Beyond the Heart – Marissa’s Story

There is still much unknown about adult Congenital Heart Disease Patients and the long term effects of their treatment in childhood. This week, Marissa, a single ventricle patient about to complete her nursing degree, shares her story of an unexpected complication, which almost cost her future in nursing. 

I call myself a “heart patient”, but why? Sure, I was born with six complex congenital heart defects, but my heart disease effects more than just one body system. As a 21-year- old Single Ventricle Fontan, I’ve always had my routine EKGs, echocardiograms, stress tests, halter monitors- you name it. Because I am a fontan, I’ve been made aware of the high likelihood of developing liver cirrhosis, so I have liver ultrasounds each summer. There can be issues with pregnancy, exercise tolerance, mental health disorders, and an overall a deterioration of the Fontan circulation. I’ve always been very realistic and knowledgeable with my own health condition, which is why I was so surprised when I heard my doctor say the words “you have T-cell Lymphopenia related to your Fontan circulation”.

In November of 2016, I underwent a diagnostic endoscopy that yielded abnormal findings. A week later, I found out I had an infection lining my esophagus. I presented with no symptoms and had been completely unaware of the infection. The treatment was simple, a two-week course of oral antibiotics, but my doctor felt I needed to undergo further immunology testing, considering this was an opportunistic infection. After three separate lab studies showing low immune cell counts, I was referred to an Immunologist in Philadelphia. Since then, I’ve seen three separate immunology specialists.

Marissa and her Mom wearing surgical masks while flying, to protect themselves.

T-Cell Lymphopenia is a secondary immunodeficiency that results in decreased levels of T cells (infection fighting cells) within the blood. The high pressures within my circulation do not allow my lymphatic system to release the proper amount of T-cells needed within a healthy individual. For me, my case is mild and was caught early, however; the newfound diagnosis almost ended my career as a nurse before it even began.

When I finally received my official diagnosis, I was in my third year of nursing school. I was attending clinical rotations twice a week, and I was already working in a hospital setting. Many specialists were unfamiliar with my diagnosis in relation to my childhood heart surgeries. Before I began my senior year of school, I visited a new doctor who advised I avoid patients with any form of a transmittable illness. I was advised to wear a mask in crowded spaces, to avoid friends or family members who were ill, and I was given immunizations intended for those with a weak immune system. Having these new restrictions was difficult, and it was a daily reminder that I will always fight this chronic illness; I’ll never be considered
“healthy”.

 

Marissa dressed as a nurse for Halloween when she was 9 years old.

When starting my last year of nursing school, I was told I couldn’t complete the program with the accommodations my doctor required. I was devastated to find that I may not be able to graduate with my nursing degree, and I may not be able to fulfill my passion to work as a nurse. I had spent three years studying and preparing to succeed in my future career, and it all came to a halt on a warm August day. I’ve always tried to treat myself as normal as possible; I’ve never wanted my congenital heart defects to define me as an individual. I knew this career would be tough, especially for someone with a single ventricle. Nursing school is the most challenging thing I’ve ever done, but hearing that I wouldn’t be able to graduate because of my heart was the worst part. No matter how hard I studied, how well I performed in clinical, and how well I prepared for my future, that didn’t matter anymore. With this crushing reality, I requested a third opinion from a pediatric specialist, hoping this physician would have more experience considering Congenital Heart Defects are from birth.

 

Marissa in her scrubs now, at the age of 20 years old.

Thankfully, after visiting a third immunologist, I now have no restrictions related to my T-Cell Lymphopenia. I will require follow-up care to monitor my T-cell and vaccination levels, however, I have NO restrictions in the clinical setting. I’m allowed to finish my degree to fulfill my dreams and work as a nurse. I’m incredibly grateful to have been able to see a specialist who was familiar with my diagnosis. Apparently, T-Cell Lymphopenia is present in a small percentage of Fontans by the time they reach their early 20’s.

 

CHD does go beyond the heart, and affects the body in various ways. It’s important that we as patients receive lifelong care, but this can be challenging when complications arise outside of the heart. Although it took over a year, I’m thankful to have received the proper diagnosis and plan of care. I want to inspire others to be an advocate for themselves and to not give up on their dreams. Life can get difficult sometimes, but through determination we can reach our goals,even when taking an unconventional path.

