A surprise heart defect diagnosis in utero: Teddy’s story

By Elle Kennedy, mom of CHOC Children’s patient Teddy

When I got pregnant with my son Teddy, my husband and I planned to follow the route we took during my pregnancy with our daughter Charlotte—a midwife and practice we were already familiar with. In the years since our daughter had been born, that office had implemented a new rule—all babies must undergo a fetal echocardiogram, an ultrasound test during pregnancy to evaluate the heart of an unborn baby.

My pregnancy had been normal, so when I went in for this fetal echo around 23 weeks, I didn’t even have my husband Jeremy come with me. We were grateful for the precautionary test, but we didn’t expect for them to find anything abnormal.

What they found was anything but normal.

Teddy was diagnosed with anomalous pulmonary artery off the aorta. Typically, the blood is supposed to go through the heart and then into the lungs and then back through the other chamber of the heart. Teddy’s diagnosis meant that one of his lungs was attached in the wrong order. This lung was getting blood that was already oxygenated by the other lung, which can make the misaligned lung really sick. The lungs are not supposed to receive oxygenated blood; they’re supposed to oxygenate the blood.

Sometimes this condition isn’t caught in utero, and the baby seems healthy at birth, but a few months down the road they have breathing issues and the detective work to find a diagnosis begins. We were fortunate that this was diagnosed before Teddy was born, so we could be prepared.

Making a new plan

I quickly understood that the rest of my pregnancy would look different than my first, and that my birth plans would change. One of my first thoughts was, “I need to find a doctor who can deliver at St. Joseph Hospital in Orange, so that we can be right across the street from CHOC.”

We found an OB/GYN and were also referred to Dr. Nita Doshi, a pediatric cardiologist at CHOC who specializes in fetal cardiology.

In the weeks leading up to Teddy’s birth, we had a big meeting with Dr. Doshi; Dr. Richard Gates, Teddy’s cardiothoracic surgeon; many other CHOC doctors; my OB-GYN and the St. Joseph labor and delivery team. There were 15 people in the room with us, ready to share the game plan for Teddy’s birth and immediate care after he was born. Everybody went around the room, in order of what would happen to me and Teddy. It was like “OK, when you come in, here is going to be the birth team. Once he’s born, here is the team taking over. When he goes into surgery, here’s that team.” Everybody got to walk us through what they were going to do and what the stages of care would look like. That experience was amazing for us. It was really nice to be able to meet everyone ahead of time.

This was an entirely new experience for us. We had a lot of fear going into it. As a parent, you hear open heart surgery and it sounds so scary. Getting to talk to people on the team and learn about their area of expertise, and have them tell you what to expect, was a very relieving experience for us. They thought through every step of the journey.

After a lengthy but healthy delivery, Teddy was born weighing seven pounds, 14 ounces. After a quick check-up to ensure his heart was doing OK and he was breathing properly, we were able to spend 30 minutes with him before he needed to be taken to CHOC.

Teddy spent four days in the cardiac neonatal intensive care unit (NICU) at CHOC before undergoing open heart surgery.

During this two-and-a-half-hour surgery, Dr. Gates removed the right pulmonary artery from the aorta and transferred it to its proper position on the main pulmonary artery.

Caring for the whole family

After surgery, Teddy spent two weeks in the cardiovascular intensive care unit (CVICU). We would visit him every day, and then go home in the evenings to put our daughter to bed. We knew our son was in good hands, and we wanted to make this time as normal as possible for his big sister.

Although the hospital staff was there to care for Teddy, they helped us prepare our daughter for her first hospital visit, as well. When Teddy was first brought to the CVICU, he was hooked up to all kinds of machines and wires. That can be scary for siblings to see. They encouraged us to wait until he was closer to going home, so that it would be a more positive experience for Charlotte. We also knew that Teddy would return to this hospital many times for other procedures and check-ups, and we didn’t want Charlotte to think of a hospital as a scary place. When she did come in, it was amazing. She loved getting to see her baby brother, and all she wanted to do was hold him. The staff was so attentive to her. Even though this wasn’t the way we intended to welcome our son into the world, it turned out to be an amazing experience.

To thank the hospital staff, and to help Charlotte feel more involved in her brother’s care, we brought small felt hearts on safety pins for her to give out to his nurses and doctors. She walked around the unit handing them out and said, “Thank you for taking care of Teddy.” It’s a tradition we’ve continued at appointments to this day.

