Lifespan – Just Like Me, in D.C.

One of the most powerful moments at the Congenital Heart Legislative Conference can come not when shaking hands with one of our nation’s leaders, but when you have the chance to connect with someone just like you, just like your child, an everyday CHD survivor. Ken Woodhouse, and adult CHD patient with tetralogy of fallot, Melanie Toth, and her son Luke, a young patient with tetralogy of fallot, share their thoughts on just such an experience in this Q&A.

 

Ken and Luke in Washington D.C,

 

PCHA:  Tell us a little about yourself. How has CHD impacted your life?

Melanie:  I was born and raised in Chicago. I married my best friend Ben at age 23. After Ben was involved in a bad motorcycle accident we realized, tomorrow isn’t promised and wanted to start a family right away. At our 20 week ultrasound we heard the words that change the world for so many families, there is something wrong with your baby’s heart. Before CHD, I never thought about volunteering and how you can help others on almost a daily basis. CHD has opened my eyes, my heart, and changed me for the better. I remind myself of that on days when CHD can really suck!

Ken: I was born in 1981 with tetralogy of Fallot and had my first open heart surgery at eight months old. Like many young adults (especially those, like me, who were asymptomatic), I thought I was “fixed” and fell out of cardiology care in late high school / early college. Between that time and 2011 (at the age of 30), when I literally fell back into care after a bicycling accident, I never really thought of myself as a heart
patient. But since that accident, I have gotten very involved with the CHD community as a volunteer and
advocate. In 2014, I had my second open heart surgery to replace my original pulmonary valve, which
had been leaking since birth.

Having a CHD has forced/reminded me to make my health a priority and to focus on the many positive
aspects of my life. (It can be a challenge to do so from time to time, especially when one lives with a
chronic health condition.) At the same time, being a part of the CHD community has introduced me to
some of the strongest, bravest, and most amazing people I know – many of whom have become dear
friends; and I am forever grateful for those connections.

PCHA: What brought you to D.C.? What inspired you to speak to Legislators on Capitol Hill and what were you hoping to accomplish?

Melanie: After Luke was born, I began questioning so many things about why this happened. I didn’t want to dwell on the negative. I found that helping other heart families with support really helped me. After many years of offering support to heart families, I wanted to do more and Legislative Conference was always on my “bucket list” to attend. Many friends both heart parents and patients attended this conference and felt so empowered after coming home. I wanted to feel that way too. I was hoping that sharing our heart journey would help heart warriors just like Luke now and in the future.

Ken: I first went to DC for CHD lobby/advocacy day in 2012, and I’ve gone almost every year since; 2014 was the only exception, as the event was shortly after my second surgery. I don’t recall a specific impetus for my initial visit; I think it just felt like the next/best way for me to get more involved in the cause.

PCHA: Melanie, tell us how you all met. As a mom, what was it like to get to meet Ken and hear his story?

Melanie: We all met by having our first Hill visits scheduled together. I had briefly met Ken before but never really had to opportunity to talk. In walking together to our first visit, we began to talk about the day ahead. In our first meeting, hearing Ken speak directly after Luke and I, starting with, “I’m the adult version of Luke,” it made me realize that one day, that could be Luke. One day that could be Luke speaking from his perspective how CHD has impacted his life. I hope Luke will grow up to be driven, smart, motivated, and a CHD Advocate like Ken.

I always feel some connection to heart families but Ken’s positive and energetic personality was an inspiration. I have met many adults with CHD over the past decade but there was something different about meeting Ken. Just a different feeling like Ken and Luke should have met that day.  Luke is pretty shy and when we came home his teacher asked him to do a PowerPoint about our trip. Luke put it together (with little help from me) and was so excited to share with his friends and teacher that he made a difference in D.C. Not being afraid to tell his story came from his interact with Ken.

PCHA:  Ken, what was it like for you to meet and hang out with Luke? Did it remind you  of your own childhood experience?

Ken: I met Luke and his mom Melanie on our walk from the conference hotel to our first Capitol Hill visits of the day. As it happened that morning, I think it’s common for heart patients and families to connect
over shared experiences, especially in a setting like the legislative conference.

I always enjoy meeting fellow CHD patients, and I think there’s an extra connection when you share the
same defect—tetralogy of Fallot, in our case. With respect to advocacy, my childhood was nothing like
Luke’s. Advocacy and lifelong care were nowhere on my radar at his age. I was asymptomatic as a child
so I never really gave my heart a second thought. When I was younger, my scar felt more like a
birthmark than anything because I didn’t remember that initial surgery as a baby. As an adult – and
knowing what I know now – it’s exciting and encouraging to see young CHD warriors getting involved to
the degree that Luke is.

PCHA:   Luke, how’d you like meeting Ken? Was it cool to meet an adult like you?

Luke: I really liked meeting Ken. He was really funny and laughed at all of my jokes. I met a few kids with special hearts at D.C., but none with the same defect like me. Ken is a SUPER cool tetralogy of fallot warrior, like me.

PCHA:  So how did your DC visit go? Were the legislators receptive to you message?

Melanie: Hill visits this year went great! In Illinois we are fortunate to have so many that attend from our state. We were partnered with heart parents, heart patients (all ages), medical staff, siblings, and bereaved families. It really showed that CHD is lifelong, never cured, with different outcomes. Our last visit with Congressman Raja Krishnamoorthi was a 100% yes as a Co-Sponsor as he is a fellow heart dad. It was a great why to end the day with a yes!

Ken:  While we got to meet Senator Durbin and Representative Bilirakis during the conference, our actual Hill visits (mine, at least) were solely with legislative staffers. In general, everyone I met with was receptive
to our message, to our stories, and to our requests (generally speaking). But the real work, I believe, is in
building the ongoing relationships to continually push for our issues. Nothing happens quickly in DC, and
in order for our advocacy work to truly be successful, we must stay in touch with our elected leaders in
Congress so that our issues remain front and center for them.

PCHA: What did your trip – and your new found friendships – teach you?

Ken: 2018 marked the sixth year that I attended the legislative conference, and I initially wasn’t sure I would learn anything this time around. The format stays pretty consistent each year, and I approached it
primarily as continuing the long but necessary journey toward increased awareness and funding for the
CHD community.