 

 

 

 

 

Marissa Mendoza lives in New Jersey and was born with six complex congenital heart defects. She was diagnosed with a Ventricular Septal Defect, Congenitally Corrected Transposition of the Great Arteries with Ventricular Inversion, Pulmonary Atresia, Bilateral Superior Vena Cavas, and Dextracardia. She underwent three open heart surgeries before the age of two, now classifying her as a Single Ventricle Fontan. She is a senior year nursing student graduating in May, 2018 and has a passion for working in pediatric cardiology. Marissa has a lifelong goal to increase awareness of Congenital Heart Defects and serve as an advocate for not only herself, but for the millions of others facing this chronic condition.

 

Action Alert: FY2019 Appropriations

Email your Senators, today, and ask her or him to sign the FY 2019 Appropriations Letter being circulated by CHD legislative champions Senator Durbin and Senator Grassley in support of congenital heart disease-related public health research and surveillance initiatives at the CDC.

Each year, we need to make sure that Congress continues to fund the congenital heart disease public health research and data collection at the National Center for Birth Defect and Developmental Disabilities (NCBDDD), with-in the Centers for Disease Control and Prevention (CDC). This budget process is called Appropriations.  Senators Durbin and Grassley have drafted a letter to the Appropriations Committee asking them to support funding for the CDC program to better understand and treat the lifelong needs of those living with congenital heart disease.  They are seeking as many Senators’ signatures as possible.

Email, call, or tweet your Senator or their health staff and ask them to reach out to Max Kanner (Max_Kanner@durbin.senate.gov), today!

Sending an email is simple!!

  1. Find your legislator’s contact information.
    1. Visit  www.senate.gov
    2. If this is your first time reaching out, use the contact form on the Senator’s website to send your email.
    3. If you have identified the Health LA, or had a previous contact with your member or a member of their staff, please feel free to use the direct email address you were given.
  2. Copy and paste the sample email, below, and personalize:
    1. Include your legislator’s name
    2. Add your own personal story where indicated
    3. Sign with your name, city, state and contact info
  3. Send it!

—— SAMPLE EMAIL —–

Dear Senator xyz,

As you are working on appropriations requests for FY2019, I urge you to show your support for continued funding of essential congenital heart disease-related public health research and surveillance initiatives at the Centers for Disease Control and Prevention.

Congenital heart disease is the most common birth defect and the leading cause of birth defect-related infant mortality. Nearly one third of children born with CHD will require life-saving medical intervention such as surgery or a heart catheterization procedure. With improved medical treatment options, survival rates are improving with a population of 2.4 million and growing. However, there is no cure. Children and adults with congenital heart disease require ongoing, costly, specialized cardiac care and face a lifelong risk of permanent disability and premature death. As a result, healthcare utilization among the congenital heart disease population is significantly higher than the general population.

As part of these ongoing public health surveillance and research efforts, the Centers for Disease Control and Prevention recently published key findings that report hospital costs for congenital heart disease exceeded $6 billion in 2013.

Congenital Heart Disease is common and costly, and attention to the needs of this community is critical.

Please show your support by signing the Appropriations Letter being circulated by Senators Durbin and Grassley.  To add your name, please contact Max Kanner with Durbin (Max_Kanner@durbin.senate.gov)
This is important to me because: (ONE-TWO SENTENCES)
Share your story briefly, here.

Odds are, someone you know has been impacted by the most common birth defect.

Join us as together, we are #ConqueringCHD.

Sincerely,
Your Name
Address

 

Advice – 10 Pieces of Advice for the New CHD Parent

Learning your child has a CHD can be overwhelming. Suddenly, you’re thrust into unfamiliar territory filled with strange terminology and new doctors. But so many families have bee there too, and they have so much knowledge to share, garnered through their experience.  PCHA recently asked the CHD Community,  families and patients, to share their advice for new parents feeling overwhelmed. This is what they had to say.

 

 

1. You Are NOT Alone

CHD is the number one birth defect, there are lot of parents or patients in your community to learn from and lean on. Parents often find it helpful to talk to each other, someone who knows what their going through.
Also, don’t be afraid to take your friends and family up on the help they offer. They love you, and are there to help lighten your load. Let them.

 

2. Take Care of Yourself

You want to be there for your Heart Warrior, but you are only human. Sometimes you need a break too. If you’re not getting the rest you need, you’re less able to effectively care for your child.

 

 

3. Go Easy on Yourself

When you learn your child has a CHD so many different emotions will flood through you. Remember this is normal. Be kind to yourself, and take comfort in that your Heart Warrior will understand you’re doing your best for them.

