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.
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.
“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.”
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.
“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.
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 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.
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.
Nico’s birth was full of surprises for his mom Jennifer. The biggest one being that he was a boy, when all along she had been expecting a daughter. That wasn’t the only unexpected realization. He lacked a fully-formed tibia (the bone that connects the knee and ankle), was missing his left thumb, and had several structural heart disease defects, none of which had been diagnosed prenatally.
Unexpected heart defects
Nico was diagnosed with unbalanced AV Canal (AVC), Anomalous Pulmonary Venous Connection (APVC), and Ventricular Septal Defect (VSD) due to the underdevelopment and abnormal formation of his heart and major blood vessels. Jennifer learned early on that her son’s heart conditions were treatable with a series of open heart surgeries and/or cardiac catheterization procedures.
The day after he was born, Nico was transported to CHOC Children’s. He underwent his first heart surgery when he was only three days old, under the care of Dr. Richard Gates, a CHOC Children’s pediatric cardiothoracic surgeon and co-director of the CHOC Children’s Heart Institute. Nico’s second heart surgery happened before his first birthday, and he’ll have a third heart surgery later this year.
“Despite Nico’s significant heart condition, he has responded well to each surgery, becoming stronger right before our eyes,” says Dr. Gates. “I look forward to his final surgery which should allow him to continue to be active and happy.”
Jennifer knew that someday Nico would need to have his leg amputated below the knee, Since Nico’s tibia bone connecting his knee and ankle wasn’t fully formed, it meant his ankle didn’t have the support it needed. Her original plan was to have that surgery performed at another facility closer to their home, when she was told they weren’t comfortable operating on his leg due to his heart conditions. Soon after, she and Nico were at CHOC meeting an orthopaedic specialist to discuss a prosthetic for his leg.
“I didn’t know what we were going to do, but during his prosthetic appointment, we learned CHOC would be comfortable performing his surgery, and could schedule it for just a few weeks later! I was thrilled we could do it earlier because I don’t want him to get to a point later on in life when he remembered or missed when he had more of a leg,” Jennifer recalls.
Shortly before his third birthday, Nico underwent surgery to amputate his leg below the knee under the care of Dr. Francois Lalonde, a CHOC Children’s pediatric orthopaedic surgeon.
After spending just one night in the hospital ―including a checkup with his cardiologist, Dr. Nafiz Kiciman, to make sure his heart looked alright after surgery― Nico was discharged.
“Nico is a brave and courageous young boy with an easy-going personality,” says Dr. Lalonde. “He has demonstrated great resilience and coping skills following surgery and wound care, and is already adapting well following the amputation of his left ankle.”
His doctors told his mom he should take it easy for a few days, but as soon as his cast was removed, he was quickly back to his old habits of climbing on furniture and jumping on pillows.
“I never want my son to have the mentality that he is limited,” Jennifer says. “Kids can sense if their parent is worried or scared, and I didn’t want him to feel like that. He’s too young to feel anxious on his own, so I made sure I didn’t bubble wrap him and let him play like his normal self.”
The importance of pediatric specialists for wound care therapy
The week after surgery, Nico and his mom started coming back to CHOC a few times per week for wound care, where specially-trained pediatric physical therapists would clean and rebandage his leg. In between those appointments, Jennifer cared for the wound at home.
“At his first appointment, he saw a new person walking into the room with all these supplies he had never seen before, and it was a lot for him to take in. He covered his eyes and wiggled around during the appointment,” Jennifer recalls.
To make Nico feel at ease, his physical therapist Sandy started letting him help her care for his wound.
“I wanted to be honest with him about what they’re going to do at this appointment, but frame it in a positive way so he knows it’s for his own good and he doesn’t have to be scared. It’s important to explain things in a way that he will understand,” Sandy says. “If you’re honest with the child, they’ll start to trust you. If you say, “this won’t hurt” and it hurts, then you’ve lost their trust.”
Once Nico was involved, he immediately understood that he didn’t have to be scared of wound care, and that it could even be fun.
Now, when Sandy finishes one step, he grabs the next supply that she’ll need. He almost sees it as a game. “Ok Nico, we’re done with this part. What part is next?” Sandy says to him.
These days, before each appointment, when Jennifer asks Nico if he wants to go see Sandy, he squeals and claps with delight.
“I tell him, “Let’s go see Sandy! She’s going to clean your owie and make it feel better.” My positive mentality was contagious for him. I was excited, so he was excited,” Jennifer says. “If the kid sees the parents squeamish during wound care or sees a worried expression on their face, then the kid will be apprehensive. Whenever we come to wound care appointments I make sure I speak to him in a very upbeat, positive way so he feels like physical therapy is fun and not something to be scared of, which helps him to relax. Positive energy is contagious.”
