Jacob Kilby

Jacob Cropped

I want to introduce you to an amazing young man who will change the way you look at the world.

Jacob Kilby is a young, 19 year old local San Diego surfer who is facing a major health challenge. You often hear about many children and adults that are battling cancer or diabetes, but congenital heart defects seem to be few and far between. Heart disease is a major problem for both the new, young, and mature people of the world. Many times when you do hear about unhealthy hearts it’s in reference to a person who has lived many years and perhaps did not treat their heart as good as they should of. Those stories that start with a newborn baby who will have his first open heart surgery at the age of 5 days old are not plastered all over the media. I want to make a difference for the San Diego community and possibly beyond, by educating and promoting organ donation awareness through the story of Jacob Kilby’s heart.

Jacob was born with Hypoplastic Left Heart Syndrome where the left side of his heart did not fully develop. This is a rare condition that is one of those congenital diseases that we don’t know how or why develops in a certain fetus. We can’t blame it on how a woman treated her body or what she did or did not eat while pregnant. It’s an unfortunate circumstance that happens and Jacob was born on Sept. 8 1995 with this disease. He was born strong and ready to take on the four open heart surgery attempts at fixing his heart, all of which failed. Jacob was very lucky to receive a life-saving heart transplant in 1998 that saved him just in the nick of time.

Jacob has lived a wonderful life. He was able to do almost anything any other boy his age could. Despite the daily doses of anti-rejection meds and the numerous doctor visits, Jacob sailed through school and was liked by everyone. His family adores him and treasures him as if he were a diamond. His smile shines so bright that he truly must be a treasure. So, the news of his need for a new heart came as a devastating shock to all of us, including him just a few months ago.

As the days have passed, I have learned a great deal about the world of organ transplantation. I learned that Jacob is on the wait list at a stage “2” where he really has no chance of getting a new heart. Those who receive organs are those who are on the list the longest and those who are the sickest. So, I guess things haven’t changed in that respect in the 17 years that he’s had his donor heart. His family watched his frail little body diminish and lose oxygen right before our eyes. And here we are again.

The amount of organs available for transplant is not even closely comparable to the amount of people on the wait list in need of an organ. There are currently 123,000 people nation-wide on the national organ transplant wait list. Eight people die every day waiting for an organ that will never come. Millions of families wait in agony every day, never knowing if their loved one even has a future.  Please help us create more opportunities for more futures for people with congenital heart defects like Jacob Kilby.

Aunt Charity

Jack Radandt

Jack 5 Jackson was born on Easter Sunday, 2001.  We brought our perfect 8lb 6oz baby home from the hospital and enjoyed two days with our new little one.  All seemed well; Jack was a wonderful, healthy baby.  At least that’s how it appeared when we were discharged from the hospital.

Things went well for the first several days at home.  However, on the evening of his fifth day of life he felt cool to the touch, became fussy and wouldn’t nurse.  I knew something was wrong when he began making a funny noise with every exhale.  Jack wasn’t improving, so I woke my husband Jason and we decided we needed to take Jack immediately to the emergency room.  Never could we fathom the journey we were about to embark upon. A journey that would last, not just days, but years, and now we know, the rest of his and our lives…

After several minutes of trying to save our baby, they told us his heart was severely compromised and asked if we wanted pastoral care, obviously insinuating they believed our child could die.  At that moment we realized it was very serious.  He was baptized in the emergency room right before the transport team came to take him to Children’s Hospital of Wisconsin.

There, we were told he had a very complex congenital heart defect; Hypoplastic Left Heart Syndrome. This meant his left ventricle was severely underdeveloped.  He would need several open heart surgeries to reconstruct his heart.  Because he had such severe physical trauma when he was just five days old, his organs needed time to recover.  This created such intense instability in Jack that they had to prolong the life-saving heart reconstruction surgery until he got stronger.