4. Treat Your Heart Warrior as “Normal” as Possible

Let your Heart Warrior find their own limits, and push them to enjoy as much of life as they can. It’s important that they also learn responsibility and accountability, like their heart healthy counterparts. They  will need to be able to manage their own care and know their condition as they grow up.

 

 

 

 

5. You are Stronger than You Think

The diagnosis is scary, and it can be overwhelming, but you are more capable than you think. And so is your Heart Warrior. Their propensity for resilience may surprise you and provide you will courage.

 

 

6. Take One Day at a Time

There will be good days and bad days, sometimes good hours and bad hours. Enjoy every positive moment, and try not to spend time worrying too much about the future.

 

 

7.  Don’t be Afraid to Advocate for Your Child

It’s OK to question your child’s doctor. It’s OK to get 2nd opinions. You are your child’s voice, and it’s OK to speak up when you need clarification or when your uncomfortable with a situation.

 

 

8. Every Child is Different

It is natural to want to know what to expect and compare our Heart Warrior’s journey with others that have the same CHD. But what goes for one child, may not be true for another.

 

9. Ask Questions and Keep Records

Your child’s doctors will throw a lot of new terms at you and explain unfamiliar procedures. Ask questions about what you don’t understand. There is no such thing as a stupid question.
Write it down. Anything that is important or difficult to remember, write it down.  Keeping records is important to keep things straight , especially if you are seeing multiple doctors at various locations.

10. Educate Yourself

Don’t Google. Make sure you find trusted resources to learn about your child’s condition. Soon you will be an expert in your own right. Be sure the material you read is reliable. Ask your child’s doctor or other heart families for recommendations of sources of information.

 

*Please note the information in this post is not meant to be a substitute for medical advice.

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.

Brie Harrison

It was the strangest thing. Something I can’t fully explain. My entire pregnancy I had a feeling that something was different. Leading up to the 20 week scan I kept saying to my husband “I’m scared. What if something’s wrong!?! I feel like somethings wrong!”.  Call it premonition, call it mother’s instinct, or simply coincidence but I was absolutely right. The sonogram tech spent 2 hours looking at my baby when finally a doctor came in and delivered the news “there’s something wrong with your baby girl’s heart.”.  We were terrified. We had no idea what this meant for our baby girl, our baby girl that we had so many hopes and dreams for. We already had a beautiful son and we were so excited to have the perfect family of four. This news was life changing.

Two days later we had an emergency appointment with a fetal cardiologist where it was confirmed that our daughter had a complex and rare heart defect, truncus arteriosus. We decided right then and there we would do everything possible to get our baby girl the best care in the country. A couple months later we uprooted our life in Florida and moved back home to our families in Maryland. We wanted our child to have all her care and treatment at The Children’s Hospital of Philadelphia and we felt a move was necessary. That’s was decision we will never regret.

I was induced and gave birth to Brie on July 27th, 2017 at 37 weeks. She weighed 5 pounds 8 oz. Brie looked perfect. It was hard to believe without surgery our little girl wouldn’t make it more than a few weeks. We are so proud of our heart warrior. Brie has been through more in her short life than most people will experience in a lifetime. We would later find out that Brie also has 22q deletion syndrome (also known as DiGeorge syndrome). 22q can have over 180 symptoms and we have no idea what will end up affecting Brie but we do know it’s why Brie has her heart defect. Brie underwent her first open heart surgery at 4 days old, has had three heart catheterizations, had stents put in to her pulmonary arteries and a few weeks ago had her second open heart surgery. We’ve had experiences that are beyond terrifying. We’ve seen our daughter bleed out from a procedure, we’ve seen her turn blue and need to be revived, we’ve had to push that horrid code blue button but she’s pulled through it all. She’s truly our miracle. Brie’s CHD has no cure and will require close monitoring with many, many doctors appointments at CHOP. We know she will need more open heart surgeries and procedures as she grows. Brie’s next open heart surgery is expected in 3-5 years. We know this is just the beginning but we are positive Brie will continue rockin’ this CHD and 22Q journey. Brie has brought hope and inspiration to so many families battling similar battles. My grand plans for her may be slightly different now but I know she has big plans of her own and I know her little stubborn self will make it happen. Life wouldn’t be the same without her, she’s given life a whole new meaning for us. We cherish the moments, celebrate each victory, and we’ve come to realize we still have that “perfect” family of four we always dreamed of.

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.

 

 

 

Jeremy Park

Jeremy was born on December 24, 2012, with Hypoplastic Left Heart Syndrome (HLHS). HLHS is a severe congenital heart defect where one side of the heart didn’t develop. In Jeremy’s case, his left ventricle, mitral valve, aortic valve, and aorta didn’t develop. HLHS requires three open heart surgeries to recreate the anatomy in order to survive.

At 4 days old, he underwent his first open heart surgery called the Norwood. He was in the hospital for 18 days. At 4 ½ months old, he underwent his second open heart surgery called the Bi-Directional Glenn. He was in the hospital for 7 days. He had complications after this surgery that required more testing and a heart catherization. At 2 years and 10 months old he had his final surgery, of the 3-stage palliation, called the Fontan. He was in the hospital for 16 days. He has had many other procedures as well.

Jeremy is 4 years old and is thriving!!  He is happy, full of energy, and his heart function is great!  He loves art, gymnastics, T-ball, and swimming. We know he will have more procedures and surgeries down the road, but we are living life to the fullest!  We are thankful for the support and love that we have gotten from everyone!

The House of Representatives is officially #CHDWise – IT PASSED!

Shortly after it’s initial passing into law in 2010, the Pediatric Congenital Heart Association began hosting the Congenital Heart Legislative Conference – in partnership with the Children’s Heart Foundation and The Adult Congenital Heart Association – in efforts to ensure the Congenital Heart Futures Act is renewed in order to meet the needs of the changing CHD community.

In June 2017, the House Committee of Energy and Commerce held a markup on the Congenital Heart Futures Reauthorization Act (CHFRA), H.R. 1222.

Today all that work has paid off and the Congenital Heart Reauthorization Act is one step closer to being put back into law. With a vote of 394 to 7 H.R. Bill 1222 was passed by the House!

This exciting news is a result of hard work, dedication, and brave voices like yours, but our work is not done!  The Congenital Heart Futures Act needs to next make it through the Senate.  Tomorrow when we meet with our Senators we will encourage them to follow the House’s lead.

You can help from home!

Are your Senators on the list of Congenital Heart Reauthorization Act co-sponsors?

If not email them, today!!!

 

Current Cosponsors (Updated 2/25/18)

Senate Cosponsors – Date Cosponsored
  • Whitehouse, Sheldon [D-RI] – 4/7/17
  • Stabenow, Debbie [D-MI] – 4/7/17
  • Klobuchar, Amy [D-MN] – 4/7/17
  • Baldwin, Tammy [D-WI] – 4/7/17

House Cosponsors – Date Cosponsored
  • Pocan, Mark [D-WI] – 3/28/17
  • Connolly, Gerald [D-VA] – 3/28/17
  • Soto, Darren [D-FL] – 3/28/17
  • Evans, Dwight [D-PA] – 3/28/17
  • Holmes-Norton, Eleanor [D-DC] – 3/28/17
  • Murphy, Stephanie [D-FL] – 3/28/17
  • Swalwell, Eric [D-CA] – 3/28/17
  • Loebsack, Dave [D-IA] – 3/28/17
  • Stivers, Steve [R-OH] – 3/28/17
  • Webster, Daniel [R-FL] – 3/28/17
  • McMorris-Rodgers, Kathy [R-WA] – 3/28/17
  • Abraham, Ralph [R-LA] – 3/28/17
  • Guthrie, Brett [R-KY] – 3/28/17
  • Bost, Mike [R-IL] – 3/28/17
  • Mullin, Markwayne [R-OK] – 3/28/17
  • Nolan, Richard [D-MN] – 3/29/17
  • Fitzpatrick, Brian [R-PA] – 4/3/17
  • Collins, Chris [R-NY] – 4/4/17
  • Velazquez, Nydia [D-NY] – 4/6/17
  • Ros-Lehtinen, Ileana [R-FL] – 4/20/17
  • Sessions, Pete [R-TX] – 5/3/17
  • Griffith, Morgan [R-VA] – 5/19/17
  • Ellison, Keith [D-MN] – 5/23/17
  • Moulton, Seth [D-MA] – 6/26/17
  • Cardenas, Tony [D-CA] – 7/13/17
  • Walberg, Tim [R-MI] – 7/13/17
  • Meehan, Patrick [R-PA] – 9/5/17

Sample email:

Send an email, today!
Sending an email is simple!!