Rather than teaching me something, I think this year’s trip just reminded me that sometimes you can
have an unexpected impact on others when you’re not even trying to and when you least expect it. I
initially got involved in CHD advocacy for myself because of my own personal interests. I never intended
to be inspiring to—or a role model for—others. But meeting people like Luke and Melanie reminded me
that being an adult thriving with CHD can offer hope and encouragement for the younger generation
and their families.

Melanie: Although this was my third conference, it was my the first time my inspiration –  Luke – was by my side. This trip taught me that no matter how long you’ve been in the CHD community, everyone has a powerful story and can inspire you when you least expect it!

PCHA:  What are your hopes for the future of the CHD community?

Ken: There are a lot of strong and passionate voices within the CHD community – and for good reason. I think that continued partnership and collaboration among all members and organizations will only make our voices stronger. At the broadest level, I think the country is facing a serious public health issue with
respect to CHD. Thanks to medical advances, people with CHD are living longer than they have in the
past; and we’re at a point where there are now more adults than children living with CHD. Yet there is a
severe shortage of certified congenital cardiologists to care for the growing and aging population. And at
the same time, we’re not – in a significant enough way – on the radars of Congress, the broader medical
community, major philanthropy, or the general public.

My hope for the CHD community is that we are able to make a real impact to significantly increase
awareness, funding, and –as a result – better longer term outcomes for our CHD warriors. When we talk
about advocacy, we frequently refer only to our work with our elected officials at the federal and state
levels. And no doubt that work is crucial! But I think CHD advocacy is much larger than that. I think
increasing awareness in all areas of our lives is just as important – whether that’s with our primary care
doctors, our families, our friends, our partners and spouses, and anyone else who will listen.

Melanie: Luke got to see what we can do as a heart community together, one voice, we can accomplish so much. My hope is for Luke to continue to use his voice in the CHD community, not just for himself, but all heart warriors from the past, present, and future.

 

 

Ken Woodhouse was born in 1981 with a congenital heart defect (CHD) known as tetralogy of Fallot. He had his first open heart surgery at the age of eight months; and like many individuals with CHD, he originally thought he was “fixed” after that surgery. Ken never really considered myself a heart patient when he was younger, and fell out of cardiology care for over a decade between his mid-teens and late 20s. A bicycling accident in August 2011 resulted in a concussion and a trip to the emergency room. While Ken was not seriously injured, a series of follow-up tests revealed that he had an aneurysm in his pulmonary artery; and the reality quickly set in that his CHD was not fixed. In fact, Ken would need additional intervention in the not-too-distant future and specialized care for the rest of his life.

Shortly after that accident, Ken became active in the congenital heart defect/disease community. As an advocate and speaker, he has had the opportunity to share his own story and to help raise awareness on Capitol Hill and at events across the country. Ken is currently a Senior Ambassador and a blogger with the Adult Congenital Heart Association; and his volunteer work in the CHD community has also given him the chance to collaborate with The Children’s Heart Foundation and the Pediatric Congenital Heart Association. Ken feels he is extremely fortunate to have been able to live a healthy and active life. He has always loved the outdoors, cycling in particular.

Melanie’s heart journey began in June 2008, during a routine 20 week ultrasound. She and husband were devastated by the news that their unborn son Luke would be born with a congenital heart defect (Tetralogy of Fallot) and required heart surgery at a week old and again at 9 months old. Feeling very scared and alone during the roller coaster ride of a CHD journey, Melanie decided that no other heart family should feel alone. In 2010, she has started a support group for heart families in Chicago. Working nationally and locally with various CHD organizations. In 2016, she began volunteering with PCHA’s new state chapters, to offer families more than just support. She is currently the State Chapter Coordinator for PCHA National helping to develop state chapters.

 

New Diagnosis – Brooke’s Story

This Week, Brooke Wilkerson shares the story of her son’s prenatal misdiagnosis, the correct diagnosis after birth, and the roller coaster of emotions the journey brought. 

“There’s something wrong with your baby’s heart.”

“Just say it,” I thought, “Just tell me that there is something wrong so that I can mentally prepare myself for what’s ahead.”

At eight months pregnant, I never thought I would think these things. I never thought that I would actually wish for that statement. But, I did. I wanted to hear those words because not knowing was agonizing. There was a lump in my throat that formed at our twenty week anatomy scan and it wasn’t able to release until I knew, for sure. I knew that there was something wrong. They thought that something might be wrong. But no one could confirm it.

Every month, I would make the long commute to Louisville, to see a specialist who told me the same thing every time- “We can’t get a clear picture of his heart. Everything else looks okay- he’s growing and active- but we can’t say, for sure, if there’s something wrong.”

Every time, multiple technician’s would have a go at my son’s anatomy scan, like a game of darts, and when no one could hit the bull’s eye, they’d eventually give up. They just didn’t know.

Every month I would get my hopes up that today would be the day that they just delivered the devastating news; that they would tell me what was wrong; that they would tell me that the reason why they couldn’t get a good picture of his heart is because it wasn’t normal. I was on the cusp of having my life turned upside down and the anticipation had me by the throat. Every appointment lasted over an hour and for about 4-5 months, I made the drive, I laid on the uncomfortable bed for over an hour just to get the same result. This limbo that we were in changed everything.

I knew that my baby was okay in the womb, and I no longer looked forward to his birth. I just wanted him to stay put- to stay safe- because his birth now came with a lot of uncertainties and fear. At least, while he was still with me, I could feel him move everyday and know that he’s okay; I could take comfort in knowing that, while his heart may not support him after birth, I could carry the weight for him right now.

As we neared my due date, the pressure was intense. Our local hospital was not equipped to deal with a baby with a CHD, and we had to decide if we wanted to deliver there or in Louisville- 1.5 hours away. By delivering locally, we risked being separated if baby Miles was transferred to the children’s hospital.

Four weeks until my due date, and we had, what was supposed to be my last fetal echocardiogram. Again, the results were the same. No one knew. They couldn’t confirm or deny. If they had to guess, he was okay- but what were the consequences if they were wrong? No one was confident enough to place a bet. The cardiologist asked me if I wanted to come back in two weeks, and try one last time and I agreed, even though I knew what would happen. I no longer expected an answer.

