Tarlie David a sonographer at UAB measures JJ in the womb for the last time estimating his weight at birth to be about 9 pounds. (Frank Couch\fcouch@al.com)
Crystal Burford wanted a big baby.
Jeremiah, her third, was scheduled to be born in just two weeks. This would be one of the final uneventful checkups for the unborn baby, who would be born fighting for his life.
Doctors diagnosed Jeremiah with hypoplastic left heart syndrome during an anatomy scan about halfway through the pregnancy. The severe congenital heart defect occurs when the left side of the heart doesn't properly develop. That prevents the circulation of oxygenated blood throughout the body, and would have been a death sentence 35 years ago.
But now there was hope. Surgeons could perform a series of three surgeries before Jeremiah turned 4. Babies diagnosed with hypoplastic left heart syndrome now have a 75 percent chance of surviving until age 1, and many now live decades longer than that. Doctors told Crystal big babies fare better than smaller newborns.
After birth, Jeremiah would need extensive medical intervention, followed by open-heart surgery in his first week of life.
His prognosis would largely depend on the outcome of the first operation, a risky and difficult surgery called the Norwood procedure. Surgeons would open Jeremiah's chest, bypass his heart and lungs, and painstakingly rebuild his tiny heart so the right ventricle can pump blood to the rest of the body. These lifesaving modifications would be measured in millimeters, and doctors wanted all the millimeters they could get.
"They want him to be at least eight pounds," Burford said.
Crystal and Jamey Burford drove to the Women's and Children's Center at UAB for a scheduled C-section birth of their son Jeremiah Burford who they call JJ. (Frank Couch\fcouch@al.com)
A devastating diagnosis
This, at least, seemed to be going in his favor. Although he would be born sick, Jeremiah would also be born big. The ultrasound technician estimated that his weight at birth would be about 9 pounds.
"Our goal is to get these babies to 39 weeks," said Dr. Richard Davis of UAB's Division of Maternal-Fetal Medicine. "Amazingly, these hearts do pretty well in the womb."
Crystal chose to deliver her baby at UAB, which is connected by sky-bridge to Children's of Alabama - the only hospital in the state where surgeons perform the Norwood procedure.
Jamey kisses Crystal as she is wheeled to the OR for the delivery, Jamey would later join her just before JJ was born. (Frank Couch\fcouch@al.com)
The Birth
Jamey Burford picked his son's birthday, April 8, because eight is his lucky number, having been born himself of August 8, or 8/8.
The family could use some good luck, considering the bad luck they had already endured. After all, fewer than 1,000 babies are born every year with hypoplastic left heart syndrome - and Jeremiah was one.
On Wednesday, April 8, the family checked in to the Women and Infants Center to deliver baby Jeremiah via C-section. Jeremiah is Jamey's first child, and Crystal's third, so although he was worried, the new father was also giddy with joy.
Jamey send Crystal into surgery with a kiss on the lips.
Jeremiah Burford arrived at 10:35 weighing in at 8lbs 12 ozs.(Frank Couch\fcouch@al.com)
Jeremiah weighed 8 pounds 12 ounces at birth, a big baby with fat cheeks, chubby thighs and a fuzzy thatch of newborn hair.
The delivery itself was routine, except for the team of doctors on hand to treat Jeremiah immediately after birth. The family barely got to see their son before nurses and doctors whisked him off to an adjacent room.
More than a dozen medical professionals descended on Jeremiah to check his reflexes and vital signs. Then they moved him to the regional neonatal intensive care unit inside the Women & Infants Center, the only one of its kind in the state.
A team of caregivers waited just outside of the OR for JJ to arrive, they gave him an initial assessment, score and prepared him for an intensive care unit. (Frank Couch\fcouch@al.com)
An army of doctors
The team caring for Jeremiah began to come together even before his birth, and included nurses, social workers, cardiologists, surgeons, intensive care doctors and obstetricians affiliated with UAB Maternal-Fetal Medicine. This fetal team met once a month at the Women & Infants Center to review upcoming deliveries.
This team approach continued after delivery, as doctors from both hospitals collaborated in the care of Jeremiah and his mother.
"Everyone works really well together," said Susan Barnes, who manages the cardiovascular operating room at Children's of Alabama. "If one of the staff notices something that the others should know about, they will let them know. We communicate very well."
Jamey watches as doctors begin lines for IV's and nurses attach monitors to Jeremiah inside an intensive care unit at UAB. (Frank Couch\fcouch@al.com)
Immediately after the birth, Jamey went to be with his son, while his wife was still in surgery. He felt torn between caring for his boy and his wife. The family was briefly reunited when staff members at UAB wheeled her bed over to the NICU for a visit with Jeremiah hours after the delivery.
The team at the Women & Infants Center inserted a central line, which could be used to administer drugs and fluids. His father, Jamey, watched from outside the room as doctors and nurses poked and prodded the howling newborn.
"I knew that the little man needed the wires, but I hated to see him going through it," Jamey said.