We were overwhelmed with this news, with equipment, monitors, tubes and lines.  It is a life no parent should have to go through, but it was now our life.  The hardest thing as a parent to accept was they couldn’t just “fix” him and he would be fine; he would have to deal with this his entire life.  Finally, at ten days old, Jack was ready for the first stage of reconstruction.  He recovered well and successfully had all three reconstructive operations by the time he was three years old.  Jack 3 While each surgery was a struggle for our little boy, he triumphed through each one and became stronger.  Other than daily medications and bi-annual cardiology visits, Jack lived a very normal life for the next 8 years.  Most people were surprised to know he had congenital heart disease let alone a very complex, life-threatening one.  While Jack was restricted physically (i.e. he couldn’t play tackle sports and fatigued seemingly earlier than other children), he excelled at school socially and academically.

Eleven years later our life was again to become a roller coaster.  At a routine clinic visit, we were told there was a change in his echo and that Jack’s heart was failing.  He didn’t really have any symptoms of heart failure so once again; this came as a complete shock to us all.  The news was much harder to process, accept, and adapt to this time because we had an eleven year old who was very aware of what was going on.  At lunch after that visit he asked us if he was going to die and also what percentage there was that he could die.  It took everything in me not to scream and sob to answer his questions.  From that appointment on, Jack’s deterioration was rapid, as his heart was failing quite quickly.  Within a month we were admitted to the Cardiac Intensive Care Unit (CICU) at Children’s Hospital of Wisconsin.  A few days later, they told us he would most likely need a heart transplant. He was put on the heart transplant list shortly thereafter.

Every day in the CICU I watched him getting weaker.  I was afraid he would over exert himself doing simple daily tasks like walking, showering or eating.  After one tough day, I admitted to myself that maybe it wasn’t God’s plan for him to make it through this. I was watching him fade away.  Every time I looked at him I would try to memorize his expressions, his smile, his hands and the sound of his laugh…everything about him.  Preparing myself that memories might be all I would have.

After two months of trying different medications and therapies, Jack continued to deteriorate physically.  Mentally however, he was as sharp as ever.  He was well aware of what medications he needed, always had questions for the doctors and kept up on his homework.  However, it was eventually decided that Jack needed more support for his failing heart.  The physicians caring for Jack decided to place a Heartware Ventricular Assist Device (HVAD) in him. The HVAD is a mechanical pump implanted in the heart to help blood flow in people with weakened hearts.  This device was necessary to bridge the time until a donor heart was available.  He was the first single ventricle child in the United States to have this device and second in the world. It was terrifying to know this was the first time they were placing this device in a child with Jack’s physiology, but there was no other choice. We had to place Jack in the best position possible to accept a new heart when one became available.

Jack 1Jack successfully had the HVAD placed on December 23rd.  He was slowly recovering from surgery when we were informed that a donor heart was available for him. He was so excited that he cried.  All he wanted was a normal life and this was his ticket home. Shortly thereafter, we received horrible news—it was too risky for Jack to take that heart because he had developed a large amount of antigens during surgery.  The hardest part was delivering that news to a little boy that just wanted to go home.  He was devastated. The
emotional blow was overwhelming.

The next day, they began therapies to try and lower his antigen levels.   As Jack continued to recovered they began talking about sending him home on the VAD–another first.  Jack 6After two months in the CICU with the device, Jack was able to come home.  We cared for him at home and had frequent visits to the hospital for immunosuppression therapy and clinic.  Jack thrived being home; he gained weight, grew taller, and eventually went back to school.  Three months after he was discharged from the hospital we received a call that there was heart for Jack.  I reluctantly asked how likely we were to accept this heart and was told it was a negative cross match (a perfect match).  On May 20th Jack received his heart and went home eleven days later.  He is doing great and even joined the basketball team, something he was unable to do before.

Life in our home has returned to normal.  A “new normal” where Jack is able to function better than he ever has.  Although he will be on medication for the rest of his life, and we now live at a bit of “heightened” sensitivity or awareness as we adapted to Jack living with a new heart, we are thankful every single day for every single breath he takes.

Jack 2

Buczko Twins

Buczko (1)Our twins, Aiden and Madison were born at The Children’s Hospital of Philadelphia in September 2012 at just 31 weeks gestation, both with critical congenital heart defects. 

Aiden was born with an isolated left pulmonary artery and right aortic arch. He had his first, and hopefully, only, open heart surgery at three weeks old.

Madison had Tetralogy of Fallot with pulmonary atresia and multiple major aortopulmonary collaterals (MAPCAs). She had her first cardiac catheterization within 24 hours of being born. She has had two open heart surgeries (MAPCA unifocalization, April 2013; VSD closure and conduit replacement, December 2013). She will need several more catheterizations, as well as additional heart surgeries as she gets older. 