The morning of my last echo, I wasn’t expecting an answer. I fully expected it to be just like the other ones- leaving disappointed. The technicians all took their turns, and then the last cardiologist in the practice, who we hadn’t seen, yet, Dr. Holland, took a go at it. He left the room, and came back with a pen and paper in hand, and when he spoke the words, “Tetraology of Fallot,” I was completely taken aback.

I don’t remember what he said after that, aside from “open-heart surgery between four to six months old.” I clearly remember tuning out his words, and telling myself to hold it together- “Don’t cry. Just smile and nod.” Did I even speak after that?

My throat was on fire, and my heart was racing. I had finally gotten what I had asked for- an answer. As he drew two hearts on a piece of paper- one normal, and one, my baby’s- I tried to focus on my restless two year old and act like this was okay- just another day at the doctor’s office. He told me that we would deliver in Louisville, and that there would be a full heart team on stand-by, but we still didn’t know what to expect or how severe his disease was.

As soon as I opened my car door, the four month old lump in my throat exploded. I hadn’t even comprehended what he had said- I didn’t even remember the name of the diagnosis and, had he not written it out on that paper for me, I wouldn’t have known what to tell my husband, who had been texting me non-stop when this appointment went longer than the others.

My baby was going to require open heart surgery to survive. I remember how the words, “open heart surgery” stung; how they felt so foreign and extreme, and I remember saying those words as if they were a question when we told our family about his CHD.

We didn’t know what would happen when Miles was born. Would he be pink? Blue? There were so many people in the delivery room, ready to jump into action. I think I held my breath when I felt them pull him from my womb- waiting to hear him cry.

“Please, cry. Scream, Miles! Let me hear you cry!” I thought, but, I didn’t have to hold my breath for very long, because he did cry. And he screamed. And when they held him up over the curtain, he was a chunky, eight pound, PINK baby!

“Thank you, Jesus.”

His first echo, out of the womb, told a different story. We were told that he did NOT have Tetraology of Fallot, that he would NOT require open heart surgery, and that the hole in his heart would heal on it’s own.

Suddenly, all of the suffering and waiting was worth it. I’d do it all over again, if I knew that they would be wrong. We shared the good news with our friends and family, left the hospital several days later and, for the first time in months, breathed a little easier.

But, that was short-lived. At Miles’ follow-up echo, a week later, Dr. Holland apologized when he told me that they got it wrong. I could tell that he felt so bad when he told me that he saw the first echo and had agreed with the cardiologist on staff when Miles was born! He couldn’t explain to me what happened- why we were on this roller coaster- but, he was confident when he told me, again, that Miles DID have Tetraology of Fallot and that he DID need open heart surgery.

Again, I was not expecting that. I remember thinking to myself, either Dr. Holland is really great at his job- so great, that he’s able to see things that no one else is seeing- or he’s really terrible and subsequently, torturing us.

My husband, our friends and family- nobody really believed him this time.

“Doctors misdiagnose all the time,” they told me.

“There isn’t a thing wrong with that baby,” they stated.

And, for the most part, they were right. Aside from a heart murmur, Miles didn’t show any symptoms of a heart defect. His O2 levels were always good; He never had breathing troubles; He never turned blue. If I didn’t know any better, I would think that he was completely normal.

And while so many people around us didn’t want to believe it, I did believe it, and I don’t know why. But, I’m glad that I did. I’m glad that I listened when Dr. Holland told me what the symptoms of congestive heart failure would be, because when Miles started showing them, he went downhill quickly.

All of a sudden, at 3 months old, Miles was having subcostal retractions that didn’t improve with medicine, he was breathing hard, and you could see his heart moving in his chest, he started to sleep all day and all night long, and he couldn’t stay awake long enough to finish eating.

His heart was failing. Dr. Holland was right.

Miles’ heart was enlarged, because, just like a muscle, when a heart is working hard, it grows- it gets bigger. And his heart had been working really hard to appear “normal” all this time. But, it couldn’t do it any longer.

At four months old, Miles had open heart surgery. We are so incredibly blessed that his surgery went well. Aside from a few complications, we were able to go home (for good) after ten days in the hospital. Miles’ heart was finally able to rest, and in no time, you couldn’t see it moving anymore. He quickly caught up on his weight, and he was happy to eat without tiring.

As it turns out, Dr. Holland is an amazing doctor, and Miles’ VSD (the hole in his heart) was actually much larger than they had anticipated. He’s now the basis for this blog- my sour patch kid, as I call him. And while, most days, he drives me insane with his mischievousness and curiosity, I can’t help but be in awe of him.

Aside from his thin, jagged scar and pacer wire scars, you’d never know that he was born with a congenital heart defect. You’d never know that, historically, Miles wouldn’t have survived. You wouldn’t know that after his open heart surgery, he required a pacemaker because his heart wasn’t working properly. You’d never know that he caught a virus in the hospital and had to be re-admitted after being released just a day prior. You’d never know that at just four months old, his life was saved.

We are forever indebted to that team of doctors and nurses.

Brooke Wilkerson is a 28 year old wife and mom to three, living in the Nashville, TN area. Brooke also started the Coffee & Chaos Blog last year, which is all about motherhood, marriage, and how to laugh at the chaos. Her middle child, Miles, was born with a CHD and required open heart surgery at 4 months old. He’s also the inspiration behind the blog, due to his mischievous personality. He’s doing great now, and there are no future surgeries planned for him! Brooke uses her platform to raise awareness for CHD and is hosting an event this October where a portion of the proceeds will be donated to PCHA.

New Diagnosis – Pulse Oximetry

This week we will hear two perspectives on the benefits of pulse ox and the effort to make this non-invasive test a standard screening of newborns. Pulse Ox screening has made it possible to detect CHD in many newborns that would have otherwise gone home undiagnosed. This week’s contributions were provided by Dr. Gerard Martin, a pediatric cardiologist, and Ms. Lisa Wandler, a pediatric nurse, from the  Children’s National Heart Institute and  Dr. Matt Oster, MD, MPH , a pediatric cardiologist at Sibley Heart Center Cardiology at Children’s Healthcare of Atlanta.

