We don’t always celebrate Mother’s Day with a tangible gift. Sometimes the day serves as a reminder of the blessings that Motherhood is on its own. Below, Sarah shares her story about what Motherhood had taught her.
There are so many valuable life lessons I have learned in my 8.5 years as a mother. Not only have I learned more than I ever imagined, I believe the discoveries are unique to each mother. For instance, I have realized and been touched by various
events that may not impact some mothers as they do for others. This is what I believe to be a special component of motherhood.
Mother’s Day has become even more special to me since the birth of our 22-month old heart warrior. While every day is a joy with both of my children, Mother’s Day is an extra reminder of how fortunate and blessed to have two children I learn so much from on a regular basis. I truly take no day for granted. While our journey is full of uncertainty raising a heart child, I treasure every moment.
If I had known what I may have experienced when becoming a parent prior to motherhood, I may not have ever become a mother! My life as a mother has been very eventful; both full of joys and many hardships – some of which many mothers never experience. However, some of these difficult times, such as the diagnosis and journey with our son’s complex heart condition have made me a better and stronger mother and person. I have since realized more so now than ever, what is important in life – what is needed and what can be lived without. Less is truly more.
Motherhood, has blessed me in more ways than I could ever describe and even more so since I became a heart mom. While the times in our journey have not always been good; they have led to where I am today and I am proud to have two loving, caring, courageous, and energetic boys who remind me what life is all about.
Each year, I look forward to Mother’s Day and am thankful I am a mother of two remarkable boys who remind me of how lucky I am, especially our little miracle child and warrior. Motherhood has helped me grow as a person and professional, for which I am so grateful.
The author, Sarah Diamond, has a passion for the non-profit sector, working with volunteers, writing, and educating. She holds a Doctor of Education in Educational Leadership and works in non-profit management.
In 2016, she partnered with two colleagues to form a non-profit, where she serves as the President and CEO of the organization, which helps families experiencing medical conditions.
She and her husband have two sons and enjoy spending time together outside doing many activities like riding horses, running, fishing, and gardening.
Most of us spend Mother’s Day with our children, but for Kristin Burns, who lost her son to CHD, the day means something different. Read her story below.
I remember the day that I become a part of the CHD community. It was August 18th, 2016. Since that day, my life has been changed forever. I have learned so much, and I have grown exponentially as a person. My first and only son, Brycen Alvin Burns, was diagnosed with CHD at 20 weeks gestation. From that day forward, he fought for his life every single day. Watching Brycen fight for his life, taught me so much as a mom and as a person. He taught me how to be strong, and how to fight with all my heart and never give up. He taught me how to be an advocate for him, and how to always trust a mother’s intuition, as its almost always right. He also taught me how to love with every ounce of my being, with the knowledge that life and death hang in the balance, and that every day we got with him was a gift. After 229 days here on Earth, Brycen was taken from us. He passed away as a result of complications from his heart defects and other medical issues. On July 29, 2017 I became a childless Mother, and the Mother of a Heart Angel. Being a childless Mother is not how I thought I would be spending Mother’s Day this year.
My first Mother’s Day was spent in the ICU at Children’s Hospital and Medical Center in Omaha. Brycen was recovering from 1 open-heart surgery, and 3 additional open chest surgeries, including a very risky tracheal reconstruction. Amid the humming of medication pumps, and the beeping monitors, we still celebrated the holiday. Brycen was battling withdrawals, but I was just so thankful that he was still here with us, that I didn’t care about the struggles. I remember trying to make the staple “First Mother’s Day” crafts with Brycen and my husband Jeremy. I remember laughing at how imperfectly perfect they were, and I remember thinking that there was always next year to make better ones. Little did I know what the future was going to have in store.
This year, everything is different. I no longer have the tangible and physical evidence that shows I am a mom. I will always be Brycen’s Mom, but now that he is no longer with me, it is hard to hold onto that identity. In fact, sometimes I like to go back to the hospital, because that is the only place that I am still referred to as Brycen’s Mom. But most times, I don’t even feel like a Mom anymore, because I don’t get to do the things that mom’s get to do. I don’t get to change diapers. I don’t get to play with my child. I don’t get to tell him to stop climbing on everything. I don’t get to do anything that mom’s get to do. Instead, I get to visit my son’s grave. I get to talk to him and call out to him in my prayers. And I get to tell people his story about how hard he fought from the day he was conceived, up until the day he passed away. These are the things that I get to do as a childless Mother on Mother’s Day.
Once you lose a child, the holidays are tough. Mother’s Day will be another “first holiday” without Brycen, and I can only imagine how difficult it will be. Social Media will be flooded with family pictures, pictures of beautiful flowers and of handmade gifts from children to their Mothers. And people will be posting about how great their Mother’s Day was. But then, there are loss moms like me. On Mother’s Day, we don’t get to wake up to any of that. Instead, everyday we wake up, we wake up to the same living nightmare that our child is gone, and we get to relive that moment, over and over again. And just like every other day after loss, we will wake up, put on our “faces” and put one foot in front of the other. This is our new world; because our “normal” died the day we lost our child. We have become experts at carrying our grief with us, each and every day. We have become experts at hiding our grief behind love and joy and even behind pain and sadness. We have had to become experts at this, because grief never goes away. Grief is forever, and lifelong, just like our love for our child. Grief is always there, but we just learn to push through it.
