A Surprise Diagnosis of Transposition of the Great Arteries: T.J.’s Story

By Buffy O’Neil, mom of CHOC Children’s patient T.J.

When my youngest son T.J. was born, we were so excited to complete our family. His nursery was ready to go and we had picked out the outfit he’d wear home from the hospital. I’d even done genetic testing, and everything about T.J. looked fine.

I wanted to deliver my son at St. Joseph Hospital in Orange because I knew if anything happened, which I didn’t expect it to, CHOC Children’s was right next door. I drove all the way to St. Joseph when I was in labor so that we’d be close to CHOC if we needed them.

Even though T.J. was a bigger baby than we anticipated and I had a rough delivery, everything looked fine right after he was born. A minute later he started to turn blue. They thought it was because of the rough delivery. They gave him some oxygen, but he turned blue again. That’s when we knew something was wrong.

My delivery team paged a neonatologist from CHOC who rushed over to evaluate him. Within minutes, there was a neonatologist in our room, and then T.J. was on his way to the neonatal intensive care unit (NICU) at CHOC, and my husband went with him. That neonatologist suspected there was a problem with T.J.’s heart, and brought in two pediatric cardiologists, Dr. Fahrouch Berdjis and Dr. James Chu. Dr Chu performed an echocardiogram, also known as a cardiac ultrasound, and diagnosed T.J. with transposition of the great arteries. They reassured me that I hadn’t done anything wrong during my pregnancy that caused this. TGA is due to abnormal development of the fetal heart, which occurs during the first eight weeks of pregnancy.

The heart has four chambers and four valves. The problem in transposition of the great arteries is that the two main arteries leaving the heart come off the wrong pumping chamber. This makes the blood flow incorrectly and makes the baby sick at birth. In order for babies with TGA to have a chance at survival, they need a diagnosis within hours of birth, and then an immediate procedure called a balloon artrial septostomy. During this procedure, a special catheter with a balloon in the tip is used to create an opening in the wall between the left and right atria, to improve the mixing of oxygen-rich and oxygen-poor blood.

On the first day of T.J.’s life, nothing happened as we had planned, but everything happened as it should. I can’t even imagine what it would’ve been like if something had gone wrong. I thank God every day that T.J. was born at St. Joseph Hospital and CHOC was right next door. The doctors told us that many babies that have to be transferred to a children’s hospital to be evaluated have died in the process. Because T.J. was born at a facility that’s literally connected to CHOC (via an underground tunnel), he was able to be diagnosed and had his procedure done within hours.

The first week of T.J.’s life was really scary. His right lung collapsed and then got a little stronger, only to have his left lung collapse. Babies with TGA need to have their first open heart surgery in the first week of life, and we weren’t sure if he was even going to live long enough to have that surgery. Doctors, nurses and respiratory therapists did everything they could to stabilize him.

Everything was very overwhelming. The baby who we had expected to be perfectly healthy was lying in a hospital crib with a lot of wires and monitors attached to him. He was so close to dying. All we could do was sit, watch and pray he would survive.

Every doctor we met with was so patient and explained everything in a way we could understand. We were overwhelmed and just trying to take it all in.

After a scary six days, T.J. was stable enough to undergo his first open heart surgery called an arterial switch. During that procedure, Dr. Richard Gates pediatric cardiothoracic surgeon and co-medical director of CHOC’s Heart Institute, cut open T.J.’s chest, cut his arteries and switched them so they were in the correct spot, and then stitched them into place.

Five hours after we kissed our son goodbye and saw him being wheeled back into the operating room, Dr. Gates came out to meet us in the waiting room. He said the surgery had been difficult but overall it went well, and now everything was all up to baby T.J.

In the week after T.J.’s open-heart surgery, he wasn’t breathing as well as we hoped he would. His team decided to do a cardiac catheterization procedure to determine why he still had a murmur and issues with his oxygen levels. During that procedure, they placed a wire, mesh device called a stent in a narrowed artery to keep it open and improve blood flow.

