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
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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.

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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.”

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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.

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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.

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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

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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.

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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.

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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.

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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.’”

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Warming Up to Wound Care: Nico’s Story

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.

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Nico, who is today a happy pre-K student, has already endured several surgeries.

“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.

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In addition to stocking our holiday toy store, donations that pour into our annual holiday toy drive bring joy to patients year round. After one wound care appointment, Nico is surprised with a new toy for being so brave.

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.

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Left: Nico was naturally apprehensive during his first wound care appointment, before he realized it didn’t hurt and could even be fun. Right: Nico during a subsequent wound care appointment, where he was rewarded for his bravery with a larger-than-life Mickey Mouse balloon.

“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.

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To help calm Nico’s fears during wound care, his pediatric physical therapist involved him in the process.

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.”

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Nico’s Star Wars-themed prosthetic.

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.

Read FAQs about pediatric wound care at CHOC

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