When Teddy was around 3 weeks old, we finally got to bring him home. His sister was so happy! He had a bandage on his chest so he couldn’t do tummy time right away, but other than that it was a normal routine.

Teddy’s first angioplasty

Three months later, we came back to CHOC for an angioplasty. Scar tissue was building where they had stitched the artery in place, so the artery wasn’t growing properly. During this procedure, they made a small incision in an artery in Teddy’s thigh, inserted a small balloon and snaked it up to his heart, and slowly expanded the balloon in order to safely break up the scar tissue and gently stretch the artery. This time, we only had to spend one night in the hospital for observation.

Teddy had a follow-up angioplasty when he was three years old. He will need another angioplasty in another three to five years, depending on how his artery grows in relation to the rest of his body. Someday, he will have a stent (a tiny mesh tube) put in to permanently keep his artery open.

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Teddy playing with toys before an angioplasty,

Throughout this process, CHOC has been amazing for us because they’re willing to take our say into consideration. Our team always asks us, “Are you comfortable with this plan?” We have been involved in the decision-making progress every step of the way.

Teddy today

Teddy is smaller than the average 3-year-old boy. Personally, I think it’s a blessing that he’s growing a little slower than other kids his age. It’s giving his heart time to grow at a slower pace because the rest of his body isn’t growing as fast. Other than his smaller than average stature, he’s a healthy kid. He underwent occupational and physical therapy for a while to help him meet milestones, but he graduated from those therapies at 18 months and now he is hitting or exceeding all of his milestones.

These days, we see Dr. Doshi every six months for a check-up. Despite many appointments and procedures over the last few years, Teddy has no fear of the doctor. When he sees a Choco Bear logo on a billboard, he points and says, “Mom that’s where I went and had my operation. They took care of me there.”

If you look at him, you wouldn’t know he had a heart condition. Unless you see him shirtless and notice his scar, you would think he was just a typical kid. He talks well for his age, and he loves anything with wheels. He’s a very brave young boy, which we knew from day one.

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After his last angioplasty, Teddy requested a photo shoot dressed as his favorite superhero. Bumblebee Boy.

A message to other moms

To any other moms with hospitalized babies, know that you are not alone. For me, the hardest part of this journey was not knowing anyone else whose baby was hospitalized after birth. Know that there are other parents out there who understand what you’re going through.

This journey could have been a scary experience for our family, but I never imagined it could actually be a positive experience. I thought we would just have to get through this, but Teddy has so much fun laughing and playing with the nurses who take care of him before and after procedures.

CHOC has done a really great job of thinking through everything from a patient perspective and a parent perspective. They try to create the best possible experience from start to finish for everyone involved. They make it a reassuring process for parents, a happy experience for siblings, and a safe experience for patients.

At CHOC, everyone’s goal is to make a child’s experience a positive one.

Learn more about the Heart Institute at CHOC Children's

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Contending with Coarctation of the Aorta: Ella’s Story

Lindsey and Oliver Nam had hoped to hold a special celebration to mark their daughter Ella’s 100th day of life – a tradition in some Asian cultures to commemorate an important milestone for a family.

But a big celebration with friends and family would need to wait.

Shortly after birth, Ella was diagnosed with coarctation of the aorta, a congenital heart defect. While the family awaited next steps, they couldn’t risk her being in a large crowd and getting sick.

“It was hard,” Lindsey says. “That’s the scariest thing to think about. You just brought them into the world and now something is wrong.”

The journey begins

Tests conducted just after Ella’s birth in December 2017 first revealed a heart murmur, an unusual sound made by blood circulating through the heart’s chambers or valves, or through blood vessels near the heart.

After two extra days in the hospital, Ella had progressed nicely and the new family of three headed home. On the horizon were follow-up medical appointments to determine whether Ella’s murmur was harmless or caused by an underlying heart condition.

At home, Ella was gaining weight, doing well and reaching milestones – so when a pediatric cardiologist made his diagnosis, her parents were surprised.

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Coarctation of the aorta means that a large artery in Ella’s heart that carries oxygen-rich blood from the left ventricle to the body was too narrow. This can restrict the amount of blood that could travel to the lower part of Ella’s body.

If the condition went untreated or the aorta didn’t widen on its own, Ella’s heart would need to work harder and harder to pump blood, leading to a possibility of stroke or heart failure.

