February is heart month, so what better way to celebrate than by showing off one of our most precious heart warriors! Most people don’t even realize that 1 in 100 babies are born with a congenital heart defect (CHD). Of the babies born with CHD, 1 in 4 of those defects will be considered critical and require intervention within the first year of life. CHD is caused by abnormal formation of the heart during growth in the womb. In most cases there is no known reason for it, but some types of CHD can be linked to an abnormality in the number of a baby’s chromosomes.
On February 23, 2018 Jaxon graced his parents, and the world with presence. His parents Cassie and Bobby thought they had a perfectly healthy baby boy. Within hours of birth, Jaxon started to get very sick and showed signs of distress. He was taken from Anniston where he was born, and transferred to Brookwood in Birmingham where they had the resources to adequately care for and diagnose Jaxon. Upon his arrival, Jaxon had a echocardiogram done so they could look and see all of the internal structures of the heart and its pumping power. The echo showed Jaxon was not only 1 in 100, he was also the 1 in 4 who had a critical CHD. He was then transferred again to Children’s of Alabama and admitted to the CVICU (cardiovascular intensive care unit) where he would spend the next 6 months.
After several days, and a whole lot of images Jaxon had a diagnosis and a plan. His first issue was an interrupted aortic arch (type B). The aorta is the heart’s main pipeline, carrying oxygen-rich blood to the body. Normally, it’s shaped like an arch or curve. In an interrupted aortic arch, part of the aorta is missing, leaving a gap. The ends of the aorta on either side of the gap are closed, so blood does not flow into the gap. Body parts that are fed by blood vessel branches beyond the gap (such as the legs and belly) will be damaged if the flow of oxygen-rich blood is not restored. He also had large atrial septal defects and ventricle septal defects, meaning that the walls of the chambers of the heart have holes in them letting oxygen-rich and oxygen-poor blood mix and flow incorrectly. Then there was the pulmonary atresia which means the valve that controls blood flow from the heart to the lungs didn’t form at all. In babies with this defect, blood has trouble flowing to the lungs to pick up oxygen for the body. Jaxon also had a fistula (opening) from his coronary artery to the pulmonary artery allowing for incorrect blood flow and more anatomical abnormalities with an aberrant right subclavian artery. None of which had any ability at stopping Jaxon.
Jaxon was ultimately diagnosed with DiGeorge Syndrome (DGS). This is an immunodeficiency disorder characterized by various congenital abnormalities. Proper functioning of the immune system relies on the thymus gland. In DGS, the thymus and parathyroid glands are either not fully developed or completely absent. In Jaxon’s case, his thymus gland was not working. DGS can also be responsible for most of the cardiac abnormalities Jaxon was facing.
So imagine for a moment that you gave birth to what you thought was a healthy baby, only to find out that he had a very long road ahead of him, including open heart surgery at 11 days old. ELEVEN DAYS OLD! A newborn baby’s heart is the size of walnut, and you have to trust a stranger to fix the problems on something so small. Nothing can prepare you for something like that. Jaxon had his second open heart surgery at just 6 weeks old.
With Jaxon’s extended stay on the ventilator, the time came for the tracheostomy talk. Now, Jaxon had to deal with severe tracheobronchomalacia. Essentially, his airway was more like a floppy cooked spaghetti noodle rather than a sturdy thick straw. Once Jaxon got his trach, he also started sharing his precious personality with the world. A short time after the trach was placed, Cassie and Bobby started seeing Jaxon’s sweet signature smiles. I have a hard time remembering seeing him without one. This child is pure joy 24/7.
On November 5th, 2018, 255 days later, after countless procedures, surgeries, needle sticks, tears, training, and more training, Jaxon busted out of Children’s Hospital and went home for the first time. This kid has climbed more mountains and overcome more hurdles in his short little life than most of us ever will, and lucky for him he has two parents that will fight tooth and nail for him every step of the way.
Because Jaxon likes to keep everyone on their toes at all times, he also ended up developing SVC syndrome which means the main vein that carries blood to the heart is blocked or compressed. This can lead to swelling which became a big problem for Jaxon. In Jaxon’s case, the superior vena cava clotted off so he wasn’t getting adequate back to his heart from the rest of his body. Eventually, Jaxon developed collateral veins that took on the job of the SVC and his swelling decreased tremendously. Thanks to some pretty amazing cardiologists Jaxon now has a working SVC. This kid is an overcomer!
At this point, Jaxon is days away from his 3rd birthday! He only uses his ventilator when he is asleep, he loves Mikey Mouse, music, and being cute as a button. It’s truly amazing to see just how far this kid has come from when he left the hospital. Once he gets a working thymus gland so he won’t be immunocompromised, this kid will be unstoppable.
I think the most moving piece of proof of Jaxon’s strength would have to be his beads of courage. It’s an insanely long strand of beads. Each single bead represents a piece of Jaxon’s journey, cath procedures, surgeries, IV sticks, blood transfusions, admission days, and so on. The long colorful strand shows his struggle, his determination to live, his strength to still smile, and the mountains he’s climbed. Jaxon’s family decorates their Christmas tree with the beads every year and it’s long enough to wrap around not just once, but twice. Like his mom says, “seeing how long the string of beads is, and feeling the weight of them almost brings me to my knees”.
I always end these with a Q and A session I had with the mom. I asked Cassie what the scariest part about going home with a trach/vent baby was, she told me “it was how sick Jaxon still was. He needed a lot of support and going home meant we were solely responsible for his well being. We lived in the hospital for almost nine months, we were used to having doctors and nurses close if something happened. I remember learning how to use the ambu bag and being terrified that we would need to use it at home and not be able to save him if he went into respiratory distress.” This is where I would like to add, I was never worried about these guys, I knew Jaxon would be getting just as great at home as he was at the hospital. They are rockstars!
What’s your greatest mom hack? “100% has to be using circuit covers. They make such a huge difference when it comes to condensation build up. Before we started using them, there were so many incidents when that water would accidentally dump into Jaxon’s trach. Now it’s something we hardly have to worry about. I started making my own instead of buying them off Etsy. They’re super easy to make and I’ve been able to gift a few to other vent families. I wouldn’t call it a hack since so many vent moms use it.”
What’s your best piece of advice for other trach/vent moms? “Be as involved in your child’s care while inpatient as possible. It will make the process of training to go home much easier. Once you get home you’ll start to learn different ways to do things that work better for you and your baby and that’s ok. What works for some may not always work for others. The first couple of months at home are the scariest. Things will get easier as you figure out your own routine.”
Jaxon is a well deserving hero of heart month. He is still overcoming hurdles and smiling the whole way!