Luke's breathing/respiratory story begins at Luke's beginning and as you will see things can and do change on a day to day and sometimes hour to hour basis.
Day 1: When Luke was born (by c-section) he squeaked a couple of times and then was not really breathing on his own. We knew something was most likely not right with Luke so there were neonatologists standing by. They intubated Luke and after a short look at him they whisked him away and put him on a ventilator. He was on “room air” which Kevin, my husband, told me was really good for being on a ventilator. Neither of us really knew what this meant but boy oh boy we would learn. Room air means that he is not on any supplemental oxygen, which is really good.
Day 2: Luke was doing well and they thought he was ready to breath without a ventilator the day after he was born but they wanted to give him an MRI first. Since you have to be still for an MRI they put babies under and they don't have the reflexes to breath on their own so he would need the vent for the MRI and they would take if off after the MRI was done. They wanted to do an MRI because they though he had a neurological disorder and they wanted to find out more and hopefully make a diagnosis with the help of the MRI. This was a huge time of unknowns. Remember this was in the first 48 hours of our experience with Luke and we had a full intervention birth after starting with a home birth plan. At this point they were not sure why he wasn't breathing on his own, Luke was a full term plus baby (41.5 weeks) and Luke was not sucking. Kevin and I do not remember if they had noticed the lack of facial expression yet.
Day 3: The MRI was scheduled for his second night and when we came to visit our sweet Luke in the morning he was on a nasal cannula (just oxygen through the nose). By the afternoon he was put on CPAP because he wasn’t breathing well enough on his own. CPAP for a baby entails putting a large prong thing in his nose that is attached to tubing which is attached to a setup that keeps Luke's airways open a little bit all the time and thus he doesn't have have to totally close and open his airways for every breath making it a little easier to breathe. I am not sure if he wasn't keeping his oxygen levels up or if it was that he had too high of CO2 levels. I am thinking CO2 because I don't remember Luke ever being on oxygen in the NICU (expect for right after surgeries and for a few brief moments here and there until he got sick in the fall of 2009). I don't remember what PEEP (positive something about airway pressure) he started on but I remember a lot of PEEP 6 and 5 for the time he was on CPAP. A PEEP of 5 is the lowest they will go on CPAP and the next step after a PEEP of 5 is a nasal cannula. Luke was getting breastmilk through a feeding tube in his mouth at this time and doing great with it.
Day 4: We got to hold Luke for the first time. Luke's ventilator and and umbilical catheter were removed and so Luke was ours for cuddling. The results of the MRI came back to say that all neurological parts where there but he did have two old subdermal (I think) hemorrhages from in the womb and that the blood would drain from those on its own. They didn't see these hemorrhages as explaining anything going on with Luke, breathing, sucking or anything. My note from this day said that Luke is getting better in not having so much mucus so they thought that he may be swallowing and that his motor functions were getting better. When Luke was born he was hypertonic or very stiff but sometime at some point he became hypotonic meaning he had really low tone and continues to have hypotonia. (We would later find that the servre low muscle tone is the most probable cause of his breathing issues.) The plan was to put Luke on nasal cannula the next day and we all expected great things out of him. We would then move from working on breathing issues to working on sucking and swallowing. (What was little known to us then was that Luke’s breathing would be and still is a long road to travel.) Luke is doing well digesting breastmilk and if he continues he will get his IV out. Luke was a good eater then and is still a good eater or should I say digester since food starts in his stomach.
Day 5: The doctors decided to wait one more day until trying Luke on nasal cannula. Not sure of the reason.
Day 6: Luke was doing well on the CPAP and so on Luke's 6th day they put him on the nasal cannula around two in the afternoon, I am not sure how much oxygen though. He also got his IV out because he is doing well and getting all his nutrition from breastmilk. We had now seen the neurologist and geneticist and they said that we may never find a diagnosis for Luke but they would look because with a diagnosis you can plan and know what the future may hold. We actually figured out the diagnosis after our friend Leslie, who has Moebius, heard Luke's story and said she thought he had Moebius Syndrome, but more on this later.
