"Abel has been a full spirited fighter since before he was born." - Abel's CDH Story
Abel’s left sided CDH was found at our routine twenty-week ultrasound. “It’s a boy” was quickly followed by a grimaced concern and the words “you have to see a specialist within the next week. They said “I suspect your son has Congenital Diaphragmatic Hernia”. Upon walking into the specialist’s office the following week we were prepared to have another ultrasound, we were taken into a room by a genetic counselor and her intern, but nothing could prepare us for what would come next. We were asked not once, but five times if we were “sure” we didn’t want to abort the pregnancy. Our firm answer was no. The ride we were in for during our high-risk pregnancy was full of appointments. We had over ten ultrasounds, multiple MRIs, echocardiograms, stress tests, and over twenty appointments in five different locations.
Weeks later I was scheduled for an induction at thirty-seven weeks to let Abel know that the world was ready to meet him. His diastolic flow diminished and his heart rate was dropping. He was telling us that he would make his entrance when he was ready. We were on Abel time. With over fifteen people present in the Operating Room, it was certainly the crew Abel needed for his grand entrance. Upon delivery he was immediately intubated and whisked away, transported by bridge over to the Med/Surg ICU at Boston Children’s Hospital.
It was then that his fight began. A consent was signed and Abel was put on the schedule at three days old to have his CDH repair. That very day we were told that if he was to need ECMO, they were leaning towards not putting him on it, due to the amount of brain bleeds present on his initial MRI. On the fourth day of intubation, sedation, and life on an osculating ventilator, he took a turn for the worse. In under twenty minutes the decision was made and he was cannulated for ECMO. With severe pulmonary hypertension the 31 days he spent on ECMO cannot be easily summed up with words. The options were as follows: take Abel off of ECMO successfully or take him off unsuccessfully. He couldn’t sustain life with a machine doing the work of his heart and lungs forever. He had to come off. Our three week “cap” of ECMO being the only solution had quickly turned into one month of ECMO.
During Abel’s ECMO run he was genetically tested for Cornelia de Lange Syndrome because of the facial characteristics that we noticed at birth. The blood test was to take six to eight weeks to come in, and frankly we didn’t invest any attention into the test (which later returned with a positive diagnosis) because we weren’t sure our son would live that long.
Abel was sent for his left sided CDH repair at the tail end of living on ECMO. His pulmonary hypertension was severe and required Nitric Oxide for months along with medication and Oxygen therapy. There were many times we thought he’d lose his battle with pulmonary hypertension, and instead was medically paralyzed on many occasions until it was controlled.
The first four months were spent intubated and we cried tears of joy when he was put on high flow Oxygen and the breathing tube that hovered over his mouth was finally gone. Abel’s respiratory failure didn’t quite match his predicted lung volume of twenty-six percent that was measured prenatally. He was intubated several times after initially being extubated due to his body retaining Carbon Dioxide and needing ventilator support. Abel’s additional surgeries during his stay included a GJ feeding tube placement, a gallbladder drain then ultimately gallbladder removal, and finally a tracheostomy. A majority of his stay was spent on IV nutrition, and more sedation than he’ll ever need to be told about when he’s old enough to understand.
Three hundred twenty-three days were spent bedside with our son in the Med/Surg ICU. We were adopted into an elite family known as our Boston Children’s Hospital family. By fate they became the wind beneath Abel’s sail. It has been just over one year since Abel came home to the woods of New Hampshire. He turns two years old this month. He’s followed still by over eight specialists, additionally with in home respiratory therapists, physicians and nurse practitioners, in home nursing, occupational and speech therapy, and a swallowing specialist.
Abel has been a full spirited fighter since before he was born. He’s full of tricks up his sleeve and particularly likes to keep physicians, surgeons and anesthesiologists guessing and strategizing. He’s impressive with his physical strength and is sitting independently and walking with assistance. He is currently G-tube fed and takes occasional baby puree’ by mouth. At home we’re controlling pulmonary hypertension and reflux with medication. Most importantly we’re busy chasing Abel around with his cart that holds his ventilator and equipment in tow. Abel has recently come off of Oxygen with only an occasional need for it. Our home is full of perseverance that’s powered by the heart and soul of our sweet Abel. He’s deemed to be an old soul by the glimmer in his eyes and his serious, stoic face. When music is playing and he has one of his favorite toys, you can catch him smiling so big that your soul catches fire. Those who meet Abel have no choice but to fall in love with him, and most anyone who knows him will tell you how full of fight he is. He’s showing to be cognitively and developmentally delayed by both a long hospitalization and sedation course accompanied with his Cornelia de Lange Syndrome, but that hasn’t stopped him one bit.
Abel has taught us to remain strong on the darkest of days, and the ability to advocate as his voice each and every day. He is light, love, and hope. Defying the odds doesn’t come close to hitting the nail on the head for Abel’s true ability and strength. Where there is love there will always be a way. Our greatest adventures are yet to come with our little wild child. CDH and CdLS are a part of Abel forever, but they do not define his capabilities. He has so many wonderful things in this life to look forward to, Boston Children’s Hospital was just a stop along his journey. My Hero Calls me Mama (myherocallsmemama.com) is a legacy of apparel and blogging we’re building at home to advocate and bring awareness to Abel’s dual diagnosis’ as well as talk about trach, ventilator, and tube feeding at home.
Hope is understanding that it’s not the size of the mountain that lies ahead, but the deep breaths you take as you embark up that mountain. Faith is knowing that when you’re weak, perseverance will help carry you through. Our story doesn’t end here! We welcome anyone to join along as we navigate this journey.
-The Taylor Family