After this, T.J.’s oxygen levels improved, but his heart and lungs had been through a lot and needed to grow stronger before we could go home. Our next big hurdle was getting T.J. to eat on his own. Our feeding specialist was surprised at how fast he caught on to sucking and swallowing, but because his body was working so hard to recover from surgery, he was burning more calories than he was taking in. The doctors decided it was best to place a percutaneous endoscopic gastronomy tube (more commonly known as a G-tube or feeding tube) directly into T.J.’s stomach so he wouldn’t have to work so hard to eat, but he’d still get the calories he needed to grow stronger.

Another unexpected hurdle was when T.J. started having withdrawals from the pain medication he had been placed on after surgery. A registered nurse/pain specialist was assigned to us in order to evaluate how best to help T.J. cope. She put him on a 30-day weaning plan and after a few days he started improving, and soon we were on our way home!

We were so happy to finally take our baby home, almost six weeks after he was born. We went home with a lot more than we came with, including a medication schedule with six different drugs outlined by his nurses, plus a breathing machine.

We knew T.J. would need another open-heart surgery before his first birthday. We had periodic appointments with his cardiologist Dr. Chu, and a feeding specialist as well. A few months later, Dr. Chu performed a cardiac ultrasound or echocardiogram to see how T.J.’s heart was working. He told us that even though T.J. had grown a lot and was healthy, it was time for his surgery. His arteries were not growing enough to keep up with him.

T.J.’s second open heart surgery occurred when he was six months old, and he had a third surgery when he was 18 months. Each of those were to expand arteries to allow for better blood flow. As a parent, I always appreciated Dr. Gates’ honesty. He told us that these were intense surgeries, and that T.J. would need blood transfusions during them to compensate for blood loss during surgery.

after-heart-surgery-transportation-of-the-great-arteries
T.J. after surgery

After the second and third surgery, we spent a week in the cardiovascular intensive care unit (CVICU) at CHOC. We were blown away at how the nursing staff cared for our entire family during that time. We were putting our son in their hands, but they didn’t just take care of him. They took care of all of us. Years later, the nurses that cared for him when he was a baby still want to hear updates about T.J. and how he’s doing in school.

For the first 18 months of T.J.’s life, it felt like we were at CHOC all the time. It was an unexpected start to my son’s life, and at the time, it was devastating. I felt like I was the only mom experiencing this, and if there’s any moms reading this I want you to know that you’re not alone. There are people out there who understand what you’re going through. Ask your child’s care team if they can put you in touch with other families who may be willing to share their stories with you. For my husband and me, being able to meet people with the same conditions, and to share stories with other adults who have been through what you’ve been through was incredibly helpful.

TJ-today
Today, T.J. is a happy eight-year-old who loves sports and is a straight-A student.

Today, T.J. is a happy eight-year-old. He’s very athletic and loves to play sports, and I’m proud to say he is a straight-A student. When he feels self-conscious about his scars, we remind him how special and strong he is. Even though his life has been filled with several surgeries and countless doctor’s appointments, he has no anxiety about going to the doctor’s office because he’s always treated so well there. When T.J. was a baby we saw Dr. Chu monthly, and now that his heart is doing so well, we only have to go twice per year.

As for my heart, I have a very special place in it for CHOC― especially for Dr. Gates and Dr. Chu. Both of them are in photos in T.J.’s room. We remind them at every appointment that they are the reason T.J. is alive.

Learn more about the Heart Institute at CHOC Children's

Related posts:

Keeping it in the Family: Why a Disneyland Resort Cast Member Joined the CHOC Walk

For Andrew Geis, participating in the annual CHOC Walk in the Park is only natural.

After all, Andrew credits CHOC Children’s with saving his daughter’s life, and the annual fundraiser takes place throughout his office – the Disneyland Resort.

