Travis ~ A Journey of the Heart (June 5, 2014 - April 8, 2015)

Travis was born on 07/04/2013, pink and vibrant, weighing 8 lbs. and 11 oz. at birth. And he was born with Heterotaxy, a rare congenital defect.

We learned about Travis’s heart condition at 25th week of the pregnancy and thought that was the only issue. Heterodoxy as a new diagnosis only came after 1 day he was born. We had intense meetings and discussions with our medical team and consultant before and after he was born and did extensive research regarding choosing the best medical and surgical team to care for him.

Boston Children’s was our final choice. 10/02/2013 we took Travis to meet the surgeon and the team for the 1st time. The initial echo looked promising. The size of his left ventricle is good enough for a Bi-Ventricular repair which is considered the best solution for babies who are born with a single ventricle. Although he had pulmonary stenosis and pulmonary hypertension, these two conditions together have formed a nice balance that lasted him a good 11 month before we were called for the surgery. Travis grew well in the first year with no medical intervention, the only thing that’s slightly noticeable setting him apart from other babies was his purple tint lips under a gradually declining blood sat, 83 at his 11th month, 93 to begin with at birth.

The 1st surgery, then the only surgery that he would need in our mind, was scheduled on 06/05/2014. Travis was all ready. I kissed his perfect little chest for the last and waved good bye while he was busy playing the new hospital toys in his premeditated drowsy state. 7.5 hours later, we shook hand with our surgeon. “All went well”. And we exhaled. Post-surgical recovery was longer than we expected. Soon we learned that the mitral valve regurgitation was the reason of the slow recovery and it had to be repaired. Reluctantly and sorrowfully, we opened our arms to welcome the surgery #2 to take place, envisioning this marking the successful ending of Travis’s surgical journey. It was over and again” all went well”. The prolonged recovery began again. At one point his recovery took an upward turn and we were happily relieved he was finally transferred on his one year birthday to the recovery floor.  Then 07/08 came, the darkest day of my life, we were pulled to the small meeting room to talk to the team, whom informed us, unfortunately the repair didn’t last, the regurgitation remained and persisted.

But they didn’t keep us there for more to be done. They let us go home with the hope that over some period of time, Travis can grow and hopefully the regurge will tame. We set our foot to home on 08/04, exactly two months after the first surgery. Travis did enjoy his home stay after being away for so much. He did grow and we did take many pictures with him smiling under the bright sun. Then he was hit by a cold 2 weeks he came home, then the decline began.

From later August to later October, Travis’s life was thinning out in a gradually but distinctive way. He started feeling more fatigued over time, eating half of what he used to, then 1/3, 1/4, then finally 1/10. Having no interest in activities and crying through his many days and nights with his very weak voice then. And the vomiting episodes became a potential of his daily routine, from once a week, to 3 times a week, he began to throw up every other day if not daily starting the end of October. We did engage in all doctors and psychologists, physical therapists in the picture and the overall conclusion was that he just needed more time to recover, based on his mystically great looking echo reports.

We called Boston Children’s and scheduled the painful trip of truth searching.  11/23/2014, 5 months after his first surgery, we came back. Friday morning was his cath, Travis went into cardiac arrest during the anesthetic process before the test began. And the answer was granted, finally. We were relieved, with pain. The 3rd surgery, an emergent one was scheduled right away, again to repair the much worn out mitral valve, as the final hope it would last this time. 11/24/2014 Monday, an already intubated Travis was sent to the operating room. We felt nervous more than ever before, knowing how very fragile he was, with absolutely no reserves through the month’s long decline. And he came out, same puffy, same cold, same pale and same breath. The days then was all about the monitor screens, every number change, every med change, and every plan change. No luck this time, still. His blood pressure and LA pressure remained high under all measures.  7 days later, the 4th surgery of mitral valve replacement took him back again on the surgical table. Now the new game was to watch if he AV node will come back after being injury during this surgery. 12/11/2014, 10 days after the 4th surgery, a permanent pacemaker was put in his belly, marking the 5th surgery during his mostly bedridden second year of life, from 11th month to the 18th month.  The storm did die down after all. He did seem getting better in his spirit and physical being through the rest of his recovery journey, there was really nice weight gain and plenty of happy faces and playful moods, all the way until the day we came back to Chicago, on the day upon departure we were rest assured that” Travis is nowhere near the need of a transplant” by our team.

