Mother’s Day – A Mother’s Day Gift

Mother’s Day is a day to shower the women that have loved us  and cared for us with gifts in appreciation!  We bring them flowers and homemade cards, in hopes of bringing a smile to their faces. But last Mother’s Day, Melissa  Zolk  received a very different kind of gift, one that changed her daughter, Maxine’s life.  

 

 

Mother’s Day – Those two words hold a meaning unlike any other, especially if you are a heart mama.  To a heart mama, they mean countless doctor’s appointments, medications, syringes, feeding tubes, therapies, hospital stays, and surgeries.  They mean finding strength in moments when you thought you had none left.  However, they also mean understanding differences and embracing the beauty that comes with those differences.  The beauty of zipper scar lining your child’s chest.  They mean understanding true joy because you have experienced true sorrow.  The sorrow that began the moment you heard, “There’s something wrong with your baby’s heart!”  Those words start a journey filled with the unknown.  And little did we know what our journey would be like with our heart warrior.  We received the devastating news at our 20 week ultrasound and from that point on, our journey took the path unexpected.  Though now as a more experienced heart mama, I know that the unexpected is to be expected.  Our daughter, Maxine, was born on October 1, 2015 with Transposition of the Great Arteries, Pulmonary Stenosis, and Ventricular Septal Defect.  She had three heart surgeries including one open heart surgery before she turned one year old.

 

For me, Mother’s Day has even more meaning now.  It still means medications, appointments, procedures, and worry, but it also means the GIFT OF LIFE.  Last year on Mother’s Day, we received the call that changed our world – after living in the hospital and being listed for 137 days on the heart transplant list, Maxine was getting her new heart!  Oh, how the emotions came flowing.  Flowing free and fast and yet again, unexpected.  I think the most unexpected feeling was the peace that I had.  Yes, I was nervous and scared and worried and excited, but a feeling of peace came over me and lingered for a while.  Sometimes, I wonder if maybe the reason I felt so at peace was because maybe the family who chose to give us this incredible gift in their time of incredible grief felt more at peace knowing that part of their child would live on within my child.  Mother’s Day means my child received the greatest blessing, a second chance at life.  It means that this year I get to celebrate at home with my husband and our three beautiful children.  And on this Mother’s Day and every day to come, I promise to remember the mama whose heart is breaking because she lost her child last Mother’s Day.  A day she will remember for a completely different reason than the reason I get to remember.

 

To my fellow heart mamas and all mamas, Happy Mother’s Day!  Whether you are celebrating with your child here on Earth or your angel up in Heaven, I want you to know that I promise not to forget you and the amazingness you are as a mother.  You were created to be the perfect mama for your baby and you are exactly what your baby needs.  My hope is that you can remember that when the days are long and difficult because life will take unexpected turns and we will be there to help our babies get through it!  Because, well, we are mamas and that is what we do!

 

 

 

 

 

Hi there!  I am Melissa.  Mama of three crazy, beautiful children, ages 3 and under and wife to the most amazing heart daddy on the planet.  Our middle child is our heart warrior, and she is an energetic little stinker!  Besides being a heart mama, I am a high school, special education teacher.  In my free time, I like to make phone calls to doctors, pharmacies, and insurance companies. Ha, just kidding!  Our heart warrior has been on quite the journey these last few years.  If you would like to follow her journey, you can find her at Maxine the Mighty Heart Warrior on Facebook.

Mother’s Day – Love, Brett

With Mother’s Day on the horizon, PCHA begins a series honoring Heart Moms. This week, Brett Nishibayashi shares a letter to his wife about their journey becoming CHD parents and his admiration for her love and strength.

I remember when you first found out you were going to be a mother. The disbelief and overwhelming excitement all at a bubbling intensity was paralyzing. Unsure of what true emotion to feel, we embraced one another and leaned on our unconditional love and faith to guide us through. It’s ironic that these same pillars of our relationship are what we would need to rest on heavily sooner than later. Unfortunately we weren’t able to bask in the joys of pregnancy for long because we were given a different journey. Your path has never truly been the “normal” path and you have never been granted an opportunity to be a “normal” mother. You are far beyond the normal in all you do and motherhood is no exception. You are always perceptive and aware, constantly observing and analyzing, but even with those admirable qualities, nobody could predict your true calling.

