Theo's Laryngeal Cleft Story

Idaho, USA  |  October 2024

By Carolyn Schultz

Theo’s laryngeal cleft journey spanned over 20 years. During that time, there were many struggles, but also angels that helped us along the way.

Theo was born in 2004 in Montana. Immediately after birth, he was unable to cry and started to turn blue. The nurses suctioned his airway and he recovered quickly. We didn’t think too much of it, but looking back, it was the first sign of his type 3B laryngeal cleft.

From the start, Theo could not really eat. After a couple of tiny swallows, he would cough and stop. I sent him to the hospital nursery for a few hours while I napped, and later learned the nurses asked for him to be evaluated by a pediatrician because of feeding difficulties. The doctor looked him over, and declared him fine. We took him home, and that’s when I realized something was very wrong. My son could not swallow without coughing and was barely eating. It seemed so odd, and I was scared and confused.

Within days of his birth, I took Theo to the pediatrician. I asked if I could feed him so the doctor could see what was happening. The doctor declined and then reassured me that all was well. I took Theo home, but was back at the pediatrician’s office within days. This time, he told me, “I see no red flags. Don’t bring him back for at least two weeks.” I cried the whole drive home, knowing deep inside that something was very wrong. 

Our first angel appeared at this time. She was a speech therapist treating my older son. She came to our house and watched as I tried to feed Theo. Alarmed and perplexed, she showed me how to use baby cereal flakes to thicken milk to a pudding consistency. I poured the sludge into a bottle, and used a knife to cut slits in the nipple so Theo could suck the mixture out. It wasn’t perfect, but he could finally control the “milk” enough to swallow better. He still coughed and choked, but for the first time in his life, it wasn’t constant.

I then brought Theo to the only other pediatric practice in town for a second opinion and this doctor took the problem seriously. He ordered a swallow study, which immediately showed aspiration. However, I was assured by the well-intentioned medical team that it wasn’t an anatomical problem. They advised it was likely an immature swallow that would hopefully get better within the next two months. So we waited.

While we waited, Theo developed serious respiratory issues. When he was two months old, he came down with croup very quickly one night after I put him to bed. I heard a weird sound from the baby monitor and went to check on him. He was struggling to breathe and retracting so significantly that he couldn’t cry. I scooped him up and drove 20 minutes to our local hospital. When we arrived Theo’s oxygen was only 72 percent. They ushered me out while a team worked hard to stabilize him. They told me later that if I had waited even 15 more minutes, they may not have been able to save him. (Theo struggled with serious croup and pneumonia repeatedly for his first several years and more mildly until he was around 7 years old.)

After two months and no improvement, we were sent to a pulmonologist. He conducted a flexible scope and said Theo’s anatomy looked unusual. He suspected a laryngeal cleft and referred us to a regional children’s hospital many hours away. An ENT from that hospital conducted a direct laryngoscopy and bronchoscopy and diagnosed Theo with a type 2 laryngeal cleft. 

While at that hospital, Theo was also diagnosed with significant reflux, which he was aspirating. His lungs were becoming compromised. He underwent a Nissen fundoplication to stop the reflux and also had a G-tube placed. These surgeries helped stabilize Theo and allowed us to finally give him water through the G-tube. Since he was now stable, we decided to return home, wait for him to grow, and hopefully find a surgeon with endoscopic cleft repair experience. This waiting period eventually ended with the discovery of our next angel - an ENT experienced with endoscopic laryngeal cleft repair in Massachusetts.

I found this ENT through an internet search when Theo was two. At that time, the doctor had performed about 30 endoscopic cleft repairs with a high success rate - far more than any other ENT we could find. Though it was very taxing both financially and logistically to get Theo to Massachusetts, we made it happen with extended family support. This ENT found that Theo actually had a type 3B laryngeal cleft. He wasn’t sure if he would be able to close it endoscopically, but was willing to try. We very much wanted to avoid an open surgery and its many risks and complications, so we were happy to have found an ENT with endoscopic repair experience.

Theo had his first endoscopic repair in 2007 when he was 3 years old. At that time, it was the deepest cleft in the world to be fixed endoscopically. Unfortunately, Theo screamed for several hours in recovery, and I have wondered if all of that screaming may have damaged the repair. For the first few days, it was like a miracle had occurred. As we were leaving the hospital, I was distracted and Theo grabbed my water bottle. He drank the water effortlessly, with no choking or coughing. We couldn’t believe it! After a few days however, some coughing slowly came back, but to a lesser degree. In the end, Theo was much improved, but not able to drink effortlessly like he had the first day after surgery.