Cumulatively, the Disneyland Resort has been CHOC’s largest corporate donor over the past 25 years, and the annual CHOC Walk in the Park is the hospital’s largest fundraiser, raising more than $32 million to date.

“I feel a sense of pride that an organization I’ve been with for 17 years has such a strong relationship with CHOC, which has done so much for my family,” says Andrew, who is part of the Disneyland Resort’s catering and convention services team. “The CHOC Walk is a small way that we give back and recognize the incredible care that we had at CHOC.”

Many Disneyland Resort cast members who have been personally impacted by CHOC participate every year. Last year, the Disney VoluntEARS walk team raised more than $90,000 for the hospital.

The Geis family’s relationship with CHOC began even before baby Sawyer was born. Imaging conducted while she was in utero revealed two possible heart defects, the severity of which wouldn’t be known until she was born.

The family started planning, and immediately after her birth, Sawyer was transferred to CHOC’s neonatal intensive care unit. There, further testing revealed a constricted aorta; an atrial septal defect (ASD), or a hole between the top chambers of her heart; and a ventricular septal defect (VSD), or a hole between the heart’s lower chambers.

Geis parents_at CHOC
Andrew and his wife Michelle with their daughter Sawyer shortly after she was born.

Sawyer would need surgery – and she’d need it quickly, specialists told Andrew.

“I don’t think you’re actually ever prepared to hear that when your child is less than 24 hours old,” he says. “It was like a kick to the heart.”

Within days, Sawyer underwent surgery to repair the defects. Dr. Richard Gates, co-medical director of the CHOC Children’s Heart Institute, and Dr. Joanne Starr, medical director of cardiothoracic surgery at CHOC, fixed the constriction in her aorta, partially closed the ASD, and placed a band around Sawyer’s pulmonary artery to equalize pressure in the two sides of her heart and force the blood to flow to the lower half of the body.

While the surgery was a success, recovery in the cardiovascular intensive care unit was tough. There, Sawyer had an irregular heartbeat, which required the activation of an external pacemaker. Then, she also developed a blood clot. That same day, Sawyer experienced a three-minute seizure.

The clot and seizures were successfully mitigated, and a CT scan following the seizure showed no signs of a stroke or blain bleed. But Sawyer remained in the CVICU healing, growing and learning to eat on her own for several weeks.

During her stay, Andrew and his wife, Michelle, took shifts, alternating who stayed with Sawyer and who went home to their 5-year-old daughter, Parker. When Parker came to the hospital to visit, CHOC staff made a point to connect with her and ensure her needs were also met, Andrew recalls.

“My wife and I felt very strongly that it wasn’t only about the care Sawyer received, but that the entire family was taken care of,” he says. “That level of compassion and total family care was evident in all interactions with team members of CHOC.”

And finally, after 32 days, a 1-month old Sawyer headed home to join her family.

Sawyer headed home from CHOC
After spending just over the first month of her life at CHOC Children’s, Sawyer was finally able to go home.

During their time at CHOC Children’s Hospital, the Geis family became increasingly aware of the long relationship between the heath system and Disney, from Walt Disney’s early fundraising efforts before the hospital was built to the Disneyland Resort’s $5 million gift toward construction of the new Bill Holmes Tower, which houses the interactive “Turtle Talk with Crush” show donated by Walt Disney Imagineering.

“I certainly enjoy working for Disney and all that it represents, and knowing that Disney is affiliated with CHOC Children’s, which did so much for my family when we were in a medical crisis – I think is a unique blend,” Andrew says.

Just after Sawyer’s first birthday, the family participated in its first CHOC Walk, now a family tradition that will continue at this year’s walk. “Team Sawyer” will strut proudly on Aug. 26, joined by its spunky and sassy, 3-year-old namesake, who knows exactly why they walk.

Geis family at CHOC Walk 2017
The Geis family, including Team Sawyer’s namesake, at CHOC Walk 2017.