Doctors treated Jeremiah with prostaglandin. The hormone would keep open a passage in the heart that usually closes after birth. Babies born with undiagnosed hypoplastic left heart syndrome usually begin to struggle soon after the hole closes, and die within days if they do not receive surgery.
Crystal spends a few minutes with JJ in the an intensive care unit at UAB before he is transferred to Children's of Alabama. (Frank Couch\fcouch@al.com)
Teams from UAB and Chidren's of Alabama prepare to move JJ across a bridge that connects the two hospitals to continue his care as they prepare for his first in a series of surgeries. (Frank Couch\fcouch@al.com)
The transfer to Children's
Three hours after his birth, the team from UAB's neonatal intensive care unit moved Jeremiah's incubator across the bridge that connects the hospital to Children's of Alabama. Nurses from both hospitals transferred each piece of equipment until Jeremiah was stabilized in the pediatric cardiac intensive care unit at Children's of Alabama.
The prostaglandin kept the oxygenated blood flowing to Jeremiah's body until surgery could be performed. While Jeremiah got settled at Children's, his mother recovered at the Women & Infant's Center a short walk away.
Emotions are overwhelming as Crystal and Jamey spend a few tender moments with JJ before his is wheeled away for his first surgery. (Frank Couch\fcouch@al.com)
An imperfect heart and a lifesaving surgery
The Norwood procedure, developed in the 1980s to treat babies with hypoplastic left heart syndrome, is the most technical and risky surgery performed on newborns' hearts at Children's. The only other available treatment for these babies is a heart transplant, which will last about 20 years, said Dr. Robert Dabal, a pediatric cardiothoracic surgeon.
"When you do a heart transplant, you start a clock ticking," Dabal said. "We would much rather have Jeremiah use the heart that he has, which isn't a perfect heart, but is a good one."
Six days after his birth, Jeremiah underwent surgery at Children's of Alabama. His parents knew the next few hours and days would be critical for Jeremiah's survival, that he would leave surgery with his sternum cracked open - wearing a patch to cover his healing heart.
Medical personnel from the cardio vascular operating room prepared to take the baby to the operating room on the morning of his operation. They paused while Crystal and Jamey Burford prayed for Jeremiah.
All attention is focused on JJ's heart as Dr. Dabal and Dr Cleveland work on young JJ. (Frank Couch\fcouch@al.com)
Fixing a tiny heart
Dabal has been fixing babies' hearts for about 10 years, and performs about five Norwood procedures a year. The night before surgery, he visualizes the operation. Jeremiah won't survive without the Norwood, but the surgery also carries a high level of risk. Many babies who die from hypoplastic left heart syndrome die immediately before, during or after the first operation. The Norwood Procedure has the lowest survival rate of the most common open heart surgeries performed at major pediatric heart centers.
Doctors practice surgery for years before they can tackle cases as complicated as Jeremiah's. Dabal said he feels the pressure every time he treats a baby with hypoplastic left heart syndrome.
"I think that you get used to the pressure, but you always feel it," he said. "It's a baby's life that you're talking about."
All attention is focused on JJ's heart as Dr. Dabal and Dr. Cleveland work on young JJ. (Frank Couch\fcouch@al.com)
Dr. David Cleveland, a pediatric cardiac surgeon, assisted Dabal during the operation.
For six hours, Dabal and his team operated on Jeremiah's tiny heart, a critical organ about the size of a ping pong ball. Dabal added a patch to his undersized aorta and used a 6 millimeter shunt - which is about the size of a stack of three nickels - to connect the right ventricle to the pulmonary artery. The procedure reworks the plumbing inside the heart, to bypass the underdeveloped left ventricle.
If successful, the Norwood procedure will allow the right ventricle to perform double duty, pumping blood to the lungs and to the rest of the body, until the lungs are mature enough to do more of the work.
The oldest surviving patients who received the Norwood procedure and two subsequent surgeries are entering their 30s. No one knows how long they may live with their modified hearts.
Dr. Waldemar Carlo, Jr. is Jeremiah's cardiologist, and a professor at the UAB School of Medicine.
"They are left with a right ventricle pumping to the body," Carlo said. "The heart is not designed to do that for a normal lifespan."
Crystal Burford stands over Jeremiah three days after surgery amid a maze of wires, tubes, pumps and monitors in his room. (Frank Couch\fcouch@al.com)
The long recovery
Bed side and operating room nurses facilitate the transfer of the patient between the intensive care unit and the operating room. Nurses must work seamlessly during the hand-off from surgery to recovery.
"There is massive, massive preparation to get this patient back to the intensive care unit and for the ICU to be prepared," said Laura Needham, director of nursing for cardiovascular services Children's.
Doctors induced a coma during the first few days of recovery, which Jeremiah spent buried in wires and tethered to machines that ventilated and fed the ailing boy.
A day and a half after surgery, the team closed Jeremiah's sternum the baby started down the road to recovery.