 

 

Dr. Gerard Martin, a pediatric cardiologist, and Ms. Lisa Wandler, a pediatric nurse, from the  Children’s National Heart Institute discuss with us how pulse oximetry screening works , as well as its benefits and limitations. 

Screening infants for Critical Congenital Heart Disease (CCHD) using pulse oximetry is recommended in the United States, but this was not always the case!  As of this summer, all 50 states and the District of Columbia will be screening for CCHD.  In many countries, this important life-saving screen is not yet standard for every newborn.  

How CCHD pulse oximetry screening works:

CCHD screening is simple, painless and takes only a few minutes to perform.  It typically takes place around 24 hours after birth either in the newborn nursery or in the mother’s room.  A sticker with a special light probe is placed on the baby’s right hand and either foot.  The measurement that the light probe takes helps the medical team determine whether the baby may have CCHD and require further assessment and testing.

How it has changed standard screening for newborns:

Prior to the implementation of CCHD screening, as many as 50% of infants with CCHD were being discharged from the hospital without anyone knowing of their heart problem.  Without CCHD screening using pulse oximetry, routine newborn screening could help identify hearing and other rare but serious conditions in babies just after birth but not heart defects.  

Benefits/importance of screening:

An undetected heart problem in a baby can lead to severe health problems for the baby and even death.  This newborn screen has helped to decrease the number of babies lost to undiagnosed heart defects and saved many lives.  The CDC continues to investigate the impact CCHD screening has had in the U.S., but an early estimate is that the number of deaths due to CCHD has gone down by 33% in states requiring this important newborn screen.

Limitations of screening using pulse oximetry:

Screening improves the detection of CCHD, however, not all types of CCHD are able to be detected using this screening method.  It remains important to follow the instructions of a baby’s pediatrician and other doctors as newborn assessment and pre-natal ultrasound remain important other ways CCHD can be identified.

Early symptoms of CCHD can include rapid breathing, difficulty feeding and bluish skin.  If your baby has these symptoms, tell the baby’s doctor.

 

The Critical Congenital Heart Disease Screening Program at Children’s National Heart Institute is composed of Dr. Gerard Martin, a pediatric cardiologist and Ms. Lisa Wandler, a pediatric nurse.  The team at Children’s National has worked on implementation, education and CCHD screening advocacy for over ten years and has provided guidance at the local, state and international levels to those interested in CCHD screening using pulse oximetry.  The team can be contacted at pulseox@cnmc.org

Gerard Martin headshot, cardiologist, children’s national heart institute

 

 

 

Dr. Matt Oster, MD, MPH  discusses the progress brought by pulse ox and the distance yet to go. 

 

In my medical career, I’ve been able to see what was once just a promise or an idea be transformed into reality – the ability to screen well-appearing newborns for critical congenital heart disease. While a baby may appear completely well by anyone who sees her, lurking beneath the surface could be subtle hypoxemia – an abnormally low concentration of oxygen in the blood.- secondary to a congenital heart defect. The application of pulse oximetry to detect such hypoxemia has thus allowed clinicians to detect many previously undiagnosed cases sooner, a change
that has led to decreased infant mortality from critical congenital heart disease.

This dramatic change in the care of newborns did not come easily. It took scientists studying the issue to determine whether this could work. It took policy makers the vision to implement this change in their regions. And, most importantly, it took the tireless advocacy of parents and others to call for, and when needed essentially demand, such change.

The application of pulse oximetry to screen for critical congenital heart disease is a true public health success story. And, it has even seen spillover effects in that many children with hypoxemia due to causes other than heart disease are being detected and treated. However, there is still much work to be done. We need to figure out the best way to implement this screening in special settings such as the neonatal intensive care units, home births, or areas of high altitude. We need to improve the quality of the program so that it is implemented consistently and correctly for all newborn. We need to help public health agencies monitor and track the success of this program.

But the biggest change we need to make is figure out a way to increase the sensitivity of the screening. Yes, the program has detected thousands of babies that may have previously gone undetected. But there are still many newborns with critical congenital heart disease that are being missed, even newborns with hypoplastic left heart syndrome. This is typically due to the fact that hypoxemia may not yet be present. We need improved diagnosed methods beyond pulse oximetry to help detect these children. This is not an easy task, but scientists are indeed working on it. And when it’s ready, we’ll need the help of advocates and policymakers to make it a reality.

 

Dr. Matt Oster, MD, MPH is a pediatric cardiologist at Sibley Heart Center Cardiology at Children’s Healthcare of Atlanta, and he holds Emory appointments of Associate Professor of Pediatrics in the School of Medicine and Associate Professor of Epidemiology in the School of Public Health. He earned his MD at the University of Pennsylvania School of Medicine and his MPH in epidemiology at Emory University Rollins School of Public Health. After completing residency training in pediatrics at the University of California-San Francisco, he did fellowship training in pediatric cardiology at Emory University. When not seeing patients, he serves as director of the Children’s Cardiac Outcomes Research Program at Sibley Heart Center. His research interests include newborn screening for congenital heart disease, the epidemiology of congenital heart disease, and long-term outcomes for patients with congenital heart disease.

 

 

 

New Diagnosis – Fetal Echo

More often now, than a generation ago, babies born with CHD are being diagnosed prenatally. This week, Dr. Sheetal Patel, from Lurie Children’s Hospital of Chicago and Assistant Professor of Pediatrics, Northwestern University Feinberg School of Medicine, explains the role prenatal echocardiography plays in diagnosing CHD and the benefit of that early diagnosis.   

 

 

Congenital Heart defects (CHDs) are among the most common birth defects, affecting 1 out of every 110 babies born. Each year nearly 40,000 babies are born in the United States with CHD, ranging from simple lesions that may not need any interventions to complex CHDs that can be fatal if appropriate treatment is not provided soon after birth. Research shows that prenatal diagnosis and early detection of these complex CHDs is associated with improved surgical outcomes. With improvement in diagnostic technology with Fetal echocardiogram today, about 70% of complex CHDs are detected prenatally. Goals for detection are targeted at 100%, and we are aiming to reach there with improved awareness and better screening techniques.

