The Incredible Highs & Rock Bottom Lows: Part I

As many of you know, Gavin had surgery on July 14th. However, only few of you know the big picture leading up to this. Which is no fault of your own. This was a year in the making, and then, suddenly, a very urgent situation. A situation that shook me. I am very aware that my threshold for “being shook” is abnormally high, but this one, like others in the past, had lifelong implications that I believed were behind us. So let me back up and explain before jumping in.

Nearly three years ago to the day, July 18, 2019, Gavin underwent a spinal fusion with growing rods surgery. The spinal fusion starts at the base of his skull and goes to T-2. The fusion refers to vertebrae that are fused together without the ability to grow or move from that day forward. From T-3 to T-9, the growing rods were placed to allow Gavin to continue to grow, where possible. The growing rods provide the ability for his lungs to grow; lungs need the length of the body more than the girth of the rib cage in order to grow to adult size. Gavin had just turned 3 at the time of the spinal fusion surgery – which means that his lungs would have stayed the size of a 3-year-old for his entire life if the growing rods were not placed. The other key component of the growing rods is the support they give to the rest of the spine and fusion. The rods allow his spine to grow straight rather than curve or twist.  The decision we made that day was not one we took lightly or without much hesitation. We knew that we would be impacting Gavin’s life in a very permanent way.

The growing rods also meant that Gavin would need a surgery twice a year to grow. Every 6 months, Gavin would go under general anesthesia for about 45 minutes while Dr. Cahill opened up Gavin’s back to adjust a mechanism for Gavin to grow a few millimeters. (Side note: there are several types of growing rods used in modern medicine. Some are adjusted with surgery, and some are adjusted much less invasively. The type of growing rod used depends on the patient and the surgeon.) Since July 2019, Gavin had the “routine growing rod” surgery performed four times. He was scheduled for the 5th procedure on April 28, 2022. That day things started to go sideways. That day in the OR, the anesthesia team could not intubate Gavin. They performed the same techniques and approaches that had worked in the past, but something just wasn’t working. They tried for over an hour to intubate him before deciding the risk of damaging his airway was too high and they needed to cancel the procedure. (Gavin remained safe and stable the entire time.) This presented a new set of challenges. Without the ability to intubate, Gavin could not go under general anesthesia and would not be a candidate for any significant procedures, including the expansion of the growing rods. Ultimately, this meant Gavin would not grow and his lungs would not fully develop. 

So we quickly went back to the drawing board. We met with Gavin’s ENT, Dr. Javia, on May 6th, to examine options to keep Gavin’s airway safe. We spoke about the simple mechanics of the jaw and how it could dramatically impact the ability to intubate. We discussed the option of a tracheostomy: an artificial airway. And, how due to Gavin’s unique anatomy, even a tracheostomy would not be a straight forward answer. We considered the idea that in an emergent situation, Gavin could not be intubated. It was a somber and serious conversation that left us with bleak outcomes. I’m not sure if it was an educated guess or shear desperation to control the only thing that seemed controllable. But we returned to the basic mechanics of the jaw. Dr. Javia encouraged us to consult with Plastic Surgery at CHOP because they are the department that oversees the TMJ (the jaw joint) clinic. Through some research, we found that there is a device, called TheraBite, that can stretch and strengthen the jaw. The plastic surgery consult was the only way to see if Gavin was a candidate for this device.

Less than two weeks after the cancelled procedure, on May 9th, we met with Dr. Swanson, the plastic surgeon. He reviewed a recent CT and discovered that Gavin’s jaw worked just fine. The mechanics were great, however, he just doesn’t open his mouth very wide. Due to Gavin’s genetic condition, Otopalatodigital (OPD) Syndrome, he has a small jaw. This disorder is also the reason why Gavin’s joints are very tight and limits the opening of his jaw. This unique combination is far less than ideal for the intubation process. Dr. Swanson measured how far Gavin could open his mouth: 15mm between his top and bottom teeth. Which is equivalent to about the width of one finger. Typically, there should be three fingers between the top and bottom teeth for a smooth intubation. Dr. Swanson explained the TheraBite technique to us: place the device between the teeth, squeeze the device in order to open Gavin’s mouth, hold for 7 seconds, repeat for 7 stretches – do this as often as tolerated. The only other option was less than ideal – but could be done in the OR prior to intubation, and included the use of force to open Gavin’s jaw.  