“Sawyer will point to her scar and she’ll say, ‘Tell me about my scar,’” Andrew says. “We’ll talk about her heart and what was wrong with it and what had to be done with it. ‘Who was with me in the hospital?’ she’ll ask, and we’ll tell her, ‘We were all with you in the hospital.’”

Register Now!

Related posts:

Tetralogy of Fallot Patient and his Family Give Back

What was supposed to be a routine visit with their pediatrician on a seemingly typical Friday morning changed Stephanie Harding and her son Trent’s life forever when their day ended with a diagnosis of Tetralogy of Fallot.

As the pediatrician wrapped up his checkup that morning, he noticed 5-week old Trent’s forehead appeared somewhat purple. He tested his oxygen saturation to find the baby’s oxygen level was low and would need to be seen by a cardiologist immediately for further examination.

A few hours later, the cardiologist determined that Trent had Tetralogy of Fallot, a heart condition made up of four related congenital defects that occur due to abnormal development of the fetal heart. Those three words were as foreign and shocking to Stephanie as the turn of events that day.

“I remember thinking we had only packed enough food and supplies for the baby for a trip to the pediatrician and back. Here we were now, at a hospital, hearing the cardiologist explain what his condition involved and everything else went blank. All I could hear is that Trent had four heart defects that needed to be repaired immediately,” Stephanie says. “We asked for prayer right away; we were in disbelief.”

A few days later, Trent had open heart surgery at Loma Linda University Children’s Hospital to repair the four heart defects.

trent-recovering-tetralogy-of-fallot-surgery
Trent recovering after his first surgery, just days after being diagnosed with Tetralogy of Fallot.

“He’s our miracle baby,” Stephanie says. “It’s a miracle that we caught his condition in time. There had been no signs or symptoms before that appointment with his pediatrician.”

In 2017, Trent, then 6 years old, underwent a second surgery, as is typical for a child with Tetralogy of Fallot, to replace the pulmonary valve with Dr. Richard Gates, a pediatric cardiothoracic surgeon and co-medical director of the CHOC Children’s Heart Institute. Stephanie and her husband, Tim, remember having to explain as best they could to their little boy what was about to happen to him once again.

“Trent is quiet and goes with the flow, yet I didn’t know how he would take it. He was so brave through it all,” Stephanie says. “I still remember as he was being wheeled into the operating room and I finally had to let go of his hand so he could go in; he looked up from the gurney and looked at me, with the anesthesia just starting to kick in, and I thought he was surely going to start crying or screaming. Instead, he just gave me this confident look like, ‘I’ve got this Mom, it’s going to be OK,’” an emotional Stephanie recalls.

The surgery was successful, and the Hardings couldn’t be more thankful with the remarkable care and compassion Trent received from CHOC staff, many of whom they keep in touch with today.

Stephanie and Trent occasionally stop by the cardiovascular intensive care unit (CVICU) at CHOC to say hello to the nurses and doctors who took care of them, and drop off gifts for other families going through what they’ve experienced. Stephanie had provided goodies to the hospital, including the CVICU, long before her son was treated at CHOC. Now, giving back to the CVICU means more than ever.

“It feels great to let another parent know they’re not alone, and that there are resources and groups out there that will support them,” Stephanie says.

Trent, now 7, puts it simply. It makes him so happy, he says, to be able to give back to other kids like him.

harding-family-Tetralogy-of-Fallot

The Harding family’s passion to help others doesn’t stop at CHOC. They are very active locally, raising awareness and funds for the congenital heart defect (CHD) community, through groups like Hopeful Hearts Foundation, an organization for families with children affected by CHD.

On Feb.  23, the Hardings are hosting a fundraiser at GritCycle, an indoor cycling gym, with the proceeds benefitting CHOC. Participants can buy a ticket to cycle at the Monarch Beach location.