A fetal echocardiogram is an ultrasound test performed during pregnancy to evaluate the heart of the unborn child and can be performed as early as 18 weeks gestation. Diagnostic accuracy for detecting complex CHD with a fetal echocardiogram is as high as 95%. It is a non-invasive procedure performed with an ultrasound probe placed over mother’s belly. Generally, the pain or discomfort that results from the probe pressure on the mother is minimal. It involves detailed evaluation of baby’s cardiac structures including cardiac chambers, valves and major blood vessels. It also evaluates fetal heart rate and rhythm. This test can detect CHDs such as missing heart chambers (such as hypoplastic left heart syndrome, hypoplastic right heart syndrome, and many other variations), abnormal great arteries (such as transposition of the great arteries, truncus arteriosus, interrupted aortic arch, etc), abnormal cardiac valves (such as atrioventricular septal defect, pulmonary valve atresia), or large hole between cardiac chambers (such as a large ventricular septal defect). There are limitations of fetal echocardiography that it may not detect minor cardiac valve abnormalities, small holes between cardiac chambers or coarctation of aorta that develops after birth.

Early detection of CHD before baby’s birth has many advantages.

Prenatal diagnosis of CHD allows for necessary preparation to provide highly specialized care that the baby will require soon after the birth and prevents the hemodynamic compromise that can result if this CHD was undetected. This preparation involves coordinated care by multiple teams with expertise in pediatric cardiology, neonatology, pediatric cardiac intensive care and pediatric cardiovascular surgery. In addition, social worker, child life specialists, and palliative care teams may be available to help parents cope with the diagnosis and treatment. An important aspect of early detection is to provide expectant parents the opportunity to have detailed counselling.  This counselling helps parents to better understand their unborn child’s heart condition and interventions that might be needed.  This aids parents in their research to choose a center of excellence for their baby’s care. The goal of this prenatal counselling is for parents to be armed with knowledge, process the information over time, and maximize the family’s preparedness for the journey and transition to a birth of their new baby. Research shows that those mothers who knew about their baby’s heart condition prior to the birth were less anxious once the baby was born as compared to mothers who found out about the defect after baby was born.

There are some standard indications for fetal echocardiography during pregnancy.

Not every expectant mother needs to have a fetal echocardiogram. However, if the risk of having CHD in the unborn child is expected to be higher than general populations, a fetal echocardiogram is indicated. These risk factors include having a prior child with congenital heart defects, maternal diabetes, maternal infections during pregnancy known to affect baby’s heart, etc. Mother should discuss with her obstetrician if a fetal echocardiogram is indicated based on the family history and her own medical history. If indicated, a fetal echocardiogram should be arranged to be performed between 20 to 24 weeks gestation which is an ideal time for accurate diagnosis of CHD. Other indications for fetal echocardiogram include abnormal findings on obstetrical screening test such as increased nuchal thickness, abnormal cardiac images during the level II anatomy scan, chromosomal abnormalities (such as trisomy 21, trisomy 18, trisomy 13, Turner syndrome, etc) detected during the prenatal genetic testing, or other organ malformations noted during the anatomy scan. These abnormal screening tests indicate higher risk of CHD in the fetus and therefore, a fetal echocardiogram is indicated.

What happens after a fetal echocardiogram detects CHD in fetus?

A pediatric cardiologist performing the fetal echocardiogram will discuss the findings of CHD in details with the expectant parents. Tailoring the counselling to the parent’s needs over time is critical as parents can be very overwhelmed during the initial hearing of a diagnosis of CHD. Counselling should include discussion about implications of this CHD on baby during the pregnancy, what support and care this baby would need soon after the birth, what interventions, procedures and surgeries would be necessary during neonatal period, and what other procedures or surgeries would be needed later in life and what is expected overall prognosis with this CHD. The goal of this counselling process is to provide information to parents that would help them with their decision making to choose their options. The options are described in details that include preparing them for this journey to have child with CHD, palliative care or other family planning options.

Each Fetal Cardiac Program have unique set up to provide this detailed counselling. At Lurie Children’s Hospital; these services are provided through our fetal cardiac program at The Chicago Institute for Fetal Health. Following the initial consultation, parents have a “Comprehensive Fetal Cardiac Consultation” which includes a follow up fetal echocardiogram to assess the evolution of the CHD (if any), consultation with a fetal cardiology team composed of a pediatric cardiologist, neonatologist, cardiac intensivist, cardiovascular surgeon, social work, and other specific team pertinent to the diagnosis. For example, a consultation for prenatal diagnosis of HLHS would include a specialist for “Single ventricle Program”. Parents may also choose to meet with “cardiac neurodevelopment team” and “Child life Specialist” if they are interested in learning more about these important aspects of their child’s quality of life in future.  The number of partners at the table during this meeting can seem overwhelming to some parents and modifications are made to this process to meet each family’s needs.  

Our goal as a comprehensive fetal cardiology team is to arm families with information, answer questions, form a united care team to provide cohesive CHD care pre and post-natally, and optimize the chances for the most successful outcome and quality of life throughout a lifetime.  

 

References:

Fetal Echocardiogram: https://www.luriechildrens.org/en-us/care-services/specialties-services/medical-imaging-radiology/diagnosis-services/heart-evaluation-testing/Pages/fetal-echocardiograms.aspx)

CDC: https://www.cdc.gov/ncbddd/heartdefects/data.html

 

Dr. Sheetal Patel is Associate Director of Fetal Cardiac Program at Ann & Robert H Lurie Children’s Hospital of Chicago and Assistant Professor of Pediatrics at Northwestern University Feinberg School of
Medicine. Her clinical interest lies in fetal, neonatal and pediatric cardiology. She is passionate about prenatal diagnosis of congenital heart defects. Her research interests are in evaluating outcomes in
congenital heart defects, with special focus on single ventricle heart defect and Fontan palliation.

Zipperstrong Project

As we continue through heart month, one amazing program, called Zipperstrong, helps honor families affected by CHD and their stories. The work done by photographer SheRae Hunter helps remind us all that even our scars can be beautiful. 