Day 7: In the early morning of day 6 Luke was put back on CPAP because his CO2 levels were too high. This was Luke's problem throughout the process of weaning to nasal cannula and probably still is. He doesn't have much trouble keeping the oxygen up but he has a hard time keeping his CO2 levels down to the levels they should be at. At this point we have no diagnosis and if we had one it would give us a better idea of what is doing on with Luke. The doctors had a couple of ideas why he wasn't doing well on nasal cannula (I don't remember what they were just knew this from a note I wrote back then). We will try nasal cannula tomorrow and Luke will get a couple of more chances before they decide to put a trach in so that they can send him home. I remember them telling me at some point that staying on CPAP long term is not good for the structure and health of Luke's upper airway and so if he needs help long term they would need to put a trach in.
Day 8: (February 12, 2009) We gave him a day of rest and on his 7th day in the morning of day 8 he was put back on nasal cannula. He lasted for a couple of hours till early afternoon when his CO2 levels were too high and then was back on CPAP. The ear, nose and throat doctor (ENT) was scheduled to come the next day and talk to us about how to help Luke breath and swallow. I am sure this was disappointing at the time for us as my note for that day started with “Today was a day of ups and downs”. I don't really remember much about my feelings then. I look back and it seems like I was taking this all so well and I would say I continue to take all of Luke's medical issues and ups and downs in stride and fairly well. I attribute this to God. There is no other way I could function and continue to be a mother, wife, daughter, friend through all of this. Our Pastor and his wife came to see us 3 ½ hours away from our home this day and it was one of the high points of the day. Luke has been with us for a week now and oh what a week it has been.
The next day, a Friday, the ENT said that Luke would get one more try to move to nasal cannula and if he didn't last then a tracheotomy was the next step. He said that if Luke could not make it on nasal cannula then he would likely need to be on CPAP long term and that it was bad for the nose and throat passages to have CPAP through his nose long term. Also Luke would not be able to go home on CPAP through the nose but would through a tracheotomy. He also said that the tracheotomy tube may bypass what is causing the problem and he may be able to breathe without the help of CPAP after the tracheotomy. I was thinking this was kind of fast and shouldn't we be doing more tests and I still sometimes think this. But back then there was a lot going on and I didn't have the energy or know to explore all of these things. The ENT did mention that he would put a scope down and look at the structure of everything involved in breathing when he did the tracheotomy. I didn't know what else to do at this point and this made sense. I was just hoping and praying my son would be able to breathe long term on the the nasal cannula.
On Sunday we were told that Luke would try his last chance at the nasal cannula on Monday. However, when Monday came they derided to wait because they heard some not so great lung sounds in the morning and instead turn him down from CPAP with a PEEP of 6 to a PEEP of 5. I was disappointed but figured his chances would be even better at making it the next day.
Luke did not last on nasal cannula on his last try. This was a hard thing for us to deal with. My son would be getting a tracheotomy. And not only a tracheotomy but most likely a g-button as well. Since no one had really seen him swallow, they didn't think with his neurological issues that he may ever swallow. They also thought that he may have aspiration issues and with the upcoming tracheotomy they decided a g-button would get food where it needed to go without the tubes down his throat or nose, which he was starting to pull out on a regular basis. During the nasal cannula trial Luke turned blue in my arms, very scary, and it was just after or during a feeding so the nurse had me lean him forward thinking he was aspiration and hoping leaning him forward would make it come out his mouth. I didn't write about this in my notes from back then but I think there was some milk or something that I saw filling up his mouth and then the turning blue. Well he got an x-ray to see if any got down in his lungs and thankfully it was clear. He would get an upper GI to make sure all his digestive track was physically there and in the right place but also see if he was aspirating so that if he was aspirating they would also give him a Nissen.