Trent will need another surgery to replace his pulmonary valve in about five years. For now, he is an active first-grader who enjoys math and jiu-jitsu. He also loves hanging out with his family, including his brothers, Stephan and Dylan, who are very supportive of their ‘miracle baby.’

Learn more about the Heart Institute at CHOC Children's

Related posts:

A Reunion of Heroes: Katie’s Story

After recently being discharged, Katie Flathom stopped by the CHOC Children’s cardiovascular intensive care unit (CVICU) again to make some introductions.

Suddenly, the 16-year-old has a group of heroes in her life: the coach who resuscitated her on school campus and the CHOC team that treated Katie for three weeks and will continue her care as she navigates life with a newly diagnosed heart condition.

During a recent athletic conditioning class in school, Katie collapsed and went into sudden cardiac arrest.

Her trained and quick-thinking conditioning coach snapped into action and resuscitated Katie with CPR until paramedics could arrive and transport her to CHOC.

“It was the longest 10 minutes of my life,” said Greg Vandermade, Katie’s coach at Mater Dei High School who also credits other students for alerting him to Katie’s condition and calling 911, as well as fellow staff who assisted by obtaining an automated external defibrillator (AED) to shock Katie’s heart into a normal rhythm.

At CHOC, Katie continued to have irregular heartbeats that required further defibrillation and cardioversion, procedures that help restore the heart’s natural heart rhythm, said Dr. Anthony McCanta, a CHOC cardiologist.

Katie also went on extracorporeal life support, a treatment that takes over the heart’s pumping function and the lungs’ oxygen exchange until a patient can recover from injury. This allowed the CHOC Children’s Heart Institute team to continue to treat her life-threatening arrhythmias with medication, Dr. McCanta said.

Dr. McCanta performed an electrophysiology study procedure and implanted beneath Katie’s skin a subcutaneous implantable cardioverter defibrillator, a device that helps prevent sudden cardiac arrest in patients.

After Katie’s discharge and further testing, she was diagnosed with Arrhythmogenic Right Ventricular Dysplasia, or ARVD.  A rare type of cardiomyopathy  where the muscle tissue in the heart’s right ventricle is infiltrated and replaced by fatty tissue and scar tissue, ARVD weakens the heart’s ability to pump blood and makes the heart susceptible to life-threatening arrhythmias.

The diagnosis also means Katie, a cross country and track athlete, will need to give up running for good.

“It was hard at first,” she said.

But instead of sitting on the sidelines, Katie’s decided to pick up golf, a sport that’s compatible with ARVD.

Katie has even begun incorporating a golf swing into her physical therapy sessions at CHOC, and she had two clubs in tow as she, her family and coach Greg visited the CVICU recently.

heroes
When Katie came back to visit the CVICU team and reunite her heroes, her CHOC care team presented her with a heart-shaped pillow, which they all signed with well wishes.

There, Dr. McCanta and the CHOC team presented Greg with a plaque recognizing him for his swift response and efforts that surely saved Katie’s life.

“Coach Greg responded to Katie with CPR on the spot and saved her life that day,” Dr. McCanta said. “His heroic actions, and those of Katie’s schoolmates and staff, including obtaining and appropriately using the AED, are the reason that Katie is alive today.”

Katie’s story underscores the importance of being trained in CPR and in the use of AEDs, Dr. McCanta said.

“Having AEDs in schools and training staff and students in CPR with an AED are some of the most important interventions that we have in saving lives of young people experiencing sudden cardiac arrest,” he said.

Getting AEDs installed in schools is among the goals of CHOC’s Life-Threatening Events Associated with Pediatric Sports – or LEAPS – program.

Coincidentally, Katie’s own grandmother, a nurse and health services coordinator in the Irvine Unified School District, has collaborated with LEAPS and helped get AEDs installed on her district’s campuses.

“Never did I think though that this would happen to one of my own family members,” said Marcia, Katie’s grandmother.