Carlo will monitor Jeremiah's heart after surgery, seeing the baby every two or three weeks to check his health.
JJ wraps his hand around his dads finger as Jamey holds his first born for the first time. (Frank Couch\fcouch@al.com)
Jeremiah's recovery was not without setbacks. He suffered three collapsed lungs and struggled to tolerate the formula fed directly to his stomach. During the next four weeks, he gradually got stronger. He traded a ventilator for a CPAP, and then a nasal cannula. His eyes opened more often. He looked for his daddy's voice whenever Jamey spoke.
Jamey, an avid Alabama football fan, hung a Crimson Tide onesie in his son's room - in the hope that his son would grow large enough to fill it out by the time he went home.
Before and after surgery, Jeremiah's parents had few opportunities to hold their newborn son.
"Every time I hold my baby, it's an act of Congress," Crystal said. "At six days old I had held him four times and Jamey has held him twice."
Jeremiah spent more than four weeks in recovery on the fourth floor of Children's. His scar faded to faint purple on his chest and he gradually shed most of the tubes and wires streaming from his body. As he grew stronger, his parents got to hold him more often.
Before Jeremiah can be discharged he and his family have to demonstrate they can care for him for 24 hours without assistance from the nurses and caregivers. (Frank Couch\fcouch@al.com)
The road ahead
By mid-May, Jeremiah's doctors began to prepare for discharge. Crystal and Jamey would be outfitted with a baby scale and pulse oximeter to measure Jeremiah's oxygen levels. Once a day, they would weigh the baby and check his oxygen level, and record it in a book. The program, called Hearts at Home, helps parents manage their children's health after discharge, in the critical period between the first and second surgeries.
"We know that if a baby does gain weight or has a significant drop in oxygen saturation, we will bring those babies back to the hospital," Carlo said.
During the months between the Norwood and the Glenn, Jeremiah's condition will still be unstable, Carlo said. After the Glenn, which will happen in the next three to six months, Jeremiah's health should improve substantially, Carlo said. The final surgery between age one-and-a-half and four years old will bring his oxygen levels to near-normal levels.
During the last two days, Jeremiah's parents roomed-in with their son, taking care of him in the hospital room under the supervision of medical staff. Jeremiah also underwent a car seat test, where he sat in a car seat for one hour to make sure his oxygen levels wouldn't drop.
Jeremiah has a serious look to him during a party celebrating his discharge. (Frank Couch\fcouch@al.com)
Over the next several months, Jeremiah will be particularly vulnerable to infection. A minor cold could quickly turn critical. An organization called Sisters by Heart, which supports families with hypoplastic left heart babies, sent a care kit to the family that included a warning sign to hang on his carrier, warning strangers not to touch the fragile boy.
Children who survive all three open heart surgeries may always be a little smaller than their peers, Carlo said. And although they can play sports, high-intensity sports such as football will be off-limits, he said.
"Our goal for him is to have as normal a childhood as possible," Carlo said. "These children can be active, they can do youth sports."
The staff at Children's threw a party for JJ before he went home and was the center of attention with Dr. Kimberly Jackson and surgeon Dr. Dabal celebrating with family and friends the day Jeremiah is discharged. (Frank Couch\fcouch@al.com)
When Jamey thinks about his hopes for his son, he recalls the Bible passage that inspired his name, Jeremiah 29:11. "For I know the plans I have for you," declares the Lord, "plans to prosper you and not to harm you, plans to give you hope and a future." Someday, he hopes his son can be an inspiration for other children with heart defects.
On Friday, May 15, the staff at Children's threw a party for Jeremiah. Nurses, doctors, surgeons, therapists and a social worker waited in a conference room for Jeremiah. Crystal carried him in, wrapped in a receiving blanket just like any other baby, without wires or monitors.
"We celebrate babies like Jeremiah and families like yours because this is not an easy journey," said Dr. Kimberly Jackson, a doctor in the cardiac intensive care unit. "It takes all these people to get home."
Carlo and Dabal took turns holding Jeremiah. Crystal praised the medical team at Children's.
"I love them," she said. "I mean, they fixed my baby's heart."
Having spent 37 days in the hospital Crystal and Jeremiah ride to the first floor of Children's of Alabama for the first time to be driven home by family members who have been supportive throughout the pregnancy, birth, surgery and recovery. (Frank Couch\fcouch@al.com)
After the party, Crystal and her family loaded the car with the oxygen tanks, high-calorie formula, medical supplies and medicine they would need to take care of Jeremiah at home for the next several months. Then finally, Crystal carried her little miracle out the door in his car seat.
Caring for Jeremiah at homes requires round the clock vigilance, Jamey said. When his son grows large enough for the Alabama onesie, he may also be large enough for his second surgery. After that, Jamey said he hopes life can become a little more normal.
"I hope that he just grows up to be a regular little boy for right now," Jamey said. "That he's able to do little boy things and play in the yard. I want him to have a fun time and enjoy his little life."
Updated at 2:01 p.m. with correct title for Laura Needham.