  1. Find your legislator’s contact information.
    1. Visit  www.senate.gov or www.house.gov
    2. If this is your first time reaching out, use the contact form on the legislator’s website to send your email.
    3. If you have identified the Health LA, or had a previous contact with your member or a member of their staff, please feel free to use the direct email address you were given.
  2. Copy and paste the sample email, below, and personalize:
    1. Include your legislator’s name
    2. Add your own personal story where indicated
    3. Sign with your name, city, state and contact info
  3. Send it!

—— SAMPLE EMAIL —–

Dear Senator XYZ,

As you are working on appropriations requests for FY2018, I urge you to show your support for continued funding of essential congenital heart disease-related public health research and surveillance initiatives at the Centers for Disease Control and Prevention.

Congenital heart disease is the most common birth defect and the leading cause of birth defect-related infant mortality. Nearly one third of children born with CHD will require life-saving medical intervention such as surgery or a heart catheterization procedure. With improved medical treatment options, survival rates are improving with a population of 2.4 million and growing. However, there is no cure. Children and adults with congenital heart disease require ongoing, costly, specialized cardiac care and face a lifelong risk of permanent disability and premature death. As a result, healthcare utilization among the congenital heart disease population is significantly higher than the general population.

As part of these ongoing public health surveillance and research efforts, the Centers for Disease Control and Prevention recently published key findings that report hospital costs for congenital heart disease exceeded $6 billion in 2013.

Congenital Heart Disease is common and costly, and attention to the needs of this community is critical.

We urge Congress to cosponsor the Congenital Heart Futures Reauthorization Act (S.477). To cosponsor this important legislation please contact Max Kanner (max_kanner@durbin.senate.gov) with Senator Durbin’s office or Shayne Woods (Shayne.Woods@mail.house.gov) with Congressman Bilirakis’ office.
This is important to me because: (ONE-TWO SENTENCES)
Share your story briefly, here.

Odds are, someone you know has been impacted by the most common birth defect.

Join us as together, we are #ConqueringCHD.

Sincerely,
Your Name
Address

Join our D.C Advocates and Participate from Home Today!

As you read this, nearly 200 fellow advocates are on Capitol Hill meeting with their Members of Congress as part of the 2018 Congenital Heart Legislative Conference. They are asking their lawmakers to support research, data collection, and awareness activities related to congenital heart disease (CHD). This includes recruitment of co-sponsors for the recently introduced Congenital Heart Futures Reauthorization Act of 2017. With the passage of the house version of the bill last night

We need you to participate from home!

Help us bring the voice of CHD to Washington by sending an email to your Members of Congress.

It’s as easy as 1, 2, 3!

  1. Find your legislator’s contact information.
    – If this is your first time reaching out, use the contact form on the legislator’s website to send your email.  Visit www.senate.gov
    – If you have identified the Health LA, or had a previous contact with your member or a member of their staff, please feel free to use the direct email address you were given.

2. Copy and paste the email below – adding your own personal story.

3. Send it!

You did it!  If you successfully connect with your legislator or their office, let us know you made contact by completing our online form available here.

This is a very exciting day and a great opportunity to work together to Conquer CHD!

__________

Sample Letter:

Subject: Support Congenital Heart Disease Research

Dear Senator [Fill in name here],

I’m writing to urge you to support federal research, surveillance and awareness for congenital heart disease (CHD).

Every 15 minutes, a baby is born with congenital heart disease, the most common birth defect and leading cause of birth defect related infant death.  Even for those who receive successful intervention, it is not a cure. Children and adults with congenital heart disease require ongoing, costly, specialized cardiac care and face a lifelong risk of permanent disability and premature death. As a result, healthcare utilization among the congenital heart disease population is significantly higher than the general population.
Continued federal investment is necessary to provide rigorous epidemiological and longitudinal public health surveillance and public health research on infants, children, adolescents and adults to better understand congenital heart disease at every age, improve outcomes and reduce costs.

We urge Congress to:

  • Cosponsor the Congenital Heart Futures Reauthorization Act (S.477). To cosponsor this important legislation please contact Max Kanner (max_kanner@durbin.senate.gov) with Senator Durbin’s office.
  • Provide $7 million in FY2019 to the Center for Disease Control and Prevention’s National Center on Birth Defects and Disabilities to support surveillance and public health research to build upon current activities to better understand the public health impact of congenital heart disease across the lifespan. Provide 36.2 billion for FY2018 and 38.4 billion for FY2019 to the National Institutes of Health to support efforts to develop innovative and cost-effective interventions for those living with Congenital Heart Disease.

If you would like any additional information please contact Amy Basken at abasken@conqueringchd.org.

Thank you-
[Your Name – your city/state]