Learn more about pacemakers and cardioverter defibriilators

Related posts:

Medical Emergency Disrupts Family’s Holiday Plans

For most American families, the Fourth of July ignites thoughts of fireworks, barbecues and outdoor fun with friends and neighbors. For the Thompsons, the summer holiday sparks memories of nurses, doctors and the intensive care unit.

It was July 3. The Thompson family, Eric, Kristen and their two young daughters Kaylee and Sarah, were getting ready to celebrate Independence Day in their close-knit Los Alamitos community. Eight-year-old Sarah was feeling a little under the weather, but was just as eager as the rest of her family for the festivities to begin.

Without warning, party preparations were interrupted by a horrific scream. Kristen raced to find her youngest seizing on the floor. She called 911, and the ambulance brought Sarah to the nearest emergency department. There, Sarah’s high heart rate led doctors to believe she had an arrhythmia. They decided to transport the young patient to CHOC Children’s Hospital.

Shortly after arriving at CHOC, Sarah’s health dramatically declined. Her heart stopped. The cardiovascular intensive care unit (CVICU) team took turns performing CPR with high quality compressions on Sarah for 95 minutes, while another specialized team placed her on ECMO (extracorporeal membrane oxygenation), state-of-the-art technology that supports the heart and lungs by taking over the heart’s pumping function and the lung’s oxygen exchange.

The platinum-level Extracorporeal Life Support Organization (ELSO) Award of Excellence in Life Support recognizes programs worldwide that distinguish themselves by having evidence-based processes, procedures and systems in place that promote excellence in ECLS. As a recipient of a platinum designation, CHOC is among the highest scoring institutions.

Eric and Kristen watched as 15-20 physicians, nurses and respiratory care therapists worked on their daughter; those performing CPR would rotate after a couple of minutes at the direction of critical care specialist Dr. Francis Kim. After she was placed on ECMO, Sarah was taken to the cardiac catheterization lab, where she was diagnosed with myocarditis. Inflammation of the middle layer of the heart wall, myocarditis can affect both the heart’s muscle cells and its electrical system. It’s usually caused by a viral infection and if severe, can weaken the heart’s pumping action. Sarah’s family was in disbelief.

ecmo
Sarah was placed on ECMO for five days. ECMO (extracorporeal membrane oxygenation) is a state-of-the-art technology that supports the heart and lungs by taking over the heart’s pumping function and the lung’s oxygen exchange.

 

“Sarah seemed to have just a mild cold, and nothing that would have prevented her from enjoying our holiday plans. It was surreal seeing her in the ICU, hooked up to all of the equipment and surrounded by so many specialists,” recalls Kristen.

CHOC kept Sarah on ECMO for five days. Her family and friends waited around the clock, hoping the feisty girl they loved would come out of the traumatic ordeal okay. Hospital staff offered encouraging words, telling Sarah’s loved ones that she was clearly a fighter. And, a scan of her brain looked good, though a complete neurological evaluation couldn’t be completed until she was awake.

Kristen got the first glimmer of hope when, after her daughter’s breathing tube was removed, Sarah said, “I want to go home.”  Soon, Sarah started to get bossy with her family members – a sign she was on the mend. Even better, her heart recovered and she suffered no neurological injuries.

Dr. Kim, the primary physician overseeing Sarah’s care, credits the skill and expertise of CHOC’s CVICU staff for her outcome — acknowledgement shared by the Thompson family. “We have an incredible, high-functioning team with vast experience in caring for the most critically-ill children. In Sarah’s case, it took skilled teamwork to put her on ECMO while performing CPR,” explains Dr. Kim. “There’s no room for error. We are thrilled she’s doing so well and has returned to pursuing all of her favorite activities.”

ecmo
Sarah has returned to all of her favorite activities, including taking dance class.

A straight A student, Sarah loves to read. Her dog and sister rank high on her list of favorites. She also takes jazz and hip hop classes, and plays soccer. And, she still looks forward to holiday celebrations with friends and family.

Related posts: