Roland’s Type 2 Cleft Journey
By Emily Hahn
I can remember the exact moment my son's medical journey began. I was sitting in my car, stuck in morning commuter traffic, 27 weeks pregnant, and I felt a distinct contraction. These preterm contractions brought about a flurry of tests that morning and I was diagnosed with polyhydramnios (excess amniotic fluid). Because no apparent structural issue was seen on his anatomy ultrasound, it was tagged “idiopathic” (a random occurrence) and I proceeded on through the rest of what was a difficult pregnancy. I knew then that my son had something that wasn't quite right, but I had no idea what it was or how to figure it out.
When he was born, Roland was a vigorous and healthy baby. He loved to eat and gained weight beautifully. He did spit up a lot, but was a “happy spitter,” so we didn't think much of it. He coughed frequently every day, particularly when lying down.
A very distinct memory is the first time I gave him solid foods, a vegetable puree. He was excited beforehand, but became so upset, gagging when he went to swallow. For several months, Roland wouldn't eat purees of typical baby food, only dry rice rusks.
When Roland was 8 months old, he had his first episode of shortness of breath. He was crawling on the living room floor and became visibly short of breath with retractions. I took him to the emergency department and since he coughed a few times, they said it was probably croup, gave him steroids and discharged him. He ended up doing fine.
However, several months later, he got RSV for the first time. He was breathing very fast and after a long night, the pediatrician instructed me to bring him to the emergency department. We were transferred to another hospital with pediatric services and he was able to be discharged the next day. But he was still short of breath, and every time he became sick with a cold that winter, he would get short of breath and have retractions.
This was the start of a long winter of coughing. In March, he got very short of breath and lethargic. Roland had RSV again and spent the night at the hospital for fluids and monitoring. This was the first time I remember that things didn't add up. The resident doctor reviewed his chart and chest x-ray before he came in the room and quickly said he thought my son could be discharged. He then took a closer look at Roland and realized he looked much worse than he imagined and was not ready to leave the hospital.
Roland was scheduled for ear tubes three weeks later and I called the pre-op nurse to ensure that his recent illness would not be an issue. She assured me it would be fine. Again, I clearly remember the cough from my son in pre-op. One of those wet, harsh, heavy coughs that could be heard across the room. Anesthesia heard it and refused to do surgery that day. I didn't realize at the time that this was another signpost on the journey. Recovery from RSV wasn’t expected to last that long in a “regular” child. Something wasn’t right.
While it probably delayed his diagnosis, we got a reprieve with the COVID-19 pandemic. My son didn't return to daycare and, exposed to so few germs, he wasn't sick for over a year. His cough cleared up and was rarely heard. He would cough occasionally with meals, but did well.
But then came the first time he had COVID. At age three, I thought he was out of the age range to get croup, but he awoke from a nap with such hoarseness and stridor, declaring he “couldn't get enough fresh air.” I recall defending his cough afterwards to his preschool, noting, “He just gets a cough every time he has a cold. He's fine to return to school.” It was hard because these were the years that every cough was suspect for a fatal illness. Between him and his younger brother, the episodes of croup were numerous. He would awaken with stridor and coughing and distress and we would bundle up to go sit outside in the cool night air until things settled down.
In this timeframe, Roland started to bark. The cough with meals and with excitement and exercise took on a loud harsh distinctive pitch, the sound of my son. Still, he had great exercise tolerance and was growing well. I began to wonder about his cough, reading about kids who had “triple scopes” and other such procedures, but his cough waxed and waned and I never pursued anything further. I began to say that he was just “plumbed wrong.”
The year he started kindergarten, we moved and began seeing a new ENT for chronic ear issues. Roland had an awful winter. He coughed for months straight with a harsh and unrelenting cough. When the school nurse asked yet again whether he had asthma and I realized time alone was not enough to resolve this cough, I finally took him to the pediatrician. Steroids did nothing to help the cough. Azithromycin cleared what we thought may have been a “walking pneumonia.”
I asked the pediatrician if he thought this could be from his enlarged adenoids and he said, “Ask Dr. Lando,” his ENT. So I did just that at a scheduled follow-up a few weeks later. I hadn't realized her interest in airways and swallowing, but we left the office with a plan for Roland to have an airway evaluation when he had his adenoids removed. It certainly helped that he had one of those big bark coughs during the appointment, though Dr. Lando told me she had a low suspicion she would find anything.
We had a very challenging appointment with an allergist during this waiting period. The allergist insisted that he had asthma as I responded affirmatively that he always coughed when he got sick. Despite my questioning of this, the allergist was so rote in his response to me. He insisted he was correct, so unwilling to consider other causes of this issue.
My son went on two months later to have his adenoids removed. The look on Dr. Lando’s face when she met us in post-op is another thing I will never forget. She beamed as she declared: “I found what it is and I know how to fix it!” She explained that Roland had a type 2 laryngeal cleft. The next few minutes were another flurry of discussion and rehashing his life, with me repeating several times that he fed very well and did not have recurrent pneumonia.
The following days became a flood of reframing my son's entire life through a new lens, one that finally began to make more sense. I also had a great sense of relief, realizing that I had been making excuses to cover for his cough for years. Further, I had a fair sense of guilt, wondering how I was never able to get this figured out before. Even working in the medical field, I didn't know who to talk to or how to ask. I often downplayed his symptoms in my mind, not wanting to risk sounding over-concerned or sensationalizing his symptoms.
There are so many things I wonder about the journey that are unlikely to be explained and certainly never proven. Was the extra fluid in my pregnancy from my son's airway and swallowing abnormality? Was his initial disdain for pureed baby foods a sign someone should have recognized? Or why didn’t he struggle more with breastfeeding, when many children with smaller clefts can't feed at all without aspirating?
A few weeks before his 7th birthday, Roland had an endoscopic repair of a type 2 laryngeal cleft performed by Dr. Tali Lando at Westchester Medical Center in Valhalla, New York. He did beautifully without any anesthesia complications. We stayed over one night only due to our distance from the hospital. It took him a week or two to get used to his new swallow, eating slowly at first.
He is doing great now. The cough is gone. If he does cough, it is still louder than your average child, but continues to improve. Initially, I thought the diagnosis was the gift, but the repair of the laryngeal cleft is the true blessing. The surgery caused him very little pain and distress. We will still need to see how he does with future illness, but the improvement so far is amazing. Some days I even find myself missing his characteristic cough.