01/25/2015. After another exact 2 months stay in Boston. We were happily returning home after the last day pictures taken with the whole Smith clang. Travis started throwing up in the taxi on the way to the airport. And he continued on during the entire flight until he ran out every single spare shirt and coat. We called Lurie as soon as we land and went straight to be admitted, the dust settled at 11:30 p.m. that night, our hearts sank into the endless dark night.

Travis didn’t get better as we and the local team had hoped. He kept throwing up multiple times at a day. Those were the toughest and wickedest days and nights with so much struggle of wanting water and milk and constantly being denied and rejected. A week later, he went into another cardiac shock while the nurses trying to replace his old IV. As much as the 3 pokes and 1 hour of hysterical crying did it, his tiny body of extreme weakness didn’t help whatsoever at all. The intensivist doc noticed me that the chance for him to be through this with a breathing tube put in is only half the chance. I refrained the selfie I was trying to take with my son whose life was again on a thin line in his forever land of hospital bed, gasping in every air he could use, holding the faith that I would take a good picture with his happy face, before I was asked to leave the room. Then the miracle boy survived through the heart wrenching night.

The team informed us that there was no way he could recover on his own unless with a new heart. With much debate in the eternal thought process, Travis, “the sickest child in Illinois”, was put on the wait list as a status 1A patient on 01/31/2015. Then our mind still shifted towards Boston, the last and the forever panacea, for the very last confirmation. With the med flight arrangements through overcoming the impossibilities, with the Boston CICU bed generously awaits, with our Boston surgeon’s one and always vision for his native heart, Travis declined the offer of leaving Lurie, with big drops of tears falling from his tightly closed eyes, as if he was saying” please let me be, no more and more”. 02/03/2015. Travis’s put back on the list. No more and more. And The Wait Began.

Midnight. 02/19/2015. We got the call with immense gratefulness.  Still and still, we talked about the decision with our consultant/family friend for another 2 good hours out of the deepest mind of dilemma. Yes came firmly after that.

Early morning.  02/20/2015. We said good bye to Travis, again. I listened to his to me still very fine heart beats and took picture of him with the surgeon whose eyes looked confident and compassionate.  8 hours later, we welcomed our new boy back, with a new heart beating in his brave chest.

Travis’s heart story didn’t end here. And it may never end. There were other unthinkable swirls of rejection issues that came with drastically invasive treatments, which did work wonders, before he was finally discharged on 04/08/2015.

We are deeply GRATEFUL with gratitude towards our little donor hero and his family, our most excellent local team and surgeon, our amazing medical consultant, our ever caring nurses and the floor staff, and our whole Boston team, our wonderful family and friends who supported us all along, and last but not least, our heartwarming fellow heart moms and their little ones who modeled and still are modeling for us, every single day, now then and ever, with their unbelievable courage and strength, All Beyond Words.

Today’s Travis is a thriving 2.5 years old, chasing his sister everywhere for the fun of life, loving the moments nonstop. He receives 5 therapies each week and has been making progress in his development steadily.

Thank you for reading Travis’s heart journey. We appreciate very much your sharing this special path of his with us.

God Bless. 

Once a Heart Mom...

When our daughter passed away, almost a year ago, I was suddenly thrust into a state of limbo.  The world of high intensity that I had come to know so well, was no longer mine to share with the heterotaxy community that I had come to call family.  And although her life was cut short and her time in the hospital was brief, I felt as though my very identity had been snatched away.  How was it possible that the monotonous sound of monitors and pumps (that were once deafening) had now become soothing and welcoming?

The transition from taxi mom to heart mom was a difficult one. There was so much heaviness at that time. Some of that stemmed from fear, some from confusion, some from my selfish desire for “normalcy” and much of it from a clear understanding that those around me simply didn’t get it.  But who could blame them?  We were talking heterotaxy here!  It’s not like there was an instruction manual or a field guide of what to expect.  Before I knew it though, the cardiac ICU became our home.  After two heart procedures and 4 weeks in the hospital, Rachel was finally moved to a step-down unit and I was coming out of the fog.  However, it was then that she, quite unexpectedly, passed.

Two weeks later, I returned to work.  It was one of the most difficult actions to carry through, but looking back, I’m so thankful that I did.  The predictability and routine was mind-numbing to say the least, but it allowed for me to keep busy.  What I wasn’t prepared for was how lost I would feel now in the world that was once my normal, everyday routine.  My CICU life and my office life didn’t collide, they actually repelled each other much like magnets do when two of the same poles come together…I was stuck in the void in the middle.