I vividly remember when you first found out you were going to be a heart mother.  I remember the anxiety of the ultrasound. I remember the tremble in your hand. I remember the tears in your eyes. The car ride home, the vulnerability and the confusion of how this could be our story all resonate deeply in my soul. I mention these moments because they are the last days that I remember of the woman I met and married. From that point on you evolved into the new and improved version of that woman and I am still amazed at that transformation daily.

Since that moment, until long after you read this, you have been a fierce advocate and a perfect example of a nurturing and invested mother. Inspiring doesn’t begin to encapsulate the way you attacked this diagnosis and all of the pertinent information that comes along with it. You immersed yourself in the CHD community learning as much as you could about Avery’s condition, its pitfalls and how we were going to mitigate its effects as best we could. None of this was easy as we stared in the face of the “what ifs” that lurked in our minds. The uncertainty of it all can leave you searching for more and more information, which once obtained, doesn’t necessarily subside the fear and anxiety of the imminent future.

I mention all of this because I want you to know that I see you. I see you hurdling these obstacles as they are thrown at you with intent and grace. I see you losing sleep and how it chips away but still finding the energy to flash a refreshing smile and hearty laugh. I see you trying to be the best mother, wife, therapist, cardiologist, activist, pre-school teacher and I’m sure many more things that you can be.  I see you trying to remain perfect in an imperfect world and it inspires me. I see how hard you push yourself but more importantly I understand why. I see you reaching out to help other heart families, recognizing that we are part of a larger community of heart parents.

I see this and so much more when I look at you. You are the perfect mother for the perfect little girl and I am truly the lucky one. The other day I mentioned to you how I really thought you were an outstanding mother and you looked back at me with disbelief. Let me assure you that there should never be a single doubt in your mind on whether or not you are a great mother. Your love, compassion, attentiveness, patience and drive are unquestionably why our family enjoys so much happiness. I want you to know that you should always be confident of how well you are doing EVERYTHING. You deserve every blessing you receive and all those that are still to come. I wish you calm and peace on this your third heart mother’s day. May the serenity you deserve be all that you need it to be. I will  always see you because I can’t take my eyes off of you.

Love Brett

 

 

Brett Nshibayashi is a 39 year old heart father from Chicago, IL. He is a high school PE teacher and has been married to his wonderful wife for 3 years. Brett considers himself honored to be a part of this community of heart parents.

Beyond the Heart – Nauman’s Story

Adult patient and medical student, Nauman Shahid, shares what he learned about the way different body systems interact with one another through Medical School and personal experience. Here, he shares the complications that developed with his lungs as a result of his Congenital Heart Disease.

 

When we think “Congenital Heart Defect” we often think about the “heart” and only the heart, but as someone who attends an Osteopathic Medical School, Ive come to realize that this is only the tip of the iceberg in the realm of CHDs. One of the tenants of the Osteopathic medicine philosophy happens to be in how the body is a unit as described by the founding father Dr. Andrew Still. Within this unit lie different organ systems that work in sync to create a balance of homeostasis. Its when we have a disturbance in this “homeostasis” is when we have a diseased state that may often require medical intervention in addition to the body’s own ability to self regulate. In this short piece, I will attempt to introduce one piece of this unit at the organ level to facilitate the understanding in how a Congenital Heart Defect is much more than just the “heart” with a focus on respiratory/pulmonary ailments.

Nauman with Representative Gus Bilirakis at the Legislative Conference in Washington D.C.

Having Tetralogy of Fallot diagnosed at a very young age, my parents were often told of the potential respiratory issues that may arise during the course of my life. As such, many Tetralogy of Fallot patients often require a pulmonary valve replacement by virtue of the initial repair surgery in which the pulmonary valve is disrupted. Coupled this with having a stenotic left pulmonary artery, this substantially increased my right sided heart pressures and decreased the blood flow to the lungs in general due to having pulmonary artery hypertension. Overtime this can cause issues in the long run as it did for me. As a result, during times of strenuous exercise or any activity in which it increased the heart rate, I would find a great amount of shortness of breathe coupled with lower extremity edema. This can be thought to have similar implications as an infant having a crying “TET” spell. This often occurred as a result of the lungs not receiving adequate amounts of blood. This resulted in a back up of blood in reverse all the way down to the lower extremities. A way to think about this is, imagine having a backup of water in your sink due to having a faulty water sink disposer. The water will continually collect unless the core of the issue is resolved. In which case, for me, was a faulty pulmonary valve coupled with a stenotic left pulmonary artery which caused respiratory issues.