Our “miracle twins,” now 20 months old, have been through more in their short lives than any child should. Between the two of them, they have had three open heart surgeries; five cardiac catheterizations; one eye surgery; three hospitalizations for illness; numerous echocardiograms, x-rays, lung perfusion scans, barium swallow studies, and other tests; many, many doctors’ and physical therapy appointments; countless prescriptions; and more than $15,000 in out-of-pocket medical expenses. In addition, last fall we learned that Aiden had a brain injury called periventricular leukomalacia (PVL) and cerebral palsy, most likely a result of being born premature with a CHD. 

However, to look at our kids yoBuczko (2)u would barely notice anything is different about them. They are beautiful and happy and are making incredible progress! Aiden has high muscle tone in his legs and feet from his PVL, requiring the use of orthotic braces, but he is walking. Madison had vocal cord damage after her first open heart surgery and her speech was delayed for a while, but recently scored above average in communication in a developmental assessment.

Norah’s Story

Norah was born in 2009, seemingly healthy. At two weeks old she was hospitalized at Blank Children’s Hospital in Des Moines, IA for having had a UTI. She was referred to the University of Iowa Children’s hospital and diagnosed w Grade IV Bilateral Vesicoureteral Reflux. After a year of prophylactic antibiotic The Lord healed her little body and she never ended up needing the expected surgical correction. Yay! 

In this same year she was diagnosed w/ a benign heart murmur, an ASD that was expected to close. It was at a sick child visit, nearly a year after the diagnosis, that a doctor recommended we revisit the cardiologist. At this point her ASD was reported to be too large to be patched via the Cath. Lab. and she would need open heart surgery soon as her heart was dilated. She was put on Digoxin while we nailed down the details for surgery. 

We were scared and didn’t even know what kind of questions to ask to better prepare ourselves for what was about to happen. Both my husband I and felt like we were responsible to make an educated decision with the doctors help, but found ourselves wondering where to even start. I figured meeting with the surgeon would be a good place to start. 

In our meeting w/ the surgeon he did a good job of explaining how he would construct the missing septum using her pericardium tissue and assured us that he was the man for the job. When I asked him if we had any other options or places to go to get a second opinion, his response was something of the sort; She will be just fine right here. This type of surgery is like the appetizer before the main course. This was a very simple surgery for him to do and it will be one of several for the day.

His response bothered me. I wanted to hear statistics, some kind of hard facts as why I could trust this man w my daughter. For pete’s sake he was talking to a Math major and Air Traffic Controller. It never sat right w me that he never directly answered my question about a second opinion, but I attibuted a lot of those unsettled feelings to just being afraid of my daughter having to undergo open heart surgery.

Norah had OHS Jan. 2012 at age 2.5. We thank The Lord that we are one of those families that have a happy ending to the story. Norah’s surgery was a success and is considered cured. Indeed the surgeon was skilled at doing this repair and we felt blessed. But, I have to be honest. When I found out that there was another facility right here in our state that had a children’s hospital and pediatric thoracic surgeon. I felt deceived and that I was forced into making an ignorant decision on purpose. I don’t want to sound ungrateful for what our surgeon did for Norah. We are forever grateful to that man. I just wish we were given more information prior and allowed to make a more educated decision for Norah. If this story had not had such a “happy ending” I can’t imagine the pain, anger, and guilt I would potentially carry.

Kieran Touney

kieranstory1

When Kieran was born, I felt like I was giving birth in a theater filled with an expectant audience—a repertoire of doctors stood around, anticipation on their faces, waiting to spring into action. As Kieran had been diagnosed with HLHS during our 20-week ultrasound, we were all ready for him—OB, residents, nurses, neonatologist, cardiology fellows, and critical care specialists. I had a very long delivery, so they were milling around for quite awhile, waiting much longer than they had planned to see him safely through his first echocardiogram and to his room in the NICU. The news came that I might need a c-section, but I was going to give it one more try. About 45 minutes later, after a long night and day, around 8 pm on August 7, 2008, Kieran was born in front of all of these “strangers,” to sighs of relief and cheers of “you did it!”

