Surgery, what surgery... oh wait yeah I have these posts sticking out of my chin

So to update on Luke's jaw distraction surgery...
(it is another long one)

Saturday afternoon Luke fell asleep asleep, no tossing, no turning, he was out.  We were thrilled.  He slept for around 4 hours and woke up to a couple of  hours of washing his body, stoma sites, post sites and then eating.  He actually went back to sleep!  He slept pretty good for quite a few hours before having some restless sleep.  Sunday morning he woke up his normal self.  It was awesome!  He woke up and pointed to all the things I needed to remove, his elbow immobilizers, his pulse ox, his Ponsetti shoes.  He knows what goes on the in morning and he was making sure I knew, very cute.  I was so excited that I scared my mom when I cried happy tears on the phone.  She thought something bad had happened and I tried quickly to control my tears and let her know all was very well.

Sunday was busy.  I slept in the morning a little bummed to be leaving sitting up fun, playing with his toys and books Luke.  But I did need sleep.  I got back to Luke and found out that all the doctors had come to see him and he was hopefully headed to the stable vent unit that afternoon.  Yeah!  So we packed up and waited for the go ahead.  We moved up 2 floors and thankfully still had our wonderful arboretum view and then some.  When we got settled I went and got a massage from Nancy's (grandma) friend Dawn.  Thank you Dawn!  I am very tight and I should work on this.  Wow have I come a long way from my hockey playing, dance company days in college.  However, my mom found out that Luke loves to exercise so when he has his pins out we will see if he likes "New York City Ballet Workout".  It is low impact and hopefully I can get loosened up.  I am sure my knees would appreciate some more muscle support as well (my knees have been giving me lots of trouble).

Sunday night my parents and Hannah arrived.  I was so happy to see her and she was so confused to see me at the end of a long car ride in a weird place that she didn't even smile at me.  She had also just woken up from a nap.  However, she was very vocal about leaving me and it was good to cuddle her.  She did give Kevin (and maybe Luke sitting next to him) a big smile when we walked into Luke's room.  It was really hard to have her in the room as she wanted to get down and go all over but then still be in my arms as she missed me.  She was also getting over tired and so my parents took her to my Uncle's to spend the night with them.  It was very hard to leave her again but I knew it was best as there was no way Kevin and I could take care of Luke and have Hannah with us in the hospital/hotel room.  On Monday when I saw here she gave me a huge smile and came right to me.  She was dressed in a cute strawberry outfit and oh so cute!

Luke was feeling good all day on Sunday, sitting up most of the day, reading and playing with toys.  Very nice to see.  Sunday night/Monday morning was also full of poo!  Two blowouts that night.  The poo has been bad.  Antibiotics are not nice to digestive systems.  Luke is taking the antibiotics to prevent infection at the pin sites and due to the internal device.  We are done in two days but the poo is awful.  However, he slept some that night and woke up a happy little guy, once his poo was removed and he had a fresh bed, on Monday.

The residents from oral surgery came down on Monday morning and turned the posts and thus started the actual jaw distraction and movement.  It seemed too easy.  We have a quite large, purple (my favorite color) screwdriver looking thing that fits over his posts and there are instructions on which way to turn and that 1 turn = 0.5 mm.  We were instructed to turn 2x per day and they estimate around 20 days.  We will follow up next Monday to check the progress.  They also said that he could go home.  What? Really?  We can go home!  We were ecstatic!  We had planned to send Hannah home with Nancy to watch her at my parents house until we came home but now we could all be home.  It was too good to be true but it was real!  We got our discharge papers and prescription and after lots of packing and a few trips to the car were ready to go.

Luke got really excited to be out and was a good car rider, even sleeping some.  However, he became really junky, needing lots of trach suctioning, as soon as we left the hospital.  This is a lot of work and we are still working on figuring it out but are hoping he is just adjusting back to the non-hospital humid air.  We had one poop blow out on the way home but fortunately the car seat was saved by the extra chuks (pads to absorb liquids that have plastic on the underside so they don't leak on things) from the hospital that we put down.

We got home and Luke all situated.  My parents brought Hannah back all ready for bed and we got to rock and nurse.  We don't have night nursing for Luke until Thursday night so Kevin and I are still switching back and forth.  Nancy came back up with us and is helping wrangle Luke and Hannah and keeping Luke from falling on or banging his posts and keeping the posts away from Hannah's exploring hands.