I had been living a dual life…one that revolved around heterotaxy at night and on Facebook and the other that consisted of the daily grind of paperwork and office tasks.  It wasn’t until Heterotaxy Awareness Day that my two lives came together.  I began posting information on my personal page about heterotaxy and the importance of raising awareness… and before I knew it, my worlds began to merge.  Of course my family, friends and coworkers had always been there, but it wasn’t until that day, that I realized what a support system I had from both worlds.  That day, I found a new purpose in this world…one of advocating for those that struggle daily with the unknown while at the same time raising awareness in a world that has never even heard of heterotaxy.  I lived the life of a heart mom for a reason, and while my time in the CICU was brief (by heterotaxy standards), I will hold tight to this truth… once a heart mom, always a heart mom.

Home

There’s a street I drive down almost every day to get back home from pretty much anywhere. It’s not a pretty one. Rundown houses and boarded-up taverns sit across from massive parking lots and big-box stores. It’s all concrete and gas stations and tiny storefronts, and the traffic is always terrible. I can remember so vividly the first time we drove the length of it. We were blindly following the GPS, heading to look at a house that we hoped to rent, a place to call our own after living in my parents’ basement for the better part of a year when what was supposed to be a short maternity leave turned into a nightmare of diagnosis and birth and my baby’s chest cracked open twice before he celebrated his first Christmas.

The whole world looked grey that day. We were facing the looming prospect of Ethan’s second surgery, walking the tightrope we call ‘interstage,’ that fragile time after the first operation when it feels like just looking at them wrong might be enough to make it all come crashing down. We had left our former life (as volunteers on board a charity hospital ship off the coast of West Africa for the better part of six years) with no idea that we’d never go back. I walked down the gangway into the hot, Congolese night, carrying only what I could pack into two suitcases, fully expecting to be unpacking again in my tiny cabin a few months later. But then, without the faintest whisper of warning, words like ‘heterotaxy’ and ‘complex, critical congenital heart defects’ wrenched everything that I’d ever held dear out of my shaking hands, and for some reason, on that gloomy day in November, the reality of it all was almost enough to suffocate me.

I didn’t want to live on land. I didn’t want to leave the vibrant community on board the ship, the community I was planning to surround myself with as I raised my children. I certainly didn’t want to be unceremoniously and irrevocably thrown headlong into this very different community, the one made up of mamas and papas fighting for their children’s lives against the shadowy spectre of heterotaxy.

I wanted to go home.

Home. To the ship and the wards and the familiarity of the ever-changing view outside my portholes. To the place where I cared for broken babies at work and went back to my cabin afterwards, certain that I could never find the kind of strength I saw in their mothers. I wanted to go back to the time before everything changed, but the vast, yawning canyon between now and then was deeper than death, and it felt like my heart was breaking all over again every time I thought about how much was wrong with my son’s. There was no way back.

I drove down that street this morning. We had just made a surprise visit to the thrift store, and the kids were happily looking through new-to-them books when Adele came on the radio. Shrieks of pure joy erupted from the back seat, and when I glanced in the mirror, both of them were holding imaginary microphones and lip-synching as though their very lives depended on it. We passed the tiny post office where the woman behind the desk knows my children by name and always has scraps of paper for Zoe to colour on while we take care of our business. We stopped at the light in front of the hole-in-the-wall restaurant that sells what I’m pretty sure is the best fried chicken in a fifty mile radius. My favourite tree, the huge one next to the tavern, reached its bare branches over my car, and I could picture the way it had looked through all the seasons, from the impossibly bright green of a nascent spring to the vibrant, red leaves that fell on us like a prayer through the fall. Zoe chanted the names of the side streets as we passed each one, calling out triumphantly as we passed the house with the purple door that we were almost home.

Home.

It looks nothing like what I expected, but as we’ve slowly filled these empty rooms with the things we’ve collected and covered the bare walls with still frames and memories, my feet are finding their way on this solid ground. I look back over the days and weeks and months between then and now, and I’m someone I barely recognize. We’ve been given the indescribably precious gift of time, and it has changed everything.

Time, when you’re spending it in the company of a medically fragile child, is a strange thing. There are stopping places along the road where it all but stands still, and you’re left staggering under the impossible weight of lost innocence for what feels like forever. There are sudden flashes of pure joy where everything is right with the world, but then you blink and it’s over and you’re left wondering whether you imagined it all in the first place. And there no way to know, just by looking at the clock, which one is coming next, so you put out your hands and you take what you’re given, and you hope with every fiber of your being that you’ll be strong enough to hold all of it.