 

He was placed in my arms and my husband Shawn and I got to hold him for about 5 minutes before he was whisked off, accompanied by my husband. But for those 5 minutes, everything felt normal. Kieran looked healthy and robust, and was a healthy weight and size. Despite the crowd of specialists and staff bustling about the room, for 5 minutes everything else stopped as I gazed at my son. Although he looked like any other baby at the time, when I look back now at pictures taken from his first weeks and months, I see how cyanotic (blue) he was. His tiny heart, made tinier by being absent a left ventricle, was desperately trying to get enough oxygen to both his body and lungs.

 

Being left alone while Kieran and my husband left for the NICU was one of the hardest times in my life. I was unprepared for how it would feel to have my baby taken away so quickly, although I was grateful we had had at least a few minutes together before the whirlwind of medical intervention began. At the same time, I had a lot of joy to comfort me through those first few hours, because Kieran looked perfect despite having such a serious heart defect. I knew, somehow, he was going to fight through what was coming.

 

I had always felt that way, from the moment my OB had called me to tell me “I can’t see all of the heart,” through the perinatologist appointment where we were given the official diagnosis, through the long Easter weekend that had followed the news. Of course, it was devastating to learn something was so wrong with our baby. But along the way, we had consistent hope that Kieran should be given as many “fighting chances” as he possibly could. We were lucky to be close to one of the best pediatric cardiology centers in the country, where we started going for prenatal echocardiograms and meeting with the cardiologist who would become such a major part of our child’s life.

 

Knowing about his HLHS before he was born made us, at least, familiar with what was happening, even though it was certainly difficult. Kieran stayed in the NICU for 7 days, and I was able to breastfeed a few times, and pump milk for him the rest of the time. He was like a typical newborn baby, in that he wanted to be held, fed, and he slept a lot. As the days wore on, however, his oxygen saturation levels were getting lower and he was getting tired eating even a little bit. It was time for his Norwood operation, although his surgery was pushed back an extra couple days to make room for emergency cases.

 

Before Kieran’s Norwood, we met with a series of cardiology fellows, a cardiology nurse, and a cardiac anesthesiologist. They explained the procedure in great detail with diagrams. They told us that Kieran was a classic case of HLHS, and that they felt he was a good candidate for the Norwood. They explained that there were two common ways of doing the Norwood procedure: by doing a BT or a Sano shunt. The Sano shunt was the newer of these two methods, and had been developed by Dr Sunji Sano. The BT shunt was named after Alfred Bialock, Helen Taussig, and Vivien Thomas, who were pioneers in pediatric heart surgery (a movie called Something the Lord Made does a wonderful job telling the story of their work).

 

Our hospital, along with other facilities, had just concluded participation in a large study comparing the BT and Sano shunts, and while there were not large differences in outcome, it appeared that the Sano shunt had better short-term outcomes, especially for lower birth-weight babies. Our hospital had concluded their portion of the study a mere day before Kieran’s surgery. His surgeon would decide in the operating room which shunt to use. He ended up having the Sano shunt.

 

Following his Norwood, Kieran did very well. At first, the number of monitors, IV drips, wires, tubes, and patches was overwhelming.  My mother-in-law was visiting from out of state, and she thought to take pictures of the equipment in Kieran’s ICU room. Looking at these photos now, I am amazed by the amount of care and hard work that is invested in the treatment of HLHS. For the first night following Kieran’s surgery, the critical care doctors in the ICU did not take their eyes off Kieran and his monitors. They watched him so closely that I felt we must be in good hands.

 

Little by little, those monitors, wires, tubes, and patches began to disappear. For us, each one was a victory. It meant progress towards healing, sufficiency, and going home. And of course, we could start to see more and more of our baby under all of that equipment! After three days, Kieran’s chest incision was closed up. Thanks to online networking and support groups, we had been prepared to see Kieran’s chest open after the Norwood. I had seen surgical pictures online, which for the average person may be alarming, but for me, it was enormously helpful in knowing what my son would look like post-operatively. For the most part, the ICU nurses kept his chest incision covered with a blanket until it could be closed, but there were a few times they had to remove the cloth. There was a clear patch over his open chest, and I could see his heart beating away right in front of my eyes. Later that day, a visiting friend told me, “You’re one of the few parents in the world who can claim she’s literally seen her child’s heart.” That was comforting to me. As a new mom, there was a lot that was “abnormal” about our first days together. In some ways, the normal bonding process and mother-child relationship had been disrupted. But there was a special kind of intimacy in being with our son through this difficult process.