So this was our only shot, the TheraBite. Because the need isn’t high, CHOP did not keep a stock of them. I checked Amazon: $600-$700 for the entire kit. I sent an email to Gavin’s PT and OT at Schreiber Pediatrics in Lancaster. They had never heard of such a thing, but they helped me find the company who makes them. I called the company, Atos Medical. Spoke to Vicki (who had a lovely Midwest accent that always makes my heart smile) for about an hour to share this story. For $445 + overnight shipping, we had the TheraBite kit within two days of meeting Dr. Swanson. I figured out how to adjust the settings and we started at the lowest/easiest setting. A rule of thumb I’ve learned with Gavin is “start low and go slow”. This way he tolerates the therapy or medication or whatever it may be much better with much more success. 

Mean while, Dr. Cahill and the orthopedic team went back to the drawing board. They knew that a surgery that required intubation and general anesthesia every 6 months was not sustainable for Gavin. Just like with the first spinal fusion and growing rods surgery, I told Dr. Cahill to discuss Gavin’s case with his colleagues across the nation to find what the best option would be. I believe there were 2-3 viable options on the table. But ultimately, we decided on removing the growing rods all together. Because of Gavin’s enormous success and completely unexpected strength from the original surgery, they were confident that he would be able to handle the instability that removing the growing rods could potentially cause. Once the decision was made, the team had to align all the stars, moons, and planets to get three very busy physicians in the OR at the same time: Dr. Cahill (orthopedic surgeon), Dr. Javia (ENT), and Dr. Ward (anesthesia). Having Dr. Javia and Dr. Ward in the room would not guarantee a successful intubation, but they were definitely bringing in the big guns, if you catch my drift. August 9th was the only day available – and it was a solid 3 months away. So we had three months to use the TheraBite to open Gavin’s jaw… and three months of anticipation and anxiety. 

Fast forward to July. We leave for vacation to see family in Minnesota. The last time we were scheduled for this vacation was also three years ago. I was packing for our Minnesota trip when I got the call that we would be heading to CHOP for the start of this journey. This trip to Minnesota was wonderful. We loved seeing (and actually hugging) our family! Gavin got to play with his cousins and even made a couple new friends. Our family & friends got to really meet this awesome child of ours and the huge personality that he hides so well – it nearly brought me to tears several times a day. Minnesota will always be the place I call home, so my time spent there is always so good for my heart and soul.

Towards the end of the trip, I took notice to a spot on Gavin’s back that has been concerning for well over a year. At the bottom of Gavin’s growing rods, there has always been a piece of hardware that doesn’t lay as flat as the others. We have been monitoring it and protecting it with special foam and bandages. Sometimes it would get red and swollen. Other times it looked like a really bad scar. There were a few times over the last three years that Gavin was put on antibiotics because we thought it might be infected. Nothing too serious until now. The team’s plan of action was always to wait and intervene only when absolutely necessary. 

We left Minnesota on Saturday, July 9th for our drive back to PA. There was a bandage on Gavin’s back to protect “the spot”. At our first stop I decided to take a peek at Gavin’s back just to double check it. It was not good – definitely looked red and swollen. I cleaned the site, took a picture, changed the bandage/dressing, added more padding and used some antibiotic ointment. Once we were on the road, I started making phone calls and sending emails (with the picture) to alert the right people that we might need to start oral antibiotics. Once we reached our half way point for the night, we checked it again. Wasn’t any better, in fact, slightly worse. We performed the same “clean the site and re-dress the wound” steps from earlier in the day and tried to get some sleep. Sunday morning we removed the dressing to find that the hardware underneath was now exposed. We could actually see the metal in Gavin’s back. So we again performed the same “wound care” plus a little extra padding and started making more phone calls and sending more emails. 

Within a few hours of arriving back home, there was a plan for the week. Gavin started antibiotics immediately, we would be seen in clinic on Wednesday, and surgery on Thursday. This meant the surgery was happening 4 weeks early and not all of the planned physicians would be available. We trust Dr. Cahill and knew this was the best option, as the risk of infection was too high, but the decision was not easy. With less time to stretch Gavin’s jaw and without the chosen team in the OR, the risk of not being able to intubate, again, was much higher. I decided to adjust the TheraBite to the next setting, which would stretch Gavin’s mouth open even wider. I wasn’t sure if only three days of this setting would be enough, but I knew that I had to try.

After more wound dressings and TheraBite stretches on Monday and Tuesday, we packed up the van and headed to Philadelphia on Wednesday morning. We met with Dr. Cahill and his team to discuss the plan and possible scenarios for the next day. The best outcome was simple: intubation goes smoothly, removal of rods, and limited signs of infection. One of the worse cases was another failed intubation attempt. If it did not work, the likelihood of it working at all was diminished. This would mean chronic/frequent wound care and long course antibiotics, likely hurting Gavin’s quality of life. It would also make future growth not possible, so Gavin’s lungs would not develop any further and remain the size of six-year-old for the rest of his life. This was hard news. We heard for the first time that if intubation did not work, other options were not (yet) known. If intubation didn’t happen, the decision we made three years ago, to give Gavin increased quality of life, had the potential to do the exact opposite.  