 

 

 

“I am the mommy of a child who is different.   All I ever want and need is for others to understand. To understand my family, to understand my son, to understand the hours of therapy, the meltdowns, and the uncertainty that we live with daily. To listen and not judge, not offer advice, and not extend pity, but to try understand us,” SheRae Hunter explains.  With her work on the Zipperstrong Project, she helps  other families, families affected by CHD, accomplish this very thing.

 

Ainsley – 2 Years Old – 2 Ventricular Septal Defects closed with open heart surgery at 3 months. ​

 

On the first day of Congenital Heart Disease Awareness Week, the Pediatric Congenital Heart Association of Virginia (PCHA-VA), in partnership with the Zipperstrong Project, shared a set of powerful images capturing the strength, vitality, and hope of children fighting congenital heart disease (CHD), as well as the reality that many CHD warriors sadly lose this battle each year.

 

Blake – Two years on Earth. Forever in our hearts.Transposition of the Great Arteries, AV Canal Defect, Pulmonary Artesia, Heterotaxy

 

Every year, 40,000 infants are born in the United States with a congenital heart defect. It the most common birth defect, yet many people are unaware. Through Zipperstrong, Winchester, Virginia Photographer SheRae Hunter helps raise awareness of congenital heart disease by giving outsiders a glimpse into the CHD world. Hunter started Zipperstrong in 2015 after becoming intertwined in the lives local CHD families and wanting to make a difference.

 

Finn – 2 1/2 Years Old – Heterotaxy, Double Outlet Right Ventricle, Total Anomalous Pulmonary Venous Return and other cardiac issues.

 

“In my photography, I see these children’s personalities shine through, despite their physical and emotional scars. Their vitality and courage is something to be admired and that is what I hope to capture through the Zipperstrong Project. That is what I want the world to see!” says Hunter. “This year’s Zipperstrong class is truly special. Many of these kids have grown up before my eyes, while others I’ve met for the very first time. All of their stories are so deep and profound that I wish I could share every word. Year after year I am reminded of how extraordinary these kids are.”

The 2018 Zipperstrong Class includes children  from across the Commonwealth, from the Shenandoah Valley and neighboring West Virginia regions to Richmond, Fredericksburg, and Alexandria. Their ages  range from newborn to 11 years old. In all, 15 children were photographed and a handful participated for the first time this year. Most of the children have multiple heart defects, and many have gone through numerous surgeries, with more in their future.

 

Jade – 11 Years Old – 2 Ventricular Septal Defects & Double-Chambered Right Ventricle

 

“Through Zipperstrong, SheRae offers the perspective of an outsider and her own artistic vision to tell the story of children living with hearts that have complex structural or functional problems hidden in plain sight,” says PCHA-VA President Laura Carpenter. “Her Zipperstrong Project has moved and captivated all of us at PCHA-VA, and so many more who have never heard of CHD. She has done a great service to the CHD community and we are thrilled to have her as a partner.”

PCHA extends great affection and gratitude to Zipperstrong founder and photographer, SheRae Hunter. Her portraits capture the incredible strength of CHD Warriors in Virginia and allow us to share that with the world!

The Zipperstrong Project kicked off CHD Awareness Week 2018 in Virginia, and we are excited to announce Zipperstrong as a program of the Pediatric Congenital Heart Association!

View this year’s Zipperstrong Project photos at www.zipperstrong.org. The photos will be shared through social media throughout Heart Awareness Month and on display at various events across Virginia, including The Hope Marietta Foundation’s Casino Night in Washington, D.C., on February 24. Arrangements can be made to connect with certain Zipperstrong families and reproduce imagery by contacting PCHA-VA Communications Director Renée Lang at rlang@conqueringchd.org.

 

New Diagnosis – Jaclyn’s Story

This week, PCHA-OH Board Member, Jaclyn Frea shares the story of her miracle baby’s diagnosis with Tetralogy of Fallot.


My husband Bryan and I were married on March 5, 2011 and we knew that immediately we wanted to start a family. Little did we know, getting pregnant would be a lot more difficult than we thought and tragedy would strike two fold. Five months after we were married, I lost my Dad to cancer and, a year and a half later, in 2014, I lost my Mom to a different form of cancer. I am an only child, so losing both my parent was a devastating loss, but we didn’t want to give up at trying to have the family we had always wanted.

Along with the loss of my parents, Bryan and I tried and tried to get pregnant with no luck, each month we thought, maybe this month will be our month, then nothing. We then decided to go through a fertility doctor, only to find out that pregnancy probably wasn’t in our future. At that time, there was no explanation why! We wanted so badly to be parents. We decided to look into the adoption process and were approved; the only thing we needed to complete the adoption process was to find a birth Mom. After 5 years of unsuccessfully trying to get pregnant and just being approved to adopt, I found out I was pregnant!!!

My pregnancy with Paul was amazing. Yes, I had morning sickness, a lot of morning sickness, but I was carrying the baby I had always wanted. I was pregnant with a miracle baby, and I was incredibly grateful and I loved every single minute of being pregnant with this child! From hearing the first heartbeat, to seeing our baby in the ultrasounds and seeing my belly grow, feeling the baby kick and being incredibly active, and then finding out that we were going to have a little boy. God had answered my prayers!

The day arrived when Paul wanted to make his debut and everything seemed to be going pretty smoothly, until, his 36 hour check up. I remember the nurse coming in and telling Bryan and I that she was going to take Paul to the nursery to complete his check up and feeling so blissful about everything being absolutely perfect. A little while later, the doctor came into tell us that they could still hear a murmur in Paul’s heart, and they wanted to send him down to the NICU to conduct an echocardiogram on his heart just to make sure everything was alright. I hadn’t been released from the hospital yet, so we were going between floors being with our son and waiting on me to be discharged.

Bryan and I walked into the NICU, and I remember being greeted at the entrance with our nurse immediately informing us that they were going to be conducting an arterial blood draw on Paul. I remember that my heart sank. I knew in my gut that something was wrong with my baby boy, and the next hour we were waiting on the NICU doctor to come and tell us that our son was born with a Congenital Heart Defect, Tetralogy of Fallot!