But because Ella was healthy and doing well, the family considered alternate timelines for treatment and sought second opinions.

A big decision

When Ella was about 7 months old, though, surgery became a reality. After more tests, experts at the CHOC Children’s Heart Institute recommended Ella undergo surgery to correct the problem as soon as possible.

“It was hard to hear,” Lindsey says. “That’s when we started to get worried.”

Dr. Richard Gates, a pediatric heart surgeon and co-medical director of the Heart Institute, would perform the procedure. By working through Ella’s back, near her shoulder blade, he would widen the narrow artery.

Heading into surgery day, Lindsey and Oliver were frightened, but maintained a brave face for their daughter.

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Ella recovering from heart surgery in CHOC’s cardiovascular intensive care unit (CVICU).

“Going into it and preparing for it, I was strong,” Lindsey said. “I didn’t want her to think anything was wrong.”

The procedure went well, but Lindsey’s strong façade crumbled when she saw Ella afterward.

“The worst part was when I saw her after the surgery,” she says. “Everything was fine but seeing her sedated and with all these tubes in her – oh, my goodness. It was a lot to handle. I broke down.”

Recovering

Ella was in great hands at CHOC’s cardiovascular intensive care unit, where she spent five days recovering after surgery. By the fourth day, Ella was able to keep down milk and even ventured outside for a wagon ride. On the fifth day, the family of three headed home.

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Just four days after surgery, Ella was well enough to venture outside for a wagon ride. Here she is pictured with her heart surgeon, Dr. Richard Gates.

“It’s the best feeling ever,” Lindsey said.

Several months after surgery, Ella has had some follow-up visits and there remains a chance that she may need surgery again someday.

For now, though, she is healthy and right on track developmentally. Ella is crawling and pulling herself up on furniture, with first steps nearly within reach.

Rather than worry about the future, the Nams are mindful to focus on Ella’s happiness and health – and they’d offer the same advice to another family contending with a heart defect.

“I would just say remain calm and just take it one day at a time,” Lindsey says. “Also, do your research and try to get second and third opinions.”

Time to celebrate

The family has also been making up for some lost opportunities. In lieu of the big 100th day celebration the Nams never got to host, they went big when it came to commemorating Ella’s first birthday a few months ago.

After all, they had so much to celebrate.

“She’s accomplished so much,” Lindsey says.

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Ella’s first birthday celebration
Learn more about the Heart Institute at CHOC Children's

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Ventricular Septal Defect: Veer’s Story

Veer’s father Jatin spent many sleepless nights searching the internet for ventricular septal defect (VSD), a condition his son has endured since birth. He was nervous awaiting his son’s upcoming appointment for a heart surgery consultation and wanted to know everything he could about VSD.

Veer was born at a local hospital and diagnosed with VSD at birth. Veer was monitored regularly by a physician, and when he was 18 months old, his parents transitioned his care to CHOC Children’s cardiologist Dr. James Chu to monitor the VSD.

“Dr. Chu kept on an eye on Veer to make sure he was doing well. He got regular echocardiograms, first every two to three months progressing to every six months and then every year since he was getting better,” says Veer’s mom, Sweta.

VSD is a hole between the heart’s lower chambers allowing blood to pass from the left to the right side of the heart. The oxygen rich blood then gets pumped back to the lungs instead of out to the body, causing the heart to work harder. Often, small VSDs can close on their own.

An echocardiogram, also known as a cardiac ultrasound, uses sound waves to create pictures of the heart. It shows the structure of the heart and its parts and how well they’re working.

A few months before Veer’s fourth birthday, Dr. Chu said the hole in his heart was no longer improving and that it might need to be surgically repaired. The hole was very close to the AV nodes (the natural pacemaker of the heart) and the tricuspid valve was regurgitating which can lead to right-sided heart failure.

Dr. Chu referred Veer to CHOC Children’s pediatric cardiothoracic surgeon Dr. Richard Gates for a consultation. Dr. Gates saw the echocardiogram and knew right away Veer would need surgery to repair the VSD.

“Before we were able to meet with Dr. Gates, we had so many sleepless nights thinking about what could happen if Veer needed heart surgery. But after meeting Dr. Gates, we felt comforted and confident about moving forward with surgery. He explained every step of the surgery to us and answered all of our questions,” says Sweta.

Surgery Day

Within days, Veer was at CHOC Children’s Heart Institute having surgery to repair his VSD.