 

Kieran slowly started to eat by mouth again, just a little more each day. I was able to pump breast milk for him through the entire hospital stay, and he could drink some orally, as much as his recovering strength would allow. The rest was fed to him through an NG (nasogastric) tube. After 10 days in the ICU, we were moved to the “step-down” unit, where we spent two more weeks. Progress was not completely linear or constant, and there were days we felt discouraged by setbacks. One of nurses told us that this was completely normal, and that the journey “is a marathon, not a sprint.” As the inter-stage period (the time between the Norwood and the Glenn operations) is a period of a high mortality for children with HLHS, Kieran’s doctors wanted to be sure he was in the best possible shape before sending him home. There were some days where Kieran wouldn’t eat enough by mouth to satisfy the cardiology team, and he had unexplained tachypnea (rapid breathing). The hospital stay began to feel long because we could hardly wait to get home with our brand new baby. Since our stay there, the hospital has been remodeled, but at the time, we had to share a room with another family and only one of us could spend the night in the room with Kieran and his roommate. It was hard to have to say goodbye to Shawn every night, as one of us left for home and the other stayed by Kieran’s bedside. We did not qualify for staying in the Ronald McDonald House because we lived within an hour’s drive of the hospital. But our drive each way was 45 minutes, and this seemed far enough away to feel distant and lonely at night. We longed for the day we would all be under the same roof for the first time.

 

The days in the hospital, however, were busy. I had milk to pump, and in the step-down unit we were able to assume most of Kieran’s baby care—feeding, bathing, changing, and rocking. We had to pass infant CPR class, learn to draw up medications, and start recording his daily weight, oxygen saturation levels, and cubic centimeters of food in a binder. We had to learn to mix high calorie formula with breast milk so that he would get enough calories every day. Kieran had to pass his car seat test, to make sure his oxygen levels remained stable when he sat upright for long periods of time.

 

Finally, after 31 days in the hospital, we were going home! It was just after Labor Day, and the weather was thankfully still beautiful. During the interstage period before Kieran’s Glenn, which would be performed just a couple months later in November, we were supposed to avoid illnesses as much as possible, which meant not taking him around big groups of people if we could help it. Instead, we took him on long walks in the stroller on our local bike trail. Even at such a young age, you could tell he loved being in the fresh air, listening to the birds and the sound of the leaves through the trees. To this day, he loves the outdoors and wants to be outside as much as possible.

 

The 10 weeks or so between surgeries were a time of both gratitude and challenge for our little family. We were thankful to be out of the hospital and have support from so many, but we were also feeling the pressure of another open heart surgery and hospital stay looming before us. We had a lot to catch up on, and a lot to learn. There were a lot of “eyes and hands” on Kieran, and although we were out of the hospital, it felt like our life was not completely our own. When we say “it takes a village to raise a child,” when it comes to HLHS, that village is made up of many medical professionals! We had an in-home nurse visiting weekly, as well as an occupational therapist and physical therapist. We continued to record Kieran’s weight, feedings, and oxygen levels on a daily basis in the hospital’s binder. Every week, a pediatric nurse would call us to review the numbers, and if any of the numbers were amiss, we would be asked to bring Kieran in for a special pediatric or cardiology appointment. We got to know our local pharmacists very well, as well as our pediatrician and clinic nurses, lab technicians, cardiologist, hospital dietician and of course, our insurance company.