We are still running on little sleep at awkward times and will be very happy to see nurse Keri on Thursday.  We are still trying to figure out how to keep Luke's posts safe, give everyone enough sleep, keep Hannah happy, keep the house running and figure out how much of Nancy's help I will need.

Luke seems like his normal self, he is even letting me wash his pin sites and turn his pins sometimes without someone holding his hands to prevent him from pulling out his trach.  He has learned to sign "mama" and other sights at his chin up around his nose to avoid the pins.  I was a little concerned he would bother the pins with his very exuberant signing but that smart little guy figured it out.  I am going to keep him contained in his high chair or with someone right next to him till the pins come out because I am so worried he will fall on them hard and do some real damage.  The risk of damage is much higher than the displeasure I have at confining him.  He didn't seem to mind today but it was only day1.  Luke had really low muscle tone and will often fall over, lean on things and rub his face on things and I do not want him to do that with the pins sticking out.  If he wasn't such low tone maybe it wouldn't be an issue but then there is Hannah.  She loves to pull his hair, his trach, anything he has out of his hands and I fear that she would think the pins are cool and pull them as well.  We were told pulling does not harm them (lots of force pushing would) but I don't want to test it out on Luke.

Also, Luke has been leaking around his trach at night (some of the air his vent is pushing into his lungs is moving around his trach and up through his mouth and nose instead of into his lung) and he has been breathing faster and shallower breaths.  I don't like this as shallow night time breathing with low volumes recorded by the vent leads over time to Luke building up CO2 and not doing as well.  The ENT is ordering a larger trach (which nurse Mike has been thinking Luke may need for awhile).  I am a little bummed because Luke has been making noises over his vent again (for the first time since maybe a year old) and I was thinking that he may vocalize with his passy muir, or similar, valve now that he has more room around his trach and realized that he can do it.  The ENT said the bigger trach may still allow him the room to do it but it may not as well.

Oh and I had this waking nightmare thought that I may have turned Luke's distraction pins the wrong way this evening but his awesome doctors (which allow me to e-mail them, thus a lot of their awesomeness) got back to me right away and said nothing bad will happen if I accidentally did move them backwards.  Yeah for not worrying but I will be really sure I am turning with the arrow from now on.

Thanks for...
Luke's surgery going really well
Luke bouncing back from surgery
getting to leave the hospital days earlier than we expected
having Nancy able to come stay with us
my parents watching Hannah and Hannah have a really good time
good doctors


So prayers for...
a good full recovery for Luke
that the distraction goes well and works
for Hannah to readjust and get through her fear of us leaving her
for us to get sleep
for our nurses not to get sick and be able to show up for their days starting Thursday
resolving the vent leaking issue
that we keep Luke's pins safe from bumping and harm and Luke happy in the process

Thanks for all your continued prayers and support!

Luke in PICU right after surgery, before his cheeks started swelling like a chipmunk.

Luke sleeping on the car ride home with his swollen cheeks.

Kevin said that Luke looked like a Saber-toothed Chipmunk :)  I love his sense of humor.

Trach out!!

Luke is at it again.  When he gets mad he is pulling on his trach and twice yesterday he pulled it out.  Someone was right there each time and I got the new trach in with Luke only getting a little blueish.  I explained to him afterwards why it is not good for him to pull on his trach.  I don't know if he understands but he seems to understand so much else I thought I would give it a try.  When he is tired and mad it's like he just doesn't think.  I see him many times reach up to pull and then stop or just touch the trach and then pull his hand away fast which makes me think that he does know that he isn't supposed to do it.  It is like he just gets too tired and upset and he doesn't have the self control.

On the outside it may not seem like this upsets me.  At the time it does me no good to break down and get all sad about it because my boy's life is at stake, I am in survival mode.  I try not to dwell on the life and death of it to often because I feel if I do I may go into a depression about it.  And then the most upsetting this was the last time he was pulling a bit, just after Hannah was born, my nursing company had the audacity to call me up and insinuate that I was being a bad mom and just letting him pull on his trach and not do anything about it.  HELLO!  Think about it, who wants to see their kid turn blue and come close to death right before their eyes.  No one.  The first 3 times his trach came out it was on a nurses shift and not caught as quickly two of the times.  When this happened I called my ENT, pulmonlogist and wrote on the trach boards to get ideas.  I got a longer trach from the ENT, a "he's just 2 and not much you can do" from pulmonoloy, and some two ideas for ways to put things at night from the trach boards that I implemented.  Was there any advice from the nursing company, no.  Sorry for the rant, it still makes me mad.

