Jacob's oxygen needs are slowly getting under control. He is down to two liters (from four) and will hopefully be continuing to do better on his own. Audri has taken the 'night off' to spend some much needed rest at home with JD. She has spent the last week staying with Jacob at the hospital while I went to work and stayed with JD.
Jacob is still in the hospital. His pneumonia has cleared up, but he is having other issues with his lungs that they have to stabilize before he can go home. He is on a high flow of oxygen and they can't send him home on that, so we will be here until they can successfully wean his oxygen needs back down. While here, Jacob has managed to pull his feeding tube out twice and keep all the nurses busy with his 'episodes'-where he drops his O2 saturation levels real low and they have to give him an extra "boost" of oxygen. They are also evaluating him for seizures, as they suspect that may be part of his 'episodes'.
The final DNA tests have come back on Jacob and they have confirmed what we suspected. He does, in fact, have Cornelia de Lange Syndrome. There is a gene, NIPBL, on chromosome 5 that causes CdLS when it is found to be mutated or changed. This is the case with Jacob. Please visit www.cdlsusa.org for more information about the syndrome, if you are interested.
A quick update on Jacob's progress so far:
He weighs 9 lbs and is 19 inches long, 3.5 months old!
His reflux and breathing issues continue. He is still on oxygen and will be for a while. The feeding tube goes in through his nose and into his intestines. The plan is to move the tube up from his intestines into his stomach next month. As long as Jacob is fed into his intestines, he has to be on continuous feeds with a feeding pump. Basically, this means he is hooked up to the pump 24 hrs a day with a slow rate of milk dripping in.
Jacob still has high blood pressure and is being monitored closely for any problems related to it, but so far he has had no issues with it. The doc thinks he will outgrow it. He will likely still need hearing aids for his hearing loss, but we don't know when that will happen. We have had 4 appointments this week, and have 3 next week, and 2 so far the week after that. Things are hectic right now, but well worth it.
We left Northside Hospital at about 2pm on Monday and our first thirty-six hours were rather hectic and will hopefully not reflect life with Jacob. It started with Audri, Jacob and I getting into our car with Joy helping us put all of his accessories into the car and the feeder quit working. We had used the hospital's feedbag which worked with a different model pump by the same brand but we could not get it to work with our pump. So, in the car, I hurried to find a new feedbag and it worked. We got home without getting stuck in too much traffic.
When we got home, we told JD we have a surprise for him and he ran into the living room to see Audri holding Jacob's car seat, which he had seen before, and he exclaimed excitedly "Baby Jacob's Car seat!" After we put the car seat down he saw Jacob and exclaimed "Baby Jacob. Baby Jacob in Mommy Daddy new house!". He has been really excited to be around Jacob and wanted to take care of him as best he can.
The rest of the evening and the night when pretty well. Audri and I worked out a system where I go to bed somewhere between 8pm and 9pm and she stays with him, dozing as she is able until about 2am when she comes and gets me and we switch. I go in and sleep on the couch and she sleeps to about 8am. It works pretty well and he is a decent sleeper. It does help that his feeds are continuous so there are not 3o minute feeding sessions, just pour it in the bag and you are done.
The incident happened about 7:30am, before he got his blood pressure medicine. I was up with him and had just laid him in his bed when he pulled his feeding tube out. His feeding tube is an NJ or TP, depending on who you ask, so it goes down through his stomach into his intestines. There is no way for us to put it back in ourselves, it requires x-rays to make sure it is in the right spot. We are trained to put in an NG tube (to the stomach) but, with all the stress, we were unable to get it in so we called all our different doctors and headed to the Scottish Rite ER.
When we got to the ER, we noticed a lot of Georgia State Patrol officers standing around, it honestly looked like they were having a convention, but the rest of the hospital seemed normal so we went on in. Audri put in a call to the GI doctor and did not check us in because they would handle the scheduling of the procedure to put the tube back in. We then proceeded to walk down to the main entrance, walking by the twenty or so GSP officers. When we got in the entrance, we did not know where else to go so we just found a column with hand-sanitizer and waited. Bear in mind Jacob has not eaten for about two hours and we were both very worried about the whole thing. The GI nurse, somehow, was able to spot us and figured out who we were (probably the scared looks on our faces) and said she had to do a few things but she would get to work on scheduling an appointment for us.
Soon after the GI nurse left, Governor Sonny Perdue came through the front doors with all the GSP officers, his staff and other people following him. We found out later he was signing a super-speeder bill that goes to fund the trauma network in Georgia. We ended up with him walking by us three times while we were sitting in the lobby, waiting.
The waiting was hard and by the time he got in for his procedure it was about 11-11:30 so he had not eaten for around three and a half hours. The procedure went pretty well, he was extremely mad the whole time but I would be too if someone stuck a tube down my nose to my gut and we packed up and went out the door. We ran into another feeding issue when we got in the car but I fixed it and we were on the way home.
About the time we were getting of the exit for our house, the feeding pump started complaining again about a clog below the pump and I was unable to remedy. I reset it and pumped milk through the extra valve on the tube and it all seemed to work fine so it had to be inside of Jacob. So back to the ER we went.
When we arrived, we checked ourselves into the ER directly and were triaged before being taken back to a room. That part was definitely smoother because we knew we had a nurse watching on things and a doctor to order the procedure and did not have to hope that the GI doctor's office would handle it. In all fairness, the GI doctor's staff did handle the first one but it is a lot more reassuring to have the nurse and doctor there, struggling with the issue and seeing what was going on. By the time we got out of the second procedure, it was about 4pm so Jacob had not eaten for about 9 hours. It was time for his blood pressure medicine again and we really did not want to leave until we were sure that everything was in good shape. They let us stay in the room until we had everything under control and Jacob was eating and had taken his medicine. The doctors and nurses with Children's Healthcare of Atlanta at Scottish Rite were wonderful and did their best to take care of all of us, not just him.
By the time we got him home, it was around 6pm and we missed the nurse for the day due to the change in our schedule. Everything that evening and the next morning went according to plan.