Zane spent his first month of life in St. Joe’s NICU… mostly trying to eat.
When he first arrived, they put an IV in to regulate his blood sugar. Because he was growing so rapidly off the placenta they felt he would need to be weaned off that amount of nutrition through an IV instead of shocking his system. He was basically getting too much nutrition from the placenta, is the theory, and when you take him off…. he’s not getting that nutrition anymore. So the major drop in nutritients would have been too much and so they started an IV right away. They also just had him on cannula oxygen. He looked pretty decent and I thought he’d only be there a couple days. They were worried he was also having tremors and he was making fast quick breaths. I thought he would grow out of the breathing issue because his lung development tests in utero always slowly got better. After a few weeks in the NICU though we found he wasn’t growing out of it and he was not eating well. He ate a lot from an NG Tube (Nasal Gastric Feeding Tube) for his whole first month.
Then we met Dr. Robey! Our ENT! He came in and did a flexible scope to see what was going on inside of his throat and airway. He was diagnosed with laryngomalacia at that time which is a floppy larynx. Click HERE to read more about Laryngo & Trachea Malacia. After that we were sent to our first swallow study and started working with speech therapists to find a way for Zane to eat. The swallow study showed that he was aspirating a little bit of formula each time he swallows. EEeek. No good! So we started thickening his food with rice cereal which got rid of the problem. I took him back to his room and he chugged down a full bottle and didn’t leave a drop left. I thought it was a miracle and I thought we would be heading home any day now! Then two days later he would only drink 20mL instead of 80mL. 😦 So now what? We tried ad lib feeding. That meant to take his NG tube out, take him off the NICU feeding schedule and let him wake up or cry to eat whenever he wants and to drink as much as he can. Completely following his own schedule. Well he did great for a couple days and even gained weight on his own. THEN he stopped eating again and lost weight. UGH 😦 It took 45minutes to convince him to drink even half his bottle. This would be too much work at home. Working super hard to get him to eat and then taking 45minutes to eat, take an hour break and do it again, around the clock. So, we decided to consent on getting him a G tube. As soon as we made the decision we were off to Children’s Hospital within days. The ambulance took him there and me and Caitlyn met him there first thing in the morning. The older boys were now in school everyday and Caitlyn came with me to the hospital everyday.
We got to Children’s and were welcomed by so many people! We had to see genetics because Zane’s story wasn’t pointing to any specific condition or cause. I didn’t think much of it because I thought nothing was wrong with him besides the laryngomalacia. Silly me. I thought we were just going there to get a g tube!
Genetics looked him over…. he had a skin tag by his nipple, and in his butt crack haha… his eyes and nose were too close together and ears lower than his nose. They explained to me how most faces are split into thirds and his middle portion of his face was less than a third. He had extra loose skin behind his neck and on his hands. He had short limbs that were unproportional to the rest of his body. Oh my! They just picked him apart! I felt kind of bad after that, but I thought he was cute and thats all that matters. No matter how funny he looks he will always know he is wonderful and has a giant peice of my heart. They needed to note these things in order to narrow down which genetic disorders to test for. Did you know there are 7000 genetic disorders in the world? (Learned that through the Global Genes Project) They first sent a test for Beckwith Weiderman Syndrome (a syndrome with large tongue, enlarged organs, large birth weight, polyhydramnios) he tested negative, but because they had considered it they ordered a belly ultrasound be done on him. During his belly ultrasound they found…… SURPRISE! A Wilm’s Tumor: NEUROBLASTOMA! ARE YOU KIDDING? Well that lead them to their new testing…. there is one genetic disorder he matched very closely with and neuroblastoma was one thing associated with that disorder: Costello Syndrome. We are still waiting for our results on that one. We also found two minor pieces of chromosome deletions through a micro array test on Zane, myself and dad. He is missing piece 1q21.1 from Dad and 15q11.2 from Mom.
We saw Neuro. Neuro came in and ran an EEG (a test measuring brain activity and waves), a brain MRI and some blood tests. They ruled out that he was having seizures because of his tremors in his arms and legs. We thought we were done with them at this point. The brain MRI also came back normal.
We met G.I…. because they had to put in his G tube! So immediately after we got to Children’s… the very next day they put in his G Tube. I felt kind of cruel at first for consenting to it, but it has made life so much easier for him.
He will be getting a much more attractive mic-key tube in one more month!
We met Endocrine! She came in a requested blood and urine I believe. They were trying to figure out why his limbs were so short. He is in the 10th percentile for height at this point. They thought he may have dwarfism? I believe they never found anything unusual on those tests. She said they can’t tell how a kid will grow until you monitor them for about a year until you can make a conclusion on that.
We also saw Physical and Occupational therapy, a social worker stopped by from time to time, medical supply, and Speech therapy on a daily basis, daily rounds of residents and fellows and our neonatologist group from St. Joe’s Hospital. My Dad calls the rounds “the laptop brigade” I wish I had a photo. hahaha.. but they had laptops on a pedstel with wheels…. anyhoo
After he had his G Tube put in…. we then found the neuroblastoma….
He was sent for a CT Scan of his neck, chest, abdomen & pelvis to check for more tumors. Then he was sent for a Bone Density scan as well. After all of that… there were no more tumors. This is great news! He had to do a MIBG Scan. They put some nuclear medicine or dye in him and he had to drink small doses of iodine several times a day which was just Gross! He hated that a lot! But the iodine protects his thyroid from this nuclear med. That scan was done then the next day which also shows his tumor hadn’t spread. They classified him low risk and stage 1 neuroblastoma.
So at that point we went to surgery to resect the tumor. This was a MAJOR surgery! And I will explain it more in the Neuroblastoma section if you want to read about it there! But he came back and was on a breathing machine for a full week. They attempted to take the breathing tube out once and he went into distress. He actually completely stopped breathing and they put the tube back in. His airway was so swollen from the tube being in that no air was getting through. They started him on diuretics/lasix, epinepherine/adrenaline and steroids to get rid of the swelling/edema in his airway. After a week we were able to successfully get it out! PHEW! Then he spent the following week weaning off cannula oxygen. Then we were discharged and able to go home! Yay! So we thought…..
Follow what comes next in the Opsoclonus Myoclonus Syndrome section.