Last night trying to get Hannah to sleep I started thinking more about Luke and his trach coming out and I cried.  It don't like to have to watch my boy every second to make sure he doesn't pull his airway out.  I don't like that he is so fragile but it is what it is and Luke is an awesome little boy.  If I could change it I would but I can't so I don't dwell often but pray that it will never come out when we are not taking it out for a change again.

And a cute picture of Luke who makes it all worth it.

One long trip and some realizations

So I decided to take my 2 month old and my 2 1/2 year old on a state tour stopping to see doctors in Ann Arbor and Grand Rapids this past Wednesday.  My mom has already said "I told you so" in that this was not a good idea.  I in my logical, not dealing with small children in cars thought this would save us 4-5 hours of driving if we did it in two appointments.  I did not think about pouring rain, a very windy day, getting 3 1/2 hours of sleep becuase my baby didn't want to go to sleep, and what would happen when shutting a teething 2 month old and active 2 1/2 year old in a car most of the day would be like.  We made it and we are home.  My mom went with me and at one point only an hour from home we were not sure if we were going to make it.  But we did...I was sick the next day and my husband had to stay home from work to take care of the kids to top it all off.   So kudos to my mom for driving through crazy weather, construction, by accidents, a crying infant and with a Luke that needed suctioning often (mostly due to the weather and dry car air) and by the end was getting upset and pulling on his trach so I was turned around in my seat suctioning him.

So why did we go on this crazy adventure?  We went to a consultation with a doctor about jaw surgery for Luke.  Another Moebius girl we know in our state used this doctor for jaw surgery and our ENT (we share most of our doctors) recommended him.  I really like him and his resident and intern.  They were friendly informative and seemed full of action.  They are gathering some information from some of our other doctors and will be getting back to us with a full recommendation soon.  Luke did pretty good and the doctor got a good look in his mouth while he had his hands in it so we only had to hold Luke down for a very short time for a better look.  Luke does not like medical professionals in the office setting.   He does not care if they are only listening to his lungs or if they are trying to get blood.  It is all defenses up for it all.  And his mouth is one of the worst places you can try to look. 

Then we went to see our pulmonologist, for this first time since sleep study results from July were back.  She did not have full results from the October sleep study, only prelimiaries so we will hear more when they come back.  However, I was thinking that Luke was on the vent at night just for sleep apenea (possibly denial on my part) but our pulmonologist was clear that he still was not breathing adequately at night to be without the vent.  She said that during the day he does good because a different part of his brain (I can't remember which part) can help with breathing during awake time.  When Luke goes to sleep this part shuts off and leaves the breathing to the pons and medela or one or the other.  These two parts are not normal in Luke's brain and have been show in the literature to be abnormal in Moebius people.  She said that his breathing problems are in his brain and that there really isn't much we can do but wait and hope he grows out of them.  WOW, it really hit me that I am looking at Luke on the vent for sleeping long term, but not necessarily forever, and that the trach will be in long term as well.  I do believe God could work a miralcle and things could change shortly but we forge on ahead with long term ventilation for Luke.

It makes me sad as I don't want my baby boy hooked to machines when he sleep and have to breathe through his neck and all the extra care that goes with that.  I want him to be as normal as possible.  I am working on really coming to terms with that but I am not sure I am there yet. 

Luke however, is forging ahead.  He is now pulling himself to stand against furniture.  He walks along the furniture as well.  He is crawling fast all over and is trying to stand on his own.  He did have more stitches due to an accident with his walker, gravity and his glasses but seems no worse for the wear. 

Hannah is 2 months old and doing great.  She is teething (my family teeths early) and so has become a fussier baby.  She is sleeping through the night though, once she gets asleep.  She is smiling a lot and babbles and I think I have even heard her giggle.  She is generally a happy baby and loves to watch Luke do whatever Luke is doing.  She was baptized a couple of weeks ago and all sorts of family came to see her and Luke.  We had a great time.

Here are some not so current pics, but more current than the previous ones...

 

Our Family

Luke exploring his books in a new more accessible location.

Luke is playing on his hands and knees a lot.  Here is is checking out his letters, he loves letters and numbers.

