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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Cochlear Implants as a Journey to Hearing: Gracie’s Story

Since Kathleen had experienced a healthy pregnancy, she and her husband Mike were expecting a healthy baby girl.

“Out she popped, and everything was perfect,” Kathleen recalls. “When she failed her newborn screening test, we weren’t that concerned. We knew that babies could fail that test for a number of reasons, even if they have no hearing problems.”

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Baby Gracie

Two weeks later during a visit with her pediatrician, Gracie failed another hearing test. Her family was referred to Providence Speech and Hearing Center.

More testing provided a diagnosis: Gracie had severe to profound bilateral hearing loss. What hearing ability she did have would not be enough to allow her to speak and learn language.

“When I realized my daughter couldn’t even hear me tell her that I love her, I was a mess,” Kathleen recalls. “It felt like the world was falling apart in that moment.”

Gracie tried hearing aids, and although she was finally able to hear her parents say her name, the hearing aids were not enough to compensate for her hearing loss.

Since Kathleen is a special education teacher, she was already familiar with cochlear implants—surgically implanted devices that offer the hope of gaining the ability to sense sound.

“Deciding to pursue cochlear implants was a pretty easy decision,” says Mike. “We didn’t really give it a second thought. As a parent, you just want to give your kid every opportunity possible—especially the chance to hear.”

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Gracie was thrilled to get cochlear implants.

The cochlear implant artificially stimulates the inner ear area with electrical signals, which sends those signals to the auditory nerve, letting a person hear. The surgery is safest in most children around one year of age.

Meanwhile, Kathleen and Mike had been teaching sign language to Gracie and her older sister.

A few months after Gracie’s first birthday, she underwent bilateral cochlear implantation surgery at CHOC Children’s Hospital with pediatric otolaryngologist (ENT) Dr. Nguyen Pham.

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Gracie was excited and happy the day of her cochlear implant surgery.

“When I first met Dr. Pham, I felt like I had known him forever,” Kathleen says. “He answered every single question we had and was honest with us throughout the process.”

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Gracie’s parents were thrilled that her bilateral cochlear implant surgery went perfectly.

Kathleen knew the benefits for Gracie outweighed the potential complications that come with surgery, but as a parent, she was naturally worried about her child’s well-being.

“Every time I saw Dr. Pham I was frantic, and he was always so calming. It was nice to have that reassurance,” Kathleen says. “Dr. Pham is a miracle worker.”

Surgery went perfectly, and Gracie spent just one night in the hospital. A few weeks later it was time to officially activate her cochlear implants.

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Gracie was all smiles on activation day for her cochlear implants.

Even though Kathleen had faith the cochlear implants would work, but she was still anxious for activation day.

“Activation day was amazing and terrifying at the same time,” she recalls. “Everyone said not to worry, but I thought it might be too good to be true. I just kept thinking, ‘How could this little device be the thing that allows my baby to hear?’”

As soon as the cochlear implants were activated, Gracie’s face lit up and she pointed to her ears. Her parents let out a sigh of relief. When they took her home, they found the most joy in the small, mundane sounds around their house. Gracie could finally hear the front door open, the dog bark, and most importantly—her parents say I love you.

Before a child with cochlear implants will begin talking, they need time to get comfortable wearing their implants and undergo speech therapy. For Gracie, that time was just a few weeks. She still undergoes speech therapy twice per week, once at Providence Speech and Hearing Center and once at home.

To say Gracie is thriving is an understatement, according to Kathleen. Not only does Gracie talk and sing non-stop with her big sister Tess, but she mastered her ABC’s just shy of her second birthday. Her language skills now exceed most normal hearing children. Gracie loves going to Disneyland, and her family appreciates any opportunity to spread education about cochlear implants to inquisitive people they meet.

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At Disneyland and other places, Gracie’s family appreciates any opportunity to spread education about cochlear implants to inquisitive people they meet.