You might be asking, “how does all of this relate to a pulmonary issue”? To answer this, we need to first define pulmonary artery hypertension, in which it is a condition where the blood pressures are increased within the arteries of the lungs. Pulmonary Hypertension is a broad spectrum term which has 5 distinct groups of etiologies as defined by the World Health Organization. In my case, having a stenosis of the left pulmonary artery increased the pressure and caused a back up of blood which in turn causes the heart to work harder. It should be noted that normal pressures for the pulmonary artery are usually below 20 mmHg. However if this pressure rises to above 25 mmHg during rest, it is by definition pulmonary artery hypertension. It is very sensitive to minute changes in pressure and hence why the right side of the heart that leads into the lungs is called the “low pressure system”.

For me as the patient, it caused a number of different issues. I often had increased exercise fatigue, shortness of breath when playing sports, and palpitations. Being in medical school, it did not help me where I would sit for long durations of time. During this time, I got a bit ill and developed pneumonia. I had repeated trips to the emergency room near my medical school. As a result, many of the ER doctors I saw were obviously not trained enough in the realm of dealing with congenital heart defects and were also confused when administering certain acute therapies including nebulizers and other respiratory treatments with a probable focus on it being a pneumonia infection. Additionally asthma was also likely present as well with the larger issue ofcourse was dealing with these EKG changes that got many doctors confused. When I initially started to get palpitations, and shortness of breath, I used to think the acute altitude changes were a contributing factor since my medical school is a bit higher in elevation in rural West Virginia. While that may also have contributed to my symptoms, it ofcourse was not the entire story. During this time, I remember my mother telling me all the time, “Nauman, your heart is telling you something, you should listen to it”. As they say, mothers are never wrong, and rightly so. The exercise fatigue, shortness of breath ended up being much more than just asthma or pneumonia alone. Finally, I got to see a Cardiologist after narrowing down that it was likely my heart that was acting up which was now affecting my lungs as well. It was only with my Cardiologist in New Jersey did I learn about the gravity of the situation and the likely intervention required.

The million-dollar question? How do you fix this? Well thankfully with the advancement of congenital heart medical care, patients with similar presentations have a multitude of options including surgical and medicinal therapeutic interventions. Initially I went in for a catherization at Cincinnati Children’s Hospital to replace my pulmonary valve via a transcatheter procedure. However, the diameter of the Right Ventricular Outflow Tract (RVOT) was simply too large as a result of a slightly dilated heart making the transcatheter procedure a non-viable option. This rendered open heart surgical intervention as the only choice which came as a blessing in disguise. Under the careful direction and precision of my surgeon at Cincinnati Children’s Hospital, they not only replaced my pulmonary valve, but repaired my left pulmonary artery,
reconstructed my RVOT, and repaired a hole in the septal leaflet of my tricuspid valve which came about as a result of my severe pulmonary valve regurgitation.

The take home message? Depending on the complexities of your congenital heart defect, it is imperative to understand that no two CHD’s will be identical with a varying degree of severity. With this, it is important to note that as in my case, it is not just about the heart in a localized fashion. This was just one example in how something as simple as 3 pieces of tissue coming together to make a valve that open and close due to pressure differentials can cause such a dramatic effect. This being, lower extremity edema, increased right heart sided/pulmonary pressures, shortness of breath and ultimately damaging the heart to a point of no return effecting the systolic (pumping function) of the heart. Furthermore, the artery connecting the right ventricle and the pulmonary trunk may get stenotic to where one may have higher pulmonary pressures and thus have pulmonary hypertension. For this reason, it is imperative to understand the human body as a unit of different organ systems working in concert to achieve the process we so often taking for granted known as life. By addressing the core etiology that disrupts our homeostasis you can address each component collectively and individually which in my case was the dysfunction between the circulatory (heart) and respiratory systems (lungs).