Veer’s surgery would require a biograft, which is like a patch, that will usually cause the valve to fall back into place. If that didn’t happen during the surgery, Veer would need more surgery done to repair the VSD at the time of the operation.

The surgery went well, and Veer only needed the biograft. “Dr. Chu told us recently that the surgery was done so well and precise that you can’t even tell on the echocardiogram where the graft was done,” says Sweta.

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Veer recovering from surgery to repair his ventricular septal defect at CHOC.

“Our experience with CHOC was amazing. All of the doctors, nurses and child life specialists took care of Veer like their own family member,” Sweta says. “The child life specialists were so friendly. Veer loves bubbles and cars, so they brought him bubbles and the Disney Cars movie. He got to hold the iPad and watch the movie before the surgery which made him so happy. After surgery, they even brought a larger bed in the room so that I could sleep next to him.”

Today, Veer is full of energy and thriving.

veer-today
Today, Veer is thriving.

“When he used to play with his toy cars, he would lie on his side because he didn’t have a lot of energy,” Sweta says. “Now he has so much energy that he doesn’t have to do that, and he’s able to run around with his older siblings.”

Learn more about the Heart Institute at CHOC Children's

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Ask a CHOC Doc: Are Heart Palpitations Dangerous?

Question: Sometimes I experience heart palpitations. I want more information on what exactly causes them and if I can do anything to reduce their frequency. At what point should I mention this to my doctor?  –Anonymous

Answer:

A heart palpitation is the feeling of your heart beating too fast, skipping a beat, or fluttering in your throat, chest or neck.

Palpitations, also known as irregular heartbeats, are most likely caused by non-heart-related triggers. Strenuous exercises as well as strong emotions of anxiety or stress, most common among the younger generation, are frequent causes. Consuming caffeine, nicotine, alcohol, certain herbal supplements, cold and cough medication, or asthma medication, which all contain stimulants, as well as illegal drugs such as cocaine are common triggers. Some people report having palpitations after certain heavy meals that contain large doses of carbohydrates, sugars or fat. Sometimes, eating foods with excessive amounts of sodium can bring them on as well. However, some palpitations are caused by actual medical conditions including cardiac arrhythmias, thyroid disease, anemia, low blood pressure, fevers, and dehydration. If palpitations ever occur with chest pain, exercise, or fainting, this could represent a cardiac arrhythmia, and you should notify a physician.

Changing your diet is another key factor in reducing heart irregularities. Cutting back on caffeine intake such as tea, coffee, sodas, and energy drinks can be quite effective.  As a replacement, drink more water. Dehydration can lead to decreased blood pressure which triggers the body’s compensatory response of adrenaline which makes your heart race. Staying hydrated throughout the day can keep your heart rate normal.

If your heart is racing unexpectedly, there are also several things you can try at home to reduce your heart rate. Vagal maneuvers are ways to send anti-adrenaline signals to your heart and blood vessels. These maneuvers include “bearing down,” blowing through a straw, forceful coughing, and placing ice or cold water on your forehead or neck, among others. Sometimes even headstands accomplish the vagal maneuver! These actions stimulate the vagus nerve, which is the nerve that connects your brain to your heart and helps control the rate at which your heart beats. If these maneuvers make your palpitations stop abruptly, that is a good thing, but it also means that you are likely to have a primary cardiac arrhythmia, like supraventricular tachycardia which requires further evaluation and treatment. If palpitations continue despite vagal manuevers, contact your physician or seek medical care.

Palpitations are most likely harmless; however, it is important to know when it is time to see your doctor. When palpitations are accompanied by severe shortness of breath, fainting, chest pain or discomfort, or dizziness seek immediate medical attention. When your heart is beating too fast, it causes your blood pressure to drop which could potentially lead to fainting. Fainting due to low blood pressure can be a possible sign of a heart problem such as a cardiac arrhythmia, congenital heart disease or a heart valve defect. Palpitations accompanied by these other symptoms are also known to have been related to cardiac arrest, strokes, coronary artery disease, heart muscle problems and heart failure.

Here are some helpful questions to keep in mind before seeing your doctor:

  • Is the onset abrupt or gradual?
  • Check your pulse once your heart starts palpitating. Is your heart beating fast or slow? Is it beating at regular intervals or irregularly?
  • During palpitations do you feel dizzy, short of breath, or experience chest pains?
  • How often do your palpitations occur and for how long?
  • What are you doing when they start? Exercise? Calm and relaxed?