 

Before we knew it, it was time to schedule Kieran’s second surgery, the Glenn. Kieran had grown very quickly and put on relatively a lot of weight, so it was time by November. His Glenn was performed the day after Thanksgiving, when he was almost 4 months old. Although this was a shorter hospital stay—14 days—this surgery was more difficult for Kieran. Usually, the Glenn is considered a more straightforward surgery, but Kieran had problems with low oxygen and lowered heart function after the Glenn. No one is entirely sure why this surgery was harder for him, although one theory is that the incision made in the right ventricle for his Norwood may have somehow damaged his heart. After we were discharged, his decreased heart function continued to manifest itself over time, and finally, at a post-Glenn echocardiogram, his cardiologist became very concerned. Kieran’s heart was working too hard. The hard work was putting pressure on his tricuspid valve, which was moderately leaking, and his right ventricle was a bit enlarged. She told us that Kieran might not be a candidate for the next surgery, the Fontan, and that he might eventually need to be listed for a heart transplant. You can imagine how hard this was for us to hear—after all he had been through, it wasn’t working? Our cardiologist, however, decided to try two different medications, although she was somewhat doubtful that it would make a difference.

 

With sadness and alarm, we went home to try the new medication regimen. This involved adding a diuretic to his daily aspirin and blood pressure medications—one that is usually used only after the Fontan. She also added digoxin, which comes from the foxglove plant. Over the next several months, we waited anxiously to see if things could improve, and miraculously, they did! No one can quite explain how Kieran’s heart was able to improve, but at his pre-Fontan catheter many months later, he was found to be a candidate for the Fontan.

 

It is hard to live knowing your child will need another major open heart surgery. But I have come to the point where I am so grateful that he even had the opportunity to receive this very surgery. As “heart parents,” we do not want to see our children suffer, but we know our children need these life-saving surgeries to live their lives and fulfill their very special places in this world. And Kieran is definitely living his life! His life is different in some ways—he is on daily medications, he gets tired more easily than most children, he gets therapy to “catch up” with kids his age, and he has more doctor’s appointments and procedures than many children. In other ways, his life is “normal–” he goes to school, takes swim class, loves music, and plays outside. And yet, his life and his experiences are exceptional in so many ways—he inspires many around him with his strength, resilience, and unique personality. He’s brought us closer together with friends, family, neighbors, and strangers through his story and circumstances. He has completely changed our lives for the better, and for good.

kieranstory2

Gregory Guzzo

Gregory Guzzo photo

Gregory has a congenital heart defect, yet we never knew until a few months ago. Gregory is an active child and 13 years old. He wrestled for 5 plus years, played soccer, never sits still unless he is playing XBox and even then he is standing and jumping around.

Out of the blue, as he was going for his wrestling physical at school in Oct of 2013, he fails. The school physician calls and says he heard what seemed to be a murmur and he felt it was significant enough to require an ECHO and I should have him seen by his Pediatrician and Children’s Hospital only to be diagnosed with Atrioventricular Septal Canal Defect, a congenital heart defect, a condition he has had from birth.

Atrioventricular septal defect (AVSD) is a multiple heart defect that involves the valves between the heart’s upper and lower chambers and the walls between the chambers. In children with AVSD, the tricuspid and mitral valves do not develop normally which then affects the growth of the atrial and ventricular septum. There is one “common” valve separating the hearts upper and lower chambers (1)(or cleft) instead of two separate valves. In addition, there is a hole in the atrial septum (2) called an atrial septal defect (his is half the size of the wall itself) and a hole in the ventricular septum with a left to right shunt (meaning the blood is lost threw the hole)(3) called a ventricular septal defect. He also has a right bundle block…which means his heart is thickened for being over worked and the electric currents have to work harder to get past the dense heart muscle.

We were informed to correct these defects Gregory was to undergo Open Heart Surgery. This was not optional. The surgery went well, and he is recovering nicely, however in Gregory’s own words, he is “FIXED not CURED”.

Since the surgery, we have established a foundation (Gregory’s Heart Warrior Foundation) to educate, promote awareness and provide charitable contributions to hospitals and families in need with CHD. We have also work with local and state legislatures to receive proclamations for CHD Awareness with the hopes of funding for research. We work in conjunction with Children’s Hospital of Pittsburgh’s Heart Institute Auxiliary Board for funding for research and we utilize social media to express the seriousness of the CHD’s with facts, statistics and CHD news. The best part is Gregory, himself speaks on behalf of his condition, he is his own advocate and the voice for so many children who are too young to express what they are going through or have lost their battle with the number 1 killer in deaths due to birth defects.

We will continue our fight for awareness, early detection, hope for a cure… as going un-diagnosed for 13 years is unacceptable and we want to prevent this from happening going forward. If we save one life, if we spread the awareness for CHDs and one child is helped… then we have done something good.