Continuity of care has also helped Nico feel more comfortable.
“It has helped him to have the same one or two physical therapists each time, so it’s a familiar face.”
Once Nico’s leg was fully healed from surgery, he resumed physical therapy, was fitted for a prosthetic, and started walking. In between all of that, he continues to love anything Star Wars, playing Ninja Turtles, coloring and attending pre-Kindergarten classes.
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.
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.”
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.
“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.
“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.”
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.
“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.
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.
After retiring from a storied career in corporate communications and marketing, Fran was looking to give back in her community. Having spent much of her adult life in Orange County, she was aware of the impact CHOC Children’s has had on the place she has called home for decades.
“When I was exploring volunteer opportunities at CHOC, I knew that I wanted a position where I could engage with patients,” Fran recalls. “When volunteer services described all that the Family Resource Center offers to patients and families, I knew it was the perfect place for me.”
Today, Fran staffs the Family Resource Center (FRC) located on the second floor of the Bill Holmes Tower. The FRC is a space where patients and families can read books and choose one to take home, rent movies, play games, utilize a computer lab, research their child’s diagnosis with medical and developmental literature, and decompress with arts and crafts.
Through her weekly shifts and as host of the FRC’s weekly Story Time, which is broadcast to all patient rooms within CHOC’s Orange campus, Fran has formed a special bond with 12-year-old patient Evelyn, who has been a CHOC Children’s patient for nearly her entire life.
Before she was born, Evelyn was diagnosed with complex congenital heart disease. Her heart defect is commonly associated with Trisomy 21 (more commonly known as Down syndrome). Two valves within Evelyn’s heart― the mitral and tricuspid valves― as well as the walls separating the heart chambers, did not develop correctly, which caused the right side of her heart to be underdeveloped and non-functional. Unfortunately, this condition can’t be “fixed,” only managed. This is typically done through three surgeries: the BT or Central Shunt, normally in the first days of life; the Bidirectional Glenn, usually between 3 and 9 months of age; and the Fontan, usually between 2 and 5 years.
In a typical heart, the right side of the heart pumps deoxygenated blood to the lungs, and the left side pumps oxygenated blood to the body. Because Evelyn’s right heart is too small to carry out its normal function, Evelyn has what’s referred to as single ventricle physiology. The purpose of the surgeries is to re-route the vessels around the heart so the one functional chamber pumps oxygenated blood to the body, and the deoxygenated blood bypasses the heart and passively drains back to the lungs.
Evelyn underwent her Central Shunt procedure at two months old to establish reliable blood flow to her lungs. Her Glenn procedure took place just after her first birthday to route the blood flow from the upper part of her body to her lungs. Her third heart surgery, the Fontan, took place when she was seven years old to re-route the remaining blood flow to her lungs. All three surgeries were performed by Dr. Richard Gates, director of cardiothoracic surgery at CHOC Children’s, co-medical director of the CHOC Children’s Heart Institute, and CHOC’s surgeon-in-chief.
In addition to check-ups for her heart every few months with pediatric cardiologist Dr. Pierangelo Renella. Evelyn makes visits to CHOC weekly for platelet transfusions. Along with her heart conditions, she was also diagnosed with idiopathic thrombocytopenic purpura (ITP), meaning she has low levels of platelets, a part of the blood that prevents bleeding.
Spending so much time in and out of the hospital and shuffling between doctor’s appointments could easily make a child scared of going to the doctor. That’s not the case with Evelyn.
“Everyone we’ve encountered at CHOC has been very kind to us. All CHOC staff is very patient with her and takes a lot of time with her,” says Evelyn’s mom Rosa. “I’m very thankful because thanks to CHOC, Evelyn is doing well.”
No matter the reason they’re visiting CHOC, Evelyn and Rosa always make a point to stop by the FRC, especially on days that Fran is volunteering.
“It makes me sad that patients are here long enough or often enough to get to know their names,” Fran says. “But I’m happy we get to offer them a distraction and sense of normalcy.”
Even though Evelyn doesn’t love reading, she loves being read to―especially by Fran. In addition to arriving early enough for appointments to make Fran’s Story Time, Evelyn loves playing blocks with her friend Fran as well.
“She feels right at home here in the FRC,” says Rosa. “Fran is very sweet to Evelyn and I appreciate everything she does for my daughter.
The admiration goes both ways.
“Evelyn is always so upbeat. Her energy is infectious. I sometimes feel that I get more out of volunteering than I give,” Fran says.
Being a bright spot in a patient’s day is what keeps Fran so connected to her role as a volunteer.
“If you can make a little one smile, then you’re doing something good.”