Charlie's Laryngeal Cleft Story

Pennysylvania, USA  |  March 2025

By Rachel

When our fourth child was born, we noticed almost immediately that he was extremely uncomfortable whenever we fed him. It seemed to be reflux related, as the symptoms really lined up - coughing and choking, arching his back, fussiness, grimacing, and crying whenever he wasn't being held in an upright position. 

When Charlie ate, we realized he was gulping and swallowing a lot of air. He also had a bad latch due to a recessed lower jaw. After about two weeks, we took him to the pediatrician for the reflux and also had a lactation consultant appointment. The lactation consultant seemed to think something anatomical was causing the issues and referred us to an ENT. 

Our little baby was six weeks old by the time we sat at our first ENT appointment, and we were exhausted. Charlie seemed to require being fed nearly constantly, but was also very fussy and upset whenever he was fed. We thought that we finally had all of the answers to our questions when the ENT explained that the crying while placed on his back was caused by tongue obstruction in his airway due to the recessed jaw. The ENT advised us to place him on his belly at all times, never on his back. The fussiness during feeding was also likely due to reflux and the recessed smaller lower jaw, so we were introduced to specialty bottles to help him drink easier. 

We got settled with appointments for a sleep study as well as meeting with a speech language pathologist for feeding therapy. We had hoped with reflux medication and feeding therapy, as well as natural catch up growth of his lower jaw, that his sleep and fussiness with feeding would improve. But Charlie continued to have very poor sleep with ongoing fussiness during and after feeding. We tried a couple of different bottles, and eventually he had his first modified barium swallow study (MBSS). The test showed that at 4 months old, he was having penetration on bottle nipples that flowed faster than a preemie nipple. At that age, he should have been able to handle the faster flow. Thankfully, he was gaining weight appropriately and meeting all of his gross motor milestones. We decided this was just something unique to him and if it worked, then that was fine. 

Over the next several months, sleep and feeding continued to be a struggle, but there never seemed to be any answers for why. It was frustrating knowing something wasn't right, but we didn't know what.  He had a total of three sleep studies, all showing only mild sleep apnea. Charlie had a chest x-ray showing findings indicative of viral or reactive lower airway disease, and a night time cough that would come and go, sometimes seeming to go hand in hand with a bad choking episode during a feed. We tried a different reflux medication and trialled asthma medications. We thought it was under control until an in-office scope during an ENT appointment showed severe cobblestoning and irritation in his esophagus. Puzzled, we decided to pursue the reason for the irritation to make sure it truly was or wasn't reflux related. We didn't want to continue reflux and asthma medication if there wasn't a medical need for it. 

Charlie had a two-hour barium milk study as well as a 24-hour PH probe test, both of which showed only mild and subtle episodes of reflux. At this point we truly didn't know what else to do to help our boy. We stopped the reflux medications and decided that there was nothing left to be done. At a follow up ENT appointment, after expressing that he was still struggling with symptoms related to sleeping and feeding, the ENT felt it would be best to discuss his case with the aerodigestive clinic and let us know what they felt would be best moving forward. 

A couple weeks later, we got a phone call. The projected plan was to complete a triple scope to see if they could get to the bottom of what was going on. This would require going under general anesthesia, which at the time seemed scary and not necessary, given that he was still gaining weight and overall a happy kid. We actually declined the triple scope at that time. But during the next few months, our little one endured several ear infections as well as adenoids that were so swollen they were blocking his airway. The decision was made, since he would be going under anesthesia anyway for adenoidectomy and ear tubes, to do the scope while everything else was being done all in one shot. I'm grateful for those ear infections, because although it was horrible for him to endure, it was the catalyst to making the decision to go ahead and do the triple scope after all.  

When the big day came, we were nervous but hopeful. Partway through the procedure, our ENT came out to tell us how that portion of the scope had gone. Everything was normal except for a deep interarytenoid notch, or a level one laryngeal cleft, which would put him at higher risk for aspiration. I had never heard of this, but a quick internet search in the waiting room made me realize it could be completely unrelated to his symptoms, or it could be the culprit. As time moved forward after the scope, it became clear that it was absolutely related at least in some ways.

We transitioned Charlie off bottles to sippy cups and immediately noticed that it didn't seem to matter what cup we used as he would frequently choke and cough while he drank. Sometimes it was mild, and other times seemed scary when it would take his breath away, his eyes would water and turn red, and he would stop drinking from his cup.

Every time I would hear him choke, it was like my whole being would cringe. We worked with feeding therapy to try narrow straws and thickened fluids to see if it helped, and it definitely did show improvement in his symptoms. We thought perhaps, because he had a good cough response when these episodes happened, he was clearing his airway properly and his body was doing what it needed to to keep liquid from going down into his lungs. But months went by, and he was still intermittently choking even with using straws. I began to want reassurance that aspiration wasn't occurring.  

After connecting with other laryngeal cleft families, we found out that aspiration is not always caught on a swallow study, and chronic irritation can occur when there is constant coughing.  Our SLP even explained that sometimes the body begins to lose some of the cough reflex over time due to the constant irritation and choking.  We were reluctant because of the radiation exposure but decided to pursue another updated swallow study, as it had been over a year and cups were different from bottles. The swallow study showed that he was having discoordination during his swallow and penetration with open cup swallowing, so we are still using straw cups and working with a SLP on feeding therapy. 

As of right now in our journey, Charlie’s cleft is unrepaired and we have no date on the table to schedule a repair. Because he hasn't had aspiration pneumonia or failure to thrive, we haven't felt an emergent need to get a repair done. He does still have issues with chronic cough and snoring, and a recent chest x-ray showed that he's still got some diffuse peribronchial thickening going on. It's frustrating because viral infection can also cause the same chest x-ray findings, not just chronic aspiration. 

It's difficult to know what the best decision is, and we are still stuck in the "wait and see" approach. Charlie is already three years old and still struggles at times. We do have a plan in place to see an ENT that is experienced with laryngeal cleft repair and of course continue to follow up with pulmonology to make sure his lungs are healthy. As of right now, Charlie’s cleft story does not have an end, but a "to be continued." We know we will do everything possible to help our little boy thrive and succeed, and he has the most supportive family and team surrounding him.