To save four hours of round-trip travel and that time with Gavin’s back pressed in his car sear, we decided to get stay close to CHOP after the appointment. This theoretically would have also meant we could sleep-in (a rarity on a surgery morning), but Greg and I could not sleep. The nerves about the day ahead would not allow it. I did the TheraBite stretches with Gav one more time. I used the measuring tool to see if we had made any progress. Gavin’s mouth was now opening between 25 and 30cm. We almost doubled it! Giving us two finger widths, rather than one. Not quite three fingers. My only hope was that it was enough to positively impact the intubation process.

We walked to CHOP for our arrival time. We met with the team. Dr. Cahill came in and chatted with us. The ENT came in: introduced himself – we’d never met him before, and he was brand new to Gavin’s case. He explained that Dr. Javia was on vacation, however, they were able to have a lengthy discussion about Gavin and his needs for today. Then we met with the anesthesia team: again, never met them before and brand new to Gavin’s case. We walked through how they were going to approach intubation. Everyone knew that the success of today was hanging on their shoulders, but no one really wanted to say it out loud. They also reassured us that they had spoken at length with the anesthesia team from April. Their words, “We are standing on the shoulders of giants. The information they provided to us is invaluable.” I knew that everyone was as prepared as they could be. I just wanted to get this show on the road. The anticipation was killing me.

We waited for two hours in pre-op before being informed that we would have to wait for a few more hours. There were no available rooms in the ICU and without one, Gavin’s surgery would be on hold. So, we anxiously waited, all of us growing hungry. They allowed us to give Gavin a very small amount of clear liquids to hold him over. They took Gavin back to the OR around 2:20pm. Just like every time before this one, we walked his bed down the hallway, said our goodbyes, waved the ASL sign for I love you, and gave kisses. As he wheeled through the double doors, my heart just ached, and my stomach was instantly nauseous. 

They promised that they would call us as soon as he was intubated (or not intubated). I knew that the call wouldn’t happen for at least 20 minutes. Highly likely that it would be 30-45 minutes later. We checked into the OR waiting room, gave them our phone numbers to text updates, and explained that we were going to leave to get something to eat. We walked back to the hotel room. Greg was getting pretty hangry at this point and was very focused on what and where we were eating. I remember also being so hungry but so nauseous that I couldn’t even think about food without getting even more nauseous. I told him, “I can’t eat yet. I need to know that he’s intubated first.” 

My phone rang, it was CHOP – this seemed way too early, can’t be a good sign, – false alarm, it was a lady from admissions wanting us to sign paperwork. At 2:48pm, my phone rang again. CHOP. It was Andrea, one of my favorite OR nurses (yes, we know them that well.) She explained that Gavin did awesome and was intubated on the second try! The first “try” was technically just to look and see what they were up against. They decided to use a smaller intubation tube on the second attempt, and it worked without any issue! I tried my best to keep my shit together as I thanked Andrea. But I couldn’t. I sobbed. Hung up the phone. And sobbed. Full on ugly cry, for what seemed like 5 solid minutes, as tears of joy flowed down my cheeks and the weight of the world was lifted off my shoulders. 

Once I gathered myself, Greg and I walked to one of our favorite restaurants, White Dog Cafe. Ordered celebratory beverages and an enormous amount of food. As we ate what felt like the best meal of our lives, we compared the similarities and differences of our roles as Gavin’s parents to other parents. We discussed that we both face tough decisions, try to encourage growth, strength, and bravery in our little ones, find pure joy in watching them experience life, and avoid watching them go through difficult situations with little control of the outcome. The difference is that we don’t face that on a soccer field, or a piano recital, or on a bike, or at a playground. We face them outside the mundane activities of life – in an emergency room, or an operating room, or during a doctor’s visit. Our stress and anxiety has a baseline that is heightened at all times. Our highs are incredibly high. Our lows, incredibly low. We may live somewhere in between for weeks, months or even years at a time. But we always know that those highs and lows are lurking in the shadows waiting for their turn. 

The surgery was an all-around success, best case scenario. We did an overnight in the hospital and came home the next (Friday) evening. Gavin was seen by the Rehabilitation team prior to discharge to determine if he would need a short stay at the rehab hospital for any weakness that removing the rods may have caused. In true Gavin fashion, he exceeded their expectations and revealed his supernatural strength. They suggested a few core strengthening exercises and released him without any restrictions on activity. We were tired, but also enjoying this high. Gavin was put on a 3-month antibiotic course (precautionary, to avoid potential infection of the remaining hardware) and shortly, we all were sleeping soundly in our own beds.