Mirabel Atkinson

Nine-year-old twins, Mirabel and Sofia, share a special bond.  Born at 32-weeks, they were more exceptional than most identical twins in that they shared a placenta and an amniotic sac.  But one significant difference they have is that Mirabel was born with a congenital heart defect, or several to be more precise, including hypoplastic right heart, transposition of the great arteries and a couple others.  We were fortunate to know about all of these complications before our girls were born, but that didn’t change how challenging the pregnancy and early years have been.

Being born early, they both required time in the NICU.  Sofia mostly just needed to grow and was discharged at about one month.  Mirabel’s case was different and the doctors anticipated her needing the first of three staged surgeries (the Norwood, the Glenn and the Fontan) within days of birth.  But because she was so small, we all waited and watched—through many good and bad days.  And then at two months, when the doctors finally determined that she could hold off on the Norwood procedure and have any necessary changes to her heart combined with her Glenn, they discharged her.  We frequented the cardiology clinic from that point on, often with several visits a week.

Mirabel had her first heart catheterization at about 6 months.  I thought it was the end of the world when we handed her over to the anesthesiologist and I watched her terrified eyes silently asking me what was happening.  But I learned that wasn’t anything compared to surgery; she had her first open heart, the Glenn, at 8 ½ months.  Mirabel’s strength and tenacity had already been proven and she did remarkably well during her Glenn and was discharged after only 6 days.  We maintained regular weekly and then monthly visits to her cardiologists.  She had a second catheterization, mostly for diagnostic purposes in preparation for her next surgery, at about 15 months old and then the Fontan surgery at age two.  Once again, she responded well and was discharged after only 8 days.

We knew Mirabel wasn’t “cured” at that point.  The regular cardiology check-ups, her slower growth (compared to her identical sister), daily medications, how quickly she caught colds and the flu, and her general lack of energy all served as reminders to us that she was different.  But we tried to act and live as normally as we could.

And then at age 5 she needed another heart catheterization to close a fenestration (vent hole) in the hope of improving her oxygen saturation levels.  We had always been open about her heart condition and frequently looked at photos, but she didn’t remember any of the previous interventions, and so when we told her she needed to have the cath, she didn’t take the news well.  It was really hard listening to her ask questions like, “how big of a knife are they going to use to cut me open…will it be like the ones in the kitchen?”  But, guided by an excellent social worker, we made it through with only a few minor complications.  Her kindergarten class made a giant heart card with well wishes and all of their signatures.   And we thought we were done for a LONG time.

Then during the summer when she was 6, we visited the cardiologist for a regular follow-up and he told us that she needed another “surgical intervention” right away.  Eleven days later she was in surgery to enlarge a hole (VSD)—a “minor” open-heart surgery, as they go.  Mirabel was the calmest of anyone in the family; Sofia struggled the most.  Sofia didn’t like the attention Mirabel was getting and was scared about what she was hearing.  We all had to work through this together as a family.  She healed a little slower than the previous two open-hearts, but was still only in the hospital 8 days.  And she started first grade four weeks to the day after her surgery.   But it takes time for everyone to recover from something like this.

Two and a half years later, Mirabel is doing well and we are relatively “normal” again.  Her last surgery granted her more strength and energy than we had ever seen.  She has been well enough to play soccer with her sister in a neighborhood league, a far distance from when eating or crying too hard would wear her out and she would turn blue.

While Mirabel’s road hasn’t been easy, and her journey to keep her heart working as best as possible isn’t over, we have met many exceptional people and made some wonderful friendships with those having similar experiences.  We know what a courageous little girl she is and when we see fits of stubbornness we are quickly reminded that her stubborn attitude is what has kept her going through rough times.  Sofia has been her constant cheerleader and their bond and love they share will remain strong forever.

Cooper bowtie

My son, Cooper, is my heart hero.

Cooper came into our lives completely blue, motionless, not breathing, and with a faint heart beat. We watched helplessly as a doctor and nurse performed CPR on him the first two minutes of his life until he finally took his first breath.

From the moment he was born, he was a fighter, a trooper, and a heart warrior. We thought that the worst was over, but hours later our world came crashing down. When the nurse came in to take him for his routine newborn testing, she found a noticeable difference in the oxygenation levels between his upper and lower extremities, no femoral pulses, a heart murmur, and that his lower extremities were bluish while the rest of his body was nice and pink.

