A short (haǃ) recap of Timmy’s first 15 ½ weeks…
Timmy was born 7 weeks early on September 29, 3lbs 9oz, 16.5 inches long. For the first several days of his life we thought his problems were normal preemie issues. He was transferred to Children’s Hospital in Columbus when he was four days old for further testing. Over the next week or so we learned that T was born with a number of birth defects, ranging from extremely minor (missing a set of ribs and having an extra vein into his heart) to severe. The biggie was a Type 3 laryngeal cleft. This means that a portion of the wall that should separate his trachea and esophagus was missing. Obviously this is a pretty big problem—much of the milk that he had been fed for his first several days of life (through a feeding tube) had ended up in his lungs, causing pneumonia. The first several days of Timmy’s life when he was “just a preemie” were extremely difficult for us, but then being told that our tiny three and a half pound baby needed major surgery in order to be able to live was heartbreaking. The next several weeks of adjusting to life in the NICU were scary—saying good-bye to Timmy as he went to the OR (for a scope under anesthesia) for the first time, watching him struggle to breathe, learning to be comfortable with cords and tubes, watching him drop down to 3lbs, 5oz, and seeing plans for his first surgery canceled because he was too tiny.
Finally on October 26 Timmy went down to the OR for major airway reconstruction. At that point we were nervous but so relieved that things would finally be “fixed”. We thought he’d have his surgery, everything would heal up, and we’d be teaching him to eat a month later. The surgery went great—the next week was pretty rough (he spent his first month birthday paralyzed to keep him from moving and damaging the repair), but we handled it pretty well, knowing that it was what he needed to live a normal life. The surgeon had given us approximate 70% odds of complete success. Though we knew that Timmy’s condition was extremely rare, we were fairly confident that everything would go well, especially after a bronchoscopy one week post-surgery confirmed that everything was healing perfectly. Unfortunately a week or so after that we got the news that the cleft repair had started to pull apart. A week or so after that we got the news that there was very little left of the original repair. And so we were back to square one.
On November 9 Timmy went down to radiology to have a fairly routine procedure done. He’d been taking all his nutrition via IV (TPN) for his first six weeks of life, and he needed to have a j-tube threaded through his existing g-tube (placed in his stomach at the same time as his airway surgery) so that he’d be able to take milk straight into his intestines. A couple of days later, Timmy started showing signs of extreme distress. We discovered on November 12 that the j-tube had perforated his intestines, causing a life-threatening infection. Because Timmy was too critical to be moved downstairs to the OR, the entire surgical team (and all their equipment) came up to the NICU and performed emergency surgery to try to repair his intestines. They were unable to fix the perforation, so they put a tube in to patch up the hole. At the same time, because of concerns about repeated intubation and extubation damaging what was left of the cleft repair, ENT came up to the NICU and performed a tracheostomy. That night, sitting in the NICU waiting room, we were faced with the very real possibility that Timmy wouldn’t survive the procedure. But he’s a little fighter, and pulled through. The next few days were scary—he had something like 9 different IV pumps and was on the ventilator again (though the first thing we learned to love about the trach was that we could finally see his whole face for the first time!).
A few weeks later, once Timmy was a bit more stable, we started to learn how to do all his trach care. It was incredibly intimidating at first-the first time we thought his trach had come out, my heart stopped and I started shaking all over. But gradually we learned to be more comfortable with it, and at the beginning of December they started talking about discharge in a few weeks. At that point we knew that there was nothing left of the original repair and that Timmy would need another airway reconstruction in 2-6 months time, along with a second abdominal surgery to repair the perforation in his intestines at some point down the road. Unfortunately, though, he took a turn for the worse, and due to a number of factors, we decided to have him transferred to Cincinnati Children’s Hospital on December 17 to see what the doctors there could do for him.
People from all over the country bring their kids with laryngeal clefts to Cincinnati. We were amazed when we first got here—we went from having a child with a condition that few of our doctors had ever seen to having a child with a condition that was almost routine! A few days after arriving in Cincinnati, ENT, pulmonary, and GI each examined Timmy down in the OR and our world was turned upside down once again. Before Timmy’s scope, Mark and I had been doing all his trach care. We were completely comfortable getting him out of bed, and we were learning to treat him (somewhat) like a “normal” baby. After the scope his airway was designated as critical—we were no longer allowed to do his trach care or even hold him. They wanted to schedule a second repair attempt ASAP, and they diagnosed him with severe laryngo/tracheo/bronchomalacia (airway floppiness).
December 28 was his second big airway surgery. This time around they used a rib graft to reinforce the repair. Now, two and a half weeks out from that surgery, things are still looking good. Yesterday they took him down to the OR for yet another bronchoscopy (his eighth). An A+ would have been a repair that was completely intact that had also fully mucosalized (essentially meaning integrating with the tracheal/esophageal wall). Though the repair still hasn’t fully mucosalized, a good portion of it has, and there aren’t any holes, which is great news! We’re finding that we’re having a hard time celebrating, though—I think that’s because we’ve dealt with so many ups and downs over the last several months that we’ve adopted a kind of “we’ll believe it when we see it” mentality.
So, as the situation currently stands, Timmy will have his trach for at least a few years (we’re not really sure how long). When it comes time for decannulation (removal of the trach) he may need to have a second airway reconstruction. He’ll likely come home on a ventilator, but we’re hoping that he won’t need that for longer than several months. He has a G-tube (feeding tube into his stomach) which isn’t currently being used for anything except a drain. He also has a surgically placed J-tube (the tube that was used to plug the perforation in his intestines) which we’re using to feed him. He’s getting over his most recent bout of tracheitis—this time around it’s pseudomonas and serratia (in the past he’s also had staph infections in his trach).
Our plans for T are fairly up in the air right now, but tentatively it looks like another abdominal surgery (to remove that J-tube and patch up the perforation) in 3-7 (ish) weeks. A few weeks after that, we might be able to take him home! In the meantime we’re waiting for the graft in his airway to fully mucosalize. Once that happens, T will do a swallow study so that they can make sure it’s safe for him to eat. If that goes well, we might get starting to teach him to eat! From what we understand, the learning to eat may not be an easy process, since we’ve skipped the period when babies usually learn to coordinate sucking, swallowing, and breathing. But at least getting to put stuff besides his pacifier in his mouth will be a huge milestone! Once he goes several months being able to take all his feeds orally, we’ll hopefully get to pull the tube in his stomach.