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.

Beyond the Heart – Anna’s Story

The effects of Congenital Heart Disease aren’t limited to the heart. Patients will often experience complications with other body systems. This week Anna Archer shares her how her late CHD diagnosis caused a brain abscess.

Growing up and Diagnosis
I was your average, ordinary, little girl for the first decade of my life and lived an idyllic life with my (incredible) parents and eight sisters and brothers (yes, you read that right), in the Pacific Northwest. When I was ten, I started showing symptoms of my undiagnosed CHD. I started becoming slightly short of breath and cyanotic with exertion, I was easily fatigued, and I probably slept more than most kids my age. After running through a whole gamut of testing, we finally received my diagnosis of Tetralogy of Fallot with a VSD, Eisenmenger’s Syndrome, and Pulmonary Arterial Hypertension.

Surgery to repair my heart was not an option for me, so life meandered on after my diagnosis, but I maintained my health for most of my second decade, it declining only in the last year or two. A few months after I turned 20, I was listed for a double-lung and heart transplant. Many years later, I learned that my parents thought they might lose me that winter, but I survived, and, a few years later, I was thriving again. I became healthy enough to go off the list after six years on it. In September of 2012, I got married and moved to Southern Nevada, where I currently live.

To better understand the plot twist, you need to have some understanding of my heart. I have what is called a bidirectional shunt with my VSD. It acts as a “pop-off” valve if you will, allowing my blood to change directions as needed, to relieve the pressures between my heart and lungs. Bacteria that should get caught in my lungs and give me a cold, is able to bypass them, through the shunting in my VSD, and maneuver its way up to my brain. There it made itself comfy cozy and became a brain abscess.

Plot twist! AKA: The Brain Abscess
On Monday, February 25th, 2013, I had a headache. Now, this isn’t abnormal in and of itself, except it didn’t go away. I still had a headache on Tuesday, and Wednesday. It was a pulsing kind of headache. Sometimes it would be a dull ache, then I would get these waves of intense pain. By Thursday, I was becoming concerned that I had injured myself while working out with my husband the weekend before. Friday came and went with no improvement, despite caffeine, ibuprofen, oxygen, and resting all day. On Saturday, I had decided if it wasn’t better by Monday, I would call my cardiologist’s office and see what they recommended. I had only seen my new cardiologist twice at this point, so I still wasn’t very familiar with how everything worked at their office.

Around 3am on Sunday, March 3rd, I started vomiting. This is always taken very seriously, as I dehydrate quickly, and what little energy I normally have is drained almost immediately. After a second round of vomiting, I woke my husband up and asked him to take me to the ER; something was definitely wrong.

The ER was fairly quiet that night, and I was taken back almost immediately. They worked through everything respiratory/heart/lung related first, chest x-ray and bloodwork, the most obvious concerns with a CHD/PAH patient. After everything came back clear, they sent me for a head x-ray. I remember someone coming into my room and asking”have you ever had anything show up on your scans”, I said “not that I knew of,” and thought it kind of a silly thing to ask (little did I know). Then I was sent for a head MRI… I wasn’t as concerned as I, perhaps, should have been. I supposed that since my head hurt, it made sense they were checking it out. I never thought something would actually show up on those scans.

The ER doctor came in, and told me I had a mass in my brain, and I needed to have a brain biopsy done. I was shocked, and in some sort of denial, because I told him flat out that surgery wasn’t happening. They couldn’t do surgery on me, it was too risky, it was reserved for life and death situations, and this was obviously not a life or death situation. He insisted that I needed to wrap my mind around it, because it would have to be done. Then he told me to look up glioblastoma, then left the room after reiterating that I would need to have surgery. I knew what a glioblastoma was, one of our family friends had died from it. I looked it up anyways; one to two year survival rate with successful treatment. I called my Dad and cried as I explained what little I knew.

I have been blessed with amazing parents, my Dad is a respiratory therapist and my Mom went to nursing school. It took my Dad almost an hour to calm me down when I called him about my brain scans, and finally, he said he would call my Mom and tell her what was going on. I didn’t think I could do it and be any amount of comprehensible. A little while later my Mom called, and I cried with her. Somebody, I’m not sure if it was myself, or one of my parents, let the rest of my family know what was going on. I have also been blessed with amazing siblings. Each and every one of them has supported me through thick and thin. They worked things around in their lives so that Mom could fly out to Las Vegas to be with me for the impending surgery and recovery.