I personally have mixed emotions about Mother’s Day now. I find myself wondering if I should even be celebrated, as I no longer have my son with me physically. But then again, I am Brycen’s mom, and no one can ever take that away from me. I guess my wishes for Mother’s Day are simple, and they include all of the loss moms/bereaved moms in the world. Please don’t forget about us. Please don’t avoid us, and please don’t avoid saying our child’s name. They existed and will always be our children. But please don’t be offended if we distance ourselves for a little while to collect our emotions. Please be patient with us, and know that we are hurting. While we watch you play with and hug your children, our arms and our hearts are aching to hold our children just one more time. On Mother’s Day we will celebrate your joy with you, but we will be experiencing pain. A pain that is unimaginable. So please don’t forget about us.
To all of the mom’s that have lost a child, or multiple children, this is for you. This is to show you that you are not alone on Mother’s day, or any other day. We are all in this together. We are all members of this club that we never asked to be in. Just know that it’s okay, to not be okay. It’s okay to distance yourself, and to take time for you. Your loved ones will understand. Grieving mothers are some of the strongest women in the world, and so we celebrate you on Mother’s Day, and every other day! From one loss mom to another, I see you and I understand. Happy Mother’s Day!
Kristin Burns is a 30 year old Heart Angel Mom, from Council Bluffs, IA. She is a Physical Therapist Assistant and has been married to her husband Jeremy for 3 years. Their son Brycen was diagnosed in utero with several medical issues including Congenital Heart Defects. Due to Brycen missing his left lung, seeing his entire heart anatomy was difficult due to positioning, and they were given several different diagnoses. After his first open-heart surgery his full diagnosis was VSD, ASD, Overriding Aorta, Hypertrophic Right Ventricle, Double Aortic Arch and a Vascular Ring. Since becoming a part of the CHD community back in August of 2016, at Brycen’s first diagnosis, Kristin and her husband Jeremy have been strong advocates for CHD research and raising CHD awareness. With the help of another Heart Angel Mom, Kristin is helping start a non-profit organization to help other CHD families at the local Children’s Hospital and Medical Center in Omaha. They are also helping start support groups for inpatient families and also for bereaved families.
In a follow up to PCHA’s series on complications resulting from CHD, Dr. Nidhy Varghese, of Texas Children’s, adds insight to the pulmonary implications in patients affected by Congenital Heart Disease.
Pediatric Pulmonary Complications of Congenital Heart Disease
Dr. Nidhy Varghese, pediatric pulmonologist at Texas Children’s
Congenital heart disease is the most common birth defect in the world, affecting the hearts of thousands of children each year. However, congenital heart disease (CHD) affects more than just the heart. Abnormal cardiac development can have significant effects on other organ systems such as the lungs. The lungs are directly connected to the heart and they share a unique relationship of development and physiology. Effects of CHD can be seen in lung growth, in anatomy and in lung function. Although these can be considered as prenatal and postnatal consequences, the pulmonary complications of congenital heart disease represent a spectrum of consequences.
What is the function of the lungs?
The lungs are the gas exchanger for the body. A multilevel organization of airways present oxygen and remove carbon dioxide through contact with a rich network of blood vessels in the lungs. The lungs are connected to the heart through the pulmonary artery and the pulmonary veins. They’re located in the chest, on the sides of heart.
The lungs are made up of conducting airways, air sacs (known as alveoli) and blood vessels. These structures begin to form by the end of the first month of embryonic development. The development of the vascular system within the lungs and the branching of alveoli are directly related to circulation. Abnormalities in blood flow due to CHD can cause alterations in development of the lungs’ vascular system (arteries, capillaries and veins) and the division of the airspaces into the millions of alveoli needed for adequate gas exchange. This results in simplification of the alveolar and vascular networks, which can produce symptoms of chronic lung disease and pulmonary hypertension.
In the setting of CHD, the flow of blood within the heart and through the blood vessels is abnormal. Different parts of the heart or the blood vessels themselves may become enlarged or may be atypically located within the chest, causing compression on the nearby lungs and airways. CHD examples in which this can be seen are anomalous innominate artery and conditions causing cardiomegaly (an enlarged heart). Compression can cause respiratory distress, stridor, wheezing and recurrent areas of lung collapse.
In certain types of CHD, there may be increased blood flow to/through the lungs (for example, patent ductus arteriosus, ventricular septal defect). The arterial system in the lungs is not used to receiving such large amounts of blood flow. Subsequently, the pulmonary blood vessels can become scarred and narrowed. While this effectively reduces the flow through the lungs, the pressure in the lungs can increase: a condition known as pulmonary hypertension.
Pulmonary edema is another complication of CHD. This is a condition in which high blood pressure occurs in the vessels close to the alveoli. As the pressure rises, water diffuses from the blood into the airspaces. The effect on gas exchange can be variable. Pulmonary edema is more likely in conditions such as pulmonary vein stenosis and mitral valve abnormalities.
In summary, CHD can cause many abnormalities in the lungs. The respiratory system in children with CHD is more likely to be simplified which may affect gas exchange. Certain types of CHD can cause further symptoms such as airway compression, pulmonary hypertension and pulmonary edema. Pulmonary complications in children with CHD are part of a spectrum and can vary significantly in each child. It is therefore important that children with CHD undergo respiratory assessment for early detection of these consequences.
As a pediatric pulmonologist, Dr. Nidhy P. Varghese’s specific area of interest is pulmonary hypertension and pulmonary complications of sickle cell disease. She enjoys working closely with families and colleagues to deliver the best care possible to care for children, so that they may thrive. She earned her medical degree from Albany Medical College, completed her pediatrics residency at New York University School of Medicine and her pediatric pulmonary fellowship at Baylor College of Medicine.
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.
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.
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.
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.
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.
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.
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.
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…
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
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.
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 email@example.com. You can also follow Gavin & Taosies’ CHD adventures on Instagram.