Liam's Story - Congenital Diaphragmatic Hernia (CDH) & Omphalocele

LIAM means strong-willed warrior.

We knew his name at ten weeks gestation. We were those impatient parents who couldn't wait until the 20-week anatomy scan for the gender reveal. Little did we know, his name was going to be our "hope" and motivation over the next year.

May 14, 2018

My 28th birthday. The day we were finally getting to see our sweet, precious Liam. I remember the awkward silence that filled the room during his anatomy scan. The ultrasound tech didn't speak. She scanned and took pictures, and when we asked a question, we received a short answer. I told my husband something felt off as we waited to speak with our midwife. When she came into the room, the look on her face immediately brought tears to my eyes. She said, "Liam has an abnormal abdomen." She explained that it seemed as if his intestines were outside his body and immediately referred us to our closest Children's Hospital to see their MFM team.

May 16, 2018

After a long day of tests (MRI, Echocardiogram, Ultrasound), the specialist sent us to a room where we sat for what felt like an eternity. When he came into the room with his nurse, he explained to us that Liam not only had an "abnormal abdomen" but that he had two major congenital disabilities. First, he said Liam had an Omphalocele. In Liam's case, his liver was incased inside his umbilical cord, outside his body, due to his abdominal wall muscles not forming properly. He then proceeded by saying, "His second defect is worse." This would be the first time we would hear the term left-sided Congenital Diaphragmatic Hernia (CDH). Liam, already measuring extremely small, had his stomach, bowel, spleen, and even some liver inside his chest cavity. We were given two diagnoses with no earthly idea how to spell either of them. We asked them to be written down or even a printout defining these defects. We asked what we were up against, what Liam's chances were, and without hesitation, they told us they couldn't give us that information. Less than ten minutes after receiving this heart-wrenching information, a genetic counselor was sent into our room to explain our termination options. We immediately declined and proceeded with receiving an amniocentesis due to the high possibility of genetic issues. 

Almost two weeks had passed, and during that time, I spent every free moment researching. Never finding a survival study on a child who had both birth defects. I felt lost and defeated. I decided to seek out a support group on Facebook, looking for encouragement. I shared our story and, within an hour, received a message from a CDH mom who told me that she knew of a little boy who also had both an "O" and "CDH." Her daughter and this little boy had received care from the same doctor. She gave me the contact information for this other mom, and I immediately felt overwhelmed with hope. I had so much hope that I knew I wasn't giving up on Liam, no matter the outcome.

I returned to the children's hospital we were originally referred to, but this time, with lots of research and the Tiny Hero questionnaire from the website. We were scheduled to speak with their Chief of Surgery. I remember going into the meeting with the thought, "If we don't get the answers we expect, we know we have the option for a second opinion." This hospital had a 3-week ECMO cut-off. They would not perform surgery while on ECMO, and we were told, "Quite frankly, any surgeon who does that is crazy." They proceeded to tell us ECMO wouldn't even be discussed until Liam was born and had seen if his lungs were "worth" putting him on ECMO. They had never treated a child with both congenital disabilities or seen it. Finally, we were told, "If he did happen to survive, his quality of life would be very poor." I did not shed one tear while hearing these harsh words because we had a plan, and we knew we had to follow through.

At the end of June 2018, we met Dr. David Kays at Johns Hopkins All Children's Hospital. He spent over an hour going over every detail of Liam's potential outcomes. He was honest and very blunt about the severity of his case. Then, the words I had longed to hear were said, "We will do everything we possibly can to give Liam his best fighting chance." I didn't need to hear anything else. We knew relocating from Missouri to Florida was Liam's best chance at survival.

September 9, 2018

Liam was born during an emergency C-section due to placental abruption. He was intubated immediately before being rushed upstairs. Dr. Kays and his team stopped at the head of my bed and lifted Liam up for my husband and me to see him. He was blue, but his eyes were wide open and looking right at us. In that split second, I knew he was our little fighter. Liam was placed on ECMO within the first hour after birth. He had his CDH repair surgery the following day, with only having 1% of his diaphragm, and he stayed on ECMO for 23 days total. Our next five months were nothing short of a rollercoaster ride. Liam self-extubated four times. He coded four times, receiving chest compressions with each code, with the longest being 7 minutes long. He had four failed extubation attempts leading up to his tracheostomy surgery on January 3, 2019. We were discharged from JHACH on February 12th with his home vent, trach, G-tube, and his unrepaired omphalocele, but with all that we went through, I wouldn't change one minute of it.

Over and over, we literally watched our son fight for his life. He humbled my husband and me, all while making us stronger individuals and making our marriage stronger. There will never be enough words to show our appreciation to Dr. Kays and his entire CDH team at JHACH. They became family to us. They loved Liam as if he were their own child. Tiny Hero was our biggest support system. They led us to our hope. Every story of every child was read leading up to Liam's birth, and I prayed for the day we might get to share ours. Liam is now 14 months old and lives his life as if he knows nothing ever happened to him. He is such an extremely loving and happy little boy. Liam ran the CDH Marathon and has continued to overcome obstacles daily.

Update:
Liam is currently doing great! He’s awaiting surgery scheduling for tonsillectomy, adenoidectomy, and double-stage laryngotracheoplasty. We figure this surgery will happen before the end of the year, but this is a big step forward towards trach decannulation! He’s attending school Monday through Thursday, 3 hours a day! He loves his family, two cats, straws, and pulse ox stickers! He had a rough start to the year with two unplanned surgeries involving his patch getting infected/replaced, and his Nissen fundo had to be redone due to a small leakage from his esophagus. He is such a happy boy, though!