I never really knew how much these issues were effecting me until I got treatment. Although my recovery from open heart surgery was anything but normal due to having pleuritis and other issues, I have come out well. In fact aside from my ability to return back to my fitness regimen, I noticed a great increase in my intellectual capacity to where I have been ranked in the top tier of my medical school class as I am no longer fatigued or tired during the rigors of medical school. While I know, many of my fellow heart warriors are not looking forward to a possible surgical intervention, I can safely say everything indeed happens for a reason. If it wasn’t for my pneumonia, they may not have ever picked up on my EKG changes. As my mother said it best, “Listen to your heart, its telling you something”, and finally my heart is content with this outcome because while I may not be where I want to be, I am simply glad I am not where I used to be.

Nauman Shahid is a 31 year old Tetralogy of Fallot Patient who is well known in the congenital heart community as a regular contributor on various social media platforms. He is involved in various congenital heart awareness initiatives including being a Hearts Unite the Globe (HUG Network) Volunteer as a Guest Host for their CHD radio show (Heart to Heart with Anna), Chief Operations Officer/Board Member for the Tetralogy of Fallot Foundation, recipient of the PCHA Scholarship to attend the 2018 Congenital Heart Legislative Conference in Washington DC where he met lawmakers to advocate for H.R. 1222 (Congenital Heart Futures Reauthorization Act of 2017), and selected for the 2018 PCHA Calendar for the month of November. However one of his strongest contributions comes as a result of him being a dedicated medical student where he lead an awareness campaign on campus during Congenital Heart Awareness week on Feb 9, 2018 where he along with a few dedicated classmates distributed purple ribbons along with relevant literature in an effort to inform the next generation of breeding physicians about CHDs. When Nauman is not studying, he is often found to either be boxing in the gym, BBQ/cooking some of his favorite foods, touching base with fellow heart warriors and their families to lift their spirits or resting up listening to his favorite soft rock tracks. Nauman will be attending a 2 week clinical internship at Cincinnati Children’s Hospital’s Heart Institute to observe the various complexities of cardiac ailments in the clinic and surgery. He has earned a Bachelors of Science in Biology and a Masters of Public Health (Honors) with a focus on Health Policy. He attributes much of his success to his family and aspires to help heart warriors with congenital heart defects as a future physician.

Recap – New Diagnosis

Becoming part of the CHD community can be a shocking and overwhelming experience. Our series on New Diagnosis offers new family a view into the fist steps of others who have come before them, in hopes they may ease the the journey if only just a bit.

 

New Diagnosis – Prenatal Conquering CHD Kits

 

New Diagnosis – One Day At A Time, A Heart Mom’s Story

New Diagnosis – Mary Beth’s Story, Diagnosed in Adulthood

New Diagnosis – Baby Shower

New Diagnosis – Jaclyn’s Story

New Diagnosis – Fetal Echo

New Diagnosis – Pulse Oximetry

New Diagnosis – Brooke’s Story

Pre-Existing Condition by Dr. Stephen Caldwell

After the diagnosis of his son, Dr. Stephen Caldwell, used music to chart his family’s experience with CHD. Watch below, and find the lyrics to “Pre-Existing Condition” and Dr. Caldwell’s thoughts on his work. 

 

University of Arkansas Schola Cantorum

Dr. Stephen Caldwell, Director

Pre-Existing Condition*                                                Stephen Caldwell (2018)

                      1. Congratulations!

                      2. Small Hands

                      3. Angel One

                      4. CVICU

                      5. Scars

                      6. The Deepest Secret

 

Hannah Mindeman, Piano

Nikola Radan, flute

Kyleigh Pell, marimba

Chris Scherer, vibraphone/glockenspiel

Collette Soriano, drum

 

 

*World Premiere Performance – https://youtu.be/uQjxhjrcnpc

Funded in part by Fulbright College of Arts and Sciences 2017 Summer Faculty Research Grant

 

 

Notes from the Composer:

In 2016, my wife and I were expecting our first child. The joy and anticipation of a routine ultrasound turned into nightmare, an experience that many CHD families share. The technician became very quiet. He zoomed in very carefully, taking more pictures than normal of our child’s heart. He sat very still. “Mr. and Mrs. Caldwell, I am going to get the Doctor…”

Like 40,000 other babies each year, our son was born with a Congenital Heart Defect. His diagnosis left us speechless: Tetralogy of Fallot with conoseptal hypoplasia, right-sided aortic arch, left-ventricular systolic dysfuction, and left branch pulmonary stenosis. The next several months unfolded like a dream that included multiple medivac helicopter rides, more than 100 days spent in the CVICU, two open heart surgeries (a palliative procedure to install a Central Shunt at 6 weeks, and complete repair at 9 months), millions in medical bills, 20,000 miles driven between where we lived in Fayetteville, AR, and Arkansas Children’s Hospital in Little Rock, and stress on an indescribable scale.

Pre-Existing Condition chronicles that journey, from the onslaught of medical terms thrown at parents when they receive the diagnosis, through the birth of a medically fragile child not knowing if they will live, through helicopter rides, 911 calls, ambulances and ERs, the stress of extended CVICU stays with their uncomfortable chairs and constantly beeping monitors, and the cloud of emotion that runs around these events.

At the center of the chaos, however, is a child. A child that needs love, attention, and support. A child that shows their parents what courage is. Children born with CHDs are remarkably fierce in their determination to live, and are immensely proud of the scars they bear. Though labeled for life by medical and insurance companies as having a “Pre-Existing Condition” these children fight against the tide of that label, to prove to everyone that every life has equal merit.

 

 

Pre-Existing Condition

I. Congratulations!

Mister and Missus, I’m going to get the Doctor.

Anomaly…results of the ultrasound…

Results of the echocardiogram…your child has

Anomaly of the heart…Tetralogy of Fallot…

Ventricular septal defect….congenital

No blood flow to the lungs…and the aorta isn’t in the right position

May not survive.

 

Right ventricular outflow tract…right-sided aortic arch

Pulmonary artery is too small…won’t be able to breathe

Here’s a drawing of a healthy heart – this is what your baby looks like…

Won’t be able to breath…pulse-ox is low…

May not survive.

 

Surgery.

 

Perfusion, myocarditis, cyanosis, Tet Spell

Diminished systolic function.

Truncus arteriosis, transannular patch, NPO

Transposition of the arteries, ECMO

Capilary refill, pacing wires

Hypoplastic Left Heart Syndrome

Conoseptal Hypoplasia

Captopril…milrinone…stenosis…

Coarctation of the aorta…probably dysphagia

Patent ductus arteriosus…expect emisis…tachycardia

And watch for aspiration…bradycardia…

Diminished Systolic function.

May not survive.

It’s not the end of the world.

There’s a risk of mortality.

It’s not your fault.

We need your consent.

Do you have insurance?

It’s a boy!

The ICU is $8,000 per day.

May not survive.

Congratulations!

 

II. Small Hands (from Somewhere I have never travelled, gladly beyond, by ee cummings)

nothing which we are to perceive in this world equals

the power of your intense fragility: whose texture

compels me with the colour of its countries,

rendering death and forever with each breathing

 

(i do not know what it is about you that closes

and opens; only something in me understands

the voice of your eyes is deeper than all roses)

nobody, not even the rain, has such small hands

 

 

III. Angel One

The ascent seems slow,

Like a rollercoaster clicking away at your fears,

But the fall never comes.

The light is heavy, full of guilt,

The engine’s pulsing onslaught reminding you of your every breath,

Giving the vibrations in the air memory.

The restraints dig and cut.

Frantic haste masked by stillness.

The grass is so green! The sky so blue!

As the Angel slices through the cloudless view,

Beating its heart in a race against another,

Extinguishing like the moonlight at dawn.

 

IV. CVICU (after Li Po)

Amongst the chaos, I am along with him.

Staring at a cup, half-full of water,

Brought by a nurse, who said I looked thirsty.

I ask the moon to drink with me.

Then I sigh, for the moon cannot drink.

Surrounded by people, but alone, I sing to him:

“Hush now, little baby boy.