Is there anything else unusual about when your palpitations start? Are you sick with fevers, vomiting or diarrhea? Have you consumed less water that day? Are there other stressful and/or unusual events occurring in your life?

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Dr. Anthony McCanta

-Dr. Anthony McCanta, a pediatric cardiac electrophysiology specialist at CHOC Children’s

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Surprise Heart Defect Diagnoses at Birth: Noah’s Story

When Noah was born last May, his parents Lauren and John were expecting a healthy baby boy. They were shocked to learn that prenatal ultrasounds had missed his pulmonary atresia with intact ventricular septum (PA-IVS), a condition where the right side of the heart is underdeveloped, and there is no connection from the heart to the lung, compromising blood flow to the lungs and other parts of the body.

Noah’s pulmonary and tricuspid hypoplasia means that he was born with birth defects of the pulmonary and tricuspid valves, which control blood flow to the right side of the heart and eventually to the lungs. He was also diagnosed with a right coronary artery fistula, an abnormal connection between the coronary artery carrying oxygen-rich blood to the heart.

“When I was pregnant, I did everything I was supposed to do to grow a healthy baby. I gave up caffeine, ate well, and took the stairs every day to the ninth floor until I was 33 weeks pregnant,” says Noah’s mom Lauren, who is an occupational therapist at CHOC Children’s.

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Noah and his mom Lauren, who is an occupational therapist at CHOC Children’s.

The evening Noah was born, he had low oxygen and platelet levels and was brought to the neonatal intensive care unit within the hospital where he was born. Dr. James Chu, a CHOC Children’s pediatric cardiologist who was making rounds that evening, suspected Noah had a heart defect and ordered a cardiac ultrasound, or echocardiogram, a non-invasive procedure used to assess the heart’s structure and function.

Dr. Chu returned to Lauren’s room as soon as he had a better idea of Noah’s diagnoses, even though it was 3:00 a.m. He knew Noah’s parents wanted to know what was wrong as soon as possible.

“He drew us diagrams and gently explained Noah’s exact heart defects, their severity, and detailed the surgeries he would have to endure,” Lauren recalls.

Dr. Chu told Lauren and John their son needed a higher level of care.

“He gave us a few options of where we could transfer Noah, and when I told him I really wanted to go to CHOC, he reaffirmed my choice,” Lauren recalls. “Once we arrived at CHOC, another cardiologist, Dr. Ahmad Ellini, confirmed the diagnoses, explained everything again, and answered all of our questions.

“We didn’t have a lot of time to think about a game plan immediately after he was born,” Lauren says of Noah’s surprise heart conditions. “But I knew that CHOC was the best place for him to be.”

noah-mom-lauren-occupational-therapist-feeds-bottle
As an occupational therapist at CHOC, Lauren has been caring for patients for years. She never imagined her son would become a CHOC patient shortly after he was born with surprise heart defects.

Surgery for PA-IVS

Noah spent a week in CHOC’s NICU before undergoing his first in a series of three heart surgeries. That first week was an emotional rollercoaster, Lauren recalls. Noah’s team of neonatologists, Dr. Amir Ashrafi, Dr. John Cleary and Dr. John Tran, helped his parents remain calm.

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Noah’s dad reads him his favorite book as he recovers from surgery to repair heart defects in the CVICU.

“The team of neonatologists were great. They answered all my questions, spent lots of time with us, and were super available― even if I had a question at 2:00 a.m. Everyone on his care team was very collaborative,” Lauren recalls, adding that she found the attention to detail and calm nature of Dr. Richard Gates, director of cardiothoracic surgery and surgeon-in-chief at CHOC, very comforting. “Dr. Gates knows his patients through and through. Even though I have a medical background, I’m still a parent. He describes things in a way my husband and I understand, especially when we’re sleep deprived and scared.”