Here is daddy with his kids :)

One of Hannah's many faces

Hannah holding her head up high.

 Luke signing "daddy"

Luke basking in the sun.

Luke coloring.

Fall and Winter 2009 – 7 to 11 months

Okay just to warn you this is a long post but is separated by month.

September – 7 months


Luke went to GR for an EEG and MRI and some other appointments at the end of September. Luke started with an arm hug (blood pressure) at the Nephrology office. His blood pressure was fine and they decided to keep him off the medicine but still to closely monitor. Very good news!!

We checked into the hospital for the MRI because he needed to be up in the Peds ICU when he got his EEG because of his vent. Luke was put under general anesthesia for the MRI because he is on a vent. He was wheeled into the MRI on time and and it was one of the smoothest moving procedures that I have been witness to. We were busier than busy when we first got there because Luke needed a different trach to go into the MRI since the one he usually wears has metal in it (Neo Bivona Flextend). The new trach did not have a flex end (Shiley) and thus was right against his neck and in his neck rolls. It was hard to keep them vent on and when you put it back on you had to move his little neck rolls. Poor dude. They put his normal trach back in while he was still sedated and that was much better. The anesthesia and MRI went smoothly for Luke and he seemed fine. He was a little restless that night, but who can blame him with everything going on, and the doctors decided to start the EEG right after them MRI even though the sedation was still in him. We were supposed to wait until Tuesday morning to start the EEG. We started hearing we were leaving on Tuesday instead of Wednesday. I of course freaked because I had a pulmonology appointment on Wednesday afternoon that was very hard to get. And this would give us the problem of having to be in the Runcci house with Luke overnight again because we didn't want to go home and come back for the appointment, way to much driving (3.5 hours one way). So I called Kevin and he brought the crib and humidifier with him. On Tuesday morning when it seemed for sure we were leaving that afternoon I called pulmonology and God was working, we were able to get an appointment for Tuesday after the Cystic Fibrosis clinic. So we may have to wait awhile because the clinic usually runs late but we would get to head home on Tuesday evening!!!
Pulmonology said we were doing great! We are going change his rate to 8 (down from 20) during the day next week and then in two weeks we will call them and let them know how Luke is doing. Assuming Luke is doing well we will take him off the backup rate during the day all together! They said it is usually three months on the Pressure Control and CPAP before turning him down more. However, we will see them in two months and discuss our options then. I am thinking Luke man will be doing fine and we will continue lowering settings then. They also decreased his dose of the only medicine he is taking all the time! We will stay on this dose through flu and RSV season though.

And great news! No Grand Rapids trips in October! Our first month with no trips. We have two November appointment days where we fit all our appointments!

In late September Luke's MRI results came back as normal which is good. This means there is no evidence of seizure activity, the fluid collections he had as a newborn are gone and that the nerves are present they are just not functioning correctly. In early November we finally got word about the EEG which showed no seizures and everything as normal.

Luke has been doing lots better with his gross motor skills. He has never really been behind in fine motor skills especially with his hands. Luke now holds his head up and sits up with just a lit bit of help. And today he even sat on my lap facing me with no help. However, I can't get him to sit up by himself when he is not on my lap. He is now pushing up with his hands when on his tummy instead of lifting his head totally with his back muscles or however he was doing it. He has even rolled from his back to his stomach (with the bar braces still on his feet) except for one arm. He can't seem to get that bottom arm around by himself and he yelps for help when he gets too frustrated. He is also very good at moving around by bending his legs and pushing his bar on the ground and the lifting his but up and moving his upper body around. Luke is still getting help from physical, occupational and speech/feeding therapists which is good. He is still signing but isn't full tilt with it yet and some days is all about signing for things and other times not. He does babble with his hands like some babies babble with sound. Even with the trach Luke does make sounds that are really cute but he has now added frustrated grunting to the mix. He has so many things to be frustrated with I don't blame him. He does sign and vocalize when he is not being fed fast enough for him which we like because then we know what he wants. Luke is over 17 pounds and is 26 inches long. Sometimes I look at him and he just looks so big. All in all Luke is doing very well and is a fun cute little guy that seems to steal hearts wherever we go.