So we returned to Massachusetts again in 2008. The ENT found that while most of the repair was well healed, the top was open. So he again fixed it endoscopically, and that was the start of many years of stability for Theo. His G-tube was removed at the age of 4, and he was able to eat and drink on his own. Because of this doctor, Theo had a relatively normal childhood from the age of four until his late teens. We are very grateful for that time, and the wonderful care this doctor has provided for nearly Theo’s entire life.

In 2019, when Theo was 15, we started to notice some coughing and choking again. It crept up gradually and we weren’t really aware of it at first. With him now in high school, I started thinking it might be a good idea to have him scoped again before college. Then covid hit and our scope in 2020 was canceled.

Theo graduated from high school in 2022, and that summer we returned to Massachusetts. The same ENT found that the top of the repair had opened up yet again, so he fixed it endoscopically for the third time. This time, he warned us that he would only be able to repair the cleft one or two more times endoscopically. We returned home and Theo isolated for six weeks before college started. He was able to drink with no coughing or choking and we were thrilled and relieved.

Six weeks after the third endoscopic repair, we dropped Theo off at college for his freshman year. Two days later, he called with a 104 fever and covid. He came back home and missed his entire first week of classes. Unsurprisingly, there was a lot of coughing. He returned to college, and a couple of weeks later told us the coughing and choking was back.

So in May of 2023, we headed back to Massachusetts for what was supposed to be Theo’s fourth endoscopic repair. However, this time the ENT discovered a tracheoesophageal fistula had formed in the lower half of the healed repair. This meant an endoscopic repair was no longer possible. Theo was now facing an open repair, which we had tried so hard to avoid for 19 years. I was devastated.

The doctor performed the nine-hour open surgery in August of 2023. Theo was on ECMO (a heart-lung bypass machine) for four hours in order to allow surgeons to safely work in his airway. He was given a tracheostomy during the surgery, and had another G-tube implanted in Idaho six weeks prior.

The surgery went as expected, but we knew there were no guarantees the repair would heal correctly. After surgery, Theo was placed on a ventilator in a medically-induced coma for four days in order to keep him still and help with healing. It was an extremely difficult time. Coming off the vent with a new trach tube was brutal. After eight days, Theo was stable enough to be moved from the ICU to a complex surgical recovery unit.

Theo was hospitalized for a total of 18 days, and then we stayed for several more weeks in nearby hospital housing. It was a tough time. Every day, he had to change his trach ties, and though we received training in the hospital, Theo struggled to change them without causing a horrible round of coughing and retching. This was terrifying, because we knew coughing and retching could damage the repair yet again. I was desperate to find a solution, and then our next angel appeared.

This time it was another laryngeal cleft parent who had heard about Theo’s surgery and reached out through Facebook. Her own son was born with a type 4 cleft and had lived with a trach for over eight years. She called and advised us of some changes to make in the tie-changing process, and it made all the difference. The coughing and retching stopped. We are forever grateful to her for reaching out and helping during such a crucial time.

After five weeks in Massachusetts, we returned home to Idaho and spent the next eight months in isolation. We felt it was necessary to allow Theo’s repair the best chance to heal without the risk of him getting sick, coughing, and damaging it yet again. After three endoscopic repairs, an aborted fourth, and an incredibly invasive open repair, we weren’t willing to take any more chances. During his isolation, Theo took the year off from college, lived at home, and took some online classes.

There were many ups and downs in the eight months after his open repair, but I’m incredibly grateful to end Theo’s story on a very positive note. We returned to Massachusetts in April 2024 for another rigid scope. We were beyond thrilled to hear the repair looked perfect, and Theo’s trach and G-tube were removed. During the open surgery, the ENT had been able to fully close the cleft, leaving no residual cleft as is often the case with open repairs. With the repair now fully healed, Theo is very lucky to be able to eat and drink normally and we don’t anticipate any future repairs will be needed.

Theo is now looking forward to a summer job as a cabin counselor at a camp for children with medical conditions. This fall, he will return to college, where he is studying microbiology. Theo now has an incredibly bright future, and we are so thankful to everyone who supported us through this journey.