Babies with PA-IVS typically undergo three procedures:

  • Blalock-Taussig (BT) shunt: a surgeon inserts an artificial tube to aid blood flow to the lungs. This procedure is usually done in the first week of life.
  • Glenn procedure: Usually done between 4–6 months of age, this operation allows blood returning from the upper part of the body to flow directly to the lungs without passing through the heart. Now the left ventricle only has to do one job, pumping blood to the body.
  • Fontan procedure: Typically occurring between 2 and 4 years of age, this surgery connects the pulmonary artery and the inferior vena cava (vessel returning oxygen-poor blood from the lower part of the body to the heart), allowing the blood coming back from the lower body to go to the lungs. Once this procedure is complete, oxygen-rich and oxygen-poor blood no longer mix in the heart. The surgeon may leave a small connection between the oxygen rich and oxygen poor chambers (a fenestration).

Lauren describes Noah’s surgeries to her family as a “miracle bandage” since they will not make PA-IVS go away. Noah may need a heart transplant someday.

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When Noah was recovering from surgery, his CVICU nurses helped him get ready for visitors by dressing up his bandages.

“When Noah was born his heart was the size of a walnut. Each of these surgeries are temporary, and it’s Noah’s job to keep growing, and eventually, to outgrow each of these repairs and need the next one,” she explains.  “Unfortunately, these surgeries cannot make his heart “normal” and he’ll always have serious heart disease, but we’re so grateful we have these operations to give him the best chance possible.”

After his first surgery, Noah spent five weeks in the cardiovascular intensive care unit (CVICU) at CHOC. For the first 48 hours of his recovery, he required extracorporeal life support (ECLS) (also known as extracorporeal membrane oxygenation or ECMO), a special procedure that takes over the heart’s pumping function and the lungs’ oxygen exchange until a patient can recover from injury or illness.

“I knew there was a possibility he’d need to be on ECMO after surgery, but it wasn’t something I allowed myself to think about,” Lauren says. “It was hard to see him hooked up to so many machines and be so fragile. Dr. Joanne Starr did an extraordinary job managing Noah’s care while he was on ECMO and she also cared for us as his parents too. She checked on Noah at all times of the day and night, and even ordered me to go take a walk in the butterfly garden to get a break from being at his bedside 24/7.”

Dr. Starr, director of ECMO and medical director of cardiothoracic surgery at CHOC, has long been committed to caring for a patient’s entire family.

“In caring for children and teens, it’s vital that we as physicians remember we are not only taking care of the patient, but the parents as well. Parental stress and anxiety may have an effect on the patient and the healing process. If parents aren’t practicing self-care, they might not have a clear enough mind to be able to understand their child’s condition and make decisions on their behalf,” explains Dr. Starr. “Having a family’s full support is an important part of the healing process, and something that goes a long way in ensuring a positive long-term outcome for my patients.”

After five weeks in the CVICU, Noah’s parents were thrilled to be able to bring their baby home for the very first time. But a mere 30 hours later, they were readmitted to CHOC as Noah fought a central line infection.

Things calmed down a few weeks later. He went home, continued growing, and started hitting developmental milestones and developing a big personality. During the next few months, the family was still coming to CHOC as frequently as a few times per week for blood and platelet transfusions. Ever since Noah had a low platelet count at birth, his parents knew that he would need transfusions― they just didn’t know how many. That turned out to be as many as three transfusions per week.

Lauren and her dad had a history of donating blood. For instance, if they were at a hospital visiting a family member, they would always go find the blood donor center and give “because it was easy and it was just the right thing to do,” she says.

“I always knew that donating blood and platelets was important, but having a baby who needed blood and platelets changed my respect for what a gift it really is,” Lauren says. “When my baby needed to go on oxygen, and then they gave him a red blood cell transfusion and all of a sudden, he doesn’t need supplemental oxygen anymore, it’s a game changer. To literally watch a kid who couldn’t oxygenate well on his own, suddenly not need help breathing because of a blood transfusion, is amazing.”

Over the past several months, Noah has been able to meet several of the donors who have given him much-needed blood and platelets.

“It is so humbling to meet his donors. Whenever we come to the Orange campus for appointments, we visit the blood donor center and have gotten to meet and thank some of his donors,” Lauren says. “The people who give regularly are my heroes. Being helpless and not being able to cure your child is heartbreaking. We rely on these strangers and their generosity. They don’t know us but they help us.”

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Noah meeting one of his platelet donors Kathleen, a CHOC staff member.

The need for regular donors ―platelets especially― is so great because the shelf life on blood products is not long. Red blood cells have shelf life of 42 days, but platelets only have a shelf life of 5 days, half of which is taken up by necessary safety testing before a patient can receive the donation. That means there is a window of about 48 hours where patients can receive donor platelets before they expire.