October – 8 months

October started with a trip to Grand Rapids of all things. Instead of getting a month off, we made a unplanned visit to get Luke's g-button replaced. The valve on Luke's g-button we use to feed him broke and he every time you put his feeding tube in our out he was loosing a lot of stomach fluid that contains important nutrients and this is very very messy. He now has a Mic-Key button (18Fr 1.7cm for those who want to know) that we can replace ourselves (and have replaced many times). This feeding tube on this button locks as well which is nice. However, the valve is open when you are feeding Luke so that instead of things only going in they can go in and out and out out out the top as we have found out lately. No leaking stomach juices though.

October also started a long couple months of sickness for Luke. :( Luke had been very "junky" (has lots of secretions out of his mouth and nose and from his trach) lately and the pulmonologist had us take him into the ER for an x-ray and culture. I thanked God that his lungs were clear clear clear and he only had an upper respiratory infection. No x-ray needed but a culture was taken from his trach secreations and in 48 hours we would know if he had something more serious. He was also put on increased Atrovent and Pulmicort, two medicines to help him keep his lungs clear. Luke seemed not to be too bothered by all this and was still fighting sleep at bedtime and naptimes.

As I wrote notes and updates about Luke on Luke's CarePage many people asked "How is Emily?" So this is what I wrote. “I am doing well. I thank God for the outlook he gives me and my ability to just roll with the flow more than I ever used to. There are so many parents I talk to who are really scared and worried about procedures and come ask me about them because Luke has had them. I don't remember feeling that scared or worried. I don't want to sound like bragging or like I am better than anyone else. I just think God (and thank him) has given me this peace about things. I am one of those who questions questions questions and I still do that. But there were a lot of things with Luke that I just accepted and accept and keep moving. His trach and g-button surgery were not huge worrysome events for me (Or maybe I am just forgetting my worry) but I just feel okay with things going on with Luke. I may wish they were different or hope things move faster with healing than they are but generally God has just let me be with who Luke is.

I am a little in your face about washing your hands before touching Luke and washing all his things. I am working in baby steps to be better about this. I am doing a lot of praying and handing over again and again my worries of Luke getting sick or really sick to God. My biggest struggle right now may be having people in my house all the time. I know I need them and they are great nurses but it wears on you to have someone in your house everyday.

So far I am not going stir crazy about getting out of the house. I think I may be a closet homebody. I struggle when I am away from Luke for any length of time and at the same time struggle to want more free time.”

Now, almost 2 years later, I am getting a lot better about the germaphobia things. I am lots better in our house but still am pretty careful with Luke when we are out in public.

November – 9 months

November started with Luke is doing better, not back to normal things were looking in the right direction. I had a tough first week mentally with Luke still being sick and earlier that week he didn't seem to be getting any better and all the doctors were telling me was to keep doing what we were doing and that it could take 8 weeks. Yes I said 8 weeks for Luke to get back to normal. I am not a patient person but dealing with Luke's medical issues has definitely helped me be more patient. However, by the end of the week I was better and Luke was getting better. He was sitting up really well and propping with his arms while sitting and on his tummy. Another sign he was getting better was that he was willing to do tummy time for the first time in a few weeks ago without throwing a fit. Luke is more alert to people and things and is exploring more of his environment. He even discovered that his vent tubes can be a toy when he had dropped all his other toys. He grabbed onto his vent tubes and was shaking them. It was a portion that was up higher and so he didn't actually pull them off but it was pretty funny. Also, during this time he started to pull his vent tubes off and hold them in his hand, sometimes making it so that the air coming out of the tubes was blowing on his face.

This week Luke goes to the orthopedic doctor and his Ponsetti brace wearing hours went from 23 hours to 12 hours a day!! It was great to see his little feet at last, and he gained freedom he hadn’t seen in 6 months.