How could this be happening, after everything we had been through, a CHD???? Neither Bryan, nor I were prepared, let alone even thought about a CHD! I remember sitting in the chair, sobbing, and the only words I remember hearing were Tetralogy of Fallot, open heart surgery necessary; if he didn’t have the surgery, he wouldn’t survive to be a teenager…..what???!!!

My husband is a Firefighter and Paramedic, and I thank God that he was there because he was so strong and he understood everything the doctor was saying. Paul was considered a pink tet baby, as his O2 saturation remained near 100. Looking at him, one wouldn’t even know that he was sick. In a normal case of Tetralogy of Fallot, the heart shunts blood from the right side to the left side, sending oxygen poor blood to the body. Paul instead shunted blood from the left to right, keeping his oxygen levels very high, but causing constriction of the pulmonary blood vessels and risking permanent lung damage due to this increased blood flow.

Paul was released from the NICU later that day, and that is when the litany of doctor’s appointments and preparations began. Bryan and I kept Paul quarantined pretty much until his surgery, because we wanted to try and keep him as healthy as possible. When he was 4 1/2 months old, he had his complete repair via open heart surgery in 2016 by Dr. Toshiharu Shinoka. Paul is followed closely by Nationwide Children’s Hospital, but now only requires yearly echocardiograms and check ups.

Paul is now 22 months old and he is doing phenomenally well; exceeding every milestone set before him.  Paul is a happy, mellow baby (toddler), who loves the water and can’t get enough of the bathtub and the swimming pool. He is always smiling, beginning to talk, and using lots of sign language (we began to teach Paul sign language when he was 6 months old).  He LOVES music, dancing and trying all kinds of foods.  He is very compassionate with a strong love for animals (he loves to give kisses and hugs where ever he goes). He is an only child, a total flirt
(who no doubt will use his scar to flirt with the ladies in the future).  Paul is a dynamic young man, who is also a giant book worm (he gets to go through about 25 to 30 books each day), walking and running everywhere, is super chatty, and is climbing on and into everything.

All who hear about him are inspired by his story and genuine passion for life.  Paul is, without a doubt, our miracle baby. I had to have an emergent hysterectomy 6 months after Paul was born, only to find out that I had severe endometriosis. The doctors said there is no scientific or medical reason why I should have ever gotten pregnant. Paul is a miracle, and is meant for great things in his very bright future!

Jaclyn Frea  is a wife, to Bryan, and a Mom, to Paul her heart warrior. She loves her family, animals and OSU Buckeye Football. She is a PCHA – Ohio Board member, as well as the Communications Coordinator for Congenital Heart Network of Central Ohio.  Jaclyn also volunteers as a wish grantor for the Make-A-Wish Foundation.  In her spare time, she loves to spend time at the zoo, with her family, coloring, and Jaclyn loves makeup and all things beauty related.

Recap – Wellness

When living with chronic illness or caring for a chronically Ill child, it’s important to maintain a healthy lifestyle. PCHA’s Wellness Blog Series contributors offered tips on how to do it!

The Most Wonderful Time Of Year

The Holiday Season is meant to be filled with family and joy. It can also be a time of great stress, especially for families with chronically ill loved ones. In today’s post, Becky Hunt shares her experience with losing a child to CHD as well as managing her own illness, and explains how to de-stress from it all around the holidays, or any stressful time of year.

 

CHD and Exercise

With the brand new year, many of us are making resolutions to better ourselves and our lives. Exercising more is often at the top of the list! This week, Kathleen Baschen, an exercise physiologist at Ann & Robert  H. Lurie Children’s Hospital of Chicago, shares tips on how patients with CHD can get FITT. 

 

Balance

In the latest post in the Wellness Series, Megan Horsley, heart mom and Pediatric Dietitian at Cincinnati Children’s Hospital Medical Center, discusses how to find balance with your nutrition.

 

 

New Diagnosis – Prenatal Conquering CHD Kits

This week, our State Chapter Coordinator, Melanie Toth, shares her experience with us as  a new mom finding out her son would be born with a congenital heart defect and how PCHA helps to make sure other families don’t have to go through what Melanie’s did alone.

 

Like many heart families, when diagnosed with their child’s heart defect, your world is flipped upside down. It was no different for our family. I remember us walking out of the room not really understanding or knowing what just happened. We had only walked into the doctors office for our 20 week ultrasound, and left finding out our child would be born with a heart defect, that I couldn’t even pronounce. While I have always had a love and hate relationship with congenital heart disease, over the past decade I have learned a lot. I can’t change the diagnosis we were given that day, but I did wish I could change the information that our family was given when we left the doctors office.

We weren’t aware that CHD was the #1 birth defect or that too many families walked in similar shoes as us. We left feeling hopeless and alone, with a paper that said, “Tetralogy of Fallot”. My husband and I went online to get more information on our son’s diagnosis and, to say the least, were completely overwhelmed. There was different information on various websites, and we just felt more hopeless.

If 10 years ago our doctors would have handed us a PCHA Prenatal Conquering CHD Kit, life would have been just a little easier. From resource cards to navigate important CHD information, to guided questions on what to ask your medical team, and most importantly, how to connect with other heart families, the prenatal kit is like a life raft for new families. Through PCHA State Chapters and working with hospitals, prenatal kits have offered the much needed information and personal connection that every heart family deserves. If 10 years ago our family was given a prenatal kit, instead of walking out with our sons defect written down, our family would not have felt so alone in our heart journey, during a difficult first year with our son’s surgeries.

I feel honored and blessed to help our PCHA State Chapters as National State Chapter Coordinator. Personally helping heart families and helping set up chapters to empower families, is the best pay it forward our family can offer.

 

Melanie’s heart journey began in June 2008, during a routine 20 week ultrasound. She and husband were devastated by the news that their unborn son Luke would be born with a congenital heart defect (Tetralogy of Fallot) and required heart surgery at a week old and again at 9 months old. Feeling very scared and alone during the roller coaster ride of a CHD journey, Melanie decided that no other heart family should feel alone. In 2010, she has started a support group for heart families in Chicago. Working nationally and locally with various CHD organizations. In 2016, she began volunteering with PCHA’s new state chapters, to offer families more than just support. She is currently the State Chapter Coordinator for PCHA National helping to develop state chapters.