The transport team from a larger hospital came immediately and took him away for further testing on a possible heart defect.They felt the defect was too severe and needed to call the Children’s Hospital who came and took him away on his second ambulance ride at less than two days old. They confirmed that he had a critical congenital heart defect that needed to be operated on in order to survive along with various other heart defects. His heart was not perfect and our hearts were completely broken. He looked absolutely perfect and it was so hard to accept that something could be so wrong with him.

At six days old, the doctors and surgeons finally felt he was ready to have open-heart surgery. The surgery was successful and even though he had dozens of tubes, wires, and monitors attached to his body, he was perfect in our eyes. Although he will need at least two more open-heart surgeries in his lifetime, he is amazingly strong and inspiring. There has not been a single day, since he was born, that I do not think about all that he went through his first two weeks of life and the special heart that he has. He is my heart hero.

Cooper cape

Zoe Lihn

Zoe Lihn

In December of 2009, Stacey earned her title as “heart mom” when her unborn daughter was diagnosed with Hypoplastic Left Heart Syndrome at 18 weeks gestation. Since birth, Zoe’s undergone three open heart surgeries (the Norwood, hemi-Fontan and Fontan) and is living well in Phoenix, Arizona with her mom, dad (Caleb) and big sister (Emerson).

Zoe continues to inspire her mom, who advocates on a wide-scale for improved outcomes in the field of pediatric cardiac medicine. While Zoe’s journey with her congenital heart defect has been challenging (which is always an understatement), her strength and courage remain a testament to our CHD communities’ resiliency and power. Together, we can make a positive impact for our children’s future.

Rayna

Rayna Schuh

In June of 2006 my husband Tim and I found out that our family would be blessed with the gift of baby. As you can imagine we were excited and anxiously awaiting that first Dr.’s appointment to hear our new baby’s heartbeat. We had no idea if our baby was a boy or girl. Nor, that the heartbeat we heard at that appointment was coming from a heart that had a critical congenital heart defect.

Rayna was born February 16, 2007. She was perfect, or so we thought. We took Rayna home and just thought she was a tiny little girl who tired easily and didn’t gain any weight. After many trips to the doctor we were reassured she was fine and I was a paranoid mom.

In the summer of 2010, Rayna was diagnosed with strep throat and the doctor heard a disturbing heart murmur. I thought, “Well, everyone has a heart murmur.” After some thought, we took her back 2 weeks later and requested that her heart be checked. Rayna’s little brother had a hole in his heart that we needed to check, and I had that “mom feeling” that something was not right. That appointment would become the beginning of our journey into the world of Congenital Heart Defects.

Rayna had many appointments to determine what her defect was. In September of 2010 she underwent a sedated MRI and we were told she has a heart defect known as Total Anomalous Pulmonary Venous Return -TAPVR – and a very large ASD or hole in her heart. Her defect is usually fatal if not detected in the first few weeks of life but she was 4 ½ years old. We were told that this is unheard of and left the hospital wondering and questioning everything we did. At one of her first cardiology appointments I had a doctor take my hand and look me in the eye saying, “Mom you did not do anything wrong to cause this. By the time you heard her heartbeat for the first time, her heart already had this defect.” These words still echo in my heart. On October 17, 2011 we set out for the hospital and it was the longest ride of my life. From the backseat my beautiful girl called for me and as I turned around and our eyes locked her words got me through the day: “Mommy today is the day they are going to fix my heart and I am going to feel all better.” We turned our beautiful girl over to an amazing team that afternoon. As they wheeled her away, the most wonderful nurse looked at my husband and I and said to us “I will protect as if she were my own.” 7 ½ hours later she kept that promise and we saw our little girl with tubes, needles and hoses everywhere, but she was out and doing well. The relief in my heart and mind was indescribable.

Rayna is now 6. She is stronger inside and out and continues to be our driving force to find the beauty in this world of heart defects and doctors appointments. As each appointment nears we still wait with excitement to hear her heartbeat, just like we did the first time.

Rayna’s heart is mended but we are forever changed. The sound of that heartbeat is priceless.