Luke's recovery was short lived and we were in the ER on Saturday and then again on Sunday night with Luke on 1.5L of oxygen (way high for him) and needing lots of suctioning. We ended up being transferred down to the Children's Hospital in Grand Rapids by ambulance on Monday morning. Our local hospital will not take pediatric vent patients so it wasn't even an option to stay and be admitted there. In the ambulance, for the first time Luke put his arms up for me to pick him up and it was very hard for me because I couldn't pick him up as we were driving down the road and he was all strapped in. Poor baby. This was our first major sickness outside the NICU and because of that we needed to be admitted. We found out Luke had tracheitis (an inflamed trachea), like bronchitis but up farther in the trachea instead of down in the bronchial tubes. Luke has had tracheitis since and we are able to treat it at home. While we were in the hospital our pulmonologist even told us that once we figure out how Luke responded to medicine then she was fine treating him at home. By Tuesday Luke was much better but the docs wanted to keep us in till Wednesday morning to make sure Luke stayed well on 24 hours of meds that we would go home on. This trip gained us a lot of Luke experience. We learned a lot about him and I learned that there needs to be two full time people down with Luke. My mom helped a lot but she worked during the day and I didn't sleep well enough in Luke's room at night to take care of him 24/7. I don't feel comfortable with Luke in the hosptial, even the PICU, and nurses who do not know him not in the room and monitoring only his stats by screen. Especially at that time Luke needed suctioning and other things usually before his stats really showed it. So I did not and will not leave him alone in the hospital. Luke also started making noise again and “talking” to us. He had not done this in awhile since his trachea was inflamed and we were happy about him being on the road to recovery for real this time.


As Luke got better he started rolling from his tummy to his back all by himself. We also saw the geneticist to make sure Luke didn't have anything in addition to Moebius. Our opthomologist still worried that we had something in addition since the Moebius people he had dealt with did not have the problems Luke did with breathing. Well the geneticist said he just had Moebius, that all his symptoms fit within the Moebius Spectrum. She also said she believed that we didn't have but may a 1-2% higher chance of having another Moebius child than the general public. Yeah! (We not have Hannah, who does not have Moebius.)


Luke has his first Thanksgiving over at my parents house with lot of family and friends to entertain and love on him. He had lots of fun and was very warn out by the end of the day.


I always planned on babywearing, carrying your baby in a wrap or sling, and I started putting Luke in the Moby wrap with the vent on my back or sitting next to me. He loved it and I loved having him close. It also helped him calm down and sleep during a lot of the holiday festivities when he was over stimulated.



December – 10 months

Luke took his first visit to the dentist, who actually got a look in his mouth! He told us to come back for a cleaning when Luke could open his mouth more and would allow him to get in. This hasn't happened yet and I he may need to be put under if someone really wants to clean his teeth. I am hoping that the oral motor and sensory therapy we are doing will allow us to take him to the dentist next year.


We had some problems with Luke's tummy this month. One day he became very lethargic and his button got tight. By the end of the day he was so bad that we took him to the ER. The ER docs called the surgeons who put the button in and they gave instructions through the ER doc and Kevin and I took his button out and a volcano of stomach juices and ickyness came out. We put the button back in and cleaned it up and Luke instantly became his normal self again. They said that sometimes with a Nissen (this makes it so food and liquids can't go up the esophagus easily) it is hard to remove air and pressure can build up especially if something irritated the stomach. So, once the pressure was relieved he was as good as normal. Here we had another Luke experience under our belts. Things never got this bad but many times in the following months we removed the button and released pressure and let things drain. We are not truly sure what caused this but after a few months it seemed to go away and it hasn't been a problem since.


Luke also started having these weird episodes at night where he would continually high pressure. Usually he was alseep but obviously uncomfortable. Holding him would help for maybe 30 seconds and nothing seemed to really make the high pressuring stop. Luke having a high pressure is like him breathing out really hard, or a big sigh may do it. In this case he was obviously not aware. We didn't know what was going on but his stats and everything else seemed fine. Very weird.


Luke saw the neurodevelopmental team again and they thought he was doing well and progressing along nicely. He was not where he needed to be for his age but he was always making progress which was really good they said.


Luke experienced his first Christmas with more family and friends. The whole Calhoon clan even came up for New Year's to meet Luke and of course they fell in love with him.



January – 11 months

A month with lots of doctor visits. At pulmonlogy (the lung doctor) we got answers and changes. Luke is weaning full force again. He had stopped weaning when he got sick in the fall and I was very anxious to get moving again. He was on a backup rate of 12 during the day (20 is where he started and he had to get to 8 to go to the next level of weaning) for 2 weeks and then we would do 8 for 2 weeks and then call to talk about the next step. Luke was teething and had a lot of secretions because he just doesn't swallow well enough and I was afraid that would slow things down but fortunately it did not.

Luke secretions were a bit thicker and a little on the yellow side so they did a trach culture to see what is growing and we will know the results Monday. My dad thought the thicker could just be all the dry car air that is blowing through as it super heats the frigid temps. Luke had been downstate 2 times the week before. As time has went on we have noticed that Luke's secretions are very sensitive to the weather and his environment (car vs. house).