Gracie’s family has much to celebrate this year, but there’s something a few years down the road that Kathleen is most excited for.

“I am excited for her to go to kindergarten at a typical school. You have all these visions of your kids growing up, and for me to just drop my daughters off at one school is a big thing.”

Meet the cochlear implant team

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Stella’s Journey with Bronchopulmonary Sequestration (BPS)

By Sharlene Ramisingh, mom of CHOC Children’s patient Stella

My daughter Stella first came to CHOC Children’s when she was just a baby, but our journey really started before she was even born. At my 20-week ultrasound, my husband Havinder and I were so excited to find out the sex of our baby. We were having a girl!

That excitement quickly turned into a feeling of unease when the physician noticed an abnormal growth in the baby’s lung area. From that point on, we had to go back to the doctor’s office for monthly and eventually weekly ultrasounds to monitor the size of the mass. After additional imaging, doctors diagnosed her with bronchopulmonary sequestration (BPS). This means that abnormal tissue or a cyst formed outside her lung but wasn’t connected to her airway.

It was determined that the size of the mass wouldn’t affect delivery, and I was able to carry her to term and proceed with my birth plan. When Stella was born, there was a room full of doctors and nurses in case she wasn’t breathing. Time stood still when she came out and the room was so quiet. Everyone was waiting to hear her cry. And when she let out a tiny little sound, it felt like everyone in the room exhaled at once.

Despite the mass on her lung, she was healthy, so we got to take her home a few days after she was born. Stella’s first few months of life were filled with a series of appointments and tests. When we met Dr. Peter Yu, a pediatric general and thoracic surgeon at CHOC, he was able to give us confirmation that Stella needed surgery to remove the mass. At that point, Stella had grown bigger and stronger and was better prepared for the anesthesia required for surgery. We knew that our daughter was in good hands with Dr. Yu. He was patient, caring and we really felt like our daughter was the most important thing to him.

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Sharlene with her daughter Stella on the morning of surgery to remove her bronchopulmonary sequestration (BPS).

Stella was just five months old when she underwent surgery at CHOC Children’s Hospital. Thankfully, surgery went perfectly, and the whole team was great. The pediatric anesthesiologist was amazing and able to deal with my daughter’s small veins.

Before Stella’s diagnosis, I had never heard of BPS. During my pregnancy and up until the surgery I was part of a private Facebook group for parents of babies with BPS. Their success stories helped me manage the stress and anxiety that I experienced during this time. I was also able to ask questions to moms who went through the surgery and who had healthy kids. My husband and I were very private about her condition during my pregnancy, and only told our parents and siblings. We were dealing with our own emotions, and we just didn’t feel up to having to explain BPS over and over again. When it did come up, we would say she a birth defect that lead to a growth in her lungs, and that it would need to be removed.

These days, Stella is doing amazing. She sprints around the house with her five-year-old brother on a daily basis, and she has a voice that will make the windows shake. She goes to swim lessons every week.

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Stella continues to thrive after surgery to remove her bronchopulmonary sequestration (BPS).

The worrisome portion of my pregnancy and Stella’s surgery seem like such a distant memory. You would never know by looking at my daughter that she ever had any complications.

My advice to new parents facing an unexpected diagnosis is to take things one step at a time. Don’t get caught up in the negative thoughts that may happen and trust your child is in good hands with Dr. Yu and at CHOC. Always remember that this too shall pass.

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Big Sister Gives Back to Honor CHOC Children’s

Lily’s big sister, Makenna loved her before she was even born. She was actually the one who chose Lily’s name. Their bond only grew stronger when Lily was born with a rare chromosomal abnormality requiring specialized treatment and extra help at home.

Lily and Makenna
Lily and Makenna

“Even though Lily is different than Makenna and her brother, Ethan, they just see her as their sister,” says mom, Danielle. “They’ve helped Lily learn sign language and practice pronouncing words.”