And this is the beauty of this life, a life without guarantees. Every second of it is precious because none of them were promised. Every hour is made of gold, the awful and the awesome alike, and I think the reason I can’t see myself when I try to look back to before the day when everything changed is because I’m blinded by the truth of everything that’s come after it.

And the truth is that I’ve found my home. It’s the memories of the years spent on the ship, and it’s the pool of sunshine on my living room floor where my broken-hearted boy and his big sister sit together and play. It’s the hallways of the hospital where they cut him open and pieced him back together again, and it’s the playground where we jump in mud puddles. It’s here and there and everywhere in between, because when you’re not promised tomorrow, home is just where you are today.

Welcome home.

(Ali Chandra - Mom to Heterotaxy Hero, Ethan)

Reflections

As 2015 comes to a close, it brings reflection. Reflection on the year that has just flown by within the blink of an eye, and yet so slowly at the same time. It is a very odd combination and one you truly understand when your child has Heterotaxy.

One year. What does that truly mean? For most, it is filled with family fun and a carefree attitude. For us, it was deciding to move across the country and subsequently having to find a new care team for our daughter, Violet. It was finally taking the plunge to remove her feeding tube after having it for nearly three years. It was giving hope to newly diagnosed families and making connections. And it was, unfortunately, watching friends lose their children due to Heterotaxy-related complications. A year can have so many different twists and turns that it can be hard to plan for the future, even just the next year. Living in the moment is something that many of my fellow Heterotaxy parents abide by because we are afraid to make plans for a child that may not be there.

But I won’t do that this year. I can’t do it this year. I am deciding that it would be healthier for me to finally start looking forward to the future. We will plan first for Violet to go to school in March once I graduate school and am working. We will plan for a vacation in the summer, perhaps to Disneyland or back home. We will put her into preschool in the fall. We will buy presents all year long for Christmas. But what I won’t plan for is what “may happen” in regards to her health. We deserve to have a year where our first thoughts are not on a diagnosis, but rather how well she is doing and rejoice in that. 

All of the changes we faced this past year has made us a stronger family and we look forward to 2016 being just as prodigious. We wish all of our Heterotaxy families a happy, prosperous, and healthy year!

Danielle Leppo ~ Mom to Heterotaxy Hero, Violet

One Family's Decision for the Bi-Ventricular Conversion over the Fontan

Zachary and Dr. del Nido almost 3 years ago after his bi-vent in Boston and then again at the Heterotaxy Connection Conference.

February 7, 2011 our son Zachary was born with Heterotaxy syndrome that was not prenatally diagnosed. He has ASD, VSD, Pulmonary Stenosis, TGA, DORV, Complete AV canal defect, asplenia, and malrotation (among others). He underwent a BT shunt at 6 weeks old and we were told at that time his next surgery would be at 6 months when they would either “fix everything” (bi-vent) or do a Glenn surgery. When he had surgery at 6 months it was the Glenn. The surgeon said he would tell us after it was over if bi-vent was an option. After the Glenn the surgeon just said he was not a bi-vent because of the AV canal defect and would not discuss with us further. He was Fontan only. The more reading we did on the Fontan the more our concerns grew. We began our own research and found Boston Children’s Hospital was  #1 in the Nation for Pediatric Cardiac Surgery. We kept hearing the name of Dr. del Nido and found his email on BCH and emailed him directly. He responded within 24 hours telling us he would look over Zachary’s records. We sent the records and he and Dr. Gerald Marx emailed and said they would like to have Zachary come to Boston for testing.

We went for testing in May, 2012. I still remember the day we received the email containing the letter that stated they felt Zachary would be a candidate for a bi-vent repair. I cried. I finally felt like we had hope. We discussed it with Zachary’s primary cardio here in GA. I asked him point blank, “If it was your child, what would you do?” He answered without hesitation. He said if someone offered him a bi-vent over a Fontan for his child he would do it. We knew going in that the bi-vent was not an easy road. We were told it was a lot of work up front but better in the long run and that the Fontan was more straight-forward up front but led to complications in the long run. We were told to expect recovery to take anywhere from 2 weeks to 6 months

After much prayer and consideration we decided to schedule the bi-vent in Boston for August, 2012. Yes, it was a long way from home and we had no idea how we would handle it financially, but we knew we had to try. In the meantime in July of 2012 we welcomed our 2^nd son, Dylan. He was born heart healthy.  He went with us to Boston and stayed for all except the last 2 weeks of our time there.