Luke was still having some episodes at night where he couldn't catch his breath and more but we couldn't figure out what they were. The pulmonologist had an immediate answer for us... Reflux. Some stuff goes a little but up from his stomach (can't go far because he has a Nissian which doesn't let things go up from his stomach) and triggers the Vagus nerve which also has a branch by the lungs and triggers bronchial spasms (go nurse Keri who was right on about these) in preparation for the aspiration it thinks is coming, but does not. Luke was put on Zantac but we were told it could take up to 8 weeks to really be effective. This was a relief as these episodes were weird. However, looking back I am not sure it was really reflux. Whatever it was he seemed to just grow out of it because the Zantac didn't seem to really change anything.

We are also working on taking a feeding out and so Luke will be to 6 feedings a day. After that we started adding puree foods to Luke's diet, you can read more about this in my blogs about Luke's blenderized real food diet.

At the ophthalmologist office Luke got a lower prescription on his glasses and they thought he was doing really well. He does not have any corneal sensation which worries the doctor. This means things can touch and possible scratch his eye and he does not even know it.

When we went to pick up the glasses I had a momentary freak out moment as the optometrist put some alien green glasses on him. I said "no thank you" can you just put the new lenses in the frames he has. So thankfully we left with a cute Luke in white glasses.

Luke was quite the chunk. He was 21 lbs. 4 oz. and 27 inches.

Hannah and Luke

Until a few days ago Luke basically ignored Hannah, even her loud crying very near him.  But in the past few days he has been pointing to her and signing "baby" more.  When we were going through letters the other day he was telling me some things that start with the letter we were on and when we got to "H" he pointed to Hannah.  We have been telling him Hannah's name by signing and "H" and jiggling it like we sign an "L" for Luke and jiggle it.  

Today he took off his HME and threw it into the bassinet things she was sleeping in.  I am not sure if this was purposeful or by accident but pretty funny.  Things haven't been all good.  The changes in our household have been apparent in negative ways as Luke has pulled his trach out twice in since Hannah has come home.  We have also had some long trying periods of time outs due to trach pulling.

Generally things are going well.  I still have helpers to lift Luke so we will really see what happens when it becomes 1 adult/parent to 2 kids in a few weeks.

Luke's (breathing) story - post trach part 1

So here is more of Luke's story. I know it may not be grammatically correct but mom feel free to let me know and I can change things. I just wanted to get it out there.

Luke had his trach put in on February 23, 2009 at about three weeks of age. The night and day after his surgery he would stop breathing and turn blue and have to be brought back with an ambu bag. This happened a lot in a short period of time the day after the surgery in the afternoon. I finally figured out, after talking it through with the nurses, that it was probably the Versed which they were giving him to keep him still so he wouldn't knock anything lose. They gave him a dose just before the many episodes happened the afternoon after the surgery. They halved the last dose they gave him that evening and things got better. Luke has on his record now that Versed is something he is allergic to because I don't want to see my baby blue again. I remember not liking to see Luke turn blue and ambu bagged back but I don't remember being really super upset about it. I look back and notice a lot of things now that didn't rile me like I would have expected but I think it was God knowing we had a long journey ahead and to take things in stride. This is unusual for me so God was working pretty well with me through this. Oh I cried a lot through it all and had my breakdown moments but they were usually after the fact back in my room when I had time to think about things more.

A good thing was that we were seeing a lot of Luke's face which was really nice. The only tube he had now on his face was the feeding tube, which he learned to pull out very well in the next couple of weeks before his g-tube surgery. Luke also learned how to pull off his vent tubes just a couple of hours after surgery and could manage to get them off right in front of my eyes while I thought I was holding his hand and keeping things under control. Luke does have a personality and he seemed to be saying that he did NOT like this trach vent thing and they needed to go.

They started feeding him breastmilk again the day after surgery and by the 2^nd day after surgery he was back to his pre-surgery level of feedings. Luke has always been a great digester, I won't say eater because he doesn't “eat” things by mouth. The 2^nd day after surgery went much better all the way around for Luke as he was more himself, had no blue episodes and was moving all limbs around. I did get to hold him for his afternoon feeding which was awesome since I wasn't allowed to hold and cuddle him much as they were afraid that his trach would fall out. And my holdings were only for feedings and very supervised. They were nervous about us holding him because the trach can come out and since the tissue has not healed to accommodate the trach things could get bad and more surgery could be needed. There were two strings that were attached, I never did grasp exactly how, to pull things open and stable in case the trach did come out. Luke did loose one of the strings before the week was up. At a week the ENT comes to do the first trach change and make sure everything is okay.