Go to sleep, and dream your little dreams.”

I’m glad to make the moon my friend.

But when it becomes too much, we part.

For now, I need steady emotion.

I hope someday we all will meet again,

Deep in the Milky Way.

 

V. Scars

What will they think of you and me, when they see our scars of such degree?

What will they wonder, with quiet nod, what did they do to be punished by God?

Did they not pray? Did they not sacrifice?

“My kids are fine because we worship Christ.”

They give you a smile, but once out of sight, they say to themselves,

“That kid isn’t right.”

 

What will they think of you and me, when they see our scars of such degree?

Will they think you a warrior with courage so brave?

Will they think you a hero, of the lives that you’ve saved?

Will they ask of your battles, of your stories and lore?

Of combat and fighting and bullets and war?

Should we tell them this anyway and hope they’ll see,

That we don’t need their sympathy?

 

What will they think of you and me, when they see our scars of such degree?

Scars are forever, there to see, what has happened to you has happened to me.

Wounds of the flesh one can hide. Wounds of the soul are kept all inside.

Our scars are the same, yours and mine.

Your scars of the body, my scars of the mind.

 

VI. The Deepest Secret (i carry your heart, by ee cummings)

i carry your heart with me (i carry it in

my heart) i am never without it(anywhere

i go you go, my dear; and whatever is done

by only me is your doing, my darling)

i fear

no fate (for you are my fate, my sweet) i want

no world (for beautiful you are my world ,my true)

and it’s you are whatever a moon has always meant

and whatever a sun will always sing is you

 

here is the deepest secret nobody knows

(here is the root of the root and the bud of the bud

and the sky of the sky of a tree called life; which grows

higher than soul can hope or mind can hide)

and this is the wonder that’s keeping the stars apart

 

i carry your heart (i carry it in my heart)

 

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.

 

Beyond the Heart – Rebeka’s Story

CHD can affect so much more than the heart, yet physicians unfamiliar with Congenital Heart Disease may have trouble seeing the big picture. In this week’s post Rebeka Acosta, PCHA Steering Committee member and Mom to two heart warriors, shares the struggle of coordinating care outside of cardiology.

In the last few decades, science and research surrounding congenital heart disease has uncovered many connections to other body systems. As the parent of two heart warriors with additional diagnoses and unique family histories, it is imperative for me to keep up with research, be able to educate non-cardiac
practitioners, and advocate for my kids’ entire bodies. If that sounds like a full-time job, you are correct!

 

 

My youngest son has single ventricle anatomy and experiences challenges in the neurodevelopmental and neuropsychology spheres. Difficulties with word recall, attention and focus, and auditory processing have left many clinicians with puzzled looks on their faces. Some prescribed therapies with scientifically unproven results, while refusing the connection to CHD. Others were willing to learn and adjust their techniques to best work with him. Looking back, gross motor delays around 3-5 years old were the least of his challenges!

 

My oldest son with minor anatomical cardiac differences has multiple airway and pulmonary disorders, which include treatment from multiple specialists. Who knew the airway was as complex as the heart? His airway diagnoses are part of the outdated school of thought that “kids will eventually grow out of it”, so it has been a several years battle to find clinicians that specialize in and treat them. We are all excited for his evaluation next month and the possibility of a surgical repair!

 

 

Our family has made tough choices regarding medical treatment, mental health, and education for both of our children. They were not always popular amongst family or friends, but they were best for the kids. After eight years parenting these awesome warriors on the CHD roller-coaster, we are on the right track. For everyone starting out, remember to speak up and do so loudly. You are the
expert and your child’s primary care provider.

 

 

Rebeka Acosta lives in Las Vegas with her husband, two heart warriors and two Great Danes. A researcher by nature and healthcare finance coordinator by training, she enjoys keeping up with congenital cardiac literature and always reviews insurance claims line by line. Before relocating to Southern Nevada, Rebeka was a founding member of the Washington chapter of PCHA. She now volunteers on the National Steering Committee and enjoys attending the annual legislative conferences and
transparency summits. Rebeka welcomes connections at racosta@conqueringchd.org. You can also follow Gavin & Taosies’ CHD adventures on Instagram.

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.