When looking at my notes it looked like they would explore his swallowing better after his trach was put in. They were thinking after the trach was in that he would not need ventilation and with no ventilation and no CPAP blowing air into his nose and mouth areas creating mucus from the irritation of the air constantly blowing that they could see what was really going on with normal secretions. This was huge, two major surgeries that were planned to happen within 7-10 days of each other. We would not be taking Luke home like a normal baby, but with a trach, g-tube and all the medical issues that come with it. And at this point I thought I would be going home a few weeks after the trach was put in (which would have been the case if not for the ventilator which you will hear about later). Luke slept most of the day after the failed nasal cannula trial which was good for me as I needed to try to regroup and take in all of this.
Luke has been alive for 14 days at this point and wow he has gone through a lot. I really see how much when I write it out like this. But there were good things going on in the mist of the disappointments with his breathing. He was moving more and it seemed that once food got into his stomach his digestive system worked very well, the proof was in the many many diapers.
I am not sure when it happened but I see in a note that on February 22, 2009 I wrote that our working diagnosis for Luke is Moebius Syndrome. In Luke's 2nd week I know we had thought that Luke had Moebius and started asking doctors, who when we asked said they suspected but didn't know for sure. Once we mentioned the possibility of Moebius Syndrome they were supportive and I think the announcement in this note comes from us finally getting the neonationlogist to say it. The ophthalmologist suspected it first but didn't say anything till we did because he didn't want to tell us something before more doctors came to the same conclusions. I know that at least one of the doctors wanted to make sure it wasn't just birth trauma and would go away with time. What got us thinking was my parents talking to Leslie Dhaseleer, who has Moebius Syndrome. My parents had of course shared some of Luke's story with their churches so people generally knew what was going on. Leslie happened to be at church that first Sunday after Luke was born. She heard about Luke and came up to talk to my parents after church. She started asking them if Luke had certain symptoms and Luke had the symptoms she asked about but the congregation was not told about these things. My parents were blown away and asked how she knew this and she told them she thought Luke could have Moebius Syndrome. My parents talked to me and we started asking questions of the doctors and found out that it had been thought of, as I mentioned before, in some of the notes but nothing said to us yet. Having Moebius Syndrome was really not we wanted to hear but now we had a diagnosis, we had a place to start looking for solutions to some of Luke's issues, and a glimpse of what the future may hold. Leslie has become a great resource and friend and gotten us involved in the Moebius Syndrome Community. I feel blessed by God to have her in our lives and that we were able to figure out what Luke had and then have a immediate contact to a community of people who had Moebius or were parents of Moebius children.
On February 23, 2010, before he was a month old, Luke had a tracheotomy (to put a trach in) and broncosopy (to put a scope down Luke's airways to check their physical status). I am not sure how long we waited in the waiting room but it wasn't a short amount of time. The doctor came and talked to us as soon as it was over. He said that his airways looked good and clear and it did not look like Luke's tongue or any other part was interfering with his ability to move air. He did say that Luke's left vocal cord may be paralyzed. Luke was so little that the doctor did not want to give a for sure on the left vocal cord paralysis but said it was a possibility.
Luke looked pretty bad when we got to seen him. They had him on sedative and pain drugs and he was still pretty out of it for awhile. He had two strings that were attached to his trachea so that if his trach fell out they would be able to open his trachea up and stabilize his airways. He had these for a week before the tissue healed enough to be more stable. At this point he had an IV in his had with a splint on it to keep the IV in and laying right. This splint was a good club and throughout the time with the IV it was tough to keep Luke from clubbing himself with it. Luke was on IV fluids for two days before they put him on milk and took the IV out. They put the IV in his head for his next surgery and I liked it there much better. He has the use of his hands it was more out of the way and less in danger of coming out. Luke is a really hard poke and so putting the IV in again is not something anyone in the NICU wanted to do.
So that is Luke's story pre-trach. I will post more of his story later.