During our pre-op visit in August we talked with Dr. Marx, Dr. del Nido and one of the cardiac surgery fellows. We voiced our concerns over other children that had attempted a bi-vent and it had failed. They were very up-front with us. None of the ever sugarcoated how hard it would be but I still remember Dr. del Nido looking at us and at Zachary who was toddling around the room talking and saying, “We would not try a bi-vent on a child who is doing as well as Zachary is if we were not sure it was a better option for him.” They also told us that their criteria for selecting candidates for this risky surgery are very stringent.

The bi-vent recovery was the toughest thing Zachary has ever been through. At 23 hours after surgery he suffered Cardiac Arrest and underwent 23 mins of CPR before being placed on ECMO. He was on ECMO for 6 days. He struggled to wean his oxygen and to eat. His stiff left ventricle did not want to do what it was being asked to do. But Zachary persevered and was discharged 47 days after admission with a SUCCESSFUL BI-VENT. His valves work beautifully and he has not been hospitalized in the 3 years since his surgery. A Heterotaxy kid with complex CHD has not been in patient in the hospital in 3 years. Awesome. He is having some narrowing in his RV to PA conduit and we will be traveling back to Boston in February 2016 to have a cath and try to balloon it. But we knew that was to be expected. At some point the conduit will have to be replaced. But that is considered a common procedure now. We consider this a bump in the road that we now hope leads Zachary to old age. The sweetest words I ever heard were when I asked Dr. Marx in Boston what he thought Zachary’s life expectancy was post-bivent when we went back for a check-up one year after his surgery. He said, barring unforeseen complications (which we know can happen at any time), he expected Zachary’s life expectancy to be “nearly normal”.

I am aware that a bi-vent is not an option for each child. Each of our Heterotaxy Heroes are so different and unique. Whatever route we take with Zachary’s care and whatever happens in the future, he still is our precious boy and a gift from God.

Stacie Bohannon (mom to Heterotaxy Hero, Zachary)

Nick Elizondo - Hope in Heterotaxy - Adulthood and Transplant Success

Adult heterotaxy hero, Nick Elizondo, shares his story...one of multiple diagnoses within a family, organ transplant and most importantly...HOPE.

"When I was born, I was really jaundiced and they knew right away that something was wrong with my liver; they realized that it wasn't connected. So, at 9 days old, I underwent a Kasai procedure to connect my liver to my intestines, leaving me with Biliary Atresia. I had my first open heart surgery at 1 year old... to be followed by 5 more, most recently in 2011 for aortic stenosis and total anomalous pulmonary venous return.

When I was 22 my liver began to show signs of failure. On January 18, 2013, I was placed on the liver transplant list at Barnes Jewish Hospital in St. Louis, MO. I waited for 1 year, 7 months, and 5 days before I received my liver on August 23, 2014. I have since recovered well and am looking forward to starting school in the spring.

My sister, Brittany Elizondo, was born with Heterotaxy also. She passed at 8 months though. She passed from Pulmonary Hypertension.

I was chosen to throw out the first pitch at a St.Louis Cardinals game for Transplant Day...a dream come true! My wife is my rock and I have the best family support."

(the lower left set of pics in the grid is Brittany)

Parenting an Adult Child with Heterotaxy

One mom shares about parenting her adult child with heterotaxy...

Many of you know what I went through when Thomas was a baby, because that’s where you are now, in a situation that affects the entire family.The impossible prenatal diagnosis of Heterotaxy Syndrome, testing, surgeries, around the clock tube feedings, medications, and extended hospital stays have become our normal. I somehow knew through all of this that we were among the lucky ones, and this feeling becomes stronger as I read so many stories of warriors and angels.

Where I am now is looking forward to Thomas’ 21st birthday! With no physical limitations, he has played baseball all his life. He was recruited to play in college, and is now in his junior year at Albertus Magnus College. The only time I think about his condition is before his annual cardiology visit. That’s when all the emotions come rushing back, as if there weren’t so many years since that first ultrasound.

I have never treated him as if he was different or sick in any way. His heart and intestines have been corrected, and his organs work just fine where they have been placed in his body. He wears his scars proudly, and has even told staring younger children that he was bitten by a shark! We may sometimes tease Thomas about his heart being on the wrong side, or donating some of the extra spleen he doesn’t need. Otherwise he is a healthy and strong young man doing well in college and looking forward to a successful future.

Dianne Ceneri (Mom to Heterotaxy Hero, Thomas)