Somewhere in the mess of things, I was coherent enough to call my cardiologists office and let them know what was going on, as well as telling the ER people that they needed to connect with my cardiologists office. The neurosurgeon who was going to be performing my surgery came in sometime that Sunday. He looked over my scans and to talk to me and my husband. He was much more calm, and kind about things. He told us that “the mass in your brain could be any number of 100 or more different things, and I simply wouldn’t know more until I get in there to check it out during the biopsy”. I felt much better about things after talking to him; I’d had enough of this glioblastoma talk.

Surgery was scheduled for the next day, Monday, March 4th, but it ended up being postponed, because they couldn’t get my blood thick enough (I’d been on blood thinners since I was diagnosed). After a few platelet transfusions, and a couple of vitamin K shots, it was finally thick enough to schedule the biopsy. I count this as a blessing on multiple levels, as it allowed some extra time for my Mom and one of my brothers to fly into town that day.

Tuesday, March 5th, 2013, my six-month wedding anniversary, I had brain surgery. My family was told the biopsy would take around one and a half to three hours, so you can imagine the almost heart-attack they all had when the surgeon came out after 45 minutes! He said everything went great, the abscess was right next to my skull, and not buried into my brain, so he was able to remove the entire thing and flush it out with antibiotics and antibacterials, before closing me up again. The abscess ended up being about the size of a golf-ball or meyer lemon.

 

 

For the next day or two my timeline is a little wonky. It was my first experience being intubated, and my first experience with any kind of narcotic (I’m apparently fluent in ASL when I’m on narcotics, I don’t remember this). What I do remember, is that my husband, mom, brother, mother-in-law were all there. I was surrounded by a million beeping machines, and my family. My best friend flew into town a few days later, followed by my dad the next weekend. I was surrounded by those who loved me. I remember distinctly getting my hair dry-washed by a nurse, which was the most incredible feeling in the world at the time. The night nurse, who made my life sane again. My Dad talking medicalese to the doctors, to explain that resting oxygen levels in the mid 70’s were perfectly normal for me. Slowly entering the social media field again, and the outpouring of prayers and support from family and friends across the country. Continual jokes about having a “few loose screws” now.

The “short-term” recovery ended up taking just over two months. I was released from my Infectious Disease doctor on May 8th and Neurosurgeon on May 16th. I say “short-term” recovery, because I was still recovering from some aspects six months later, a year later, two years later, even now.

Some things I wish they had done, was suggest I see a psychologist, and do neuropsych testing. My abscess could’ve been much worse, bigger, deeper into my brain, in a different spot, any number of things could’ve made recovery that much harder and longer. There wasn’t anything obviously different, or wrong, after the surgery, but lots of teeny tiny things that slowly built up, and built up, and built up, to become large problems later on. My abscess was in the frontal left lobe of my brain, and the frontal lobe functions include things like mood, personality, memory, behavior, decision making, judgement, initiative, planning, reasoning, intelligence, movement, and inhibition. I lost all semblance of self-confidence. I forgot how to cope. I became incredibly jealous. I wasn’t the same, and I didn’t know it for awhile. When I figured it out, I didn’t know how to fix it. Rewiring the brain is hard work. It’s an exhausting, frustrating business.

After I had a bad reaction to some allergy medications in the summer of 2016, along with some of life’s tougher moments, I became severely depressed, and developed anxiety. I had called my cardiologists office to see if there were any medications I could take, and as I’m bawling my eyes out with my nurse, they told me they had a psychologist I could see. We made an appointment for the next day. It’s been an incredible, life-saving, life-changing experience. For the past year and a half, I’ve seen my psychologist every week to two weeks, and I can’t recommend it enough. We’ve worked a lot on rewiring my brain, learning how to cope in various situations, and to remember that I am enough.

It’s a continual process, learning who you are, and what you are capable of, if only you give yourself half a chance.

Anna (Becky) Archer is 31 and lives in Las Vegas, NV with her husband and kitten. When Becky isn’t busy at her part-time job as a filing clerk, you can find her playing video games, knitting,
crocheting, swimming in her pool, shooting photos, hiking, going to the movies, and hanging out with family and friends. She is a Heart Warrior, PHighter, and Survivor. She has a fierce
perseverance for living.