you're saying, and my son is dying," she snapped. "So use words I get."
The resident blanched. "You're right," she said. "I'm sorry." She began to explain again, using plain English.
Traci texted the four lawyers she worked for, saying that she needed help. One was in a court hearing in Douglas County; she told the judge sorry, adios, gotta go. The other three were in meetings or depositions. All of them dropped what they were doing and came down to Children's to support her and Jackson.
The crisis ebbed, then surged back. Jackson was fighting a viral infection, a bacterial infection and a staph infection from the breathing tube. No quarter given; none sought. Samurais don't quit.
Traci spent long days at the hospital, saw Chris for fifteen minutes at dinner, collapsed while Chris rushed to take the evening shift at their son's bedside. Neither one of them had any family that lived locally, but friends and neighbors stepped up to help with Mason, with chores and meals, with a golf tournament to help with the mounting hospital bills, with getting through the emotional exhaustion.
"You really learn who your friends are," Traci says. "A lot of people say they will do everything they can because they think it's the right thing to say. They feel obligated to say it. But in our case, a lot of people actually meant it."
********
May 2: We were finally able to hold Jackson yesterday.... Jackson is addicted to the sedatives they have been giving him for the past week and a half, and they are working to wean him off of them. So he's on methadone. Man, that sucks to even admit out loud.
May 10: He was asleep when I got there today, so I just sat down in the chair with him tucked in my arms and he slept so peacefully. It was amazing therapy for us both. I stroked his head, and tried to cover the giant bald spot from the IV in his scalp. Adel [Younoszai] came by and asked how I was, and the only word that came to mind was "perfect," so that's what I said. Right then, in that very moment, I was perfect. Jackson was asleep, in my arms, not struggling to breathe, not blue from low sats, not crying from opiate withdrawals. He was just sleeping and I was just holding him. I'd call that pretty damn perfect.
June 12: And now for the update we've all been waiting for...JACKSON IS GOING HOME TODAY!! After 61 days here, 48 of them spent in the Cardiac ICU, and after nearly losing him two separate and very scary times, we are finally making a break for it.... We are so very blessed, so very fortunate, so very lucky.
********
On a rainy October afternoon, Traci takes Jackson to Children's for his weekly clinic appointment. Members of his medical team review his heart tests, oxygen levels, nutrition and much more. A nurse types information into a computer as Traci meticulously ticks off from memory the names and amounts of medications being administered to Jackson, from aspirin to antacid to diuretics to Sildenafil — a drug that helps reduce pulmonary hypertension but is better known to the adult population as Viagra.
Dr. Younoszai examines Jackson. He likes what he sees. The baby's complexion, while still mottled in places, is looking rosier all the time. Jackson grips the cardiologist's ID card, energetically drums his legs on the exam table, bats his long eyelashes.
"We've had our ups and downs," Younoszai says, "but he's looking phenomenal today."
"There were times when it was minute to minute," Traci says.
Younoszai smiles. "Now it's week to week," he says.
The minute-to-minute part didn't end with Jackson's two-month spiral of complications after the Glenn surgery. He was home only a couple of weeks before a series of additional hospital stays — a bout of pneumonia, followed by fluid retention in his lungs, followed by other crises involving aspiration and low sodium levels. During one low period in July, when the doctors felt particularly grim about his prospects, there was talk of undoing the Glenn and even contemplation of a heart transplant.
No additional open-heart surgeries have been performed, but Jackson has undergone a series of lesser procedures. Surgeons have twice used a heart catheter to tie off tiny blood vessels, known as collaterals, that tend to form when the pathways between lungs and heart are compromised; in effect, the body tries to create its own shunts to get more oxygen into the bloodstream. He's also had a Nissen, a technique for constricting the esophagus to make vomiting and gastric reflux more difficult, and surgery to remove bony tissue that was obstructing his nasal passages. The tissue is growing back, requiring at least one more surgery scheduled in a few weeks.
"He's had some chronic issues that have been conspiring against him — his airways, aspiration, reflux," Younoszai says. "It doesn't have to affect the lungs directly, but it can cause this clamping down of the blood vessels, this restriction, and that can push back on this unnatural system we've given him. It's not just his CHARGE syndrome, but I think that's behind a lot of this."
At times Jackson's ordeals have come close to overwhelming Traci. She's felt helpless to relieve his suffering, guilty about putting him through all this, a complete failure as a parent. The long hospital stays have been hard on her marriage — "When he's not here, Traci and I seem to grow apart," Chris says — and hard on Mason, who must play second fiddle to his little brother's ongoing medical drama. But they've been particularly hard on Traci's estimation of herself as a mother and a member of the human race.
July 29: I am tired of the pokes and the cuts and the tests, that never lead to anything definitive. I am tired of not getting concrete answers. I am tired of handing my kid over so they can abuse him. I miss him. I am sucking as a mother to Mason. I am sucking as a wife. I am sucking as a mother to Jackson...I am just sucking all around.
She got to the point where she could no longer bear conversations with other parents about how poor little Jess or Josh got an owie or howled while getting vaccinated. "I realized one day that I had unfriended every single person I knew on Facebook that had healthy kids," she says. "I have a friend who's pregnant, and I've maybe asked twice about how it's going. It makes me feel like a jackass, but I can't make the words come out of my mouth."
Yet in the past few weeks, Traci and her son have been faring better. To the extent that any heart baby ever truly turns a corner in the labyrinth of medical crises surrounding CHD, Jackson seems to have turned one. It's been more than a month now since his last hospitalization, his longest stay at home since his Glenn surgery. The amount of supplemental oxygen he requires has been dialed down significantly; it's now being used more in a medicinal sense, to help keep blood vessels open wide rather than because he needs it to breathe.
"To be honest, there are a lot of kids who just don't make it," Younoszai says. "But the great thing about kids is they have this plasticity, this ability to adjust — and they heal so much better than adults do. They start flying on their own. Jackson is a bit different; with his genetic syndrome, we don't know what his complete potential is. You've just got to take it in small steps. But for him, week to week is great. I'm still hopeful that we're going to be talking year to year with him."
Despite his setbacks, Younoszai says Jackson has a "very good intellectual potential prognosis." Traci and Chris know that their son is far from out of the maze. He has a blood clot in his leg the doctors would like to see broken up, but not at the risk of some piece of it traveling to his heart and possibly his brain. He needs to more than double his weight and get much stronger before he's ready for the Fontan that will complete his trio of heart surgeries; the operation is typically done on CHD children between the ages of two and five years old.
Over the past twelve months, Jackson has racked up medical bills totaling well over two million dollars — most but not all of it paid by insurance and Medicaid. He's gone through opiate withdrawal, popped Viagra, brushed up against death and bounced back into his Johnny Jump Up. He now has a physical therapist, a speech therapist and even an occupational therapist, but his mother jokes that she's the one who needs therapy.
"He's very behind, developmentally," Traci notes. "He's spent five or six months of his life in the hospital. He doesn't roll over. He doesn't sit up. He doesn't make a lot of verbal noise. He doesn't eat with his mouth. But so what if he doesn't walk on his first birthday? The way I look at it, he's alive. I will take alive and not walking."
The past year has taught the Holbrooks plenty about the frontiers of medicine and its limitations. It's taught them how to be advocates for their son, and Traci doesn't hesitate when asked what advice she offers other heart moms: "Speak up for your child. Find what works for you, the doctors and nurses who are going to talk to you on your level. And stay the hell off the Internet. Google is not your friend. The information is outdated, it's wrong, it's scary."
But most of all, having Jackson has taught them about what matters. Most of the cases Traci deals with as a paralegal are family-court cases, epic struggles over who gets the children for Christmas and who gets to keep the good china — stuff that seems to fade into insignificance now. She can't get worked up over the small bumps in the road. Not after seeing kids fighting cancer at Children's. Not after attending the funeral of an eight-month-old, the son of a heart mom she'd met just a few months earlier. She will take alive for every precious moment it's offered.
October 5: You won't find me bragging about today marking the longest that Jackson has been home since April. You won't see us dancing around the living room. There won't be champagne toasts. I am not buying him a pony. I will instead stay quiet and humble. I will say thank you to the big guy upstairs for this gift of my son at home. 23 days. 23 glorious days.... I am trying not to focus on the number and instead just live for today.
Jackson turned one year old last week. The birthday celebration has been delayed a few days to accommodate some guests, but his mother sees every day at home as a day worth celebrating. This one will just be a little louder.
"He deserves the biggest celebration," she says. "He's been through so much, and he just keeps on smiling."
