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