So the thought was that once the trach was put in that Luke would be able to breathe unassisted. It seems that usually trachs are put in because of some issues with the airways and since our ENT didn't think he had any issues with his airways we didn't know what to expect but were hoping for the best and to be vent and CPAP free soon after the surgery. The 3^rd day out of surgery Luke tried CPAP and couldn't handle it so was put back on low vent settings (I don't remember what they were).

He also did a little spitting up so they decreased his feeds a little bit. I don't remember this but read it in my notes. I knew we were looking at a g-tube when he recovered from the trach surgery and don't really remember any feeding issues before that.

Luke was breathing short shallow breaths and fast if I remember correctly which is not so good. Slower deep breaths are the preferable way to breathe. Five days out of surgery Luke was on CPAP all day and breathing a little fast but holding his own. His trach stoma site was a little redder than it had been and the nurses said they would point it out the the ENT in the morning when he came.

So about a week after the trach surgery when I got to the NICU in the morning Luke was back on the vent and on antibiotics for an infection. A culture was out to the lab to see what the infection was. This was very disappointing and I was hoping for a fast recovery and back to CPAP and hopefully lower. However, little did I know, Luke would not be coming off the vent before going home and now, at 2 years old he is still on the vent at night and naps.

The culture turned out to be staff and they were already treating with the correct antibiotics so that was good. The antibiotics kicked in and Luke's vent settings were turned down twice in one day!

Luke first trach change went well and the ENT through things were going fine. They kept the trach size at 4.0 since Luke was on the vent. The ENT said a smaller trach could have leakage and then the vent would alarm unnecessarily and not be delivering its air efficiently. At this point we started things in motion for getting trained on all the care needed for a trach baby. I was now back to holding Luke whenever I wanted, which was mainly during feedings which was great.

And a new issue, high blood pressure. Luke has had high blood pressure it seems like always. The nurses comment on it but the doctors don't seem to concerned. The new resident mentioned it to the doctor and they ordered a kidney ultrasound for the next day. From what I gather, kidneys not functioning correctly can cause high blood pressure and they may not be functioning correctly due to the UVC line that was in his belly button when he was first born because they could not get an IV. The ultrasound didn't reveal any abnormalities in the kidneys so his high blood pressure was still a little bit of a mystery although they were still thinking the UVC line might have caused some damage afflicting the blood pressure.

So this turned out to be a busy day with the ENT and first trach change, high blood pressure issues, and the eye doctor. The pediatric eye doctor said Luke's eyes still had scratches but looked good enough to take him off his antibiotic for his eyes. Yeah!

A little over a week after trach surgery and after recovering a bit from a staff infection Luke started 3 hour trials on CPAP. He did well for most of his first few but started going downhill towards the end of them. It is a step in the right direction though.

Luke went for an upper GI to proceed the putting in of a g-tube the next day. Which is good because he took his feeding tube out twice during our trip to get the GI done. The upper GI revealed some aspiration and so the docs decided to do a Nissen Fundoplication as well. The Nissen keeps food from going up from Luke's stomach into his mouth and then down his airways. There is a lot of controversy around getting these done. I had no idea of the controversy at the time but Luke's seems to have turned out well and so far we have had no problems with it. I mentioned Luke's grabby hands to the surgeon and since Luke was a good sized baby he said he could put a button in right away and not a tube that hangs out. This was great new for me as I foresaw a lot less problems. Luke was also going to get a muscle biopsy done to rule out muscular dystrophy.

Around this time I had some down times about not being able to hold and cuddle my baby as much and a little about Luke's future. I didn't and still don't like to suction him and back then he really didn't like it. He has learned to deal somewhat now. I would rather be the one than some nurse that didn't care as much as I did though. What I didn't like is that with all the tubes and stuff it wasn't easy just to scoop Luke up and cuddle him when he cries. I do enjoy the ability to just scoop him up now and give him a good cuddle.

Luke's Story - Birth to Trach

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 6^th 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 7^th 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 2^nd 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.