Direct donations, when blood and platelet donations are earmarked for specific patients, are an important way to safeguard patients who need ongoing transfusions, as they help minimize the number of different types of blood products they are exposed to during treatment. This will also help to improve Noah’s chances of being matched for a heart if he needs one in the future. Lauren outlined the ways donors helped her son in handwritten thank you notes she asked the Blood & Donor Services staff to distribute to his directed donors.

When Noah was about five months old, he underwent a cardiac catheterization procedure to determine if his heart was ready for the next surgery. This was standard protocol before part two in his series of surgeries, the Glenn procedure.

“It never crossed my mind that more bad news was coming because he looked ok. We thought he was doing fine,” Lauren says.

During Noah’s cardiac catheterization, his team noticed that the fistula in his heart had grown significantly in size. Noah’s “lucky fin” (as Lauren refers to his right ventricle) grew, which wasn’t good news for the left, healthy side of his heart. The weaker side of his heart was stealing space, blood and other resources from his stronger side. The discovery prompted the question, “Do we rush him into the Glenn procedure or go straight to a heart transplant?” ― a conversation his parents were not prepared for at the time.

“I didn’t even know what to hope for. Do we hope for the Glenn, or do we hope we find a new heart and a transplant goes well?” Lauren recalls. “His team told us to hope that his heart lasts as long as possible.”

His cardiology and hematology teams at CHOC rushed to help the family coordinate second opinions at other institutions within just a few days. They also helped the family coordinate a transplant evaluation, a three-part process to determine if the patient is medically qualified and the family emotionally prepared to care for a transplant patient.

“With invaluable input from a Southern California pediatric transplant team, and after multiple phone and in-person conferences amongst all his caregivers and his family, it was decided that Noah’s best chance at a positive outcome would be to have his Glenn procedure at CHOC,” recalls Dr. Ellini. “I have never worked at an institution that can so quickly mobilize to make sure that patients obtain the best care possible. It is even more amazing that our team at CHOC has the ability to use its regional resources to optimize the care of our complex patients like Noah.”

The consensus was clear―Noah needed a second surgery, and he needed it to go perfectly, or else he would need a heart transplant.

“That week rushing to get second opinions was a whirlwind,” Lauren recalls. “My husband and I were basically looking for any reason to stay at CHOC for surgery. Not only did we have complete confidence in Dr. Gates, but Noah’s entire care team has always treated him like he was their own child. There were so many people at CHOC totally invested in his care― everyone from cardiology, hematology, blood and donor services, the CVICU, everyone.”

After surgery, which went well, Noah stayed in the CVICU for 10 days before going home.

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With every appointment at CHOC comes a special visit to the Blood & Donor Services Center to thank blood and platelet donors for helping kids like Noah.

“I didn’t realize how hard he was working to just survive until after his second surgery,” Lauren says. “I couldn’t see how hard his heart was working to do anything because he was still happy, growing and meeting developmental milestones. But now I can just tell he feels so much better. He has more energy to play and skills are coming to him more easily now. It’s really amazing to see.”

The reason why Noah required platelet transfusions for the first few months of life remains a mystery. Thankfully, he hasn’t required platelets since his second surgery, when he was almost six months old, and his care team remains hopeful this is something he’ll grow out of.

The reason his platelets continue to be low remains a mystery, but Noah is slowly improving under the close watch of his hematology team, including Dr. Diane Nugent, Dr. David Buchbinder, Dr. Arash Mahajerin, Dr. Amit Soni, Dr. Victor Wang and Dr. Geetha Puthenveetil. Noah has an affinity towards Dr. Puthenveetil, whose last name means ‘Newhouse’ (Noah’s last name) in her home language. His family remains hopeful Noah won’t need any more transfusions, and his directed donors can now donate to help other CHOC patients in need.

Noah’s third open heart surgery, the Fontan procedure, will happen in a couple years.

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At a recent appointment, Noah’s fan club at CHOC welcomed him with a special message on the exam table.

Even though Lauren has been a valued CHOC employee for over four years, she knows the high level of care her son has received isn’t due to special treatment.

“We are treated like family here not because I work here, but because that is how CHOC treats all patients.”

Today, one-year-old Noah is “defying all odds in terms of cardiac babies,” his mom says. He is very curious, always alert, and loves flirting with his favorite nurses.

Learn more about the Heart Institute at CHOC Children's

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