A gift for Jake
Jake Hahn's only chance at survival comes from small stranger
NAPERVILLE — Whether it's the second, third or fifth child, parents usually think their next go-round with a baby is going to be easier.
Kent and Kristie Hahn were no different. So, when Kristie's water broke nearly three weeks early, it didn't send them scurrying like rookies.
Kent and Kristie Hahn were no different. So, when Kristie's water broke nearly three weeks early, it didn't send them scurrying like rookies.
Kristie even half-expected the new baby to be born ahead of schedule because her first son, Nicholas, had done the same thing two years earlier. No, this was just a small surprise on the grand scale of parenting.
Kristie even half-expected the new baby to be born ahead of schedule because her first son, Nicholas, had done the same thing two years earlier. No, this was just a small surprise on the grand scale of parenting.
It would be the first of a seemingly countless string of surprises, though.
Jake Austin Hahn was born by Cesarean section at 1:45 p.m. Aug. 1, 1999 at Edward Hospital in Naperville. He weighed in at 6 pounds 9 ounces and was 19 inches long, a little smaller than his brother was. While Jake looked like Nicholas, however, the accolades the parents heard from physicians and nurses when their first child was born were conspicuously absent this time.
As nurses took Jake away to clean and clothe him, Kent overheard a conversation between the hospital personnel.
"He better be checked for 21," the doctor told a nurse.
"Is there a problem?" Kent asked.
No, he was told.
He would later learn the test doctors ordered was for Down syndrome, a congenital disorder resulting from having an extra 21st chromosome.
These problems, though, did not even enter into Kristie's mind. She cried tears of joy when they handed Jake to her, dressed in his little pink and blue hat. Her parents, Chuck and Carol Swanson, brought Nicholas to see his baby brother, and the day couldn't have been happier.
* * *
Kristie awoke the following morning anxious for the next stage of her life to begin, but the rosy outlook turned gray almost immediately. Before her husband even arrived at the hospital, Kristie's doctor told her she wanted to test Jake for Down syndrome and check his heart.
About 30 percent of the children born with Down syndrome have a heart defect of some sort, the doctor explained. About half of those will need open-heart surgery to repair it.
Kristie couldn't believe this news. Her son looked so normal. Yet, the trained eye can catch subtle differences, she was told. Likewise, a trained ear can pick up a heart defect by listening for a "whooshing" sound. Jake showed enough differences to make doctors and nurses suspicious.
A few days later, Kristie and Kent met with Dr. Jose Quinones, a pediatric cardiologist at Edward. They learned Jake had been born with an AV Canal defect with hypoplasia, meaning the left side of his heart, which pumps blood throughout the body, was too small.
The AV canal defect was common, but the hypoplasia made Jake's case more complicated. Without three surgeries to repair the defects, he would probably die by the age of 5 or 6.
This was too much to process for Kent and Kristie, who were still in denial about Jake's possible Down syndrome. Kristie was so overcome with grief, she didn't even take a picture with her son in the hospital.
Her anguish during the weeks following Jake's birth had been set up while she was pregnant with Jake's older brother. Kristie underwent an alpha-fetoprotein (AFP) test that screens for early signs of spina bifida or Down syndrome. The test came back inconclusive, and the time she spent waiting for the results of a second test — an amniocentesis — was filled with stress. Although the test came back negative, she decided never to undergo an AFP test again.
As they sat with Dr. Quinones, though, Kristie and Kent knew advance notice wouldn't have changed their present situation. People who undergo prenatal tests can decide to terminate pregnancies, but the Hahns realized the only decision they had to make was when Jake's heart surgeries would begin.
The family was referred to Dr. Michel Ilbawi, director of pediatric cardiac surgery at the Heart Institute for Children at Hope Children's Hospital in Oak Lawn.
The first surgery would be a pulmonary artery banding procedure, which would control the amount of blood flow to the lungs. It was by far the easiest procedure — they wouldn't have to stop Jake's heart — but it was the hardest thing Kent and Kristie had gone through to that point.
And it would only get more difficult.
* * *
Jake's first surgery, on Aug. 19, 1999, was successful, and, after 12 days, he was allowed to go home.
Living with their tiny son — tests had now confirmed he had Down syndrome — was a whole new lesson in parenting for Kristie and Kent. Because of the wounds Jake had under his arms from surgery, they couldn't even pick him up the way they normally would lift a baby.
Knowing Jake wasn't due back for another surgery for seven months, the family enrolled their son in the state's Early Intervention program, which helps children from birth to age 3 who have developmental delays or disabilities.
Jake gradually learned to roll over. He was growing, but at a slow rate. He weighed no more than 15 pounds when the time came for his second surgery.
That second procedure, the Bi-directional Glenn, was the first attempt to reroute the blood flow throughout the body. The surgery on March 20, 2000, went as planned, leaving only the third surgery, known as the Fontan, as Jake's final hurdle.
When Kristie saw Jake later, it was the first time she had ever seen anyone on a ventilator. All she could do was cry.
* * *
Discovering your newborn child has severe health problems unleashes a tidal wave of emotions some people never overcome. The news can make a cynic out of the most positive mother, a recluse out of the most gregarious father.
Despite the trauma during Jake's first year of life, however, Kristie and Kent showed only sprinkles of depression that can define — and destroy — a family. They are human after all; yet, their focus was not on Jake's Down syndrome, but on how far he could advance despite his condition.
They knew when a baby is born with Down syndrome, a doctor never can say how advanced it will be, just as a physician can never predict an outcome for a normal developing child. With that in mind, the Hahns kept Jake enrolled in the state's Early Intervention program, hoping the therapy would prepare him well enough for school in a few years.
For the final heart procedure, the Fontan, Quinones suggested the Hahns take their son to the University of Chicago Hospitals, which had just launched its own pediatric cardiac surgery program under the leadership of Dr. Emile Bacha, a renowned surgeon at Boston Children's Hospital.
Bacha, a compassionate man who has photos of his young patients next to the photos of his own children, explained the Fontan was the final piece to the recirculation puzzle, which would connect the unoxygenated "blue" blood returning from the lower part of the body directly to the lungs, bypassing Jake's heart. After picking up oxygen, the blood would return to Jake's heart, which would pump it to the rest of the body with its single ventricle.
Bacha was confident the Fontan would work. "He's not the sickest I've seen," he said of Jake.
The doctor also tried to reassure the couple their child had an excellent chance at the University of Chicago because the hospital was one of only a handful which had a heart-bypass machine called an ECMO (Extra-Corporeal Membrane Oxidation) that keeps the child alive while the heart gains strength.
Bacha mentioned the ECMO, thinking they would never need it, and the Hahns decided to put Jake under his care, thinking the ECMO support would be a good idea just in case.
Bacha was wrong. The Hahns were right.
* * *
It seemed such a foregone conclusion that Jake would recover well from the Fontan, Kristie spent the last day before his surgery doing household chores she knew would not get done between long trips to the hospital.
She would grow to hate her practicality as she watched her baby falter over the next few weeks.
Things appeared to be going well on May 17, 2001, the day of the surgery. At the seven-hour mark, the parents were told the Fontan was completed. A couple hours later, however, they learned doctors had a hard time getting his heart to start pumping again.
Although it eventually responded, it was weak, and Jake was wheeled to intensive care with low blood pressure and a low heart rate.
"Things didn't go as well as planned," Bacha explained to the Hahns.
The doctor spent the night at the hospital after Jake's surgery to make sure he made it through without complications, but he could see the boy deteriorating during the next day. Jake's heart was simply not keeping up.
Late in the afternoon, Kristie was at Jake's bedside, while Kent was at the nurses' station on the phone with her parents. They both suddenly heard the sound of alarm and saw commotion headed Jake's way.
Jake had arrested. His heart had stopped.
Within seconds, physicians and nurses were on top of Jake. More professionals arrived, and Jake was quickly hooked up to the ECMO machine.
He soon stabilized, but technicians stayed with Jake around the clock. He would probably stay on the machine for a few days, Bacha told the parents, then doctors would slowly wean him off it. Gradually, the machine's power would be turned down, forcing the heart to respond and pump. They would build the strength in Jake's heart back up, as if he was running a marathon.
This race would be full of false starts, though.
After four days, they started to turn the ECMO down, but Jake's heart would always tire. They would try it again the next day, with the same result. They never were able to get below 50 percent.
"If there's one thing I learned from Jake, it's patience," Kristie said. "We need to go with his time frame."
Kristie and Kent went home for the first time three days after Jake's surgery. They decided they had to see Nicholas. About midnight, the phone rang. Jake had suffered another cardiac arrest.
By the time the Hahns got to the hospital, the baby had stabilized, but it took a defibrillator to restore a normal heart rhythm. Apparently, a mechanical pump that was giving Jake potassium had malfunctioned, delivering an overdose that sent his heart into an abnormal rhythm.
Jake survived, but it was a harbinger of things to come. He was still under ECMO protection, but his heart wasn't responding the way they hoped it would. After a week, doctors decided they couldn't wait any longer.
Bacha suggested the family consider a heart transplant.
* * *
Nothing — not Jake's Down syndrome, not his open-heart surgeries — could prepare the Hahns for this option. Their son was only 1 1/2 years old. It couldn't have come to this.
But Jake's heart was telling them it had. While the ECMO machine could buy time, it was only a temporary fix. Doctors don't like to keep children on the machine for more than a week because of problems to other organs.
Bacha introduced Kent and Kristie to Dr. David Waight, co-director of the pediatric heart transplant program at the University of Chicago Children's Hospital.
For patients that weren't in such poor condition as Jake, the chances of surviving the surgery and first year would be about 80 percent. For Jake, the odds dropped to 50 percent, Waight said.
The surgery, however, would only be the beginning. The family would be asked to bring Jake in for consistent follow-up appointments and trips to the cardiac catheterization laboratory. There were other factors to consider as well: because of the chance of rejection, Jake would have to be on heavy medication, and he would have to avoid regular contact with many people initially for fear of infections.
Kent and Kristie pondered the situation. They were afraid it would be trading one terrible situation for a lifetime of them. They weren't even sure Jake's other organs hadn't been damaged because he was on the ECMO machine for so long.
But after tests confirmed those organs were healthy, Jake and Kristie agreed to put their son on the heart transplant list. Because of his condition, he was placed in the highest priority bracket for his age and blood type.
The nationwide search for a heart began. That's when it hit the couple hard: They were waiting for something terrible to happen to someone else in order to help their son.
The weekend came and went without any suitable matches. So did the next one.
Nearly three weeks had gone by without any leads. Normally, the phone would ring periodically with organ candidates that a physician could accept or decline, but even the semi-suitable offers weren't coming.
Meanwhile, Jake turned more blue in color, and his body filled with fluids. He hadn't opened his eyes in weeks because of the paralytic drug that kept him from moving while on the ECMO machine. Children aren't meant to spend more than a week on the machine, but it was the only thing keeping him alive.
Thoughts for a transplant were being replaced with plans for a funeral.
The Hahns couldn't feel more helpless on June 7, 2001. They were starting to give up, and they feared Jake had given up, too. As they prepared to go to bed that night, the phone rang.
They knew it was the hospital. No one else would call that late.
Kent answered the phone. "Oh my God, this is over," Kristie thought.
But it was Dr. Bacha.
Kent turned to Kristie. "He's got a heart," he said.
It wasn't the best option, however. This baby was only 17 pounds, while Jake was closer to 24 pounds, a dramatic, but not insurmountable, disparity. Considering the circumstances, Bacha accepted it.
The heart was from a child in the San Antonio, Texas area. Every minute the organ is outside the body makes the procedure riskier. Six hours — the estimated time Jake's heart would be in transit — is like an eternity to physicians. Nothing short of flawless timing, from removing the heart in Texas to having Jake ready to receive it upon its arrival in Chicago, would be required to pull this transplant off.
Kristie and Kent arrived at the hospital at 4 a.m. to unexpected fanfare. When word of Jake's news got around, nurses were clapping and cheering.
The Hahns weren't sure how they were feeling. They knew June 8 would either go down on their calendar as an annual celebration day, or as the anniversary of their son's death. Kristie arrived crying, and the tears rarely stopped that day.
Kristie, holding her grandfather's rosary, took her place by Jake's side and prayed, like she had been doing every day. He had lain motionless with his eyes closed for three weeks, and she longed to see his beautiful blue-gray eyes again.
At about 9 a.m., the parade to the operating room began, as Jake, the ECMO machine, and all the other equipment connected to his tiny body were slowly wheeled away from intensive care. Already familiar with the drill of long surgeries, Kent and Kristie went to the courtyard to wait.
The updates came regularly. In the morning, they were working through the scar tissue to get into Jake's chest. Soon, they stopped his heart and had him on a heart-lung machine. A few hours later, Bacha had disconnected his heart.
Meanwhile, a cardiac surgeon had flown to San Antonio from Midway Airport in the middle of the night, removed the other baby's heart, and was flying back to Chicago. Because of the distance, the doctors did not have the luxury of having the heart in hand before they started Jake's surgery.
The new heart arrived at Midway in the early afternoon. It was the right size and a good fit in Jake's body. In the middle of the afternoon, the Hahns got the update they were praying for: the heart was in place and beating.
About 5 p.m., Jake returned to the intensive care unit. The ECMO machine, which for three weeks was both his ally and the bane of his existence, was conspicuously missing.
As Kristie met Jake and his entourage, she leaned over to kiss his head. Her son opened his eyes for the first time in three weeks. He was still bloated from fluids, but his color, which had always been a little blue, was now a fresh pink. His new heart was doing the job his old one never could.
Bacha smiled.
* * *
Maybe it's human nature to find signs and omens during crisis situations. A package awaited Kristie when she returned home that day following Jake's successful transplant. She opened it to find her grandmother's wedding ring, sent to her from a cousin living in New Mexico, for good luck.
Whether it was the work of God, a crowning medical accomplishment or a combination of the two, nobody can say. People didn't hesitate to call Jake a miracle baby, though.
Jake turned 3 last week, but his new heart celebrated its one-year anniversary a couple months ago.
It didn't take long after his transplant for him to start scooting on his bottom again. He stands with the help of something to grab, and he takes ginger steps while holding his parents' hands. He laughs and he fights with his brother.
Without seeing his scars, you wouldn't know Jake had been through four major surgeries.
It has by no means been an easy life since the transplant. A bed sore on the back of his head grew to enormous proportions while Jake was on the ECMO machine, and it took months for the wound to close. He even shook uncontrollably at times — withdrawal symptoms from the paralytic drugs.
His appointment schedule and medication regimen are unrelenting as well, though it has eased with time. Jake hates blood draws and cries at the sight of some doctors because he associates them with pain.
Still, he is a happy child. He hands out hugs like a salesman hands out brochures, he's never slow to flash his infectious smile, and laughing is second nature to him.
Jake will make the transition from Early Intervention to school-based therapy this fall. He has other challenges ahead of him — like learning to talk. Regardless of how far he advances, however, he is alive, and his parents understand that's all that matters.
Someday, they hope he will, too.