Lily’s entire family has been involved in her care from day one. Makenna, age 11, would regularly tag along to Lily’s physical and occupational therapy sessions. She was keen to learn how she could help take care of her sister. She watched closely as physical therapist Cathy Lopez taught Lily how to move her body, and occupational therapist Lauren Newhouse taught her how to eat solid food (her favorite is avocado).

Seeing the way CHOC’s rehabilitation services team cared for Lily and the progress she made in therapy inspired Makenna to give back.

Makenna’s fundraiser

When Makenna’s school hosted a fair for students to showcase causes important to them, she chose to honor CHOC. She and a few friends watched YouTube videos to learn origami and created dozens of animal and emoji-themed bookmarks. She quickly sold out of them and donated the proceeds to the rehabilitation department at CHOC.

Lily backpack

“Raising money for CHOC is the least I can do to thank them for taking such good care of my sister,” Makenna says. “Whenever we are there for an appointment, all the doctors, nurses, therapists and security guards are so nice to us.”

Organizing this fundraiser has inspired Makenna to continue her philanthropic streak.

“It was feels good to do it and it was so easy. It makes me want to do more to raise more money for CHOC,” she says.

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Lily and Makenna present a Makenna’s fundraiser proceeds to physical therapist Cathy Lopez.

Lily’s diagnosis and early days

When Danielle and her husband, David, found out they were expecting their third child, they were thrilled. A few months into Danielle’s pregnancy, a blood test showed that Lily had Turner Syndrome, a genetic condition affecting a female’s growth and fertility. That turned out to be a false positive, but a follow-up amniocentesis correctly diagnosed Lily with Mosaic Trisomy 12, a rare chromosomal abnormality about which not much is known.

Lily was born full-term via C-section at a local hospital. Immediately after birth she had trouble breathing and turned blue, and she was quickly whisked away to that hospital’s neonatal intensive care unit (NICU).

Lily needed a ventilator to help her breathe. She also required use of a CPAP, also known as continuous positive airway pressure, which helped keep her airway open. Danielle pumped her breastmilk so Lily’s care team could nourish her through a feeding tube.

Just outside the hospital, CHOC geneticist Dr. Neda Zadeh was starting to make her way home for the day when she heard about Lily’s condition.

“Dr. Zadeh was on her way out, but she turned around and came back to the hospital to run more tests and get a better idea of what was affecting her,” Danielle recalls. “Dr. Zadeh is amazing.”

In the first few days of Lily’s life, her parents weren’t sure if she would make it or not. When she was 3 days old, she was transferred to CHOC’s NICU for a higher level of care.

Lily spent the next 24 days in the NICU. When she learned how to breathe and eat on her own, she was ready to go home for the first time.

While the family spent much of their daughter’s first month in the hospital, NICU nurses made sure the family didn’t miss out on their first holiday together — Father’s Day. With her nurses’ help, Lily created a special Father’s Day craft for David using her footprints.

These days, Lily sees a mighty brigade of pediatric specialists at CHOC on a regular basis. Although not much is known about her Mosaic Trisomy 12, her care team remains fully committed to ensuring she is safe and healthy. In addition to Dr. Zadeh, Lily is under the care of neurosurgeon Dr. William Loudon, endocrinologist Dr. Timothy Flannery, and otolaryngologist Dr. Nguyen Pham. She also sees a neuro-ophthalmologist at UC Irvine.

Danielle is proud of the progress Lily has made during therapy at CHOC and seeing how that progress sparked Makenna’s giving spirit makes her proud as well.

family

“Makenna probably doesn’t realize this, but Cathy and Lauren at CHOC really cared for our entire family, not just Lily,” Danielle says. “The entire rehabilitation team at CHOC was incredibly supportive to our entire family. They were the weekly and sometimes daily reminders that we could do it. They impacted our entire family in a way that words simply cannot express. When we look back on Lily’s first years, they are in our memories and always will be!”

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