Our family began on September 1, 2012, when Josh and I said our vows outdoors on a hot, humid and beautiful Saturday evening. Our wedding was perfect and we couldn’t wait to start our life together.
Fast forward to a few days after Christmas 2012. On a hunch, I took a pregnancy test and what do ya know, I WAS PREGNANT!!! We were surprised and SO excited!! The pregnancy went well with hardly any morning sickness, and I was feeling pretty good besides being tired. We found out at our 18-week ultrasound that we were having a boy! From the beginning, we knew that his name would be Gabriel Josiah. We also were told that Gabe had mild to moderate ventriculomegaly, which is enlarged brain ventricles. When we heard this news, I immediately had a sinking feeling in my stomach. From the day I found out I was pregnant, I had struggled with constant worrying about this baby and now it seemed like the worst was happening (little did I know, “the worst” was going to get a lot worse). After that appointment, we went in for monthly ultrasounds. At the next two ultrasounds the peri-natologist couldn’t find his stomach, and in June, the doctors informed us that my amniotic fluid level was rising. With no stomach sighting and my polyhydramnios (high amniotic fluid level), there was a good possibility that Gabriel had a tracheoesophageal fistula (TEF) and esophageal atresia (EA). TEF means that the trachea and the esophagus are attached to each other allowing food to pass into the lungs. EA means that the esophagus isn’t attached to the stomach, therefore, no food can reach the stomach. Although we tried to prepare ourselves for the possibility that Gabe would have surgery to correct the TEF and EA soon after he was born, Josh and I still held onto the chance that he would be healthy.
On July 25, at 34 weeks gestation, I was admitted to Willis-Knighton South in Shreveport, LA for pre-term labor, a complication of polyhydramnios. I stayed in the hospital on bedrest for a week and a half. On August 5 at 36 weeks gestation, my doctors decided to go ahead and induce labor because of the rising amniotic fluid levels (they had already performed one amniotic fluid reduction earlier and didn’t want to do another). Gabriel Josiah entered this world on August 5, 2013, at 3:56 pm. He weighed 5 lbs. 11 oz. and was 19 1/2 inches long. Those next few moments were bittersweet. Josh and I both were crying as we got to hold our son for the first time, but then he was taken from us to be examined across the room. I remember lying there asking “Is he ok? Why isn’t he crying? What’s going on?” over and over. No one could answer my questions. After Gabriel had a quick exam, Josh handed him to me for a few minutes before he was rolled up to the NICU for a complete assessment. The next couple hours were torture as we impatiently waited for news. Finally, the neonatologist came to give us the news. Gabe did have a TEF, EA, mild to moderate ventriculomegaly, unilateral choanal atresia (bony blockage of one nostril), persistent left superior vena cava (a normal variant of his heart anatomy that shouldn’t require any surgeries), one undescended testicle, ear abnormalities, and small chin and jaw. Gabe was also intubated due to respiratory distress at birth. With his multiple birth defects, it was obvious that there was some kind of genetic problem. We stayed calm as the doctor gave us our son’s diagnoses, but after he left the room we both broke down. We cried thinking of what Gabriel might have to endure; we cried because he might not be “normal”; and we cried because we were afraid. After that news, Josh went up to see Gabriel, and I followed a couple hours later. I remember when I was rolled into his NICU room for the first time and saw him with a tube down his throat, tape all over his face, and wires everywhere. I reached for his hand and almost immediately he grabbed my finger with a death grip. It was such a special moment for me because I felt like he knew I was there for him.
The next morning Gabe went down for the TEF and EA repair. That surgery went well. On his one-week birthday, the doctors attempted to extubate, but due to significant respiratory distress, Gabe was re-intubated a few hours later. Gabriel’s choanal atresia repair was performed on August 16, and on August 30, he had a jejunostomy tube (feeding tube) placed because he was refluxing and aspirating his OG tube feedings. After these surgeries, he was extubated and placed on vapotherm, a machine that delivers high liter flow of oxygen through a nasal cannula. Although he had awful stridor (loud breathing noises) and retractions, he was able to keep his oxygen saturation up while on vapotherm, so we were happy! We slowly started to see his personality emerge. He was so curious, always wanting to look around. If he wasn’t exploring his surroundings, he was staring at people. Many times while holding him, I would close my eyes for a few minutes and open them to find him staring at my face.
When he was three months old, Gabe lost his PICC line (the central line which allowed him to receive IV nutrition), therefore, no more TPN and lipids. For a month, his doctors tried different calorie and volume combinations to get him to gain weight with just his J-tube feedings, but he actually lost weight. On December 5, Gabriel went down to surgery and had a broviac (central line) placed allowing him to receive continuous TPN and lipids. We were thrilled that Gabe could gain weight now, but since the broviac has a high risk of infection, we would need to learn and be able to do sterile procedures at home to prevent line or bloodstream infections.
Since Gabe was 6 weeks old, Josh and I, Gabe’s grandparents, and his nurses had all been trying to get a smile from Gabe. He was always so interested in faces but would never crack a smile. I got discouraged and said more than once that he would never smile. But on New Years Day he proved me wrong! He finally started smiling! What a happy day!
