Father’s Day – I See You, Heart Dad

Often times, Heart Dads do the behind the scenes work, the work that the world can’t see. Yet, in truth, their job as dad, their love, their encouragement  and concern doesn’t go unnoticed by the ones that matter. This week, Diana Schneider shares with us just how special a role her husband plays in their family and the life of their warrior, Danny. 

 

I see you, Heart Dad. You may not always wear your CHD heart on your sleeve, but I can see that too. I see the furrow in your brow as you’re researching our son’s diagnosis and treatment options. You wonder if you are doing enough to help him, if there’s more you could do for him. You try to imagine what the future holds for him and how you can make it better. I see you clench your jaw when the news anchor reports the latest flu statistics in our area and you glance anxiously at our boys, praying that somehow it spares everyone in our house. I see the tension in your shoulders when I need to take him to the emergency room. Again. You linger as you buckle him into his car seat, not wanting to let him go and yet wanting him to get there as quickly as possible at the same time. I know when I see you again there will be dark circles under your eyes because you can’t sleep well until he’s home safe with you again. Even with your anxieties and fears and without much sleep, you will be up the next morning to feed and dress our other two sons and take them to school and daycare. You will be mom and dad to them until Danny and I come back home, and I love that I never have to worry about them because it’s a job you do so well.

I see you swallow your concerns and let Danny be like every other little boy his age, running, climbing, and jumping off everything in sight. You roughhouse with him and his brothers, you chase him and tickle him until he squeals, planting big dinosaur kisses ever so skillfully on his belly without disturbing his G-tube. You never tell him he can’t try something because of his heart. And you don’t use it as an excuse to spoil him either. I see you discipline him and hold him to the same standards of behavior we set for his brothers when it would be much easier to let pity and guilt over everything he’s gone through get the best of you. But you know you wouldn’t be doing him any favors in the long run.

I feel like the people who know the story of our son’s journey don’t always see or appreciate how much of his success is from you. When our friends and family ask how Danny is doing you tell them I take him to weekly appointments with the feeding specialist and how he ate his first slice of pizza. But you don’t tell them you made the pizza from scratch (thin crust, so it’s easier to chew) and let him help you make it so he could experience food as a fun, not just therapy. You tell them I take him to see a speech therapist every week to get his language development on track so he can start school next year. But you don’t tell them how many books you read to him or how many flashcards you practice together each day when you get home from work. You tell them he no longer has a gross motor delay and doesn’t see a physical therapist any more, but you don’t tell them you built him his own platform swing to work on his balance at home. You tell them about current CHD research and what it could mean for Danny, but you don’t tell anyone that you went to our nation’s capital to educate legislators and push for more research funding.

I see the way becoming a Heart Dad has molded you into an even more remarkable father than you already were. It’s made you someone I could never have imagined when we were dating in high school, and your biggest concern in life was how to start a food fight without getting suspended. As it turns out, you weren’t very good at that. But you are the most incredible Heart Dad. It has shaken you to the core, and you have put the pieces back together even better than they were before. It has made you more vulnerable, but stronger. It has made you more serious, but you’ve retained your sense of humor. You live your life differently now, taking little for granted and stopping to enjoy the small things. You plan for the future, but cherish each day knowing that tomorrow isn’t guaranteed. I’ve watched the man who can fix anything around the house struggle to accept he can’t fix his son’s heart, but instead of buckling under the stress and fear you’ve allowed it to shape you into a better husband and father than you already were. You’ve managed to find a way to thrive under the burden of being a Heart Dad and because of that, because of you, Danny is thriving too.

Thank you for appreciating how hard I work to take care of our children. But the next time someone asks how Danny is doing, I hope you will remember to take your credit where it is due. To you and all the wonderful Heart Dads out there, have a Happy Father’s Day knowing how much we love and appreciate you the other 364 days of the year as well.

 

Diana Schneider is the lucky wife of an amazing Heart Dad and mom of three wild little boys. They live near Ann Arbor, Michigan and thankfully, Mott Children’s Hospital.

 

Father’s Day – Joe’s Dad

In May, PCHA honored Heart Moms for Mother’s Day. This month, we’re taking time to shine a light on the great Heart Dads out there, who conquer CHD along side us and are often unsung in the fight. This week, Joe Valente, an adult CHD patient, thanks his dad for the sacrifices he made a long the way and talks about why sometimes men seem to be in the background when it comes to  the CHD Family journey.

 

My father used to have a fond place for box kites and I remember many a summer being dragged across the windy beaches of Northern California by his kites.

As I sit hear with my next chapter in my CHD journey unfolding I am reminded of all that has been sacrificed to get me here and as Father’s Day is approaching it is hard to not think of all the CHD dads that make this world go around including my own. The world of Congenital Heart Disease is in a rapid state of flux. Since the day I was born over 36 years ago survival rates for critical congenital heart defects have improved beyond belief, but maybe more importantly the community of patients and parents that are able to connect through the internet and organizations like the PCHA have drastically changed the knowledge and support for everyone affected by CHD.

