P’s Type 1 Cleft Journey
Texas
by Caetyn
In 2023, our son was born with a type 1 laryngeal cleft, food protein induced enterocolitis (FPIES), and gastrointestinal issues.
He was very, very active in my womb and shortly after birth he was very eager to eat. His brother had difficulty latching and feeding as a newborn, so it was a sigh of relief that P had such a strong desire to eat and a good latch. We never expected the tremendous difficulties with eating that would ensue.
Hours after birth he vomited a large amount of fluid, though we were told this is normal for a C-section baby. But the projectile vomiting continued. I noticed his lips would turn blue frequently and his neck would retract while breathing. He would fall completely asleep while eating, after just the first few eager sucks. He was unable to stool and his jaundice worsened.
I noticed he hadn’t gained weight, so our pediatrician recommended we try pumping breastmilk and adding some formula to fortify calories. His coloring, energy, and stool improved slightly, but I knew something was still wrong.
I am a pediatric speech-language pathologist (SLP), so I am familiar with airway disorders professionally and personally. My oldest son had persisting laryngomalacia and oral ties, so we went to our pediatric dentist who specialized in ties and had P’s severe lip tie revised. We took him to my colleagues for feeding therapy and physical therapy. We implemented every compensatory strategy possible for infant feeding and swallowing.
We saw a gastroenterologist (GI), who placed P on the first food he could tolerate: an amino acid formula. He began gaining weight and started to show signs of personality and development, but he still struggled to eat. He still choked on his profuse reflux, he still turned colors, and he still struggled to meet his minimum hydration needs.
We finally got him his first modified barium swallow study (MBSS) which showed oropharyngeal dysphagia and other signs of a laryngeal cleft. He is allergic to the most common thickeners (rice and oatmeal), so we tried GelMix, which he miraculously tolerated. With thickened feed, we saw another little jump in his weight and development, but he continued to struggle with all of his symptoms. His GI and ENT finally agreed to perform a triple scope, and we were told he would "outgrow everything" in a few months.
We were heartbroken and frustrated. Why was our “normal” baby so unwell? Why did every single day revolve around getting him to fall asleep so he could eat and why did feeding take over one hour to maybe complete his bottle? With some findings from the endoscopy regarding his allergies, we were able to move to a speciality team at Texas Children’s Hospital (TCH). His GI/allergy team referred us for a second opinion to TCH’s aerodigestive team.
There, our teams collaborated on P’s care and responded to emergent issues quickly and thoroughly. P had an NG tube placed. In March 2024, we finally received my son’s correct airway diagnosis after a second opinion with the aerodigestive team at TCH in Houston. He had another MBSS study confirming oropharyngeal dysphagia, but this time our SLP also noticed silent aspiration. The SLP was part of the aerodigestive team, so I felt confident in her specific skill set. While I am an SLP, we all have particular areas of competence based on the settings in which we train and work. My skill set is pediatric-focused, but more broad than an SLP who works in a children’s hospital on specialty swallowing teams (e.g., craniofacial, aerodigestive, etc.)
After the swallow study, P had another triple scope that confirmed his type 1 laryngeal cleft, laryngomalacia, and mild tracheomalacia. He also had a G-tube placed. His ENT, Dr. Elton Lambert, injected filler in hopes it would help him at least temporarily. The filler was almost immediately successful. It was incredible to see the eating and drinking my son was capable of when his anatomy worked properly!
He had another swallow study to confirm the injection worked. It did! P still showed some signs of oral discoordination, since he never learned to swallow properly, so we continued feeding therapy. As anticipated, the filler dissolved by six weeks and his dysphagia symptoms immediately returned. Dr. Lambert spoke with us about P’s progress and response to the filler. We decided a formal stitch repair would benefit our son.
In May 2024, P had his formal stitch repair completed. Dr. Lambert placed two stitches to close the cleft and performed a supraglottoplasty on his left aryepiglottic fold. P recovered well from the surgery and even requested water (thickened, of course) two hours after waking up! Two months later, P successfully weaned off thickener to thin liquids! He is still learning to use various cups and straws safely, and that will take time. His swallow is brand new to him, so we are focused on giving him time to learn. We are so happy he can splash in the bath with his brother and water refills simply entail placing his cup under the faucet!
My hope with sharing our story is to encourage families to advocate for their babies! Just because a provider is educated and wonderful, does not mean they are educated on this rare disorder. Your provider(s) and you are a team! You each bring crucial information to the table.
I also hope to raise awareness amongst the variety of providers on the front lines of treating children who may have a laryngeal cleft. To the pediatricians, lactation consultants, speech pathologists, occupational therapists, ENTs, and GIs: it is a gift to the child and their family to recognize when a referral or second opinion would be helpful. We ALL have the same goal - a healthy, happy, developing child! I am forever thankful for TCH, our local pediatrician, and our local therapy team.