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)