Being a male in the CHD world is difficult, there are not many emotional resources for the men that need support, the fathers who are helping their child fight, or those that are grieving a loss. Maybe it is our own doing and what society expects from us, but it is often a lonely road to walk. I enjoy seeing the fathers that are public about their battles and the men that share their journey with CHD with the world. So, while so much has changed the men are often stuck in yesteryear as they are expected to be strong through all else and have little escape or resources to coupe with daily struggles that accompany CHD.

As I was going through some childhood belongings the other day I found an old guide to CHD from the American Heart Association, copyrighted 1978. It is quite an enlightenment to read, as it was the only real resource that my parents had outside of the hospital. In this information era it feels like reading a tri-fold brochure when you want an encyclopedia. It is a good reminder for all of us fighting for more to not forget to be thankful of how far we have come.

As most fathers mine was observing my birth but for him it was in the same hospital that he had already delivered so many babies as an OBGYN. So, when I was delivered and was extremely cyanotic I wonder at what point he really knew. I know my mother tells the story of the pediatrician telling her I was being transported to UCSF and to call the priest. But I know my father knew before that—maybe from the moment he saw me—or was he blinded by a fathers love like so many parents who don’t see what may appear as obvious looking back?

I was blessed that my father was one of my biggest advocates when I was young, being a doctor he was knowledgeable enough to know when things were not right. I can say without hesitation that he saved my life. I know his grief was significant as anyone that was there would tell you he could barely walk into the hospital to find me after my transport. And when I was not on the floor they thought they assumed the worst. Like so many father’s I am not sure who was there for him, of course my mother and family, but who was his strength? Maybe all he needed for his strength was me to be there and to keep fighting.

My dad never let his medical knowledge and any fear of looking like a dumb parent get in the way of following his gut. When I was 9 months old one week after getting a the “see you in a few months” clearance from cardiology I began to go downhill. My father new something wasn’t right and immediately called my cardiologist. As my pediatric cardiologist still does, and I believe any good doctor should, he listened to him told them to come down as soon as they could. Upon setting eyes on me my cardiologist turned pale. He had never seen a baby decline so fast, he had only seen me a week before and I was “healthy.”

My father didn’t stop there when the surgeon came and gave the consult on a Friday afternoon and told my parents he would operate on Monday my father told him I didn’t have until Monday. The surgeon was adamant that I would be fine and that when he got back from his weekend vacation he would do the surgery. That wasn’t good enough, so my dad kept pushing finally he agreed to operate same day. After the surgery, the surgeon came out of the operating asking my father how he knew, he questioned how many hours I had left, not days, as I had formed an aneurysm in my trans annular patch that was placed when I was just 2 days old, and it was about to rupture.

As I have grown into a man, I think of all the nights my father was out delivering babies and how at 76 he still works 4-5 days a week. Not much has changed in my 36 years, except now I am the one in charge of my care. The lessons my parents taught me are what has led to me to search for a life of meaning. Not only for me but for everyone I love. I have had many moments in my life when I was required to trust my gut, and I have never seconded guessed it, because I learned at a young age the significance of doing so. I will always appreciate the medical professionals who take a pause and listen when I say something isn’t right.

I know that fathers may often seem hidden in the world of chronic illness and CHD, but it is not representative of their role. Many are at home and work, keeping the rest of the world moving while we fight our battles in hospitals and doctor offices. Many are out there advocating for us. They may not be the faces on our Facebook pages, but we know what they do and have done for us, and we are forever grateful. Maybe one day it will be the posh thing for dads to wear their emotions on their sleeve for the world to see, but until that day we must not forget of everything they contribute to us and our families. Regardless of their fame or the lack thereof we know their love is unwavering.

So, when I say:

The twine on your kite is as long as you want it to be. Always set it loose and reach for the blue skies. Let it fly high in the wind. If a breeze catches you let it pull you to where you were meant to be. Never give up on life. Fly free and you will be amazed at what you can be.

I think of my father and being pulled across the beach as I look up for all that can be. A happy Father’s Day to my dad and all the CHD dads out there that mean so much to us and our families.

 

Joe was born with Tetralogy of Fallot. He required his first open heart surgery at 54 hours old and suffered a stroke after surgery. He has now had four open heart surgeries, the last two days before his 30th birthday. He has struggled with PTSD for most of his life and believes a strong mind and building a team with a roster full of supporters is the key to successfully living with a chronic illness. He is a Board Certified Patient Advocate for families, children, and adults with congenital heart disease—helping them to navigate the emotions, diagnoses, and challenges they face in finding and understanding appropriate CHD care.

 

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.

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.

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.

New Diagnosis – Pulse Oximetry

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

 

 

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

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

How CCHD pulse oximetry screening works:

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

How it has changed standard screening for newborns:

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

Benefits/importance of screening:

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

Limitations of screening using pulse oximetry:

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

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

 

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

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

 

 

 

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

 

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

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

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

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

 

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

 

 

 

New Diagnosis – Fetal Echo

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

 

 

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

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

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

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

There are some standard indications for fetal echocardiography during pregnancy.

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

What happens after a fetal echocardiogram detects CHD in fetus?

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

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

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

 

References:

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

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

 

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

Zipperstrong Project

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

 

 

 

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

 

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

 

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

 

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

 

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

 

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

 

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

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

 

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

 

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

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

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

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