 

Recap – Arrhythmias and Cardiac Devices

Complications from surgery, scar tissue left behind, and an underlying disease can bring about arrhythmias in CHD patients and lead to need for a cardiac device. Gathered here are the contributions and resources from PCHA’s Arrhythmia Blog Series. 

 

Jarvik: For Smaller Hearts

PCHA’s next series introduces the various Cardiac Devices involved in the treatment of Congenital Heart Disease and the associated conditions. In the first post, Dr. Adachi tells us about the Jarvik for small hearts, a ventricular assist device used to help pump blood through the body.

 

Terri’s Story

Often times, CHD patients face issues with the rhythm of their hearts. In some patients, this can mean additional treatment is necessary, with either medication or a cardiac device. In this week’s post, Terri Elliott, an adult CHD patient, shares her experience with an arrhythmia that led to her receiving an implantable cardioverter defibrillator. or ICD.

 

The Elephant in the Room

With each CHD, there is no certain path, no one course looks like the next, and complications vary widely. Though a patient may have a specific structural diagnosis, different arrhythmias may develop over time, however patients and families are not always made aware of this possibility immediately. That’s why the diagnosis of an arrhythmia may surprise parents and send them for a loop, just when they think they’ve seen it all.  This also makes it difficult for parents to know just how much to share with teacher and other adults in their children’s lives, just as Alison Connors shares with us this week.

 

Bradycardia Explained (Part One)

This week former Cardiac Nurse, Carol Raimondi, provides us with the first of a two-part resource on arrhythmia and the various diagnosis and treatment. Up first: Bradycardia.

 

Tachycardia Explained (Part Two)

New Diagnosis – Baby Shower

When a mom -to-be  receives a CHD diagnosis for her baby, questions and uncertainty swirl. Laura Carpenter shares, this week, how her baby’s Truncus Arteriosus diagnosis affected her perspective on baby showers. 

 

I remember reading an advice column many years ago. Someone was seeking guidance about her struggle to attend a friends baby shower after a pregnancy loss. I remember thinking that was selfish of her to not attend and probably only fed her grief.

Oh how time and life experiences round the rough edges.

I am now the one who hesitates at the thought of attending baby showers, even as I’m most genuinely excited for babies-on-the-way. Sometimes I accept the invitations and I attend. Other-times I forgive myself and excuse myself.Gwenyth, my heart baby was born with a heart defect called Truncus Arteriosus on St. Patricks day in 2010. She passed away when she was two months old. Baby showers are hard for me now.

When I do attend baby showers, it’s not just the absence of my baby that can be magnified, it’s the memory of the baby shower that I didn’t have, that often summons the ever present heartache to the surface.

If I could do things over again (how often I tread this path in my mind) I imagine the baby shower I wish had for Gwen.

If I could do it over again…I would say YES when my friend offered to plan a shower instead of deciding it was awkward to ask people to attend a celebratory event when her survival to birth was uncertain. I would decide to celebrate her life as given – her life as it was, already deeply a part of ours and Gwen already much-loved in our hearts. However, I would not want anyone to pretend everything was okay – but rather to be there with me, to be present and allowing for all the range of emotions that may come at such “type” of a of baby shower. They could be there to witness her story unfolding and her life as given. They would be part of her life story.If I could do it over again…I would use her baby shower as an opportunity to share the details of her diagnosis and begin spreading CHD awareness then and there (this is my hind-site 20/20 feeding my vision, so of course I’m a well-versed CHD advocate at my HeartBaby shower in this scenario). I’d share the stories of new connections I made with other mom’s expecting CHD babies. Everyone would hear about the CHD survivors that live right under our noses and be well aware of the HOPE that exists for heart kids. They would would all leave knowing how underfunded CHD research is and, through my crystal clear, 20-20 hindsight, I’d share a special “HeartBaby Registry” with them which would offer guidance on the “baby stuff” needs of a heat parent.

This special list would help everyone know which baby items would be most useful for hospital life. They would serve to bring a little sunshine and warmth to some of the lonely times caring for a baby in the hospital as I would remember the shower and think about the person who gifted it to us and was rooting for Gwen.

From the unfair vantage point of hind-sight, I wish I had told my friend, YES, please plan a shower. The reality is, my family and friends would have been honored to celebrate Gwen with me, even it was a different kind of baby shower, perhaps a little more heavy and less light and giggly than most. And what’s more – it would have given us one more very special memory of her life to cherish.

 

 

 

My volunteer work within PCHA Virginia this past year has centered around completing our prenatal care package for families like mine, who learn their baby will need open heart surgery early in life. In this “Conquering CHD kit,” PCHA offers guidance for parents regarding what questions to ask their care team, directs them to trusted sources online and shares stories of CHD Warriors that inspire HOPE. There is also a special page, created partly in response to my relentless hindsight about a missed baby shower. With anecdotes and lists, the page offers suggestions on what to pack for the anticipated hospital stay. I hope it will help families get a confidence-boosting glimpse of what caring for a heart baby in the hospital looks like. And, with this in hand, I sincerely hope families facing a prenatal diagnosis will embrace the opportunity to have a baby shower for their heart child and will even find themselves having fun creating their registry, feeling empowered as they know exactly what to ask for as Heart Parents!

http://conqueringchd.org//wp-content/uploads/2018/01/Heart-List.pdf

 

Laura, originally from the Hudson Valley area of New York, lives in Harrisonburg, Virginia where she met her husband, Myers when they were undergrads at James Madison University.  Their heart-healthy kiddos are Lillian and Arthur. Their middle child, Gwenyth was diagnosed prenatally with Truncus Arteriosus. She was born in March, 2010 and had open heart surgery when she was two days old. After about one month of enjoying life at home Gwen passed away from a sudden overwhelming sepsis (Neonatal Alloimmune Neutropenia is suspected in her sudden illness.)  Gwen’s life has inspired Laura to stay involved with the CHD community.  She is the President of the the Virginia chapter of the Pediatric Congenital Heart Association and is passionate about helping parents find strength for this journey, confidence in their role as their child’s advocate and connection with other CHD families for invaluable sources encouragement and education. She teaches art part-time at a small private school and enjoys gardening, hiking and spending quality time with friends and family.