At the beginning of January, Gabriel had a bronchoscopy and laryngoscopy to determine the exact cause of his stridor and retractions. They found multiple airway anomalies which included anterior airway (making him a very difficult intubation), laryngomalacia (collapsing of the larynx when inhaling), malformed larynx, and possible tracheomalacia (collapsing of the trachea when inhaling). The doctors told us there was no good or right solution to his extensive airway issues. If we were to go home on just vapotherm, there was a significant chance that eventually Gabriel would just give up breathing because it was so difficult for him and he would probably die en route to an adequate ER. His other option would be a tracheostomy but that had a downside too. A tracheostomy, if placed long-term, can result in scar tissue and stenosis later on, and trach patients require 24/7 monitoring and extensive care. When three doctors whom you trust tell you there is no good answer to your son’s problem, it’s devastating. It seems like I cried all day that day, even though it was only a couple hours. It was such a HUGE decision, and I felt at a loss. I think in the back of my mind I knew what we needed to do, but I didn’t want to believe it. I called Josh to tell him the news and it was so hard on him too to hear the severity of Gabriel’s problems. But when I laid out the pros and cons of both options, his answer was almost immediate. Gabe needed the trach. It felt like a weight lifted off my shoulders that we agreed and it really seemed like the only answer. The next day, Gabe went down for a tracheotomy and second choanal atresia repair.
The trach was scary at first. We had to learn what sounds were normal, when his trach needed to be suctioned, what the hiccups sounded like (haha, sounds like short gasping breaths), how to do trach care and trach change, how to always be prepared to go into emergency mode, how to tell if he’s crying, etc. The first day he had the trach, after he came off the vent, I remember standing by his bed and he started desatting (I was very used to handling desatting episodes before the trach). The doctor was passing by so I ran out into the hallway and made him come in Gabe’s room. He told me over and over “relax, he’s fine”. After a couple weeks we did relax and trach life became normal to us. We soon realized that the trach was the best decision we could’ve made for Gabe! He started cooing and making baby noises, smiling more, and became much more energetic. The trach also made him more mobile. Since he didn’t have to have oxygen while awake, he was able to go on his first stroll around the NICU, which he loved. He’s a ladies man so he just soaked up all the attention from the nurses on our daily strolls. A couple days later, we took him outside! The outside trip wasn’t too much of a success because the sun hurt his eyes, but I was so excited that he finally got to experience that!
Soon after Gabe had the trach placed, the surgeon discovered that he had a stricture in his esophagus, which isn’t uncommon in babies born with TEF. The solution to a stricture is a routinely scheduled surgical procedure using a balloon to dilate the esophagus. This procedure has to be done incrementally over a long period of time so there’s less risk of the esophagus tearing. Gabriel had his first 3 esophageal dilations while he was still in the NICU, and since he’s been home he’s had 8 more. Every dilation has gone extremely well and his esophagus is almost to the right size again. Now that his esophagus is almost to the normal size, we are scheduled for a swallow study to see if Gabe’s previously non-existent swallowing skills have developed. If he passes his swallow study, we will be able to start oral feeds which will be an AMAZING accomplishment. The oral feeds will make his stomach grow (he has a tubular stomach which made it impossible for the surgeon to place a gastrostomy tube; instead he has a jejunostomy tube that is a pain in the butt), and when his stomach is large enough they will be able to switch his feeding tube from his jejunum to his stomach which will allow him to have bolus feeds which will in turn make him gain weight faster which will in turn make everyone happier. So we are hoping and praying for good results at his swallow study!
But I’m skipping ahead. Let’s go back to the NICU…At the end of Febuary, I walked into Gabe’s room and was floored with some news. They were sending us home by the end of the week!! I knew we were ready, and I had been expecting it soon but it was still shocking. From that point on, it was a mad rush to get the house clean, all his equipment set up, and our services in place. Then on Febuary 28, after almost 7 months in the NICU, we went home with our son. What a mixture of emotions – elation to finally be going home, panic that Gabe’s life was our responsibility now, and sadness at leaving the security of the NICU and the friends we had made there. By the time we arrived home, the only emotion I was feeling was panic. I’m pretty sure that I was yelling at everybody who was there to help us, even though all the while I was telling myself to calm down. We made it through the first night just fine, and then the night after that, and then the night after that.
Since we’ve been home from the NICU, Gabe has been re-admitted 4 times – twice for line infections, once for cold/pneumonia, and once for stomach bug. We’ve been back for another 8 esophageal dilations and countless doctor appointments. He’s been diagnosed with significant hearing loss (possibly no hearing; we will find out more soon) and moderate hydrocephalus. Although Gabe has no official diagnosis yet, the geneticist wants to say he has CHARGE syndrome, but she’s waiting on his DNA testing to come back. We have an appointment with the neurosurgeon on June 18 to talk about the plan of treatment for his hydrocephalus, and he has a procedure scheduled on June 24 to have tubes placed in his ears, another hearing test, and a CT scan of his ears to look at the extent of the abnormalities.
Gabe loves his bed and his toys and books. He’s becoming more okay with being held which is awesome. He likes watching TV and really anything and everybody. He makes the craziest faces when he’s happy and loves hitting himself in the head with whatever he can get his hands on. When he’s really excited he’ll kick his legs like he’s riding a bicycle and becomes a little maniac. He just recently started playing with his feet and now sometimes it’s like his hand is attached to his foot. He has a huge personality and wants all the attention. We make him practice holding his head up everyday, and just the past couple weeks he’s made big strides in that area (he does better for Papa than for Mama). But when he’s tired of holding it up, he just throws it back and refuses to do it anymore, haha. He can roll from his stomach to his back if his arms are fixed just right under him and he’s working on back to stomach. He sleeps super good at night and takes 1-2 naps in the day. He has 6 teeth and weighs somewhere between 13 and 14 lbs. He scored high on his cognitive and social skills, normal in fine motor skills, and low in adaptive and gross motor skills.
In Gabe’s 10 months of life he’s endured more than anyone should have to in a lifetime. It’s been hard on mom and dad too. No parent can ever prepare for a chronically ill child. But we wouldn’t trade our special little boy for anything and we feel so blessed that he was entrusted to us.